Systemic Lupus Erythematosus

Aka: Systemic Lupus Erythematosus, Lupus, SLE, Lupus Serositis, Lupus Cerebritis

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II. Background

  1. Named in 1851 for wolf (lupus) bite-like facial rash
  2. First described by Moritz Kaposi

III. Epidemiology

  1. Prevalence
    1. England: 200 per 100,000 women aged 18 to 65 years
    2. U.S
      1. Adults: 40 to 50 per 100,000 persons (up to 1 in 1000)
      2. Children 3.3 to 8.8 per 100,000 persons
  2. Over-diagnosed in United States
    1. Of 2 million U.S. cases, only 25% have true disease
    2. As of 2008, estimated true Prevalence in U.S.: 300,000
  3. Age distribution
    1. Young adult onset is most typical (but onset may be at ages 15-64 years)
    2. Children comprise 10 to 20% of cases (median onset 11 to 12 years, uncommon before age 8)
  4. Gender
    1. More common in women (especially child-bearing age) by ratio of 9:1 (women 90%, men 10%)
    2. Ratio 4-5:1 male to female in children
  5. Ethnic and racial predisposition
    1. Native American
    2. African American (twice as prevalent than in white patients)
    3. Hispanic
    4. Chinese
    5. Filipino
  6. References
    1. Duarte-García (2022) Ann Rheum Dis +PMID: 35577385 [PubMed]
    2. Kamphuis (2010) Nat Rev Rheumatol 6(9):538-46 +PMID: 20683438 [PubMed]

IV. Causes

  1. Idiopathic
  2. Drug Induced lupus
    1. More than 80 drugs associated, esp. Hydralazine, Procainamide, Beta Blockers

V. Pathophysiology

  1. Tissue damage by Antibody and immune complex deposition
  2. Autoantibodies form to cell nucleus components

VI. Findings: Presentations

  1. Consider SLE when multiple organ systems are involved
  2. Most common presenting symptoms (90% of cases)
    1. Fatigue
    2. Weight loss
    3. Fever without focal infection
  3. Women of child-bearing age (age 15 to 50 years old)
    1. Fever
    2. Malar Rash
    3. Arthralgias (or myalgias)
  4. Men
    1. Less skin and joint involvement in men than women and children
    2. Hematologic disorder
    3. Renal disorder
    4. Neurologic disorder (esp. CNS)
  5. Children
    1. Fever
    2. Malar Rash
    3. Arthralgias
    4. Alopecia
    5. Hematologic disorder (Anemia, Leukocytopenia)
    6. Renal involvement
  6. Drug-induced Lupus (occurs equally in men and women, typically closer to age 50 years old)
    1. Fever
    2. Rash
    3. Arthralgias and myalgias
    4. Pleuritic Chest Pain
    5. Rare renal involvement and rare CNS involvement
    6. Resolves in most cases after offending drug is stopped (see list of drug causes above)

VII. Symptoms

  1. Arthralgias and myalgias (95% at presentation)
  2. Fatigue (Prevalence overall: 90%)
  3. Fever
  4. Malaise
  5. Weight loss
  6. Malar Rash (31% at presentation)
  7. Photosensitivity (23% at presentation)
  8. Pleuritic Chest Pain (16% at presentation)
  9. Raynaud Phenomenon (16% at presentation)
  10. Oral Lesions such as Oral Ulcers or mucositis (12% at presentation)

VIII. Signs: General

  1. Dermatologic
    1. Discoid Rash (LR +18)
      1. See Cutaneous Lupus Erythematosus
    2. Malar "butterfly" rash (50% of cases, 31% at presentation, LR+ 14)
    3. Photosensitivity (23% at presentation, LR+ 11)
    4. Vasculitis
    5. Alopecia
    6. Oral Ulcers or mucositis (often painless Oral Ulcers on Palate)
    7. Sicca Syndrome
  2. Rheumatologic or musculoskeletal symptoms (95% of cases)
    1. Arthralgias or Arthritis with symmetric Polyarthritis (esp. small joints)
    2. Myalgias or Myositis
    3. Raynaud Phenomenon (16% at presentation)
    4. Fibromyalgia (often comorbid in SLE patients)
  3. Abdominal
    1. Lymphadenopathy
    2. Splenomegaly
    3. Nephritis
    4. Mesenteric Vasculitis
  4. Neuropsychiatric
    1. Organic brain syndrome
    2. Seizures (LR+ 13)
    3. Psychosis (LR+ 13)
    4. Thrombotic Cerebrovascular Accident
    5. Transverse Myelitis
    6. Cognitive dysfunction (spectrum from mild cognitive deficits to Dementia)
    7. Migraine Headaches
    8. Peripheral Neuropathy
  5. Cardiovascular
    1. Pericarditis
    2. Myocarditis
    3. Acute Coronary Syndrome
    4. Venous thrombosis (DVT)
      1. Suggests Antiphospholipid Antibody Syndrome
  6. Pulmonary
    1. Pleuritis
    2. Pneumonitis or Pneumonia
    3. Interstitial Lung Disease
    4. Pulmonary Arterial Hypertension
    5. Pulmonary Embolism
  7. Ocular changes (20% of SLE cases)
    1. Anterior eye disorders
      1. Keratoconjunctivitis Sicca (25% of patients)
      2. Uveitis
      3. Episcleritis and Scleritis
      4. Keratitis
      5. Blepharitis-like Discoid Lupus eyebrow involvement
    2. Retinal Disorders
      1. Cotton wool spots
      2. Retinal Hemorrhages
      3. Proliferative Retinopathy
    3. Neurologic conditions
      1. Optic Neuritis
      2. Ischemic Optic Neuropathy
      3. Amaurosis Fugax

IX. Diagnosis: EULAR/ACR Criteria 2019

  1. Background
    1. New 2019 EULAR/ACR guidelines included many of the SLICC Criteria (2012), which replaced original ACR Criteria (1997)
    2. Test Sensitivity: 96%
    3. Test Specificity: 93%
  2. Antinuclear Antibody (ANA) positive is required for all SLE Diagnoses
    1. Titer >1:80 on HEp-2 Cells or equivalent positive at any time in patient's past history
    2. Titer over 1:320 is very suggestive
    3. Prior discriminatory value was 1:40 dilution
  3. Adjunctive Criteria Background
    1. SLE diagnosis requires at least one clinical criterion and at least 10 total points
    2. Criteria may have occurred previously and need not occur simultaneously with other criteria
    3. Only count criteria that can be attributed to SLE, and not to another more likely diagnosis
    4. Only count the highest weighted criterion from each domain, when a domain has more than one positive feature
  4. Adjunctive Clinical: Constitutional
    1. Weight 2: Fever (>38.3 C or 101 F)
  5. Adjunctive Clinical: Hematologic
    1. Weight 3: Leukopenia (<4000/uL)
    2. Weight 4: Thrombocytopenia (<100,000/mm3)
    3. Weight 4: Autoimmune Hemolysis or Hemolytic Anemia
      1. Findings include Reticulocytosis, low Haptoglobin, increased Indirect Bilirubin, increased LDH, positive Direct Coombs test
  6. Adjunctive Clinical: Neuropsychiatric
    1. Weight 2: Delirium
      1. Altered consciousness for <2 days, fluctuating throughout the day, affecting cognition or behavior, mood or affect
    2. Weight 3: Psychosis
      1. Findings include Delusions and/or Hallucinations without insight, and without comorbid Delirium
    3. Weight 5: Seizure Disorder (generalized or partial/focal)
    4. Findings
      1. Neurologic changes or Lupus Cerebritis is ultimately present in 75% of SLE cases
      2. Other neurologic criteria per SLICC (not part of EULAR/ACR Criteria)
        1. Thrombotic Cerebrovascular Accident
        2. Transverse Myelitis
        3. Cognitive dysfunction (spectrum from mild cognitive deficits to Dementia)
        4. More subtle, less specific or dramatic changes (Migraine Headache, Peripheral Neuropathy)
  7. Adjunctive Clinical: Mucocutaneous (findings must be observed by clinician)
    1. Weight 2: Non-scarring Alopecia
    2. Weight 2: Oral Ulcers
      1. Typically painless, oral or nasopharyngeal lesions
    3. Weight 4: Subacute cutaneous or Discoid Lupus
      1. Annular or papulosquamous (psoriaform) skin eruption typically in sun exposed regions (and phoosensitivity)
      2. Raised erythematous patches with adherent keratotic Scaling
      3. Follicular plugging
      4. Atrophic scarring in older patients
    4. Weight 6: Acute Cutaneous Lupus
      1. Generalized maculopapular rash
      2. Malar Rash (present in 30-50% of SLE cases)
        1. Erythema (may be raised) over the cheeks and sparing the nasolabial folds
      3. Photosensitivity
        1. Atypical sunlight skin reactions
      4. Biopsy findings
        1. Perivascular lymphohistiocytic infiltrate
  8. Adjunctive Clinical: Serosal (Lupus Serositis)
    1. See cardiac complications and pulmonary complications described below
    2. Weight 5: Pleural or Pericardial Effusion
    3. Weight 6: Acute Pericarditis
    4. Findings
      1. Pleuritic Chest Pain, friction rub or Pleural Effusion
      2. Positional Chest Pain worse supine
        1. Improved sitting or leaning forward
        2. Pericardial Effusion or friction rub
      3. EKG changes with diffuse ST Elevation, PR Depression and no reciprocal changes
  9. Adjunctive Clinical: Musculoskeletal
    1. Weight 6: Joint involvement (SLE Polyarthritis)
      1. Synovitis (swelling or effusion) involving 2 or more joints OR
      2. Joint tenderness and >=30 minutes morning stiffness affecting 2 or more joints
    2. Findings
      1. Polyarthritis is present in 90% of SLE cases
      2. SLE Polyarthritis is non-erosive and involves 2 or more joints
  10. Adjunctive Clinical: Renal
    1. Weight 4: Proteinuria >0.5g/24 hours (or equivalent Urine Protein to Creatinine Ratio)
    2. Weight 8: Renal biopsy class 2 or 5 Lupus Nephritis
    3. Weight 10: Renal biopsy class 3 or 4 Lupus Nephritis
    4. Findings
      1. Renal disease or Lupus Nephritis is ultimately present in 60% of SLE cases
      2. Persistent Proteinuria >500 mg/day (>3+ Urine Protein)
      3. RBC Cellular Casts (or mixed casts) may also be present
  11. Adjunctive Immunologic: Antiphospholipid Antibodies
    1. Weight 2: Anti-cardiolipin antibodies (medium or high titer) OR anti-B2GP1 Antibodies OR Lupus Anticoagulant
  12. Adjunctive Immunologic: Complement Proteins
    1. Weight 3: Low C3 OR low C4
    2. Weight 4: Low C3 AND Low C4
  13. Adjunctive Immunologic: SLE Specific Antibodies
    1. Weight 6: Anti-dsDNA Antibody OR Anti-Smith Antibody
  14. References
    1. Aringer (2019) Arthritis Rheumatol 71(9):1400-12 +PMID: 31385462 [PubMed]

