Sjogren's Syndrome

Aka: Sjogren's Syndrome, Sjogrens Syndrome, Sjogren Syndrome, Keratoconjunctivitis Sicca, Dry Eye Syndrome

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II. Epidemiology

  1. Incidence: 4 per 100,000
  2. Prevalence: 1-2 Million in United States
  3. Gender: Predominately women (93% in one study)
  4. Age of onset: Middle aged (mean age of onset 52 years old in one study)
  5. Garcia-Carrasco (2002) Medicine 81(4): 270-80 [PubMed]

III. Causes and Associated Conditions

  1. Primary: Specific Autoimmune Disease (Sjogren's)
  2. Secondary associations (Keratoconjunctivitis Sicca)
    1. Rheumatoid Arthritis (occurs in 25% of RA cases)
    2. Hypothyroidism (10-15%)
    3. Systemic Lupus Erythematosus
    4. Scleroderma
    5. Polymyositis
    6. Felty's Syndrome
    7. Pulmonary Fibrosis

IV. Pathophysiology

  1. Environmental stimulus triggers autoimmune reaction directed at exocrine glands
  2. Postulated triggers: CMV and EBV
  3. Association with HLA-DR haplotype
  4. B-Lymphocyte mediated chronic exocrine gland destruction
    1. Lacrimal glands affected in Keratoconjunctivitis Sicca
    2. All exocrine glands affected in Sjogren's Syndrome
      1. Salivary Glands
      2. Lacrimal glands
      3. Sebaceous Glands
      4. Vaginal glands

V. Symptoms: Most Common (93-98% of patients at presentation)

  1. Xerostomia (Dry Mouth)
    1. Frequent sips of water
    2. May impact speaking, eating and Swallowing
    3. Exam demonstrates dry mucus membranes (secondary redness and ulcers may be present)
    4. Dental Caries are more common
    5. Parotid Glands tender to touch in some cases
  2. Xerophthalmia (Dry Eyes): Keratoconjunctivitis Sicca
    1. Symptoms worse toward latter part of day
    2. Foreign Body Sensation
    3. Painful or itching eyes
    4. Conjunctival injection
    5. Keratoconjunctivitis
    6. Corneal clouding in severe cases

VI. Symptoms: Other Associations

  1. Gastrointestinal
    1. Dyspepsia
    2. Altered stool habits
  2. Genitourinary
    1. Renal conditions (e.g. Glomerulonephritis, Interstitial Nephritis, Renal Tubular Acidosis)
  3. Hematologic
    1. Lymphadenopathy
  4. Musculoskeletal
    1. Non-erosive Arthritis
  5. Neurologic
    1. Peripheral Neuropathy
  6. Respiratory
    1. Nasal dryness
    2. Sinusitis
    3. Chronic Cough or recurrent Bronchitis
    4. Imaging
      1. XRay may show chronic Interstitial Infiltrates
      2. CT Chest may demonstrate alveolitis or fibrosis
  7. Skin
    1. Dry Skin
    2. Cutaneous Vasculitis
    3. Raynaud's Phenomenon
  8. Constitutional and other effects
    1. Fatigue
    2. Major Depression
    3. Fever

VII. Diagnosis: Revised International Classification Criteria (4 of 6 criteria required)

  1. Lip Salivary Gland biopsy positive (see diagnostics below)
  2. Autoantibodies Anti-Ro (Anti-SSA) and Anti-La (Anti-SSB) present
  3. Ocular symptoms: One or more of the following
    1. Dry Eyes Sensation everyday for at least 3 months
    2. Recurrent Eye Foreign BodySensation (sand or gravel Sensation)
    3. Artificial tears instilled more than 3 times daily
  4. Ocular signs: One or more of the following
    1. Schirmer Test
    2. Rose Bengal Test (or other ocular dye test)
  5. Oral symptoms: One or more of the following
    1. Dry MouthSensation everyday for at least 3 months
    2. Salivary Gland swelling recurrent or persistent
    3. Dry food intake requires frequent Swallowing of liquids
  6. Oral signs: One or more of the following demonstrating Salivary Gland involvement
    1. Nonstimulated Whole Salivary Flow Collection (<1.5 ml in 15 minutes)
    2. Contrast parotid sialography demonstrates diffuse sialectasia
    3. Salivary scintigraphy with delayed uptake and decreased concentration and excretion
  7. References
    1. Vitali (2002) Ann Rheum Dis 61(6): 554-8 [PubMed]

