A 72-year-old male patient with known diffuse cutaneous systemic sclerosis (SSc) presented with severe haemoptysis and blood and protein in the urine. In light of his known interstitial lung disease, he had been repeatedly treated for recurrent community-acquired pneumonia. Immunological testing demonstrated a strongly positive perinuclear antineutrophil cytoplasm antibody with a high titre antimyeloperoxidase antibody. The patient was diagnosed with pulmonary-renal syndrome as a consequence of antineutrophil cytoplasm antibody-associated vasculitis. He started immediate plasmapheresis in combination with methylprednisolone, followed by cyclophosphamide and rituximab, with good clinical outcome.