Vasculitis with mesangial IgA deposits complicating relapsing polychondritis.
The authors report the case of a patient presenting with cutaneous, renal and neurologic vasculitis in the course of relapsing polychondritis (RPC). A 78-year-old man presented with a palpable purpura of the lower limbs, high fever arthralgias, delirium, and nephrotic syndrome. He had a history of relapsing polychondritis treated by corticosteroids. Renal biopsy showed diffuse endo- and extracapillary proliferative glomerulonephritis with mesangial IgA deposits. A spectacular regression of the symptoms was observed in response to pulse intravenous methylprednisolone. Relapsing polychondritis is complicated by vasculitis in 25% of the cases. This vasculitis is characterized by cutaneous, neurologic and renal manifestations, usually occurring in elderly patients. Renal involvement is characterized by segmental and focal or diffuse necrotizing glomerulonephritis. The mesangial IgA deposits observed in our patient are rarely present in the course of RPC. Renal manifestations identify severe forms of RPC, justifying systematic screening for renal complications.
Barzegar
C
C
Internal Medicine Department no. 4, Louis Mourier Hospital, Colombes, France.
Vrtovsnik
F
F
Devars
J F
JF
Mignon
F
F
Pradalier
A
A
D000368:Aged; D001706:Biopsy; D005920:Glomerular Mesangium / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology; D008297:Male; D011081:Polychondritis, Relapsing / Q000150:complications* / Q000276:immunology* / Q000473:pathology; D014657:Vasculitis / Q000150:complications* / Q000276:immunology* / Q000473:pathology
D007070:Immunoglobulin A