Abstract
IgA vasculitis or Henoch-Schönlein purpura (HSP) is an acute immunoglobulin A (IgA)–mediated disorder characterized by a generalized vasculitis involving the small vessels of the skin, the gastrointestinal (GI) tract, the kidneys, the joints, and rarely, the lungs and the central nervous system (CNS). It is the most frequent vasculitis in childhood. The disease is much less common in adults, who often have a more severe course of the disease. Renal involvement in HSP affects the prognosis of the disease in adult patients. The aim of our study was to examine the clinical characteristics of this renal involvement and to identify factors influencing renal prognosis. Retrospective study including all patients with histologically confirmed HSP (EULAR classification criteria) with renal involvement treated in our department from 1990 to 2019. We analyzed renal survival and identified the factors influencing renal prognosis Twenty-eight patients were included (sex ratio M/F = 2.5), their average age at diagnosis of HSP was 38.14 years. In 22 patients HSP and the kidney damage were discovered at the same time. The other patients were followed for HSP and kidney damage occurred after an average follow-up time of 20.16 months. An Infectious disease was identified as a contributing factor in 6 patients. Smoking was noted in 10 cases. Fourteen patients had a normal renal function. Proteinuria was positive in 24 cases and 9 patients met the criteria for nephrotic syndrome. Acute nephritic syndrome was present in 4 patients. Histological analysis of the skin biopsy was performed in 15 cases and showed leukocytoclastic vasculitis in 8 patients with Ig A deposits in 4 patients. The most common histological lesion was segmental and focal glomerulonephritis found in 11 cases. The most important renal prognostic factors identified were gastrointestinal involvement including vomiting (p=0.022) and initial acute renal failure (p=0.0004). Our study shows that renal involvement during Ig A vasculitis can be relatively severe with poor renal prognosis. The identification of prognostic factors may be useful to guide prospective therapeutic studies.
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