Rheumatology Book

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Sjogren's Syndrome

Aka: Sjogren's Syndrome, Sjogren Syndrome, Keratoconjunctivitis Sicca, Dry Eye Syndrome
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  1. 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
  2. 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
  3. Etiology
    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 exocrine gland destruction
  4. Pathophysiology: Chronic destruction of exocrine glands
    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
  5. Symptoms and Signs: 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
  6. Symptoms and Signs: 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
  7. 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 Body sensation (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 Mouth sensation 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. Vitali (2002) Ann Rheum Dis 61(6): 554-8
  8. 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
  9. 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
  10. Labs: Serology
    1. Rheumatoid Factor positive (32-90% positive)
    2. Antinuclear Antibody positive (55-97% positive)
      1. Autoantibody Anti-Ro (Anti-SSA) present (16-70%)
      2. Autoantibody Anti-La (Anti-SSB) present (7-50%)
    3. Garcia-Carrasco (2002) Medicine 81(4): 270-80
  11. Labs: Other
    1. Erythrocyte Sedimentation Rate elevated
    2. Complete Blood Count
      1. Leukopenia
      2. Anemia
    3. Liver Function Tests abnormal
  12. 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
  13. 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
  14. Complications
    1. Non-Hodgkin Lymphoma (40 fold increased risk over general population)
    2. Dental Caries
    3. Sialadenitis
    4. Corneal Ulceration
    5. Chronic oral infection
  15. References
    1. Fox (2005) Lancet 366(9482): 321-31
    2. Kassan (2004) Arch Intern Med 164(12): 1275-84
    3. Kruszka (2009) Am Fam Physician 79(6): 465-70

Dry Eye Syndromes (C0013238)

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) 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
ICD9 375.15
ICD10 H04.12
SnomedCT 155182003, 46152009, 193980001
English Syndrome, Dry Eye, Syndromes, Dry Eye, Tear film insufficiency, NOS, Tear film insufficiency, unspecified, Dry Eye Syndromes, dry eye syndrome (diagnosis), dry eye syndrome, Dry eye syndrome (disorder), Tear film insuffic NOS, DRY EYE(S), Dry eye, EYE(S), DRY, Keratoconjunctivitis Sicca, Dry Eye Syndromes [Disease/Finding], syndrome dry eye, dry eyes syndrome, dry eye syndromes, Dry eye syndrome, Tear film insufficiency, Tear film insufficiency (disorder), dry eye; syndrome, syndrome; dry eye, Dry Eye Syndrome
Dutch droog oogsyndroom, niet-gespecificeerde traanfilminsufficiëntie, droge ogen; syndroom, syndroom; droge ogen, Droogoogsyndromen, Droogoogsyndroom, Syndromen, droogoog-, Syndroom, droogoog-
French Insuffisance de film lacrymal, non précisée, Syndrome des yeux secs, Syndromes de l'oeil sec
German Traenenfilminsuffizienz, unspezifisch, Trockene Augen-Syndrom, Syndrome des trockenen Auges
Italian Sindrome dell'occhio secco, Insufficienza del film lacrimale, non specificata, Sindromi dell'occhio secco
Portuguese Síndrome de olho seco, Insuficiência do humedecimento lacrimal NE, Síndromes do Olho Seco
Spanish Insuficiencia de la película lagrimal, no especificada, Síndrome de ojo seco, Tear film insufficiency, Dry eye syndrome, insuficiencia de la película lagrimal (trastorno), insuficiencia de la película lagrimal, síndrome del ojo seco, Síndromes de Ojo Seco, Sindromes de Ojo Seco
Japanese 眼乾燥症候群, 涙液層障害、詳細不明, ｶﾞﾝｶﾝｿｳｼｮｳｺｳｸﾞﾝ, ﾙｲｴｷｿｳｼｮｳｶﾞｲｼｮｳｻｲﾌﾒｲ
Swedish Torrögdhetssyndrom
Czech syndromy suchého oka, Nespecifikované poruchy slzného filmu, Syndrom suchého oka
Finnish Kuivasilmäisyysoireyhtymät
Russian SUKHOGO GLAZA SINDROMY, GLAZA SUKHOGO SINDROMY, ГЛАЗА СУХОГО СИНДРОМЫ, СУХОГО ГЛАЗА СИНДРОМЫ
Croatian Not Translated[Dry Eye Syndromes]
Polish Zespoły suchego oka
Hungarian Száraz szem syndroma, Könnyfilm elégtelenség, nem meghatározott
Sources
Derived from the NIH UMLS (Unified Medical Language System)


