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Systemic sclerosis, or scleroderma, is a connective tissue disease that causes fibrosis of the skin and potentially internal organs (1). The most common lung findings in those with scleroderma are interstitial lung disease and pulmonary hypertension (1). Here we describe a 58-year-old man with scleroderma, interstitial lung disease and pulmonary arterial hypertension. He presented with atypical pulmonary manifestations and an acute kidney injury caused by a new diagnosis of ANCA-associated vasculitis.
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