Rheumatology Book


Systemic Sclerosis

Aka: Systemic Sclerosis, Scleroderma, Systemic Scleroderma
  1. Epidemiology
    1. Incidence: 10-20 cases per 1 million persons annually
    2. Prevalence: Up to 250 cases per 1 million persons
  2. Pathophysiology
    1. Idiopathic Collagen Disease
    2. Progressive Muscle atrophy and fibrosis
    3. Esophagus changes
      1. Loss of peristalsis
      2. Sphincter reduction
  3. Types
    1. Limited cutaneous Systemic Sclerosis (60% of cases)
      1. Typical skin involvement is distal to elbows and knees
      2. Associated with Pulmonary Hypertension
      3. Associated with CREST Syndrome
        1. Severe Gastroesophageal Reflux is common
        2. Raynaud's Phenomenon are common
      4. Other associated findings
        1. Telangiectasia
        2. Calcinosis cutis
        3. Sclerodactyly
        4. Digital ischemia
    2. Diffuse cutaneous Systemic Sclerosis (35% of cases)
      1. Skin involvement may be proximal to elbows and knees and may affect face
      2. Associated with Interstitial Lung Disease (pulmonary fibrosis)
      3. May present with renal disease
      4. Skin pigment may be altered
      5. Tendons friction rubs may be present
    3. Systemic Sclerosis sine Scleroderma (5% of cases)
      1. Internal organ manifestations only
  4. Symptoms
    1. Gradual symptom onset
    2. Anorexia
    3. Dyspnea
    4. Dysphagia
    5. Reduced sweating
    6. Arthralgias
  5. Signs (see specific types above)
    1. Key features
      1. Scleroderma
        1. Skin hardening
        2. Skin contracted with immobility and symmetric deformity (tight skin)
      2. Raynaud's Phenomenon
    2. Other findings
      1. Low grade fever
      2. Muscular weakness
      3. Limited chest expansion
      4. Limited jaw opening
      5. Digital pitting or ulceration
  6. Labs
    1. Screening
      1. Antinuclear Antibody
        1. Present in 95% of Systemic Sclerosis (all subtypes)
    2. Confirmation
      1. ANA Nucleolar Pattern
      2. Anti-centromere Antibody
        1. Most associated with limited cutaneous subtype (60-80% of cases)
      3. Antitopoisomerase-1 Antibody (Anti-Scl-70)
        1. Associated with diffuse cutaneous subtype (esp. more severe cases)
        2. Associated with worse prognosis (increased mortality, higher risk of Interstitial Lung Disease)
        3. Test Sensitivity: 43%
        4. Test Specificity: 100%
  7. Differential Diagnosis
    1. Amyloidosis
    2. Eosinophilia-Myalgia Syndrome
    3. Nephrogenic Fibrosing Dermopathy
    4. Scleromyxedema
    5. Toxic oil syndrome
  8. Complications
    1. Esophageal Dysmotility
      1. Substantial Gastroesophageal Reflux Disease
      2. Barrett's Esophagus
    2. Pulmonary fibrosis
    3. Pulmonary Hypertension
    4. Scleroderma renal crisis
    5. Digital infarction
  9. Management
    1. See Raynaud Phenomenon
    2. See Pulmonary Fibrosis
    3. See Pulmonary Hypertension
    4. Skin Fibrosis
      1. Chemotherapy agents
    5. Esophageal Dysmotility
      1. Chew food well and drink adequate liquids with food
      2. Gastric acid reduction
        1. H2 Blocker (e.g. Ranitidine)
        2. Proton Pump Inhibitor (e.g. Prilosec)
  10. Resources
    1. Scleroderma Foundation
      1. http://www.scleroderma.org
  11. References
    1. Hawk (2001) Semin Cutan Med Surg 20(1):27-37 [PubMed]
    2. Hinchcliff (2008) Am Fam Physician 78:961-8 [PubMed]
    3. Mitchell (1997) Med Clin North Am 81(1):129-49 [PubMed]
    4. Steen (2006) Autoimmun Rev 5(2):122-4 [PubMed]

Systemic Scleroderma (C0036421)

