The profile and clinical outcomes of patients with renal involvement due to IgA vasculitis: is azathioprine a good option for treatment?

Abstract

Background: The Henoch-Schönlein Purpura (HSP) orIgA vasculitis is the most common vasculitis of childhood and may occur with renal involvement, with hematuria and / or proteinuria, and may cause severe and non-reversible sequelae.Objectives: To establish the profile of patients with renal involvement due to IgA vasculitisand to describe our experience with the use of azathioprine to treat patients with nephritis. Methods: Clinical data were retrospectively collected from medical records of patients with IgA vasculitiswho attended the pediatric rheumatology unit between 1995 and 2017. Patients were separated into two groups based on whether or notthey weretreated with non-glucocorticoid immunosuppressants. Results: From the178 patients with IgA vasculitis,nephritis was found in67 patients (37.6%), 13 of whom receivedtreatment with non-glucocorticoid immunosuppressants. Ten patients responded well to azathioprine and 1 patient to cyclosporine. Forty patients received oral glucocorticoids, whilst 16received intravenous glucocorticoids. Conclusion: Azathioprine may be beneficial in the treatment of IgA vasculitis with renal involvement.

Leflunomide therapy for IgA vasculitis with nephritis in children

“…Cys A may also cause severe adverse events [19]. Azathioprine, a prodrug of 6-mercaptopurine, has antileukemic, anti-inflammatory, and immunosuppressive properties, and is also often used in the treatment of IgA vasculitis with nephritis [9,13]. Previous studies reported administration of tacrolimus and rituximab for treatment of HSPN [20,21], but there is limited evidence of their efficacy and further research is required for confirmation.…”

“…Currently, clinicians often treat these patients with a combination of corticosteroids and immunosuppressants [9], including cyclophosphamide (CTX) [10], mycophenolate mofetil (MMF) [11], cyclosporine A (CysA) [12], and azathioprine [13]. In our clinical experience, we found that many patients and families do not accept CTX therapy, mainly due to its gonadal toxicity.…”

Adjunctive treatment with infliximab in pediatric immunoglobulin A (IgA) vasculitis: A case report

“…3 Internationally agreed consensus recommendations have recently been provided. 4 In most cases, the disease course is benign and self-limited, 5 and glucocorticoids are required for selected patients. 4 The use of immunosuppressive agents is limited to severe IgAV nephritis cases.…”

IgA Vasculitis (Henoch-Schönlein Purpura) in Argentina

“…Despite of the limitations of a retrospective study, we believe that our research contributes to the understanding of IgA vasculitis by demonstrating the more aggressive clinical phenotype of the disease in adults for the first time in the Latin American population. [2][3][4] and these cohorts have thus far focused only on pediatric populations. These aforementioned studies present data similar to that reported by Brom et al, as well as our group, demonstrating a less severe renal outcome among pediatric cases of IgA vasculitis.…”