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level: Level 1 of Ch9: Sjogren Syndrome

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level questions: Level 1 of Ch9: Sjogren Syndrome

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What is Sjogren syndrome?chronic autoimmune inflammatory disorder, lymphocytic infiltrate in exocrine organ, diminished lacrimal and salivary gland function, result in dryness of eyes and mouth. Clinical features are exocrine glandular features and extraglandular features. Usually primary or secondary to other autoimmune diseases like RA and SLE.
How is epidemiology of Sjogren?women 50-60 years old, men, kids. Increase with age, third most common autoimmune disease, Sicca symptoms are much more common than SS with a higher prevalence in older adults
How are clinical features of Sjogren?Exocrine features (Sicca [dry eyes and mouth], parotid gland enlargement) Extraglandular features (arthralgia, arthritis, raynaud, myalgia, pulmonary disease, GI disease, leukopenia, anemia, lymphadenopathy, neuropathy, vasculitis, renal tubular acidosis, lymphoma)
How is the american-european classification system of Sjogren?3 out of 4 (ocular symptoms [dry eyes for more than 3months], Oral symptoms [dry mouth, swollen salivary glands], Ocular signs [Schirmer test without anesthesia <5mm in 5 min], Oral signs [abnormal salivary scintigraphy, abnormal parotid sialography findings, abnormal sialometry findings <1.5 ml saliva in 15 min]) And 1 out of 2 (positive minor salivary gland biopsy finding, and positive anti-SSA/SSB result)
What are symptoms of Sjogren?Ocular symptoms (dry eyes for 3 months, sensation of sand in eye, tear substitute 3 times aday) Oral symptoms (dry mouth 3 months, wake at night to drink, drink liquids to aid in swallowing, swollen salivary glands)
How is physical exam of Sjogren?Presence of salivary gland enlargement, salivary hypofunction [caries at cervical and incisal portion of teeth, hyperlobulated tongue, absence of salivary pooling under the tongue]
What is the ACR/EULAR classification of primary Sjogren?Score 4 positive Labial salivary gland with focal lymphocytic sialadenitis and focus score of ≥1 foci/4 mm2 3 pt Anti-Ro/SSA positive 3 pt Ocular staining score ≥5 (or van Bijsterveld score ≥4) in at least one eye 1 pt Schirmer test ≤5 mm/5 minutes in at least one eye 1 pt Unstimulated whole saliva flow rate ≤0.1 mL/minute 1 pt
What are exclusion criteria of Sjogren?Past head and neck irradiation, HepC, AIDS, Prior lymphoma, sacroidosis, GVHD, anticholinergic drugs, IgG4
What are the complications of Sjogren?• Secondary Sjögren syndrome, such as SLE and RA • Infection of the parotid gland, staphylococcal, streptococcal, or pneumococcal • unilateral worsening of symptoms, along with tenderness, warmth, and erythema • Emergence of parotid tumors • watch for unusually hard or unilateral parotid enlargement • Pregnant patients with antiRo/SS-A antidodies are at risk for fetal loss, complete heart block in the fetus ,and neonatal lupus syndrome in the newborn • Emergence of pseudolymphomas and non-Hodgkin B-cell lymphomas
What are diagnostic tests for Sjogren?Labs (leukopenia, thrombocytopenia, anemia, hyperglobulienmia, proteinuria, hematuria) Ophthalmo (Schirmer test/ ocular surface staining) Salivary hypofunction (gland imaging [US/MRI], sialometry) Evidence of systemic autoimmunity (serology [SSA, SSB, ANA], systemic rheumatic disease, exclude SS mimics) Labial salivary gland biopsy (confirms dx, rule out other etiologies, stage of SS, prognosis)
How is pathophysiology of Sjogren?Glandular dysfunction in Sjögren's syndrome (SS) is generally presumed to result from autoimmune-induced inflammation and resultant damage and destruction of the tissue responsible for tear and saliva production Glandular inflammation by overexpression of IFN apoptosis by SSA Ag to salivary and epithelial cells, lymphocytic infiltrates in salivary and lacrimal glands, focal aggregates of lymphocytes involve the lobule, migration of lymphocytes in response to chemokines, and adhesion molecules