Definition (CHV) a rare, chronic disease characterized by excessive deposits of collagen in the skin or other organs
Definition (CHV) a rare, chronic disease characterized by excessive deposits of collagen in the skin or other organs
Definition (CHV) a rare, chronic disease characterized by excessive deposits of collagen in the skin or other organs
Definition (CHV) a rare, chronic disease characterized by excessive deposits of collagen in the skin or other organs
Definition (CHV) a rare, chronic disease characterized by excessive deposits of collagen in the skin or other organs
Definition (NCI_NCI-GLOSS) A disease that is marked by hardening and thickening of skin, connective tissue that surrounds other tissues and organs, and blood vessels.
Definition (NCI) A chronic disorder, possibly autoimmune, marked by excessive production of collagen which results in hardening and thickening of body tissues. The two types of systemic scleroderma, limited cutaneous and diffuse cutaneous are classified with focus on the extent of affected skin. A relationship exists between the extent of skin area affected and degree of internal organ/system involvement. Systemic scleroderma can manifest itself in pulmonary fibrosis, Raynaud's syndrome, digestive system telangiectasias, renal hypertension and/or pulmonary hypertension.
Definition (MSH) A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA.
Definition (CSP) systemic disorder of the connective tissue; manifested by hardening and thickening of the skin, by abnormalities involving the microvasculature and larger vessels, and by fibrotic degenerative changes in various body organs including the heart, lungs, kidneys, and gastrointestinal tract.
Concepts Disease or Syndrome (T047)
MSH D012595
ICD9 710.1
ICD10 M34.0, M34, M34.9
SnomedCT 89155008, 128457007, 268049000, 201440007, 156451000
English Progressive systemic sclerosis, Scleroderma, Systemic, PROGRESSIVE SYSTEMIC SCLEROSIS, Systemic Scleroderma, Sclerosis, Systemic, Systemic sclerosis, unspecified, PSS - Progressive syst scleros, PSS - Progressive systemic sclerosis, Diffuse Sclerosis, Diffuse Scleroderma, Scleroderma, Diffuse, Scleroderma, Systemic [Disease/Finding], scleroderma, sclerodermas, Scleroderma;progressive, Progressive system sclerosis, systemic scleroderma, Scleroderma syndrome (disorder), systemic sclerosis, Systemic Sclerosis, Systemic scleroderma, Systemic sclerosis, SS - Systemic sclerosis, Scleroderma syndrome, Systemic sclerosis (disorder), progressive systemic sclerosis, PSS (progressive systemic sclerosis), sclerosis; systemic, progressive, sclerosis; systemic, sclerosis; system, system; sclerosis, systemic; sclerosis, progressive, systemic; sclerosis, progressive scleroderma, progressive system sclerosis
Dutch progressieve systemische sclerose, sclerose; systeem, sclerose; systemisch, progressief, sclerose; systemisch, systeem; sclerose, systemisch; sclerose, progressief, systemisch; sclerose, Systemische sclerose, niet gespecificeerd, gegeneraliseerde sclerose, Systemische sclerose, Progressieve systemische sclerose, Sclerodermie, systemische, Systemische sclerodermie
French Sclérose systémique progressive, Sclérose générale, Sclérodermie généralisée, Sclérodermie systémique, Sclérose systémique
German progressive systemische Sklerose, Progressive systemische Sklerose, Systemische Sklerose, nicht naeher bezeichnet, Systemische Sklerose, systemische Sklerose, Sklerodermie, systemische, Systemische Sklerodermie
Italian Sclerosi sistemica progressiva, Sclerosi sistemica, Sclerodermia sistemica
Portuguese Esclerose sistémica progressiva, Esclerose sistémica, Escleroderma Sistêmico, Esclerose Sistêmica
Spanish Esclerosis sistémica progresiva, esclerosis sistémica progresiva, trastorno: síndrome de esclerodermia, en sus formas localizada y sistémica (concepto no activo), síndrome de esclerodermia, esclerodermia (trastorno), esclerosis sistémica (trastorno), esclerosis sistémica, Esclerosis sistémica, Esclerodermia Sistémica, Esclerosis Sistémica
Japanese 進行性全身性硬化症, ゼンシンセイコウカショウ, シンコウセイゼンシンセイコウカショウ, 全身性強皮症, 全身性硬皮症, 強皮症-全身性, 硬化症-全身性, 全身性皮膚硬化症, 全身性硬化症, 鞏皮症-全身性, 全身性鞏皮症
Swedish Sklerodermi, systemisk
Czech skleróza progresivní systémová, Systémová skleróza, Progresivní systémová skleróza, systémová sklerodermie, sklerodermie systémová, systémová skleróza
Finnish Systeeminen skleroosi
Korean 전신 경화증, 상세불명의 전신 경화증, 진행성 전신 경화증
Polish Twardzina układowa
Hungarian Progresszív systemás sclerosis, Systemás sclerosis
Norwegian Systemisk sklerodermi, Systemisk sklerose, Sclerosis systemica
Derived from the NIH UMLS (Unified Medical Language System)

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