VIII. Diagnostics: Dry Eye evaluation

  1. Schirmer's Test
  2. Rose Bengal Test
  3. Slit Lamp Exam of Cornea
    1. Decreased tear meniscus
    2. Punctate erosive keratopathy

IX. Diagnostics: Dry Mouth evaluation

  1. Nonstimulated Whole Salivary Flow Collection
  2. Minor Salivary Gland biopsy from lip (pathognomonic findings)
    1. Acinar gland lymphocytic infiltration with secondary degeneration, necrosis, atrophy
    2. Positive biopsy: one or more foci of dense inflammatory infiltrate >50 lymphs/4mm

X. Labs: Serology

  1. Rheumatoid Factor positive (32-90% positive)
  2. Antinuclear Antibody positive (55-97% positive)
    1. ANA Test Sensitivity: 48%
    2. ANA Test Specificity: 52%
    3. Positive and Negative Likelihood Ratios approach 1.0
  3. Sjogren Antibodies
    1. Autoantibody Anti-Ro (Anti-SSA) present (16-70%)
    2. Autoantibody Anti-La (Anti-SSB) present (7-50%)
    3. Increased risk of Sjogren Syndrome when antibodies present in Rheumatoid Arthritis
    4. Increased risk of cytopenias, Discoid Lupus, Lupus Nephritis when antibodies present in SLE
  4. References
    1. Garcia-Carrasco (2002) Medicine 81(4): 270-80 [PubMed]

XI. Labs: Other

  1. Erythrocyte Sedimentation Rate elevated
  2. Complete Blood Count
    1. Leukopenia
    2. Anemia
  3. Liver Function Tests abnormal

XII. Management: General

  1. Dry Eyes (Xerophthalmia)
    1. See Dry Eyes for management
  2. Dry Mouth (Xerostomia)
    1. See Dry Mouth for Management
  3. Dry nose
    1. Saline Nasal Spray
    2. Humidifier
  4. Dry Skin
    1. Skin moisturizing creams and ointments after shower
  5. Vaginal Dryness
    1. Vaginal Lubricants
    2. Estrogen Replacement Therapy
    3. Vaginal Estrogen cream
  6. Infection
    1. Observe for Thrush or Yeast Vaginitis

XIII. Management: Systemic Medications

  1. Dry Eyes and Dry Mouth
    1. See Muscarinic Agonists (e.g. Pilocarpine)
  2. Arthralgias
    1. NSAIDs
    2. Hydroxychloroquine (Plaquenil)
    3. Corticosteroids
  3. Monoclonal antibodies
    1. Rituximab (Rituxan): anti-CD20 - decreases B-Lymphocyte activity

XIV. Complications

  1. Non-Hodgkin Lymphoma (40 fold increased risk over general population)
  2. Dental Caries
  3. Sialadenitis
  4. Corneal Ulceration
  5. Chronic Oral Infection

XV. References

  1. Fox (2005) Lancet 366(9482): 321-31 [PubMed]
  2. Kassan (2004) Arch Intern Med 164(12): 1275-84 [PubMed]
  3. Kruszka (2009) Am Fam Physician 79(6): 465-70 [PubMed]

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Related Studies

Ontology: Dry Eye Syndromes (C0013238)