X. Labs: Protocol

  1. Lab interpretation described specifically below
  2. Indications for ANA titer
    1. Unexplained involvement of Two or more organ systems
    2. ANA positivity at low titers <1:320 is very prevalent in normal population (esp. children)
  3. Initial Screening
    1. ANA titer positive if 1:80 dilution (prior cut-off was 1:40)
  4. Secondary testing if ANA titer positive
    1. Complete Blood Count
    2. Comprehensive metabolic panel including Serum Creatinine
    3. Urinalysis
    4. Antiphospholipid Antibody
    5. Anticardiolipin Antibody
    6. Double Stranded DNA Antibody (Anti-dsDNA)
    7. Smith Antibody (Anti-Smith or Anti-Sm)
    8. Anti-ribonucleoprotein (Anti-RNP)
    9. Anti-Beta2-Glycoprotein1
    10. C-Reactive Protein
    11. Direct Coombs
  5. Alternative diagnosis labs to consider
    1. Thyroid Stimulating Hormone (Hypothyroidism)
    2. Blood Cultures (endocarditis)
    3. HIV Test
    4. Rheumatoid Factor and anticyclic citrullinated Antibody (Rheumatoid Arthritis)

XI. Labs: Interpretation

  1. Complete Blood Count
    1. Anemia
    2. Lymphopenia
    3. Thrombocytopenia
  2. Urinalysis
    1. Consider 24 Hour Urine Protein
    2. Consider 24 hour Creatinine Clearance
    3. Lupus Nephritis findings
      1. Persistent Proteinuria > 500 mg/day (>3+ Urine Protein) or
      2. RBC Cellular Casts (or mixed casts)
  3. Coagulation Factors
    1. Prothrombin Time
    2. Partial Thromboplastin Time (PTT)
      1. Increased (prolonged) in Antiphospholipid Antibody Syndrome
  4. Other initial basic labs
    1. Comprehensive metabolic panel
    2. Direct Coombs test
  5. Primary Antinuclear Antibodies (and other autoantibodies)
    1. Antinuclear Antigen (ANA)
      1. Positive in 94-98% of true SLE cases
      2. Typically positive in Drug-induced Lupus
      3. Only 5% of ANA positive patients have SLE
    2. Smith Antibody (Anti-Smith or Anti-Sm)
      1. Positive in 20-30% of SLE cases
      2. Highly specific for SLE (nearly pathognomonic)
      3. Does not correlate with disease activity (unlike Anti-dsDNA)
    3. Double Stranded DNA Antibody (Anti-dsDNA)
      1. Positive in 50-70% of SLE cases
      2. Specific for Systemic Lupus Erythematosus
      3. Associated with Lupus Nephritis
      4. Associated with Lupus CNS Involvement
      5. Also positive in Syphilis and Bacterial Endocarditis
  6. Antiphospholipid Antibody Syndrome related labs
    1. Antiphospholipid Antibody
    2. Anticardiolipin Antibody
    3. Partial Thromboplastin Time (PTT)
      1. Prolonged in Antiphospholipid Antibody Syndrome
  7. Other Autoantibodies
    1. Anti-ribonucleoprotein (Anti-RNP)
    2. Beta-2 GlycoproteinAntibody
    3. Anti-ribosomal P (Lupus sensitivity: 20-30%)
      1. Highly specific for lupus erythematosus
      2. Associated with Lupus Psychosis
    4. Anti-Ro (Anti-SSA)
      1. Positive in 40% of SLE cases
    5. Anti-La (Anti-SSB)
      1. Positive in 10-15% of SLE cases
    6. Histone Antibody (Anti-histone)
      1. Positive in 50-70% of SLE cases (especially drug induced lupus)
  8. Other labs used historically in Lupus evaluation
    1. Complement Levels
      1. Complement C3, Complement C4, Complement CH50
      2. Typically unreliable in predicting acute lupus flare
      3. Low complement levels are more consistent with SLE
    2. Syphilis Serology (VDRL or RPR)
    3. Erythrocyte Sedimentation Rate >100 mm (LR+ 5.3)
    4. C-Reactive Protein
      1. May be used to gauge lupus activity

XII. Diagnostics

  1. Electrocardiogram
    1. Pericarditis
      1. See EKG in Pericarditis
  2. Lumbar Puncture
    1. Evaluate differential diagnosis of Lupus Cerebritis (e.g. Meningitis or encephilitis, Multiple Sclerosis)
    2. Findings suggestive of increased CNS Lupus activity
      1. CSF White Blood Cells increased
      2. CSF Protein increased
      3. Immunoglobulin synthesis or Immunoglobulin G increased

XIII. Differential Diagnosis

  1. Children
    1. Cancer (e.g. Leukemia, Lymphoma)
    2. Juvenile Rheumatoid Arthritis (Juvenile RA)
    3. Immune Thrombocytopenic Purpura (ITP)
    4. Infections
    5. Thyroid disorders
  2. Adults
    1. Behcet Syndrome
    2. Chronic Fatigue Syndrome
    3. Dermatomyositis
    4. Endocarditis
    5. Fibromyalgia
    6. HIV Infection
    7. Hypothyroidism
    8. Fibromyalgia
    9. Inflammatory Bowel Disease
    10. Lyme Disease
    11. Malignancy
    12. Mixed Connective Tissue Disease
    13. Psoriatic Arthritis
    14. Reactive Arthritis
    15. Rheumatoid Arthritis or adult onset Still's Disease
    16. Sarcoidosis
    17. Systemic Sclerosis
    18. Sjogren's Syndrome
    19. Thrombotic Thrombocytopenic Purpura (TTP)
    20. Vasculitis

