Pulmonary renal syndrome in an adult patient with Henoch-Shönlein purpura

Abstract

Henoch-Schönlein purpura (HSP) is a small vessel vasculitis characterized by purpuric skin rash, haematuria, abdominal pain, gastrointestinal bleeding and arthritis. Nephritis is more frequent and severe in adults than in children, with relatively more adults developing renal insufficiency. Another, fortunately rare, manifestation of HSP that increases mortality significantly, is diffuse alveolar haemorrhage. We report a rare case of an adult male patient with full-blown HSP that followed a respiratory tract infection. He successively, but not concurrently, developed all the clinical manifestations of HSP, i.e. arthritis, abdominal pain and bloody stools, a non-thrombocytopenic purpuric rash, and renal involvement; nephrotic range proteinuria first and haemodialysis-requiring nephritic syndrome later. Most interesting he developed life-threatening pulmonary haemorrhage fulfilling the criteria of the pulmonary-renal syndrome. An immunosuppressive regimen consisting of intravenous cyclophosphamide and corticosteroids was administered with success. In conclusion, HSP should be considered in the diagnosis of pulmonary-renal syndrome. In our opinion, the severity of the condition justifies the use of aggressive immunosuppressive treatment, like the one applied successfully to our patient.