Definition (MSHCZE) Vysychání spojivky a rohovky oka, které může vést k jejich těžkému poškození. Srov. xerostomie, Sjögrenův syndrom, sicca syndrom, vitamin A. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI) A syndrome characterized by dryness of the cornea and conjunctiva. It is usually caused by a deficiency in tear production. Symptoms include a feeling of burning eyes and a possible foreign body presence in the eye.
Definition (NCI_CTCAE) A disorder characterized by dryness of the cornea and conjunctiva.
Definition (MSH) Corneal and conjunctival dryness due to deficient tear production, predominantly in menopausal and post-menopausal women. Filamentary keratitis or erosion of the conjunctival and corneal epithelium may be caused by these disorders. Sensation of the presence of a foreign body in the eye and burning of the eyes may occur.
Concepts Disease or Syndrome (T047)
MSH D015352 , D014985
ICD10 H04.12
SnomedCT 193980001, 155182003, 1249004, 46152009
English Syndrome, Dry Eye, Syndromes, Dry Eye, dry eye, Dry Eye Syndromes, dry eye syndrome (diagnosis), dry eye syndrome, Keratoconjunctivitis Sicca, Dry Eye Syndromes [Disease/Finding], syndrome dry eye, dry eyes syndrome, dry eye syndromes, Dry eye syndrome (disorder), Dry eye (finding), Dry eye, Dry eye syndromes, EYE(S), DRY, DRY EYE(S), Dry eye syndrome, dry eye; syndrome, syndrome; dry eye, Dry Eye Syndrome
Swedish Torrögdhetssyndrom
Czech xeroftalmie, syndromy suchého oka, Syndrom suchého oka, keratitis sicca, KCS, keratoconjunctivitis sicca
Finnish Kseroftalmia, Kuivasilmäisyysoireyhtymät
Russian KSEROFTAL'MIIA, SUKHOGO GLAZA SINDROMY, GLAZA SUKHOGO SINDROMY, ГЛАЗА СУХОГО СИНДРОМЫ, СУХОГО ГЛАЗА СИНДРОМЫ
Croatian KSEROFTALMIJA, Ceratoconjunctivitis sicca, SUHI KERATOKONJUNKTIVITIS, Sindrom suhog oka
Polish Zespoły suchego oka, Zeschnięcie spojówki i rogówki, Zeschnięcie gałki ocznej
Spanish Síndrome de ojo seco, Síndromes de Ojo Seco
Portuguese Síndrome de olho seco, Síndromes do Olho Seco, Xeroftalmia
Dutch droog oogsyndroom, droge ogen; syndroom, syndroom; droge ogen, Droogoogsyndromen, Droogoogsyndroom, Syndromen, droogoog-, Syndroom, droogoog-
French Syndrome des yeux secs, Xérophtalmie, Syndromes de l'oeil sec
German Trockene Augen-Syndrom, Syndrome des trockenen Auges, Xerophthalmie
Hungarian Száraz szem syndroma
Italian Sindrome dell'occhio secco, Sindromi dell'occhio secco, Xeroftalmia
Japanese ｶﾞﾝｶﾝｿｳｼｮｳｺｳｸﾞﾝ, 眼乾燥症候群
Norwegian Tørre øyne, Xeroftalmi, Tørt øye-syndrom, A-vitaminmangel med konjunktival tørrhet
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Keratoconjunctivitis Sicca (C0022575)

Definition (MSHCZE) Keratokonjunktivitida vznikající v rámci sicca syndromu. Nedostatek slz činí spojivky i rohovku náchylnějšími k infekci a poškození. Postižení mohou mít pocit cizího tělesa nebo pálení očí. Kromě celkového vyšetření a sledování se z oftalmologického hlediska podávají preparáty s „umělými slzami“. Srov. xeroftalmie. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (MSH) Drying and inflammation of the conjunctiva as a result of insufficient lacrimal secretion. When found in association with XEROSTOMIA and polyarthritis, it is called SJOGREN'S SYNDROME.
Definition (CSP) drying and inflammation of the conjunctiva as a result of insufficient lacrimal secretion.
Concepts Disease or Syndrome (T047)
MSH D007638
SnomedCT 78946008, 201444003, 156455009, 268050000, 302896008
English Sicca, Keratoconjunctivitis, KCS - Keratoconjunctivit sicca, Keratoconjunctivitis Sicca, KCS, Keratoconjunctivitis Sicca [Disease/Finding], kc, kcs, kerato conjunctivitis sicca, keratoconjunctivitis sicca, Keraconjunctivitis sicca, Dry eye, Keratoconjunctivitis sicca, KCS - Keratoconjunctivitis sicca, Keratoconjunctivitis sicca (disorder)
Swedish Keratoconjunctivitis sicca
Czech keratokonjunktivitida suchá, Keratokonjunktivitis sicca
Finnish Keratoconjunctivitis sicca
Russian KERATOKON"IUNKTIVIT SUKHOI, КЕРАТОКОНЪЮНКТИВИТ СУХОЙ
Polish Zapalenie rogówki i spojówki suche
Spanish Queratoconjuntivitis seca, queratoconjuntivitis sicca (trastorno), queratoconjuntivitis sicca, Queratoconjuntivitis Seca
Portuguese Queratoconjuntivite seca, Ceratoconjuntivite Seca
Dutch keratoconjunctivitis sicca, Keratoconjunctivitis sicca
German Keratokonjunktivitis sicca, Keratoconjunctivitis sicca
Hungarian Keratoconjunctivitis sicca
Japanese 乾性角結膜炎, ｶﾝｾｲｶｸｹﾂﾏｸｴﾝ
Norwegian Keratoconjunctivitis sicca
French Kératoconjonctivite sèche
Italian Cheratocongiuntivite secca
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Sjogren's Syndrome (C1527336)