XIV. Imaging

  1. Brain MRI
    1. Indicated in suspected Lupus Cerebritis or other neuropsychiatric findings
    2. Findings are typically non-specific
  2. Echocardiogram (or Bedside Ultrasound)
    1. Indicated for suspected Myocarditis or Pericarditis
    2. Evaluate for overall Left Ventricular Dysfunction and Pericardial Effusion

XV. Complications

  1. Immunocompromised state
    1. Hyposplenism
    2. Immunosuppressants used to treat systemic lupus
  2. Lupus Nephritis (50% lifetime Prevalence)
    1. Lupus Nephritis is a Glomerulonephritis of several different types (worst is diffuse proliferative nephritis)
    2. Lupus Nephritis is a predictor of increased mortality risk
    3. Renal biopsy is typically required for diagnosis of specific Glomerulonephritis type, which in turn drives management
  3. Lupus Cerebritis
    1. Various presentations (see above under diagnosis) of CNS disease are seen in 75% of Lupus patients
    2. Consider MRI and Lumbar Puncture (see above under imaging and diagnostics)
  4. Cardiovascular disease (28 to 40% lifetime Prevalence)
    1. Coronary events
      1. Coronary atherosclerosis
        1. Autoantibody related Vasculitis
        2. Lupus medication adverse effects
      2. Coronary Vasculitis with secondary ST Segment elevation Myocardial Infarction (STEMI)
      3. Spontaneous Coronary Artery Dissection
    2. Pericarditis (most common)
      1. Risk of Cardiac Tamponade (has even occurred in children)
    3. Myocarditis
      1. Presents with conduction abnormalities, Arrhythmias
      2. Associated with Cardiomyopathy or Heart Failure
  5. Pulmonary disease
    1. Lung disease affects 50% of Lupus patients and is a presenting symptom in up to 5% of cases
    2. Pleuritis and pulmonary infections are most common presentations
    3. Interstitial Lung Disease, pneumonitis, Pulmonary Arterial Hypertension, Pulmonary Embolism may also occur
  6. Venous Thromboembolism (VTE)
    1. Higher risk by a factor of 100 in SLE (and at a younger age)
      1. Occurs in up to 26% of lupus patients (contrast with 0.2% in the general population)
    2. Increased risk with Antiphospholipid Antibody positive status
    3. D-Dimer has less less utility in SLE due to a high False Positive Rate in this population (less specific)
      1. However, a negative D-Dimer in SLE suggests a very low likelihood of VTE
      2. Wu (2008) Clin J Am Soc Nephrol 3(6): 1628-36 [PubMed]
  7. Antiphospholipid Antibody Syndrome (Hughes Syndrome, Anticardiolipin Syndrome)
    1. Affects 15% of Lupus patients
    2. Risk of thromboembolic complications (despite prolonged PTT)
      1. Venous Thromboembolism
      2. Acute Coronary Syndrome
      3. Acute thrombotic stroke
      4. Acute Limb Ischemia
      5. Mesenteric Ischemia
      6. Renal artery thrombosis
      7. Raynaud's Phenomenon (with risk of digital ischemia)
  8. Hematologic (most SLE patients)
    1. Thrombocytopenia
    2. Autoimmune Hemolytic Anemia
  9. Malignancy
    1. Non-Hodgkin Lymphoma (risk increases 3-4 fold over general population)
    2. Lung Cancer
    3. Cervical Cancer
  10. Pregnancy-related complications
    1. Preeclampsia
    2. Preterm Labor
    3. Miscarriage or Stillbirth
    4. Pregnancy may trigger increased SLE acitivity and flares
    5. Risk Factors for increased pregnancy complications
      1. Active disease 6 months before pregnancy
      2. Lupus Nephritis
      3. Discontinuation of Hydroxychloroquine

XVI. Management: General Principles

  1. Reevaluate every 3-6 months
  2. Employ measures to relieve Fatigue
  3. Sunscreen and other protection due to photosensitivity
  4. Reduce risk of infection (e.g. Immunizations)
  5. Birth Control is critical during exacerbations
  6. Monitor Disease Activity: Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K)
    1. https://www.mdcalc.com/calc/10099/systemic-lupus-erythematosus-disease-activity-index-2000-sledai-2k
    2. Mild Disease: Score <=6 on SLEDAI-2K
    3. Moderate Disease: Score 7 to 12 on SLEDAI-2K
    4. Severe Disease: Score >12 on SLEDAI-2K

XVII. Management: Hospitalization Indications

  1. Acute lupus presentation
    1. Female aged 15 to 50 years old with fever, Arthralgias and Malar Rash and associated findings (esp. nephritis)
    2. May benefit from admission for diagnosis and acute management
  2. Significant febrile illness
    1. Lower threshold for hospital admission given underlying Immunocompromised state and potential Hyposplenism
  3. Acute lupus flare with severe pain
    1. Consider IV cytotoxic agents and Corticosteroids
  4. Lupus Nephritis (acute onset or worsening)
    1. See below
    2. Heralded by abnormal Urinalysis (Hematuria, 3+ Proteinuria, RBC Cellular Casts) or increased Serum Creatinine
    3. Renal biopsy is typically required for diagnosis of specific Glomerulonephritis type, which in turn drives management
    4. Nephritis is a predictor of increased mortality and morbidity, including progression to Renal Failure
    5. Acute Induction Management
      1. Systemic Corticosteroids AND
      2. Mycophenolate or Cyclophosphamide
  5. Lupus Cerebritis (e.g. Seizures, Psychosis, Dementia)
    1. Initial evaluation typically with MRI Brain and Lumbar Puncture
    2. Hospitalization for further evaluation and acute management
  6. Acute cardiovascular conditions
    1. Acute Coronary Syndrome
    2. Pericarditis
    3. Myocarditis (may cause life threatening, Acute Heart Failure)
  7. Venous Thromboembolism
    1. Suggests Antiphospholipid Antibody Syndrome (higher risk of thrombotic complications)
  8. Acute Abdominal Pain
    1. Hospitalization indicated where diagnosis is unclear or for associated Lactic Acidosis
    2. Normal Complete Blood Count or non-diagnostic CT Abdomen does not exclude serious cause in SLE
    3. Acute Abdominal Pain in SLE presenting to the emergency department is associated with 57% morbidity and 11% mortality
      1. Vergara-Fernandez (2009) J Gastrointest Surg 13(7): 1351-7 [PubMed]

XVIII. Management: System Based

  1. See complications and hospital indications above
  2. Musculoskeletal (Arthralgias, myalgias in 95% of cases)
    1. First-Line Medications
      1. NSAIDs
      2. Corticosteroids
      3. Hydroxychloroquine
    2. Medications in Refractory cases
      1. Methotrexate
      2. Mycophenylate
  3. Skin Involvement (70-80% of cases)
    1. Use sun screen (minimum SPF 15)
    2. Avoid Photosensitizer medications
    3. First-Line Medications
      1. Topical Corticosteroids (and consider Systemic Corticosteroids)
      2. Topical Calcineurin Inhibitors
      3. Hydroxychloroquine
    4. Medications in Refractory cases
      1. Methotrexate
      2. Retinoids
      3. Dapsone
      4. Mycophenylate
  4. Hematologic effects (e.g. Leukopenia, Anemia, Thrombocytopenia)
    1. Thrombocytopenia
      1. Monitor Platelet Count weekly if Platelets <50,000 cells/mm3
      2. Glucocorticoids AND Immunosuppressants (e.g. Azathioprine, mycophenylate, Cyclosporine)
      3. Consider Rituximab in refractory cases
    2. Automimmune Hemolytic Anemia
      1. Monitor Hematocrit, Hemoglobin And Reticulocyte Count weekly in severe Hemolytic Anemia
      2. Glucocorticoids AND Immunosuppressants
    3. Leukopenia
      1. Reduce infection risk
  5. Lupus Nephritis
    1. Lifetime Prevalence 50%
    2. Screen Urinalysis and Serum Creatinine every 3-6 months (reflex to Urine Protein to Creatinine Ratio)
    3. Renal biopsy indications
      1. Proteinuria >=1 g per 24 hours OR
      2. Hematuria > 0.5 g per 24 hours OR
      3. Cellular Casts
    4. Acute Induction Management
      1. Systemic Corticosteroids AND
      2. Mycophenolate or Cyclophosphamide
    5. Chronic Maintenance Management
      1. Mycophenolate or Azathioprine
  6. Cardiovascular Risk
    1. Acute Coronary Syndrome risk increased in women 35-44 years old by factor of 52
    2. Reduce Cardiovascular Risk Factors (Tobacco Cessation; Hyperlipidemia, Hypertension, Diabetes Mellitus management)
  7. Neuropsychiatric symptoms
    1. Consider MRI Brain for Headache or Seizure
    2. Risk of Cerebrovascular Accident

