A 42-year-old female has palpable purpura with rash over buttocks, pain in abdomen, and arthropathy diagnosis is(AIIMS Nov 2012)
1). Henoch-Schonlein purpura is a systemic disease characterized by a petechial rash on the extremities, arthropathy, abdominal pain, and glomerulonephritis.
2). The glomerular lesion is identical to that found in IgA nephropathy, Henoch-Schonlein nephritis and IgA nephropathy may be part of a spectrum of manifestations of a single disease.
3). Nephritis is present in 80% of the patients and manifests as a nephritic urine sediment and moderate proteinuria.
4). Macroscopic hematuria and nephritic range proteinuria are uncommon.
5). Light-microscopic appearance can vary from mild mesangial proliferation and expansion to diffuse proliferation with glomerular crescents.
6). The sine qua non for diagnosis is the presence of mesangial IgA deposition on immunofluorescence microscopy.
7). IgG and C3 are also detected. Electron microscopy reveals mesangial immune deposits.
8). Immune complexes may also be present in the peripheral glomerular capillary wall and para mesangial areas.
9). Biopsy of involved skin reveals dermal IgA deposition and leukocytoclastic vasculitis.
10). IgA deposition is also seen in the areas of uninvolved skin.
11). Since there is no proven therapy for Henoch-Schönlein nephritis, treatment is symptomatic. Steroids and/or cytotoxic agents are often tried in patients with severe disease.
12). The prognosis is generally excellent.