Disease Directory Primary Sjogren Syndrome
Immune

Primary Sjogren Syndrome

Also known as: primary Sjogren's, sicca syndrome, autoimmune exocrinopathy

Prevalence

60-100 in 100,000; predominantly female (9:1 ratio)

Onset

Middle adulthood; predominantly women aged 40-60

Type

Systemic autoimmune exocrinopathy

About Primary Sjogren Syndrome

Primary Sjogren Syndrome is a chronic systemic autoimmune disease driven by lymphocytic infiltration of exocrine glands, particularly salivary and lacrimal glands, causing the hallmark features of dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia), with frequent extraglandular manifestations affecting joints, lung, kidney, peripheral nervous system, and skin. Serological hallmarks include anti-Ro/SSA and anti-La/SSB autoantibodies, elevated polyclonal immunoglobulins, and cryoglobulins, with a markedly increased risk of non-Hodgkin B-cell lymphoma compared to the general population. Targeted biologics including belimumab, ianalumab, and CD40 pathway inhibitors are under active investigation in clinical trials.

Common Clinical Features

Persistent dry eyes with foreign body sensation Xerostomia with difficulty chewing and swallowing Parotid gland enlargement Inflammatory arthralgia and non-erosive arthritis Fatigue (often severe and disproportionate) Peripheral and autonomic neuropathy Renal tubular acidosis from tubulointerstitial nephritis

Clinical Trial Eligibility Tips

What to know before applying to Primary Sjogren Syndrome trials.

Fulfillment of 2016 ACR/EULAR classification criteria (score ≥4) with positive lip biopsy or anti-Ro/SSA serology is typically required; gather biopsy pathology, salivary flow measurements, and Schirmer test results

ESSDAI score (disease activity) and ESSPRI score (patient-reported symptoms) are the standard outcome measures; baseline scores are often used as eligibility thresholds — ask your rheumatologist to calculate these at your next visit

Lymphoma history is a common exclusion criterion; if you have had cryoglobulinemic vasculitis or lymphoproliferative complications, disclose these at screening as they may affect eligibility differently across trials

Patient Resources

Patient Organization

Sjogren's Foundation

Visit website ↗

Natural History Registry

Sjogren's Research Exchange

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:289390)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

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Find recruiting Primary Sjogren Syndrome trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Primary Sjogren Syndrome. Updated daily.

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