XIX. Management: Pregnancy

  1. General measures
    1. Aspirin 81 mg orally daily started after 12 weeks (due to risk of Preeclampsia)
    2. Consider Anticoagulant (e.g. LMWH) during pregnancy and Postpartum Period in Antiphospholipid Antibody Syndrome
    3. Consult Maternal-Fetal Medicine and Rheumatology for co-management during pregnancy
  2. SLE Control Medications
    1. Continue Hydroxychloroquine during pregnancy
      1. Decreases pregnancy complications and controls SLE
    2. Other medications that may be used in pregnancy
      1. Prednisone
      2. Azathioprine
      3. Tacrolimus
    3. Contraindicated Medications in Pregnancy (transition to safe medications at least 3 months before conception)
      1. Methotrexate
      2. Mycophenolate
      3. Cyclophosphamide
      4. Leflunomide
  3. Monitor for Fetal Cardiac Complications (cardiac neonatal lupus)
    1. Risk of congenital Heart Block in 1 to 3% of seropositive SLE
    2. Hydroxychloroquine during pregnancy reduces risk of cardiac neonatal lupus
    3. Risk Factors: Cardiotoxic Antibodies
      1. Antiphospholipid Antibodies positive
      2. Anti-Ro or SS-A Antibody Positive
      3. Anti-La or SS-B Antibody Positive
    4. Obtain serial fetal echo between 12 and 26 weeks gestation if Anti-Ro or Anti-La Antibody Positive
      1. Incomplete Heart Block (treated with Dexamethasone)
      2. Complete Heart Block (no known treatment as of 2023)

XX. Management: Severity Based for Non-Renal SLE

  1. Background
    1. Severity based on Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K)
      1. https://www.mdcalc.com/calc/10099/systemic-lupus-erythematosus-disease-activity-index-2000-sledai-2k
    2. Goals of therapy
      1. Remission (SLEDAI-2K of 0, on Hydroxychloroquine and no Corticosteroids) OR
      2. Low disease activity (SLEDAI-2K of <=4, on Hydroxychloroquine and Prednisone <=7.5 mg/day)
  2. Mild Disease: Score <=6 on SLEDAI-2K
    1. First-Line
      1. Hydroxychloroquine AND
      2. Systemic Corticosteroids (as needed)
    2. Refractory
      1. Add Methotrexate
  3. Moderate Disease: Score 7 to 12 on SLEDAI-2K
    1. First-Line
      1. Hydroxychloroquine AND
      2. Systemic Corticosteroids (as needed) AND
      3. Other agents (choose one to add)
        1. Methotrexate
        2. Azathioprine
        3. Calcineurin Inhibitors
        4. Mycophenolate
    2. Refractory
      1. Add Belimumab (Benlysta)
  4. Severe Disease: Score >12 on SLEDAI-2K
    1. First-Line
      1. Hydroxychloroquine AND
      2. Systemic Corticosteroids (as needed) AND
      3. Other agents (choose one to add)
        1. Mycophenolate
        2. Cyclophosphamide
    2. Refractory
      1. Add Rituximab (Rituxan)
  5. References
    1. Fanouriakis (2019) Ann Rheum Dis 78(6):736-45 +PMID: 30926722 [PubMed]

XXI. Management: Medications

  1. Corticosteroids
    1. Longterm goal (once controlled) is to minimize the use of Glucocorticoids
    2. Indicated as a mainstay of acute flare management at lowest effective dose (then taper off or to dose <=7.5 mg/day)
      1. Low dose: Indicated for most patients
        1. Prednisone 7.5 to 10 mg orally daily or less
      2. High dose: Indicated in Lupus Cerebritis, nephritis, Thrombocytopenia
        1. Prednisone 40-60 mg orally daily
        2. Acute organ-threatening disease may require IV doses 250 to 1000 mg/day for up to 3 days
    3. Systemic Corticosteroids in moderate to severe exacerbations
      1. Prednisone 0.5 to 1 mg/kg/day up to 4 weeks or
      2. Solu-Medrol 15 mg/kg IV for 3 days
    4. Skin lesions (no significant evidence to support use)
      1. Topical Corticosteroids
      2. Intralesional Corticosteroids
    5. Monitoring
      1. Serum Glucose every 3 months
      2. DEXA Scan every 1 to 2 years
      3. Monitor for symptoms of avascular necrosis (e.g. Hip Pain)
      4. Use every visit to attempt to taper Corticosteroids, and employ steroid-sparing agents instead
  2. Salicylates and NSAIDs
    1. Indicated for mild to moderate pain from Arthralgias, Headache or other lupus-related conditions
    2. Contraindicated in renal disease
    3. Preparations
      1. Enteric Coated ASA 650 mg PO every 4-6 hours prn
      2. Ibuprofen 400-800 mg PO tid-qid prn
    4. Monitoring
      1. Obtain Serum Creatinine and Complete Blood Count annually
  3. Cytotoxic agents (and other Immunosuppressants): First-Line
    1. Hydroxychloroquine (Plaquenil)
      1. First-line agent in Systemic Lupus management (prevents flares, organ injury, thrombosis and decreases mortality)
      2. Also indicated for Lupus Nephritis, Arthritis and lupus-related skin lesions
      3. Dosing: 200-400 mg/day (limit to <5 mg/kg/day to reduce Macular complication risk)
      4. Effects are delayed for 2 to 8 weeks
      5. Monitoring
        1. Annual dilated Eye Exam and Visual Field testing (including baseline exam)
        2. Low risk patient dilated Eye Exams may be spaced to every 5 years
  4. Cytotoxic agents (and other Immunosuppressants): Second-Line
    1. Azathioprine (Imuran)
      1. Indicated in Lupus Nephritis and severe SLE
      2. Dosing 1.5 to 2.5 mg/kg/day
      3. Therapeutic response and toxicity monitored with thiopurine metabolites (6-MMP, 6-TGN)
      4. Monitoring
        1. Complete Blood Count, comprehensive metabolic panel every 3 months
        2. Observe for hepatotoxicity, lymphoproliferative disorders, myelosuppression
    2. Methotrexate
      1. Indicated in Arthritis, Cutaneous Lupus, serositis and severe SLE
      2. Dosing: 7.5 to 15 mg per week
      3. Monitoring
        1. Complete Blood Count, comprehensive metabolic panel every 3 months
        2. Observe for hepatotoxicity, myelosuppression
  5. Cytotoxic agents (and other Immunosuppressants): Third-Line
    1. Cyclophosphamide
      1. Indicated in Lupus Nephritis or severe SLE
      2. Daily dosing: 1.5-2.5 mg/kg/day or
      3. Monthly dosing: 10-15 mg/kg OR 500 to 1000 mg/m2 IV every 4 weeks
      4. Monitoring
        1. Complete Blood Count, comprehensive metabolic panel, Urinalysis every 3 months
        2. Risk of hemorrhagic cystitis, myelo and Immunosuppression, malignancy
    2. Mycophenolate Mofetil (Cellcept)
      1. Therapeutic response and toxicity monitored with Mycophenolic acid (MPA)
      2. Indicated in Lupus Nephritis and refractory SLE
      3. Typical doses: 2-3 grams/day
      4. Monitoring
        1. Complete Blood Count and comprehensive metabolic panel every 3 months
        2. Observe for signs infection, myelosuppression
    3. Voclosporin (Lupkynis)
      1. Oral Calcineurin Inhibitor
      2. Indicated in Lupus Nephritis (in combination with Mycophenolate and steroids)
      3. Dose 23.7 mg orally every 12 hours
      4. Monitor GFR at baseline, then every 2 weeks for first month, then every 4 weeks
      5. Monitor Blood Pressure every 2 weeks
  6. Monoclonal antibodies (indicated as third-line agent for severe SLE)
    1. Anifrolumab (Saphnelo)
      1. IgG 1 Kappa Monoclonal Antibody against Interferon (type 1)
      2. Dose: 300 mg every 4 weeks via IV infusion
      3. Risk of respiratory infection, Hypersensitivity
    2. Belimumab (Benlysta)
      1. B Lymphocyte Stimulating Factor
      2. Dose: 10 mg/kg IV OR 200 mg SQ once weekly
      3. Risk of serious infections, malignancy
    3. Rituximab (Rituxan)
      1. Dose: 1 g IV repeated in 2 weeks (one time delivery of 2 doses)
  7. Anticoagulation
    1. Indicated in Antiphospholipid Antibody Syndrome (to prevent thrombotic complications)
    2. Warfarin (Coumadin) with goal INR of 2.5 to 3.0
  8. Additional measures
    1. Ophthalmology Consultation for dilated Eye Exam
      1. Initial exam on starting steroids or Plaquenil
      2. Repeat exam yearly in high risk patients

