Lung inflammation in scleroderma: clinical, radiographic, physiologic and cytopathological features

Twenty-five patients with systemic sclerosis were studied by chest radiography, lung function, esophageal motility, gallium-67 (67Ga) lung scanning and bronchoalveolar lavage (BAL). Alveolar inflammation, as defined by an elevation of proportional BAL lymphocyte or neutrophil counts, or increased thoracic uptake of 67Ga was found in 16 patients. An NIH gallium index greater than 65 index units identified a subgroup of patients with a significantly higher proportional BAL lymphocyte count (13.7 +/- 8.5 vs 5.6 +/- 3.1, p less than 0.0005). The presence of an abnormal chest radiograph correlated with physiologic evidence of lung restriction (p less than 0.01), and an elevation of proportional BAL lymphocyte count (15.5 +/- 8.2 vs 6.6 +/- 5.1, p less than 0.01). Eight patients receiving oral penicillamine therapy had significantly lower BAL lymphocyte counts compared to untreated patients (4.7 +/- 3.6 vs 11.3 +/- 7.7, p less than 0.05). We suggest that alveolar inflammation in scleroderma is characterized by lymphocyte accumulation and increased thoracic uptake of gallium.