Sjögren's syndrome in progressive systemic sclerosis

Area of Science:

• Rheumatology
• Immunology
• Pathology

Background:

• Progressive systemic sclerosis (PSS) is an autoimmune disease.
• Sjögren's syndrome is an autoimmune disorder affecting exocrine glands.
• The co-occurrence and diagnostic criteria for Sjögren's syndrome in PSS patients require further investigation.

Purpose of the Study:

• To evaluate the prevalence of Sjögren's syndrome in patients with progressive systemic sclerosis.
• To investigate the clinical significance of minor salivary gland fibrosis in PSS patients without confirmed Sjögren's syndrome.

Main Methods:

• Prospective evaluation of 35 consecutive patients with progressive systemic sclerosis.
• Biopsy analysis of minor salivary glands.
• Clinical assessment for Sjögren's syndrome diagnostic criteria.

Main Results:

• 17% of PSS patients met the criteria for Sjögren's syndrome.
• 48% of PSS patients exhibited significant salivary gland fibrosis without sufficient inflammation for Sjögren's diagnosis.
• This subgroup of PSS patients with fibrosis showed aggressive disease and high mortality.

Conclusions:

• The prevalence of Sjögren's syndrome in PSS is higher than previously reported in some studies.
• Minor salivary gland fibrosis in PSS, even without overt Sjögren's syndrome, is associated with a poor prognosis and aggressive disease.
• These findings highlight the importance of salivary gland evaluation in PSS management.