XXII. Prevention

  1. See Adult Health Maintenance Screening
  2. Co-management with rheumatology
  3. Clinic follow-up every 3 to 6 months in stable disease (more often in moderate to severe disease)
    1. Symptom screen
    2. Physical exam
      1. Cardiopulmonary exam
      2. Musculoskeletal Exam
      3. Skin exam
      4. Lymph Node exam
      5. Mental health and Neurologic Exam
    3. Screen for signs and symptoms of malignancy
      1. Hematologic Malignancy
      2. Non-Hodgkin Lymphoma
      3. Lung Cancer
      4. Cervical Cancer
    4. Medication monitoring
      1. Each medication has specific monitoring recommendations (see above)
  4. Labs every 3 months (or at each clinic visit)
    1. Complete Blood Count
    2. Urinalysis
      1. Obtain Urine Protein to Creatinine Ratio (or 24 Hour Urine Protein) if Lupus Nephritis suspected
      2. See Lupus Nephritis management above
    3. Specific metabolic tests (comprehensive metabolic panel is needed if on indicated medications as above)
      1. Serum Creatinine
      2. Serum Glucose (if on Corticosteroids)
    4. Lupus activity markers
      1. Anti-dsDNA
      2. Serum complement levels
  5. Immunizations
    1. See Adult Immunization
    2. Prevnar 20 (PCV20) if Lupus Nephritis or on Immunosuppressants longterm
      1. Pneumovax (PPSV23) is also needed if PCV15 is used
    3. Avoid Live Vaccine (e.g. MMR) if on Immunosuppressants
      1. Do not give Live Vaccine within 1 month of Immunosuppressant
  6. Other diagnostics as indicated
    1. Dilated Eye Exam yearly (if on Hydroxychloroquine)
    2. DEXA Scan yearly (if on Corticosteroids)
  7. Cardiovascular Risk Reduction
    1. See Cardiac Risk Management
    2. Tobacco Cessation
    3. Manage Hyperlipidemia and Hypertension
    4. Screen for and optimize control of Diabetes Mellitus
    5. Encourage Exercise and Healthy Diet
    6. Obesity Management
  8. Contraception
    1. Intrauterine Device
    2. Avoid Oral Contraceptives (due to Hypercoagulable state, esp. in Antiphospholipid Antibody Syndrome)

XXIII. Prognosis

  1. Drug-induced Lupus typically resolves spontaneously after stopping the offending agent
  2. Overall five year survival: 91-97%
  3. Worse prognosis for childhood onset
    1. Lupus Nephritis occurs in 40% of children with SLE (half of these will have CKD 4)
    2. Neuropsychiatric manifestations in one third of children
    3. Increased risk of infection, cardiovascular disease
    4. Much higher mortality in children (10 fold higher than adults)
      1. Untreated mortality approaches 90% at 5 years
  4. Other worse prognostic factors
    1. Seizure Disorder
    2. Lupus Nephritis
    3. Azotemia
  5. References
    1. Ambrose (2016) Lupus 25(14):1542-50 +PMID: 27147622 [PubMed]
    2. Cimaz (2016) Nat Rev Rheumatol 12(7):382-3 +PMID: 27305850 [PubMed]

XXIV. Resources

  1. Lupus Foundation of America
    1. http://www.lupus.org

XXV. References

  1. Edworthy in Ruddy (2001) Kelly's Rheumatology, 1105-19
  2. Green (2014) Crit Dec Emerg Med 28(10): 2-9
  3. Orandi (2024) Mayo Clinic Pediatric Days, lecture attended 1/18/2024
  4. Sercombe in Marx (2002) Rosen's Emergency, p. 1607-13
  5. Ali (2018) Am Fam Physician 98(3): 164-70 [PubMed]
  6. Gill (2003) Am Fam Physician 68:2179-86 [PubMed]
  7. Lam (2016) Am Fam Physician 94(4): 284-94 [PubMed]
  8. Lam (2023) Am Fam Physician 107(4): 383-95 [PubMed]

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Ontology: Lupus Erythematosus, Systemic (C0024141)

