Interstitial lung disease in scleroderma. Analysis by bronchoalveolar lavage

Area of Science:

• Pulmonary Medicine
• Rheumatology
• Immunology

Background:

• Interstitial pulmonary fibrosis is a frequent complication of scleroderma (systemic sclerosis), significantly impacting patient morbidity and mortality.
• Clinicopathologic evidence suggests that interstitial and alveolar inflammation may precede the development of fibrosis in scleroderma lung disease.

Purpose of the Study:

• To characterize the inflammatory process in the lower respiratory tract of patients with scleroderma using bronchoalveolar lavage (BAL).
• To investigate the relationship between inflammatory markers in BAL fluid and the severity of pulmonary involvement in scleroderma.

Main Methods:

• Bronchoalveolar lavage (BAL) was performed on 19 non-smoking patients with scleroderma.
• Analysis of BAL fluid included differential cell counts (neutrophils, eosinophils) and immunoglobulin levels (IgG).
• Pulmonary function was assessed, including lung diffusing capacity for carbon monoxide, and radiographic features of interstitial fibrosis were evaluated.

Main Results:

• 58% of patients (11/19) showed increased neutrophils and/or eosinophils in BAL fluid.
• 50% of patients (5/10) had elevated IgG levels in BAL fluid.
• Neutrophil presence in BAL fluid was significantly associated with decreased lung diffusing capacity (P < 0.05) and more advanced radiographic interstitial fibrosis in patients with disease duration > 1 year.

Conclusions:

• Scleroderma-related lung disease may be characterized by an inflammatory alveolitis.
• The presence and extent of this inflammatory alveolitis appear to correlate with the severity of pulmonary involvement in scleroderma.