Sjögren's syndrome in progressive systemic sclerosis.

Area of Science:

• Rheumatology and Immunology
• Autoimmune Diseases
• Ophthalmology and Otolaryngology

Background:

• Progressive systemic sclerosis (PSS) is an autoimmune disease affecting connective tissues.
• Coexistent autoimmune conditions, like Sjögren's syndrome (SS), can complicate PSS management.
• Early diagnosis of coexistent SS is crucial for comprehensive patient care.

Purpose of the Study:

• To determine the prevalence of Sjögren's syndrome (SS) in patients with progressive systemic sclerosis (PSS).
• To characterize the clinical and serological features of SS in the context of PSS.
• To investigate the diagnostic utility of labial salivary gland biopsy and specific symptoms.

Main Methods:

• Prospective evaluation of 44 unselected PSS patients.
• Diagnosis of SS based on labial salivary gland (LSG) biopsy (focal lymphocytic infiltration ≥2+), keratoconjunctivitis sicca (KCS), and/or xerostomia.
• Assessment of LSG biopsy scores, parotid flow rates, and anti-Ro (SSA) antibody presence.

Main Results:

• A 20.5% prevalence of SS was found in PSS patients (9 out of 10 with LSG score ≥2+).
• Parotid gland enlargement was more common in PSS patients with SS (44.4%).
• Anti-Ro (SSA) antibodies were detected in 33.3% of SS patients.

Conclusions:

• Sjögren's syndrome is a common comorbidity in progressive systemic sclerosis.
• SS in scleroderma patients may present with subtle exocrine gland symptoms but shares clinical and serological similarities with primary SS.
• LSG biopsy is a valuable tool for diagnosing coexistent SS in PSS.