Definition (NCI_NCI-GLOSS) A chronic, inflammatory, connective tissue disease that can affect many organs including the joints, skin, heart, lungs, kidneys, and nervous system. It is marked by many different symptoms; however, not everyone with SLE has all of the symptoms.
Definition (MSH) A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Definition (CSP) chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes; it is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system; the disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Concepts Disease or Syndrome (T047)
MSH D008180
ICD9 710.0
ICD10 M32 , M32.9
SnomedCT 201439005, 201435004, 156450004, 55464009
LNC LA15300-9
English SYSTEMIC LUPUS ERYTHEMATOSIS, LUPUS, ERYTHEMATOSUS, SYSTEMIC, Lupus Erythematosus Disseminatus, Lupus Erythematosus, Systemic, LE SYNDROME, DISSEMINATED LUPUS ERYTHEMATOSUS, LUPUS ERYTHEMATOSUS SYSTEMIC, SLE, Disseminated lupus erythematos, SLE-System lupus erythematosus, Systemic lupus erythematos.NOS, Systemic lupus erythematosus, unspecified, SYSTEMIC LUPUS ERYTHEMATOSUS, SYSTEMIC LUPUS ERYTHEMATOSUS SYND, Systemic Lupus Erythematosus, disseminated lupus erythematosus, lupus, systemic lupus erythematosus (diagnosis), systemic lupus, systemic lupus erythematosus, Lupus erythematosis disseminated, Syndrome disseminated lupus erythematosis, Systemic lupus erythematosis, Systemic lupus erythematosus synd, Lupus erythematosus systemic, LE syndrome, LE systemic, Lupus syndrome, Syndrome lupus, Syst lupus erythematosus, SLE NOS, Systemic lupus erythematosus NOS, Lupus Erythematosus, Systemic [Disease/Finding], les syndrome, sles, syndrome lupus, systemic lupus erythematosus (SLE), lupus erythematosis, lupus syndrome, le syndrome, erythematosis lupus systemic, lupus erythematosus disseminatus, lupus syndromes, Systemic lupus erythematosus NOS (disorder), Systemic lupus erythematosus (SLE), Systemic lupus erythematosus, Disseminated lupus erythematosus, SLE - Systemic lupus erythematosus, Systemic lupus erythematosus (disorder), erythematosus; lupus, systemic, lupus; erythematosus, systemic, system; lupus erythematosus, SLE - Lupus Erythematosus, Systemic
French LUPUS ERYTHEMATEUX DISSEMINE, Syndrome lupique, Syndrome du LE, LES, Syndrome de lupus érythémateux disséminé, Syndr de lupus érythémateux systémique, LE systémique, LUPUS ERYTHEMATEUX SYSTEMIQUE, SYNDROME DE LUPUS ERYTHEMATEUX, Lupus érythémateux généralisé, Lupus érythémateux disséminé, Lupus érythémateux systémique, LED (Lupus Érythémateux Disséminé)
Portuguese LUPUS ERITEMATOSO SISTEMICO, Síndrome de LE, Lúpus eritematoso disseminado, Lúpus eritematoso sistémico, LES, LE sistémico, Síndrome lúpica, Síndrome de eritematose lúpus disseminado, Síndrome de lúpus eritematoso sistémico, Lúpus Eritematoso Disseminado, Lúpus Eritematoso Sistêmico, LUPUS ERITEMATOSO SISTEMIC, SINDROMA DE LUPUS ERITEMAT, Lupus eritematoso sistémico, LUPUS ERITEMATOSO DISSEMINADO
Spanish LUPUS ERITEMATOSO SISTEMICO, LUPUS ERITEMATOSO DISEMINADO, Lupus eritematoso diseminado, Síndrome similar al lupus, LE sistémico, Síndrome lúpico, Síndrome de lupus eritematoso diseminado, Síndrome LE, LUPUS ERITEMATOSO, SINDROME, LES, lupus eritematoso sistémico, SAI (trastorno), lupus eritematoso sistémico, SAI, LES, SAI, lupus eritematoso diseminado, lupus eritematoso sistémico (trastorno), lupus eritematoso sistémico, Lupus eritematoso sistémico, Lupus Eritematoso Diseminado, Lupus Eritematoso Sistémico
Dutch lupus erythematodes disseminatus, SLE, lupus erythematodes systemisch, lupussyndroom, lupus erythematosus disseminatus, LE syndroom, systemische lupus erythematosis, LE systemisch, gegeneraliseerd lupus erythematosus syndroom, systemisch lupus erythematosus syndroom, erythematodes; lupus, systemisch, lupus; erythematodes, systemisch, systeem; lupus erythematodes, Lupus erythematodes disseminatus [LED], niet gespecificeerd, gegeneraliseerde lupus erythematosus, Lupus erythematodes disseminatus [LED], LED, Lupus erythematosus disseminatus, Systemische lupus erythematosus
German Lupus-Syndrom, Syndrom, disseminierter Lupus erythematosis, LE-Syndrom, systemischer Lupus erythematosus synd, LE systemisch, systemischer Lupus erythematosus, diseminierter Lupus erythematosus, Lupus erythematosus systemisch, SLE, Syndrom Lupus, Lupus erythematosus disseminiert, LE SYNDROM, LUPUS ERYTHEMATODES DISSEMINATUS, LUPUS ERYTHEMATODES SYSTEMISCH, SYSTEMISCHES LUPUS ERYTHEMATODES, Systemischer Lupus erythematodes, nicht naeher bezeichnet, Systemischer Lupus erythematodes, systemischer Lupus erythematodes, Lupus erythematodes disseminatus, Lupus erythematodes, systemischer
Italian Sindrome da lupus eritematoso, Sindrome da lupus eritematoso disseminato, Sindrome da lupus, Sindrome lupoide, Sindrome da lupus eritematoso sistemico, Lupus eritematoso disseminato, LES, Lupus eritematoso sistemico
Japanese 全身性エリテマトーデス症候群, ＬＥ症候群, ＳＬＥ, ｾﾞﾝｼﾝｾｲｴﾘﾃﾏﾄｰﾃﾞｽ, ﾊｼｭｾｲｴﾘﾃﾏﾄｰﾃﾞｽ, LEｼｮｳｺｳｸﾞﾝ, SLE, ｾﾞﾝｼﾝｾｲｴﾘﾃﾏﾄｰﾃﾞｽｼｮｳｺｳｸﾞﾝ, 全身性ＬＥ, ｾﾞﾝｼﾝｾｲLE, ﾙｰﾌﾟｽｼｮｳｺｳｸﾞﾝ, 播種性エリテマトーデス症候群, ﾊｼｭｾｲｴﾘﾃﾏﾄｰﾃﾞｽｼｮｳｺｳｸﾞﾝ, ループス症候群, 急性び漫性紅斑性狼瘡, 紅斑性狼瘡-全身性, 全身性紅斑性狼瘡, リブマン-サックス症候群, Libman-Sacks病, 播種状紅斑性狼瘡, 急性エリテマトーデス, 全身性エリテマトーデス, 播種状エリテマトーデス, リブマン-サックス病, エリテマトーデス-播種性, 播種性エリテマトーデス, Libman-Sacks症候群, エリテマトーデス-全身性
Swedish Systemisk lupus erythematosus
Finnish Systeeminen lupus erythematosus
Russian LIBMANA-SAKSA BOLEZN', ENDOKARDIT VOLCHANOCHNYI, VOLCHANKA KRASNAIA SISTEMNAIA, VOLCHANKA ERITEMATOZNAIA SISTEMNAIA, ВОЛЧАНКА КРАСНАЯ СИСТЕМНАЯ, ВОЛЧАНКА ЭРИТЕМАТОЗНАЯ СИСТЕМНАЯ, ЛИБМАНА-САКСА БОЛЕЗНЬ, ЭНДОКАРДИТ ВОЛЧАНОЧНЫЙ
Czech Lupus erytematosus disseminovaný, SLE, Syndrom lupusu, Systémový lupus erytematosus, Systémový LE, Lupus erytematosus systémový, Lupusový syndrom, Diseminovaný lupus erytematosus, Syndrom difuzního lupusu erytematodu, LE syndrom, Sytémový lupus erytematosus -syndrom, lupus erythematosus systémový, lupus erythematosus disseminatus, systémový lupus erythematodes
Korean 상세불명의 전신 홍반성 루푸스, 전신 홍반성 루푸스
Croatian LUPUS, ERITEMSKI SISTEMSKI, SISTEMSKI ERITEMSKI LUPUS
Polish Liszaj rumieniowaty narządowy, Liszaj rumieniowaty trzewny, Toczeń rumieniowaty układowy, Toczeń trzewny
Hungarian LE-syndroma, Lupus erythematosus, disszeminált, Disseminált lupus erythematosus, Systemás LE, SLE, Szisztémás lupus erythematosus, Systemás lupus erythematosus, Systemás lupus erythematosus syndroma, Lupus-syndroma, Disseminált lupus erythematosus syndroma, Lupus syndroma
Norwegian Systemisk lupus erythematosus, SLE, Lupus erythematosus, systemisk, Lupus erythematosus disseminatus
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Systemic lupus erythematosus (SMQ) (C1869047)

