MANIFESTATIONS OF SCLERODERMA PULMONARY DISEASE
Section snippets
INTERSTITIAL LUNG DISEASE
Patients with scleroderma develop interstitial lung disease more commonly than patients with any other connective tissue disease.131 Approximately 74% of affected patients are found to have interstitial fibrosis at autopsy.35, 162 Physiologically and histopathologically, scleroderma pulmonary fibrosis resembles idiopathic pulmonary fibrosis.62
Incidence
Depending on the method used to detect PAH and the population under study, the incidence of PAH in patients with scleroderma varies from 6% to 60%.11, 16, 146, 152 Although only 9% of patients with limited cutaneous scleroderma have clinically detectable severe PAH,146 pathology studies show that 65% of these patients have evidence of PAH.166 Ungerer et al152 prospectively studied 49 consecutive patients with scleroderma (39 with diffuse disease and 10 with limited disease). They found definite
LUNG CANCER
Zatucini et al in 1953 were the first to suggest that patients with scleroderma had an increased incidence of lung cancer.167 In 1980, Talbott and Barrocas reviewed the world literature and identified 41 cases of coexisting scleroderma and lung cancer, predominantly bronchoalveolar cell carcinomas.148 In a study of 2141 patients, Duncan and Winkelman41 reported an overall cancer rate of 4% and no increase in the incidence of lung cancer compared with the general population. However, other
OTHER MANIFESTATIONS
Asymptomatic bronchiolitis is a common finding in patients with scleroderma.19 Recently, two cases of bronchiolitis obliterans organizing pneumonia, associated with limited scleroderma, have been reported.20
Diffuse alveolar hemorrhage is a rarely reported consequence of scleroderma,56, 80 and immune complexes are thought to play an important etiologic role in its pathogenesis. Capillaritis and focal fibrinoid necrosis of alveolar walls are seen on microscopy.96
Groen et al in 199357 were the
CONCLUSIONS
Pulmonary involvement in scleroderma is not an unusual occurrence and is a significant cause of morbidity and mortality. Pulmonary fibrosis and PAH constitute the most common manifestations. A multifactorial process involving alveolitis, stepped-up lung fibroblast proliferation, and capillary endothelial damage is responsible. Symptoms and signs, though often present, are nonspecific. Pulmonary function testing often reveals a restrictive ventilatory defect with a decreased DLCO. Aims of
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Address reprint requests to Alejandro C. Arroliga, MD, Department of Pulmonary and Critical Care Medicine, G-62, The Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195