Sjogren syndrome is a chronic inflammatory autoimmune disease that most commonly occurs in middle-aged women.

Epidemiology

Etiology

Primary Sjogren syndrome: idiopathic (association with HLA-DR52)
Secondary Sjogren syndrome: associated with another autoimmune disease, e.g., rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, polymyositis, or primary biliary cirrhosis

Systemic autoimmune disease characterized by lymphocytic infiltration of exocrine glands, particularly salivary and lacrimal glands.
Pathogenesis involves both humoral (autoantibodies) and cell-mediated (CD4+ T cells) destruction of glandular tissue.

Predominantly affects middle-aged women (9:1 female-to-male ratio).
Sicca Symptoms (Dryness)
- Xerophthalmia (dry eyes): Pt c/o gritty sensation, corneal ulcers, “sand in my eyes.”
- Xerostomia (dry mouth): Pt c/o dysphagia for dry foods, dental caries, “lipstick sign” (lipstick adheres to teeth).
Glandular Enlargement: Firm, non-tender enlargement of parotid or submandibular glands is common.
Extraglandular Manifestations:
- Systemic: Fatigue, low-grade fever.
- MSK: Arthralgias, non-erosive arthritis.
- Skin: Raynaud phenomenon, cutaneous vasculitis (palpable purpura).
- Pulmonary: Interstitial lung disease.
- Renal: Interstitial nephritis (Type 1 RTA).
- Neuro: Peripheral neuropathy.

Serology:
- Anti-Ro (SSA) and Anti-La (SSB) antibodies: Highly specific, especially anti-La.
  - Sjogren syndrome → Anti-SSA and anti-SSB
- Antinuclear Antibody (ANA): Positive in >95% of cases, making it a sensitive but non-specific test.
- Rheumatoid Factor (RF): Often positive even without concomitant RA.
Ophthalmologic Tests:
- Schirmer test: Measures tear production by placing filter paper in the lower eyelid; <5 mm of wetting in 5 minutes is abnormal.
- Rose bengal stain: Stains devitalized cornea and conjunctiva, revealing damage from dryness.
Biopsy: Lip (minor salivary gland) biopsy is the most accurate test, showing focal lymphocytic infiltration.

Development of associated conditions
- Autoimmune diseases, e.g., systemic lupus erythematosus, rheumatoid arthritis
- B-cell lymphomas, e.g., MALT lymphoma
  - Prevalence ∼ 5%
  - Frequently manifests as unilateral, persistent parotid enlargement
  - Predictors of lymphoma include lymphadenopathy, palpable purpura, and cryoglobulinemia.
- Renal tubular acidosis type 1
Corneal scarring, ulcer, rupture, and infection
Pregnancy: fetal loss, infant with neonatal lupus syndrome and associated complete heart block