[Pulmonary functions in patients with progressive systemic sclerosis]

Area of Science:

• Pulmonary Medicine
• Rheumatology
• Immunology

Background:

• Progressive systemic sclerosis (PSS) is a multisystem autoimmune disease.
• Pulmonary involvement is a major cause of morbidity and mortality in PSS.
• Understanding the factors influencing pulmonary function is crucial for patient management.

Purpose of the Study:

• To investigate the correlations between pulmonary function parameters and clinical symptoms or autoantibody profiles in PSS patients.
• To identify predictors of pulmonary dysfunction in PSS.
• To assess the impact of comorbidities on lung function in PSS.

Main Methods:

• Retrospective analysis of pulmonary function tests (PFC) including percent vital capacity (% VC) and percent diffusing capacity of carbon oxide (% DLCO).
• Correlation analysis with clinical data, including esophageal hypomotility and Sjögren's syndrome (SS).
• Assessment of autoantibody profiles, specifically anti-Scl-70 and anti-RNP antibodies.

Main Results:

• 85% of PSS patients exhibited abnormal pulmonary function.
• % DLCO was decreased in 76% and % VC in 40% of patients.
• Lower % DLCO in males; lower PO2 in patients with esophageal hypomotility.
• PSS with SS showed significantly decreased % VC and PO2.
• Pulmonary fibrosis occurred in 63% of patients, more frequent with anti-Scl-70 antibody.
• Annual VC decrease was 62 ml, more prominent in severe fibrosis or with anti-Scl-70.
• Milder VC decrease observed in patients with anti-RNP antibody.

Conclusions:

• Pulmonary dysfunction is highly prevalent in PSS.
• Esophageal hypomotility, Sjögren's syndrome, and anti-Scl-70 antibody are associated with worse pulmonary function.
• Anti-RNP antibody may be associated with a milder decline in VC.
• Pulmonary function monitoring and risk stratification are essential in PSS management.