Definition (MDRCZE) Chronická zánětlivá autoimunitní porucha zasahující mnoho tělesných systémů s různou závažností charakterizovanou různými protilátkami.Protilátky dvouvláknové DNA a polypeptidů Sm Protilátky antifosfolipidů (včetně antikoagulantu lupusu, protilátek antikardiolipinu a protilátek, které vedou k falešně pozitivním výsledkům testu na syfilis)Aktivace komplementu vede k formaci imunního komplexu. Léky mohou vyvolat symptomy systémového erytematózního lupusu (SLE) de novo anebo prohloubit existující symptomy. Klinické obrazy se liší závažností a nástupem.Systémové symptomy: anorexie, hubnutí, nevolnost, myalgie, artralgie a horečkaKožní manifestace (např. makulární „motýlí" erytém, diskovité léze, makulopapulární léze, erytém po stranách dlaní, červené nebo fialové makulární léze na povrchu prstů, plešatost, periungvální erytém, léze membrán sliznic, purpura a citlivost na světloPolyartritida Nefritida může dojít k proteinurii, difúzní membranoproliferativní glomerulonefritidě, abnormálnímu rozboru moči (hematurie, pyurir, válečky v moči) nebo zvýšení kreatininu v séru, hypertenzi a nefrotickému syndromu.Manifestace centrálního nervového systémuBolesti hlavy, epilepsie, psychózy, organický mozkový syndrom a změny osobnosti, mrtvice nebo přechodné ischemické ataky kvůli vaskulární okluziOstatní manifestace / komplikaceKardiovaskulární: perikarditida, myokarditida, fibrinová (Libman-Sachsova) endokarditida a nedomykavosti chlopníPulmonální: pleuritida s výtokem nebo bez nějHematologické: anemie, leukopenie a trombocytopenie kvůli hemolýzeGastrointestinální: bolesti břicha, nevolnost, zvracení a průjemOční: syndrom vysoušení, nespecifický zánět spojivky, zánět sítnice a zánět zrakového nervu
Definition (MDRGER) Chronisch-entzündliche Autoimmunerkrankung, die viele Körpersysteme mit mit verschiedenen Autoantikörpern mit unterschiedlicher Schwere angreift o Antikörper mit Doppel-Strang-DNA und Sm-Polypeptide o Antiphospholipid-Antikörper (einschließlich Lupus-Antikoagulanz, Antikardiolipin-Antikörper und solche, die einen falsch positiven Syphilistest verursachen) o Die Komplementaktivierung führt zur Immunkomplexablagerung in Blutgefäßen Arzneimittel können Symptome von systemischem Lupus erythematosus (SLE) neu auslösen bzw. die Verschlimmerung bestehender Symptome verursachen. Klinische Präsentationen sind unterschiedlich in Bezug auf Schwere und Ausbruch. o Systemische Symptome: Magersucht, Gewichtsabnahme, Unwohlsein, Myalgie, Arthralgien und Fieber o Kutanmanifestationen (z.B. makuläres „Schmetterling"-Erythem, diskoide Läsionen, makulapapuläre Läsionen, Erythem seitlich an den Handflächen; rote oder lila makulare Läsionen an den volaren Fingeroberflächen, Alopezia, periunguales Erythem, Schleimhautläsionen, Purpura und Photosensibilität o Polyarthritis o Nephritis Proteinurie, diffuse membranproliferative Glomerulonephritis, anomale Urinanalyse (Hämaturie, Pyrurie, Harnzylinder) oder Kreatinin erhöht im Serum; Bluthochdruck und nephrotisches Syndrom möglich o Zentralnervensystem betreffende Manifestationen Kopfschmerzen, Epilepsie, Psychosen, organisches Hirnsyndrom, Persönlichkeitsänderungen, Schlaganfall und transiente ischämische Attacken o Andere Manifestationen/Komplikationen Perikarditis, Myokarditis, fibrinöse (Libman-Sachs) Endokarditis und Ventilerkrankungen Pleuritis mit oder ohne Effusion Anämia, Leukopenie und Thrombozytopenie durch Hämolyse Bauchschmerzen, Übelkeit, Erbrechen und Diarrhoe Sicca-Syndrom, unspezifische Konjunktivitis, retinale Vaskulitis und optische Neuritis
Definition (MDRJPN) 種々の自己抗体によって特徴づけられる様々な重症度を呈する多数の身体器官に及ぶ慢性の炎症性自己免疫疾患である。　2本鎖DNAおよびSmポリペプチドに対する抗体　抗リン脂質抗体（ループス性抗凝固因子、抗カルジオリピン抗体、および梅毒の偽陽性検査結果をもたらす抗体を含む）　免疫複合体を形成する補体の活性化。　薬剤によって全身性エリテマトーデス（SLE）の新規症状の誘発や既存の症状の悪化が起こる可能性がある。　臨床症状は重症度や発症によってさまざまである。　全身症状：食欲不振、体重減少、倦怠感、筋肉痛、関節痛、および発熱‐皮膚症状（例、斑状蝶形紅斑、円板状病変、斑状丘疹性病変、手のひら辺縁の紅斑、掌側の指の表面の赤色または紫色の斑状病変、脱毛症、爪周囲の紅斑、粘膜病変、紫斑、および光線過敏性）。　多発性関節炎‐腎炎‐蛋白尿、びまん性膜増殖性糸球体腎炎、尿検査の異常（血尿、膿尿、尿円柱）、または血清クレアチニン値上昇の他に、高血圧やネフローゼ症候群が起こることもある。　中枢神経系症状：頭痛、てんかん、精神病、器質性脳症候群、人格変化、脳卒中、および一過性脳虚血発作。　その他の症状／合併症：心膜炎、心筋炎、線維性（リブマン‐サックス）心内膜炎、および弁膜不全‐滲出を伴うまたは伴わない胸膜炎‐溶血に起因する貧血、白血球減少症、および血小板減少症‐腹痛、悪心、嘔吐、および下痢‐乾燥症候群、非特異性結膜炎、網膜血管炎、および視神経炎。
Definition (MDRHUN) Krónikus gyulladásos autoimmun betegség, ami számos szervrendszert érinthet különböző súlyossági fokkal, és melyet különböző auto-antitestek jelenléte jellemez. Antitestek duplaszálú DNS molekulákhoz és Sm polypeptidekhez. Az antifoszfolipid antitestek (ideértve a lupus anticoagulánst, az anticardiolipin antitesteket, és azokat is, melyek szifilisz fals-pozitív teszteket produkálnak).A komplemens aktiválása immunkomplex formációhoz vezet. A gyógyszerek de novo beindíthatnak systemás lupus erythematosus (SLE) tüneteket, vagy a meglévőeket súlyosbíthatják. A klinikai megjelenés eltérő lehet a súlyos és éppen megjelenő között. Systemás symptomák: anorexia, súlyvesztés, rossz közérzet, izomfájdalom, ízületi fájdalom és láz. Cután megjelenések (vagyis pl. maculosus "pillangó" erythema, discoid laesiók, maculopapulosus laesiók, erythema a tenyér oldalakon, vörös, vagy lila maculosus laesiók a tenyér ujj felületeken, alopecia, periungualis erythema, mucus membran laesiók, purpura és photosensitivitas; polyarthritis; nephritis. Proteinuria, diffúz membranoproliferativ glomerulonephritis, vizeletvizsgálattal kóros (haematuria, pyruia, vizelet cilinderek), vagy emelkedett szérum kreatinin; hypertensió és nephrosis syndroma fordulhat elő. Központi idegrendszeri manifesztációk: fejfájás, epilepszia, psychosis, organikus agy syndroma, személyiség megváltozása, sztrók és átmeneti ischaemiás rohamok. Egyéb manifesztációk/komplikációk: pericarditis, myocarditis, fibrosus (Libman-Sachs) endocarditis, és billentyű elégtelenség. Pleuritis effusióval, vagy a nélkül. Hemolízis miatti anaemia, leukopenia és thrombocytopenia. Abdominalis fájdalom, nausea, hányás és hasmenés. Sicca syndroma, nem-specifikus conjunctivitis, retinalis vasculitis és opticus neuritis.
Definition (MDR) Chronic inflammatory autoimmune disorder affecting many body systems with variable severity characterized by various autoantibodies o Antibodies to double-stranded DNA and Sm polypeptides o Antiphospholipid antibodies (including lupus anticoagulant, anticardiolipin antibodies, and those that cause false-positive tests for syphilis) o Complement activation leads to immune complex formation - Drugs may trigger systemic lupus erythematosus (SLE) symptoms de novo or exacerbate existing symptoms - Clinical presentations vary in severity and onset o Systemic symptoms: anorexia, weight loss, malaise, myalgia, arthralgias and fever o Cutaneous manifestations (e.g., macular "butterfly" erythema, discoid lesions, maculopapular lesions, erythema on sides of the palms, red or purple macular lesions on volar finger surfaces, alopecia, periungual erythema, mucus membrane lesions, purpura and photosensitivity o Polyarthritis o Nephritis - Proteinuria, diffuse membranoproliferative glomerulonephritis, abnormal urinalysis (hematuria, pyruia, urinary casts) or increased serum creatinine; hypertension and nephrotic syndrome can occur o Central nervous system manifestations - Headaches, epilepsy, psychoses, organic brain syndrome, personality changes, stroke and transient ischemic attacks d o Other manifestations/complications - Pericarditis, myocarditis, fibrinous (Libman-Sachs) endocarditis, and valve insufficiencies - Pleurisy with or without effusion - Anemia, leukopenia and thrombocytopenia due to hemolysis - Abdominal pain, nausea, vomiting and diarrhea - Sicca syndrome, nonspecific conjunctivitis, retinal vasculitis and optic neuritis
Definition (MDRSPA) • Es un trastorno autoinmune inflamatorio y crónico que afecta muchos sistemas del organismo con gravedad variable caracterizado por varios autoanticuerpos o Anticuerpos anti-ADN de doble cadena y anti-polipéptidos Sm. o Anticuerpos antifosfolípidos (incluyendo anticoagulante lúpico, anticuerpos anticardiolipina y aquellos que causan falsos positivos en pruebas de sífilis) o La activación del complemento deriva en depósitos de inmunocomplejos • Los fármacos pueden desencadenar síntomas de Lupus eritematoso sistémico (LES) de novo o exacerbar los síntomas existentes • Las presentaciones clínicas varían en gravedad e inicio o Síntomas sistémicos: anorexia, pérdida de peso, malestar general, mialgia, artralgias y fiebre o Manifestaciones cutáneas (p.ej. exantema macular en forma de "alas de mariposa", lesiones discoides, lesiones maculopapulares, eritema en los costados de las palmas; lesiones maculares de color rojo o púrpura en la superficie volar de los dedos de la mano, alopecia, eritema periungueal, lesiones en la membrana mucosa, púrpura y fotosensibilidad o Poliartritis o Nefritis Proteinuria, glomerulonefritis membranoproliferativa difusa, análisis de orina anormal (hematuria, piuria, cilindros urinarios), o creatinina sérica elevada, puede ocurrir hipertensión y síndrome nefrótico o Manifestaciones en el sistema nervioso central Cefaleas, epilepsia, psicosis, síndrome cerebral orgánico y cambios de la personalidad, ictus o accidente isquémico transitorio o Otras manifestaciones/complicaciones Pericarditis, miocarditis, endocarditis fibrinosa (de Libman-Sachs) e insuficiencias valvulares Pleuritis con o sin derrame Anemia, leucopenia y trombocitopenia debido a hemólisis Dolor abdominal, náuseas, vómitos y diarrea Síndrome sicca, conjuntivitis inespecífica, vasculitis retiniana y neuritis óptica
