Sjogren's Syndrome Overview Epidemiology: • F > M: 9:1 • 5-6th Decades (can be any age) Autoimmune exocrinopathy multisystemic disease affecting salivary and lacrimal glands: • Focal lymphocytic sialadenitis • Keratoconjunctivitis sicca • Xerostomia • Symmetric parotid swelling • Lacrimal swelling • Skin dryness • Vaginal dryness • Primary form: not associated with other diseases • Secondary form: overlaps with RA, SLE • Mikulicz syndrome: Prominent parotid and lacrimal glandular enlargement Sjogren's Syndrome Diagnosis: • Dry eyes • Salivary hypofunction • Antibodies: Anti-Ro/SSA & Anti-La/SSB ab • Lip (minor salivary gland) biopsy • Ocular examination Labs: • Antibodies: • Anti-Ro/SSA ab (specific in primary) • Anti-La/SSB ab • RF • Antinuclear antibodies ≥ 1:320 • Cytopenia: 2% • Hypocomplementemia • Can cause TYPE IV RTA, TYPE I RTA Diagnosis: • LIP (minor salivary gland) biopsy • Histo: aggregate foci (>50 cells/aggregate) of CD4 T cells, B cells, and plasma cells around salivary ducts Clinical Features: HEENT: • Keratoconjunctivitis sicca • Xerostomia • Dental caries • Parotid swelling CNS Involvement: • Central nervous system (CNS): demyelinating disease, myelopathy, cranial nerve neuropathy • Peripheral nervous system: small-fiber neuropathy, mononeuritis multiplex, peripheral neuropathy, transverse myelitis • 8%-27% for CNS and peripheral Thyroid Disease: • 10%-15% Pulmonary • Interstitial Pneumonitis (LIP, UIP, NSIP): • Lymphocytic interstitial pneumonia • Usual interstitial pneumonia • Tracheobronchial disease • Xerotrachea • NSIP: Pleural thickening or effusion, INC risk PE, Pulmonary amyloidosis, PAH Skin: • Dry skin (xerosis) • Cutaneous vasculitis: 10%-16% Liver: • Autoimmune hepatitis, primary biliary cirrhosis: 3%-20% Renal: • Interstitial nephritis • Distal (Type I) renal tubular acidosis • NAGMA • Glomerulonephritis: 5%-6% • Membranoproliferative GN • Nephrogenic DI Hematologic: • Lymphoma, Cytopenia: 2% Systemic Vasculitis: • 7%, Cryoglobulinemia (4%-12%), Raynaud phenomenon (16%) MSK: • Arthralgia and Arthritis Malignancy Risk: • Non-Hodgkins lymphoma • B cell lymphoma • Large B cell lymphoma • MALT Lymphoma • Lung Adenocarcinoma Treatment: • Symptomatic: artificial tears/artificial saliva/mouth lubricants • Pharmacologic: • Severe oral dryness: muscarinic agonists • Active extraglandular autoimmune disease: Glucocorticoids • Immunomodulatory/Biologic: 2nd or 3rd line #Sjogrens #Syndrome #Rheumatology #Diagnosis #Management
