A case of elderly-onset Crescentic Henoch-Schönlein purpura nephritis with hypocomplementemia and positive MPO-ANCA

Abstract

Henoch-Schönlein purpura (HSP) is common in childhood and often self-limiting. There have been limited studies on elderly-onset HSP nephritis (HSPN). A 76-yr-old man was transferred to our hospital with a 1-month history of oliguria, abdominal pain, edema and palpable purpura in the legs. Three months ago, he was admitted to another hospital with jaundice, and consequently diagnosed with early common bile duct cancer. The patient underwent a Whipple's operation. Antibiotics were administrated because of leakage in the suture from the surgery. However, he showed progressive renal failure with edema and purpura in the legs. Laboratory investigations showed serum creatinine 6.4 mg/dL, 24-hr urine protein 8,141 mg/day, myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA) 1:40 and C(3) below 64.89 mg/dL. Renal biopsy showed crescentic glomerulonephritis, as well as mesangial and extracapillary Ig A deposition. We started steroid therapy and hemodialysis, but he progressed to end-stage renal failure and he has been under maintenance hemodialysis. We describe elderly onset HSPN with MPO-ANCA can be crescentic glomerulonephritis rapidly progressed to end stage renal failure.

Keywords: Anti-Neutrophil Cytoplasmic Antibody; Hypocomplementemic; Purpura, Schönlein-Henoch.

Fig. 1

Skin rash and generalized edema were showed on lower extremities. Palpable purpuric rash mixed with erythema on both edematous legs (A) and feet (B).

Fig. 2

Changes in serum creatinine concentration in the patient during the hospital course. HD, hemodialysis; op, operation; CT, computerized tomography.

Fig. 3

Microscopic findings of needle biopsy specimen. (A) Eight glomeruli, 2 of which are globally sclerotic, whereas 5 showed segmental sclerosis. (B) Glomerulus shows fibrocellular crescent with focal capsular tear on PAS staining. (C) Masson's trichrome stain shows golmerular sclerosis with fibrin deposition and interstitial fibrosis. (D) The silver staining shows some infiltrated neutrophils. The glomerular capillary basement membrane is not thickened. (E) The glomerulus demonstrates diffuse endocapillary proliferation with circumferential cellular crescent formation formation more than 50% glomeruli (Meadow classification Grade V, Jones silver stain, original magnification × 400). (F) Immunofluorescence demonstrates diffuse deposition of IgA in the mesangium and subendothelial portion of the glomerulus (anti-IgA immunofluorescence, original magnification × 400).