|Pulmonary disease is an important component of Systemic Scleroderma. It is estimated that 80% of patients with SSc have some evidence of pulmonary disease. This makes pulmonary disease second only to esophageal disease as the most commonly seen visceral component. Moreover, pulmonary involvement portends a poorer prognosis. Pulmonary disease is now the leading cause of death amongst patients with SSc with an estimated mortality from pulmonary disease of all causes to be 33%. While multiple pulmonary manifestations have been associated with SSc including pleural effusions, bronchiectasis, lung neoplasms, aspiration pneumonia and drug induced pneumonitis, the most common pulmonary manifestations of SSc include pulmonary hypertension and interstitial lung diseases (ILDs). The significant prevalence of ILD in SSc is reflected in the criteria of the diagnosis of SSc with the finding of predominantly basilar fibrosis being one of the three minor criteria utilized by the American College of Rheumatology for the diagnosis of SSc. This article reviews the specific manifestation of ILD in SSc.
Scleroderma Lung Disease Other Lung Complications in Systemic Sclerosis: Maria do Socorro Teixeira Moreira Almeida
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