Henoch-Scho[Combining Diaeresis]nlein Purpura in Adults

Abstract

Abstract. Henoch-Schönlein Purpura nephritis (HSPN) has been extensively studied in children but, its natural history in adults is much less known. A cohort of 250 adults suffering HSP was retrospectively analyzed for a median follow-up period of 14.8 yr. All patients had biopsies consistent with HSP (predominant IgA mesangial deposits) associated with purpura, bowel angina, and/or abdominal pain. At presentation, palpable purpura was present in 96% of patients, and arthritis was reported in 61%, and gastrointestinal involvement in 48%. Thirty-two percent of the patients showed renal insufficiency (Creatinine clearance [CrCl] Ͻ50 ml/min), usually associated with proteinuria (99%) and/or hematuria (93%). Endocapillary glomerulonephritis was the most frequent lesion on renal biopsy (61%). At the end of follow-up, patient survival was only 74%. The first cause of death was carcinoma (most of them of respiratory or digestive tract). Regarding renal function, 11% of patients reached end-stage renal failure, 13% exhibited severe renal failure (CrCl Ͻ30 ml/min), and 14% moderate renal insufficiency (CrCl Ͻ50 ml/min). Clinical remission defined as the absence of proteinuria, hematuria, and a normal renal function was achieved in only 20%. This is a retrospective study; therefore, it is not possible to demonstrate any steroid and/or cyclophosphamide efficacy in diminishing the incidence of renal insufficiency. Multivariate analysis demonstrated that renal function impairment and proteinuria level at presentation and, on renal biopsy, the degree of interstitial fibrosis, percentage of sclerotic glomeruli, and presence of glomeruli with fibrinoid necrosis were associated with a poor renal prognosis. The data indicate that clinical presentation of HSPN in adults is severe and its outcome relatively poor, worse than in children. Identification of clinical and histologic prognostic factors may permit the design of appropriate therapeutic prospective studies.Henoch-Schönlein purpura (HSP) is a leukocytoclastic vasculitis involving small vessels with the deposition of immune complexes containing IgA. It is characterized by the association of skin, joint, and gastrointestinal manifestations that may occur in successive episodes (1). In addition to these manifestations, renal involvement is common, and the long-term prognosis depends on its severity.HSP primarily affects children, and its incidence is approximately 15 cases/100,000 children per yr (2); it is less common in adults. Although HSP has been extensively studied in children, much less is known about its natural history in adults. Apart from a recent multicenter Italian study (3,4), data on this disease in adults are confined to small series with relatively short follow-up (5-15). In adults, however, the incidence of HSP and the severity of its clinical manifestations appear not to be the same as in children.The incidence of renal involvement in adults varies from 45 to 85% of cases, depending on the data for patients and the definition of renal involvement (16). Among c...

A Case of IgA-related Enteropathy Complicated with Gastrointestinal Bleeding and Progressive IgA Nephropathy: A Possible Variant Henoch-Schönlein Purpura?

“…Pillebout et al described in a retrospective review that the clinical findings of adult HSP include a high prevalence of palpable purpura (96%) and gastrointestinal symptoms (48%) (6). On the other hand, there was a case report of HSP in a 6-year-old girl with severe gastrointestinal lesions without purpura, similar to our case (7).…”

“…It has been reported that adult patients with HSP are at increased risk of progression from significant renal involvement to end-stage renal failure. Eleven % of adult patients reached end-stage renal failure, and 13% showed severe renal failure (CCr <30mL/ min) at a median follow-up of 14.8 years (6).…”

“…The gastrointestinal lesions occur in about half of the adult patients. Serious bleeding, requiring transfusion or surgery or leading to death, was shown in 11% of the patients, in a retrospective review of 250 French adult patients (6). The small intestine is predominantly the most involved part of the gastrointestinal tract, and the second portion of the duodenum, stomach, terminal ileum and colon are also frequently affected (9)(10)(11)(12).…”

Henoch-Schönlein purpura in adults: a case series from a multidisciplinary study group

“…Conversely, end stage renal disease is reported in up to 60% of adult HSP patients (3,5) . Nevertheless, most series studied selected patients whose disease exhibited renal involvement severe enough to warrant renal biopsy (4,6) . Others studies have also suggested that cutaneous ulcers are more frequent in adult patients (6) .…”

“…However, HSP is much rare in adults and the severity of its clinical manifestations seem to be different to the presentation in children (3)(4)(5)(6) . We retrospectively analyzed 11 adults who suffered from HSP.…”