Keratoconjunctivitis Sicca (C0022575)

Definition (AIR) WHAT: Keratoconjunctivitis sicca. Keratoconjunctivitis Sicca (KCS): an eye condition in which there is decreased tear production and inflammation of the cornea and conjunctiva. WHY: Keratoconjunctivitis sicca is found in a significant number of patients with rheumatoid arthritis. KCS has also been described in systemic lupus erythematosus, polyarteritis nodosa, and scleroderma. KCS plus xerostomia is called the sicca syndrome. The association of the sicca syndrome with rheumatoid arthritis is known as Sjogren's syndrome. HOW: Clinically, patients with KCS may present with itching, burning eyes. They may complain of a dry sensation or of a foreign body sensation of the eyes. The conjunctiva is hyperemic, and thick strands of mucus may cover the eye. With slit lamp examination there is less than 1 mm of tear width at the margins of the upper and lower lids. There is increased debris and mucus strands within the tear film, seen best after blinking. Tiny punctate opacities are seen throughout the corneal surface. The Schirmer tear test (which is most accurately performed without topical anesthesia) shows less than 5 mm of wetting of filter paper at the end of 5 minutes. Finally, rose bengal, which is a water soluble dye specific for devitalized cells and mucin, will stain the dessicated corneal and conjunctival cells a bright red color. REFS: 1) Henkind, P and Gold, DH: Ocular manifestations of rheumatic disorders. Rheumatology 4:13, 1973. 2) Havener, W: Synopsis of ophthamology. St. Louis, C.V. Mosby Company, 1979. 3) Anderson, JR; Whaley, K; Williams, J and Buchanan, WW: A statistical aid to the diagnosis of keratoconjunctivitis sicca. Quart J Med 41:175, 1972. DN19292-9.
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, 302896008, 156455009, 201444003, 268050000
English Sicca, Keratoconjunctivitis, Kerato conjunctivitis sicca, KERATOCONJUNCTIVITIS SICCA, keratoconjunctivitis sicca, KCS - Keratoconjunctivit sicca, Keratoconjunctivitis Sicca, KCS, Keratoconjunctivitis Sicca [Disease/Finding], kc, kcs, keratoconjunctivities sicca, kerato conjunctivitis sicca, Keratoconjunctivitis sicca, KCS - Keratoconjunctivitis sicca, Keratoconjunctivitis sicca (disorder), Keraconjunctivitis sicca
Japanese 乾性角結膜炎, ｶﾝｾｲｶｸｹﾂﾏｸｴﾝ
Swedish Keratoconjunctivitis sicca
Czech keratokonjunktivitida suchá, Keratokonjunktivitis sicca
Spanish Keratoconjunctivitis sicca, Keraconjunctivitis sicca, queratoconjuntivitis sicca (trastorno), queratoconjuntivitis sicca, Queratoconjuntivitis seca, Queratoconjuntivitis Seca
Finnish Keratoconjunctivitis sicca
Russian KERATOKON"IUNKTIVIT SUKHOI, КЕРАТОКОНЪЮНКТИВИТ СУХОЙ
Polish Zapalenie rogówki i spojówki suche
Hungarian Keratoconjunctivitis sicca
Portuguese Queratoconjuntivite seca, Ceratoconjuntivite Seca
Dutch keratoconjunctivitis sicca, Keratoconjunctivitis sicca
German Keratokonjunktivitis sicca, Keratoconjunctivitis sicca
French Kératoconjonctivite sèche
Italian Cheratocongiuntivite secca
Sources
Derived from the NIH UMLS (Unified Medical Language System)


Sjogren's Syndrome (C1527336)

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 (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 (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:

  • Dry eyes
  • Dry mouth

Treatment focuses on relieving symptoms.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases
Definition (NCI) 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, 156455009, 268050000, 201444003
English Syndrome, Sjogren's, SJOGRENS SYNDROME, GOUGEROUT-SJOEGREN SYNDROME, SJOEGREN SYNDROME, SJOGREN'S SYNDROME, SYNDROME SJOGREN'S, Sjogrens Syndrome, Sjogrens syndrome, Sicca syndrome [Sjogren], Sjogren's disease, Sjogren's syndrome (disorder), SJOGREN SYNDROME, Sjogren's syndrome (diagnosis), Sjogren Syndrome, Gougerot-Nulock-Houwer syndrome, Sjogren's, Syndrome Sjogren's, Sjogren's syndrome, Sjögren syndrome, Sjogren syndrome, SYNDROME, SJOGREN'S, Sicca syndrome [Sjögren], Sjogren's Syndrome [Disease/Finding], syndrome sjogren, sjogrens's syndrome, sjogrens syndrome, sjoegrens syndrome, sjogren's syndromes, sjogren's disease, sjogren's syndrome, syndrome sjogrens, sjogren syndrome, sjoegren's syndrome, Sjoegren's syndrome, Sjögren's disease, Sjögren's syndrome (disorder), Sjögren's syndrome, Sjogren, Sjögren, Sicca (Sjogren's) syndrome, Sjogren's Syndrome
Portuguese SINDROME DE SJOGREN, Síndrome de Gougerot-Nulock-Houwer, Doença de Sjögren, Síndrome de Sjoegren, Síndrome Seca, Síndrome de Sjögren
Dutch ziekte van Sjögren, syndroom van Sjögren, Gougerot-Nulock-Houwer-syndroom, Sjögren-ziekte, Sicca-syndroom [Sjgren]i, Sjögren-syndroom, Siccasyndroom, Syndroom, Sicca-
French Sjogren, Maladie de Sjogren, SYNDROME DE GOUGEROT-SJOGREN, Syndrome de Gougerot-Sjögren, Syndrome arthro-oculo-salivaire, Syndrome de Sjögren
German Sjogren, Sjoegren-Syndrom, Sjogren-Syndrom, Gougerot-Nulock-Houwer-Syndrom, Syndrom Sjogren, Sicca-Syndrom [Sjoegren-Syndrom], Sjoegren Syndrom, Dakryo-Sialo-Cheilopathie, Dakryosialoadenopathia atrophicans, Mikulicz-Syndrom, Sjögren-Syndrom
Italian Sindrome di Sjogren (sindrome sicca), Sindrome di Sjögren, Sindrome di Gougerot-Nulock-Houwer, Sindrome di Sjoegren, Di Sjogren, Sindrome di Sjogren
Spanish Enfermedad de Sjögren, Síndrome de Sjogren, Síndrome de Gougerot-Nulock-Houwer, enfermedad de Sjögren (trastorno), síndrome de Sjögren (trastorno), síndrome de Sjögren, enfermedad de Sjögren, Sjogren's disease, Sicca (Sjogren's) syndrome, Sindrome de Sjogren, Síndrome de Sjögren
Japanese グジュロー・ヌロック・フーワー症候群, シェーグレン病, シェーグレン症候群, ｸﾞｼﾞｭﾛｰﾇﾛｯｸﾌｰﾜｰｼｮｳｺｳｸﾞﾝ, 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
Swedish Sjögrens syndrom
Czech Sjoegrenův syndrom, Sjögrenův syndrom, Sjögrenova nemoc, Gougerot-Nulock-Houweruv syndrom
Finnish Sjögrenin oireyhtymä
Russian SHEGRENA SINDROM, ШЕГРЕНА СИНДРОМ
Korean 건조증후군[쉐그렌]
Croatian SJOGRENOV SINDROM
Polish Zespół Sjogrena, Zespół suchości
Hungarian Gougerot-Nulock-Houwer-syndroma, Sjögren-syndroma, Sjögren-szindróma, Sjögren syndroma, Sjögren-betegség, Sjögren-féle tünetegyüttes
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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