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



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