Definition (CHV) A chronic inflammation of the tear and salivary glands, often accompanied by rheumatoid arthritis and the presence of autoantibodies in the blood
Definition (CHV) A chronic inflammation of the tear and salivary glands, often accompanied by rheumatoid arthritis and the presence of autoantibodies in the blood
Definition (CHV) A chronic inflammation of the tear and salivary glands, often accompanied by rheumatoid arthritis and the presence of autoantibodies in the blood
Definition (CHV) A chronic inflammation of the tear and salivary glands, often accompanied by rheumatoid arthritis and the presence of autoantibodies in the blood
Definition (CHV) A chronic inflammation of the tear and salivary glands, often accompanied by rheumatoid arthritis and the presence of autoantibodies in the blood
Definition (CHV) A chronic inflammation of the tear and salivary glands, often accompanied by rheumatoid arthritis and the presence of autoantibodies in the blood
Definition (CHV) A chronic inflammation of the tear and salivary glands, often accompanied by rheumatoid arthritis and the presence of autoantibodies in the blood
Definition (CHV) A chronic inflammation of the tear and salivary glands, often accompanied by rheumatoid arthritis and the presence of autoantibodies in the blood
Definition (CHV) A chronic inflammation of the tear and salivary glands, often accompanied by rheumatoid arthritis and the presence of autoantibodies in the blood
Definition (MEDLINEPLUS)

Sjogren's syndrome is a disease that causes dryness in your mouth and eyes. It can also lead to dryness in other places that need moisture, such as your nose, throat and skin. Most people who get Sjogren's syndrome are older than 40. Nine of 10 are women. Sjogren's syndrome is sometimes linked to rheumatic problems such as rheumatoid arthritis.

Sjogren's syndrome is an autoimmune disease. If you have an autoimmune disease, your immune system, which is supposed to fight disease, mistakenly attacks parts of your own body. In Sjogren's syndrome, your immune system attacks the glands that make tears and saliva. It may also affect your joints, lungs, kidneys, blood vessels, digestive organs and nerves. The main symptoms are:

