Sjogren syndrome

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Here is a comprehensive overview of Sjögren syndrome based on authoritative textbook sources.

Sjögren Syndrome

Definition & Overview

Sjögren syndrome (SS) is a chronic systemic autoimmune disorder primarily targeting the exocrine glands — most notably the salivary and lacrimal glands. It is characterized by a dense lymphoplasmacytic infiltration leading to glandular hypofunction, with the hallmark symptoms of dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca).

Primary SS: disease confined to the exocrine glands
Secondary SS: characteristic SS features occurring alongside another autoimmune disease (rheumatoid arthritis, SLE, scleroderma, polymyositis, primary biliary cirrhosis)

— Cummings Otolaryngology Head and Neck Surgery; Textbook of Family Medicine 9e

Epidemiology

Estimated prevalence: 1–3% of the population
Predominantly affects women (>90% of patients)
Peak incidence: 4th–5th decade of life

Pathogenesis

The etiology involves a complex interplay of genetic, immune, and environmental factors:

Genetic predisposition: HLA alleles B8 and DR3 increase susceptibility
Environmental trigger: likely a viral infection that initiates an aberrant autoimmune response
B-cell overstimulation: leads to excess immunoglobulins, autoantibody production, and formation of germinal centers within glandular tissue — autoreactive B-cell clones escape tolerance checkpoints
Elevated follicular helper T-cells also contribute to disease progression

Key autoantibodies:

Anti-Ro (SS-A) and Anti-La (SS-B) — ribonuclear proteins; used to support diagnosis
Elevated RF (>1:320) and ANA (>1:320) also seen

— Cummings Otolaryngology

Clinical Features

Oral (Xerostomia)

Difficulty chewing, swallowing, and speaking
Multiple dental caries
Food adherence to buccal mucosa
Intolerance to acidic/spicy foods
Smooth, fissured tongue with atrophic filiform papillae
Oral Candida albicans overgrowth (common)
Scant or cloudy saliva from ducts
Bilateral parotid gland enlargement in 25–66% of patients (may be episodic or chronic)

Ocular (Keratoconjunctivitis Sicca)

"Gritty" or "sandy" foreign body sensation
Bulbar conjunctival vessel dilation, perilimbal injection
Irregular corneal image
Lacrimal gland enlargement (occasionally)

Systemic

Fatigue, dry skin, vaginal dryness
Upper respiratory tract dryness
Dysphagia (from reduced saliva)
Constitutional symptoms

Diagnosis

San Diego Criteria (Fox & Saito)

Criterion	Details
IA	Objective ocular dryness: Schirmer test <8 mm/5 min + positive Rose Bengal staining
IB	Objective dry mouth: decreased parotid flow + minor salivary gland biopsy with focus score ≥1
IC	Serologic evidence: elevated RF/ANA or positive anti-SS-A/SS-B
Definite SS	All three criteria (IA + IB + IC)
Probable SS	IA + IB-1 + IC (biopsy not required)

Exclusions: HIV, sarcoidosis, pre-existing lymphoma, hepatitis B or C, primary fibromyalgia, keratitis sicca from other causes

Key Diagnostic Tests

Schirmer test: tear flow measurement (<8 mm/5 min = abnormal)
Rose Bengal staining: identifies corneal/conjunctival epithelial damage
Minor salivary gland biopsy: definitive; shows focal lymphocytic infiltration (focus score ≥1)
Sialography: demonstrates sialectasis in 85–97% of patients
Lashley cups: quantify parotid flow rate

— Textbook of Family Medicine 9e

Imaging

Salivary gland ultrasound in early SS shows enlarged glands with multiple small (~2–3 mm) hypoechoic foci throughout the parenchyma, representing focal lymphocytic accumulations:

Salivary gland ultrasound in early Sjögren syndrome showing hypoechoic foci (FOC) in the parotid gland (A) and submandibular gland (B)

Histopathology

Minor salivary gland biopsy shows dense lymphocytic infiltrates destroying acinar epithelium. In advanced cases, lymphoepithelial lesions develop — a hallmark of SS — where lymphoid cells infiltrate and distort ductal epithelium:

H&E of salivary gland in Sjögren syndrome: lymphoepithelial lesion with dense marginal zone B-cell infiltration destroying ductal epithelium (×400)

Complications

MALT lymphoma (extranodal marginal zone B-cell lymphoma): most feared complication; arises in salivary glands from autoreactive B-cell clones that escape tolerance. Risk is significantly elevated in SS patients. Histology shows CD20+, CD79a+, BCL2+, CD5−, Cyclin D1− lymphoid infiltrate with lymphoepithelial lesions.
Renal involvement: tubulointerstitial nephritis, distal RTA (covered in nephrology texts)
Neurologic involvement: peripheral and cranial neuropathies
Hepatic involvement: overlap with primary biliary cirrhosis

Differential Diagnosis

Condition	Distinguishing Features
Sarcoidosis	Non-caseating granulomas, hilar adenopathy
HIV/AIDS	HIV serology positive
Lymphoma	Clonal B-cell expansion, no autoantibodies
Hepatitis C	HCV serology, hepatic involvement
IgG4-related disease	Elevated IgG4, storiform fibrosis on biopsy

Management (Overview)

Oral dryness: salivary substitutes, sugar-free lozenges, pilocarpine (muscarinic agonist), meticulous dental hygiene, antifungals for candidiasis
Ocular dryness: artificial tears, cyclosporine eye drops, moisture chambers
Systemic/severe disease: hydroxychloroquine, corticosteroids, rituximab (for severe systemic features or lymphoma)
MALT lymphoma: radiotherapy (early stage) or rituximab-based regimens

— Cummings Otolaryngology; Textbook of Family Medicine 9e

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