Eighteen non-smoking women suffering from primary Sjögren's syndrome (pSS) with previously documented alveolitis were re-examined, clinically and by pulmonary function tests (PFT), bronchoalveolar lavage (BAL), chest X-ray and high-resolution computed tomography (HRCT) after a 2 yr follow-up period. Longitudinal evaluation revealed unchanged PFT. The final BAL study showed a normal differential count in six of 14 patients with initial lymphocyte alveolitis, and a persistent alveolar lymphocytosis in the remaining eight patients, associated with an increased percentage of neutrophils in one of them. In four patients with initial mixed alveolitis, the BAL cell profile was unchanged 2 yr later. Five of 18 patients (28%) had abnormal HRCT, represented by isolated septal/subpleural lines in three patients, ground-glass opacities with irregular pleural margins in one patient, and ground-glass opacities associated with septal/subpleural lines in another. All these patients had abnormal BAL results with an increased proportion of both neutrophils and lymphocytes. The presence of alveolar neutrophils was associated with a significantly (P=0.005) greater mean rate of reduction of carbon monoxide diffusing capacity (DLCO) -- more than four times the normal rate of loss of DLCO. Chest X-ray, repeated at the end of the 2 yr follow-up period, showed parenchymal abnormalities in only one patient who had evidence of fibrosis on HRCT. This study provides evidence that lung involvement is not an uncommon extraglandular manifestation of pSS and that a BAL neutrophilia may play an important role in the pathogenesis of pulmonary disease in this autoimmune disorder.
Pulmonary involvement is one of the most frequent extra-articular manifestations of rheumatoid arthritis (RA) and represents a serious complication, being the second cause of death after infection. High-resolution computed tomography (HRCT), owing to its increased sensitivity and diagnostic accuracy respect to the conventional chest radiograph (CXR), allows to detect pulmonary abnormalities in RA patients more frequently than CXR. The aim of this study was to assess pulmonary involvement by HRCT in lifelong non-smoking RA patients without symptoms and clinical signs of pulmonary disease. Seventy-two patients (54 women and 18 men) with a mean age of 56.8±10.4 years (range, 40- 77 years) and mean duration of disease of 6.9±4.7 years (range, 2-12 years) entered the study. 52/72 (72%) were positive for rheumatoid factor (> 20 UI/ml). Standard CXR and HRCT were carried out in each patient. CXR showed a mild interstitial fibrosis in 7 patients (9.7%), whereas HRCT demonstrated pulmonary abnormalities in an higher number of them (22/72 = 30.5%). The most frequent abnormal findings on HRCT were irregular pleural margins (13.8%) and septal/subpleural lines (18%), both compatible with pulmonary fibrosis. Ground-glass opacities were found in 8.3% of the patients. Pulmonary nodules (diameter, range 0,5-2 cm) predominantly located in the subpleural portions of the lung, were demonstrated in the same percentage (8.3%) of patients. Small airway involvement, represented by bronchiectasis/bronchioloectasis, was shown in 15.2% of patients. Subpleural cysts were present in two cases (2.8%). No patient had evidence of honeycombing on HRCT. In conclusion, HRCT is an accurate, non-invasive and safe method of diagnosing lung abnormalities in RA patients without signs and clinical symptoms of pulmonary disease..
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