Treatment focuses on relieving symptoms.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases
Definition (MSHCZE) Imunopatologická choroba charakterizovaná triádou příznaků: keratokonjunktivitida, parotitida, artritida. Bývají známky zduření slinných žláz a žlázy slzné se suchostí úst (xerostomie) a očí (xeroftalmie), s polyartritickými příznaky (srov. sicca syndrom). Snížená může být i sekrece z pankreatu či žaludku. Žlázy jsou infiltrovány lymfocyty (srov. Mikuliczův syndrom) a v některých případech je pozorován vznik maligního lymfomu z B buněk. S. n. postihuje výrazně častěji ženy, zejm. v menopauze. Kurativní léčba není známa, léčba je zaměřena na postižené orgány. Používají se umělé slzy, látky stimulující slinění (např. pilokarpin), systémově imunosupresiva či antirevmatika. Důležitá je i péče o zuby, kůži. S. n. se někdy označuje jako primární Sjögrenův syndrom, zatímco obdobné příznaky sicca syndromu spojené se systémovým onemocněním jako sekundární Sjögrenův syndrom. Terminologie však není jednoznačná. Kromě imunologického vyšetření (zvýšené IgM, autoprotilátky anti SS, ENA) je v diagnóze důležitá biopsie [H. Sjögren]. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI) An autoimmune disorder affecting the salivary and lacrimal glands. Morphologically, it is characterized by the presence of lymphocytic and plasmacytic infiltrates which cause destruction of these glands. It results in dry mouth and dry eyes. It may be associated with the presence of other autoimmune disorders, including rheumatoid arthritis and lupus erythematosus.
Definition (NCI_NCI-GLOSS) An autoimmune disease that affects the tear glands and salivary glands, and may affect glands in the stomach, pancreas, and intestines. The disease causes dry eyes and mouth, and may cause dryness in the nose, throat, air passages, skin, and vagina. It may also cause inflammation in the joints, muscles, and skin; pneumonia; tingling in the fingers and toes; and fatigue. It often occurs with rheumatoid arthritis or other connective tissue diseases.
Definition (MSH) Chronic inflammatory and autoimmune disease in which the salivary and lacrimal glands undergo progressive destruction by lymphocytes and plasma cells resulting in decreased production of saliva and tears. The primary form, often called sicca syndrome, involves both KERATOCONJUNCTIVITIS SICCA and XEROSTOMIA. The secondary form includes, in addition, the presence of a connective tissue disease, usually rheumatoid arthritis.
Definition (CSP) symptom complex of unknown etiology, usually occurring in middle-aged or older women, marked by the triad of keratoconjunctivitis sicca with or without lacrimal gland enlargement, xerostomia with or without salivary gland enlargement, and the presence of a connective tissue disease, usually rheumatoid arthritis but sometimes systemic lupus erythematosus, scleroderma, or polymyositis; an abnormal immune response has been implicated.
Concepts Disease or Syndrome (T047)
MSH D012859
ICD10 M35.0
SnomedCT 83901003, 201444003, 156455009, 268050000
LNC LP36352-0, LA15301-7
English Syndrome, Sjogren's, SJOGRENS SYNDROME, Sjogrens Syndrome, Sjogren's disease, Sicca syndrome [Sjogren], SJOGREN SYNDROME, Sjogren's syndrome (diagnosis), Sjogren's, Syndrome Sjogren's, Sjogren's syndrome, Sjögren syndrome, Sicca syndrome [Sjögren], Sjogren's Syndrome [Disease/Finding], syndrome sjogren, sjogrens's syndrome, sjogrens syndrome, sjogren's syndromes, sjogren's disease, sjogren's syndrome, syndrome sjogrens, sjogren syndrome, sjoegren's syndrome, Sjogren syndrome, Sjogrens syndrome, Sjogren Syndrome, Gougerot-Mulock-Houwer syndrome, Sicca (Sjogren's) syndrome, SYNDROME, SJOGREN'S, SJOGREN'S SYNDROME, Sjoegren's syndrome, Sjögren's disease, Sjögren's syndrome (disorder), Sjögren's syndrome, Sjögren, Sjogren's Syndrome
Portuguese SINDROME DE SJOGREN, Doença de Sjögren, Síndrome de Sjoegren, Síndrome de Gougerot-Mulock-Houwer, Síndrome Seca, Síndrome de Sjögren
Dutch ziekte van Sjögren, syndroom van Sjögren, Sjögren-ziekte, Gougerot-Mulock-Houwer-syndroom, Sicca-syndroom [Sjgren]i, Sjögren-syndroom, Siccasyndroom, Syndroom, Sicca-
French Sjogren, Maladie de Sjogren, SYNDROME DE GOUGEROT-SJOGREN, Syndrome de Gougerot-Mulock-Houwer, Syndrome de Gougerot-Sjögren, Syndrome arthro-oculo-salivaire, Syndrome de Sjögren
German Sjogren, Sjoegren-Syndrom, Sjogren-Syndrom, Syndrom Sjogren, Sicca-Syndrom [Sjoegren-Syndrom], Gougerot-Mulock-Houwer-Syndrom, Sjoegren Syndrom, Dakryo-Sialo-Cheilopathie, Dakryosialoadenopathia atrophicans, Mikulicz-Syndrom, Sjögren-Syndrom
Italian Sindrome di Sjogren (sindrome sicca), Sindrome di Sjögren, Sindrome di Sjoegren, Di Sjogren, Sindrome di Gougerot-Mulock-Houwer, Sindrome di Sjogren
Spanish Enfermedad de Sjögren, Síndrome de Sjogren, enfermedad de Sjögren (trastorno), síndrome de Sjögren (trastorno), síndrome de Sjögren, enfermedad de Sjögren, Síndrome de Gougerot-Mulock-Houwer, Síndrome de Sjögren
Japanese シェーグレン病, シェーグレン症候群, ｼｪｰｸﾞﾚﾝｼｮｳｺｳｸﾞﾝ, ｼｪｰｸﾞﾚﾝﾋﾞｮｳ, ｸﾞｼﾞｭﾛｰﾑﾛｯｸﾌｰﾜｰｼｮｳｺｳｸﾞﾝ, グジュロー・ムロック・フーワー症候群
Swedish Sjögrens syndrom
Czech Sjoegrenův syndrom, Sjögrenův syndrom, Gougerot-Mulock-Houwerův syndrom, Sjögrenova nemoc
Finnish Sjögrenin oireyhtymä
Russian SHEGRENA SINDROM, ШЕГРЕНА СИНДРОМ
Korean 건조증후군[쉐그렌]
Croatian SJOGRENOV SINDROM
Polish Zespół Sjogrena, Zespół suchości
Hungarian Sjögren-syndroma, Sjögren-szindróma, Sjögren syndroma, Sjögren-betegség, Sjögren-féle tünetegyüttes, Gougerot-Mulock-Houwer-syndroma
Norwegian Sjøgrens syndrom
Derived from the NIH UMLS (Unified Medical Language System)

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