Progressive systemic sclerosis, immunosuppression and necrotising arteritis: cause or effect?

Necrotising arteritis is rare in progressive systemic sclerosis (PSS). We report a patient with PSS who died with renal failure due to necrotising arteritis and segmental crescentic glomerulonephritis more typical of polyarteritis nodosa. Possible pathogenetic mechanisms are discussed. Renal biopsy is an important investigation in PSS, but is especially important in patients without hypertension, as other pathology needs to be excluded.