Autoimmune Disease

Sjögren's Syndrome

SS — A chronic, systemic autoimmune exocrinopathy

Overview

What is Rheumatoid Arthritis?

Sjögren's Syndrome is a chronic, slowly progressive autoimmune disease that primarily targets the exocrine glands — most notably the lacrimal and salivary glands — resulting in the hallmark symptoms of dry eyes (xerophthalmia / keratoconjunctivitis sicca) and dry mouth (xerostomia). It can occur as a primary condition (pSS) or secondary to another autoimmune disease such as RA, SLE, or systemic sclerosis (sSS). Beyond the sicca complex, Sjögren's causes widespread systemic inflammation affecting the lungs, kidneys, nervous system, skin, and joints. It is associated with autoantibodies anti-Ro/SSA and anti-La/SSB, which are key diagnostic and prognostic markers.

Clinical Presentation

Signs & Symptoms

The sicca complex dominates the presentation, but systemic features affect up to 70% of patients and may precede or overshadow glandular symptoms.

Currently Licensed States:

Systemic Involvement

Organs Affected

Ocular

KCS, corneal ulceration, blepharitis, uveitis

Pulmonary

Pleuritis, pneumonitis, pulmonary hypertension

Ocular

Episcleritis, scleritis, keratoconjunctivitis sicca

Neurological

Peripheral neuropathy, cranial neuropathy, CNS vasculitis

Diagnosis

ACR/EULAR 2010 Criteria

Score ≥6/10 confirms RA. Applies to patients with at least 1 swollen joint not explained by another disease.

Laboratory & Imaging

Key Investigations

Pathophysiology

Disease Mechanism

1


Genetic susceptibility

HLA-DR2/DR3, complement deficiencies (C1q, C4), IRF5, STAT4 gene polymorphisms increase risk.

2


Triggering factors

UV light, infections (EBV), hormonal changes, and drugs (procainamide, hydralazine) can precipitate disease.

3


Autoantibody formation

B cells produce ANA, anti-dsDNA, and other autoantibodies. T helper cells and type I interferons amplify the response.

4


Immune complex deposition

Autoantibody-antigen complexes deposit in tissues — kidneys, skin, vessels — activating complement and causing inflammation.

5


End-organ damage

Persistent inflammation leads to fibrosis and functional impairment of target organs if disease remains uncontrolled.

Ready to Take the First Step?

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