Definition (MDRITA) • Patologia infiammatoria cronica autoimmunitaria che interessa molti sistemi del corpo con gravità variabile caratterizzata da vari autoanticorpi. o Anticorpi contro la doppia elica del DNA e i polipeptidi Sm. o Anticorpi antifosfolipidi (inclusi gli anticorpi anticardiolipina anticoagulanti del lupus, e quelli che causano test falsamente positivi per la sifilide) L'attivazione del complemento induce al deposito di complessi immunitari. • I farmaci possono causare i sintomi del lupus eritematoso sistemico de novo oppure esacerbare sintomi esistenti. • Le presentazioni cliniche variano per gravità e manifestazione. o Sintomi sistemici: anoressia, perdita di peso, malessere, mialgia, artralgia e febbre. o Manifestazioni cutanee (ad es., eritema maculare a "farfalla" , lesioni discoidali e maculopapulari, eritema sui lati dei palmi, lesioni maculari rosse o viola sulle superfici delle dita palmari e plantari, alopecia, eritema periunguale, lesioni alle membrane mucose, porpora e fotosensibilità o Poliartrite o Nefrite: proteinuria, glomerulonefrite membranoploriferativa diffusa, urinanalisi anormale (ematuria, piuria, sedimenti urinari), o creatinina sierica aumentata; si possono verificare ipertensione e sindrome nefrotica o Manifestazioni del sistema nervoso centrale Mal di testa, epilessia, psicosi, sindrome cerebrale organica e alterazioni della personalità, ictus o attacchi ischemici transitori o Altre manifestazioni/complicazioni pericardite, miocardite, endocardite fibrinosa (Libman-Sachs) e insufficienze valvolari pleurite con o senza effusione anemia, leucopenia e trombocitopenia dovuta a emolisi dolore addominale, nausea, vomito e diarrea sindrome sicca, congiuntivite aspecifica, vasculite retinica e nevrite ottica
Definition (MDRFRE) • Affection autoimmune inflammatoire chronique affectant plusieurs systèmes du corps selon différents degrés de sévérité et caractérisé par divers anticorps o Anticorps anti-ADN double brin et anti-polypeptides Sm o Anticorps antiphospholipides (comprenant l'anticoagulant lupique, les anticorps anticardiolipine et ceux produisant des résultats faux positifs pour la syphilis) o L'activation complémentaire entraîne la formation de complexe immun • Les médicaments peuvent déclencher les symptômes de novo du lupus érythémateux systémique (LES) ou exacerber les symptômes existants • La sévérité et la manifestation des symptômes varient selon les présentations cliniques o Symptômes systémiques : anorexie, perte de poids, malaise, myalgie, arthralgies et fièvre o Manifestations cutanées (par exemple, érythème maculaire en ailes de papillon, lésions discoïdes, lésions maculopapulaires, érythème des côtés des paumes, lésions maculaires rouges ou pourpres sur la face palmaire des doigts, alopécie, érythème périunguéal, lésions de la muqueuse, purpura et photosensibilité o Polyarthrite o Néphrite Protéinurie, glomérulonéphrite membranoproliférative diffuse, urines anormales (hématurie, pyurie, cylindres urinaires) ou augmentation de la créatinine sérique ; hypertension et syndrome néphrotique peuvent survenir o Manifestations du système nerveux central Céphalées, épilepsie, psychoses, syndrome de cerveau organique et changements de la personnalité, accident vasculaire cérébral ou crises ischémiques transitoires o Autres manifestations/complications péricardite, myocardite, endocardite fibrineuse (Libman-Sachs) et insuffisances de valves Pleurésie avec ou sans effusion Anémie, leucopénie et thrombocytopénie dues à l'hémolyse Douleurs abdominales, nausées, vomissements et diarrhée syndrome de Gougerot-Sjögren, conjonctivite non précisée, vascularite rétinienne et névrite optique
Definition (MDRDUT) • Chronische inflammatoire auto-immuunziekte die vele lichaamsstelsels met variabele mate van ernst beïnvloeden, gekenschetst door diverse auto-antistoffen o Antistoffen voor dubbelstrengig DNA en SM polypeptiden o Antifosfolipiden-antistoffen (met inbegrip van lupus-anticoagulans, anticardiolipine-antistoffen en die welke valspositieve tests voor syfilis veroorzaken) o Complement-activering leidt tot de depositie van immuuncomplex • Geneesmiddelen kunnen de novo symptomen van systemische lupus erythematodes triggeren of bestaande symptomen verergeren • Klinische presentaties variëren wat ernst en onset betreft o Systemische symptomen: anorexie, gewichtsverlies, malaise, myalgie, artralgieën en koorts o Cutane manifestaties (bijv. maculair vlindervormig erytheem, discoïde laesies, maculopapulaire laesies, erytheem aan zijden van de palmen; rode of paarse maculaire laesies op volaire vingeroppervlakken, alopecie, periunguaal erytheem, slijmvlieslaesies, purpura en lichtgevoeligheid o Polyartritis o Nefritis proteïnurie, diffuse membranoproliferatieve glomerulonefritis, abnormale urineanalyse (hematurie, pyurie, urinesediment) of verhoogd serumcreatinine; hypertensie en nefrotisch syndroom kunnen zich voordoen o Manifestaties van het centraal zenuwstelsel Hoofdpijn, epilepsie, psychosen, organisch hersensyndroom, persoonlijkheidsveranderingen, beroerte en transient ischaemic attacks o Andere manifestaties/complicaties Pericarditis, myocarditis, fibrineuze (Libman-Sachs) endocarditis en klepinsufficiënties Pleuritis met of zonder effusie Anemie, leukopenie en trombocytopenie als gevolg van hemolyse Buikpijn, misselijkheid, braken en diarree Sicca-syndroom, niet-specifieke conjunctivitis, retinavasculitis en neuritis optica
Definition (MDRPOR) • É uma afecção autoimune inflamatória e crónica que afecta muitos sistemas do organismo com gravidade variável caracterizada por vários autoanticorpos ou Anticorpos anti-ADN de cadeia dupla e anti-polipéptidos Sm. o Anticorpos antifosfolípidos (incluindo anticoagulante lúpico, anticorpos anticardiolipina e aqueles que causam falsos positivos em testes de sífilis) o A activação do complemento leva à formação de depósitos de imunocomplexos • Os fármacos podem desencadear sintomas de Lupus eritematoso sistémico (LES) de novo ou exacerbar os sintomas existentes • A apresentações clínicas variam em gravidade e início o Sintomas sistémicos: anorexia, perda de peso, mal-estar geral, mialgia, artralgias e febre o Manifestações cutâneas (p. ex. Eritema macular em forma de "asas de borboleta", lesões discóides, lesões maculopapulares, eritema nos lados das palmas; lesões maculares de cor vermelha ou púrpura na superfície palmar dos dedos da mão, alopecia, eritema periungueal, lesões na membrana mucosa, púrpura e fotossensibilidade o Poliartrite o Nefrite Proteinúria, glomerulonefrite membranoproliferativa difusa, análise da urina anormal (hematúria, piúria, cilindros urinários), ou creatinina sérica aumentada; pode ocorrer a hipertensão e a síndrome nefrótica o Manifestações do sistema nervoso central Cefaleias, epilepsia, psicose, síndrome cerebral orgânica e mudanças da personalidade, acidente vascular cerebral e ataques isquémicos transitórios o Outras manifestações/complicações Pericardite, miocardite, endocardite fibrinosa (de Libman-Sachs) e insuficiências valvulares Pleurite com ou sem derrame Anemia, leucopenia e trombocitopenia devido a hemólise Dor abdominal, náuseas, vómitos e diarreia Síndrome sicca, conjuntivite não específica, vasculite retiniana e neurite óptica
Concepts Classification (T185)
English Systemic lupus erythematosus (SMQ)
Dutch Systemische lupus erythematodes (SMQ)
French Lupus érythémateux systémique (SMQ)
German Systemischer Lupus erythematodes (SMQ)
Italian Lupus eritematoso sistemico (SMQ)
Spanish Lupus eritematoso sistémico (SMQ)
Portuguese Lúpus eritematoso sistémico (SMQ)
Czech Systémový lupus erythematosus (SMQ)
Japanese 全身性エリテマトーデス（ＳＭＱ）
Hungarian Systemás lupus erythematosus (SMQ)
Derived from the NIH UMLS (Unified Medical Language System)

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