Treatment Of IgA Vasculitis Research Articles

Treatment of IgA Vasculitis With Nephritis in Children

Treatment of IgA Vasculitis With Nephritis in Children

IgA vasculitis - similarities and differences to IgA nephropathy

IgA vasculitis - similarities and differences to IgA nephropathy Abstract. IgA vasculitis (IgAV), formerly called Henoch-Schönlein Purpura (HSP) is an immune complex-mediated vasculitis of small vessels typically affecting the skin, gastrointestinal tract, and the kidneys. Based on distinct histopathological and pathophysiological commonalities of IgAV and IgA nephropathy (IgAN) they are viewed as part of a spectrum of IgA-mediated inflammatory syndromes. While the disease course in children is most often benign, IgAV has a high risk of renal and gastrointestinal complications when first appearing in adulthood. Acute morbidity and mortality in IgAV are determined by gastrointestinal complications such as intussusception or perforation, while chronic morbidity depends on renal involvement. Nephritis symptoms can appear, subclinically, many weeks after the initial manifestation of IgAV, and can therefore be missed. Continuous monitoring of renal parameters and blood pressure is therefore recommended even after apparent remission of the disease. As there are no data from randomized controlled trials available, the treatment of IgAV is currently based on consensus-based expert opinions.

Consensus evidence-based recommendations for treat-to-target management of immunoglobulin A vasculitis

IgA vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is the most common cause of systemic vasculitis in childhood. Given its potential life-threatening systemic complications, early and accurate diagnosis as well as management of IgAV represent a major challenge for health care professionals. This study was carried out to attain an evidence-based expert consensus on a treat-to-target management approach for IgAV using Delphi technique. The preliminary scientific committee identified a total of 16 key clinical questions according to the patient, intervention, comparison, and outcomes (PICO) approach. An evidence-based, systematic, literature review was conducted to compile evidence for the IgAV management. The core leadership team identified researchers and clinicians with expertise in IgAV management in Egypt upon which experts were gathered from different governorates and health centers across Egypt. Delphi process was implemented (two rounds) to reach a consensus. An online questionnaire was sent to expert panel (n = 26) who participated in the two rounds. After completing round 2, a total of 20 recommendation items, categorized into two sections were obtained. Agreement with the recommendations (rank 7–9) ranged from 91.7–100%. Consensus was reached (i.e. ⩾75% of respondents strongly agreed or agreed) on the wording of all the 20 clinical standards identified by the scientific committee. Algorithms for the diagnosis and management have been suggested. This was an expert, consensus recommendations for the diagnosis and treatment of IgAV and IgA vasculitic nephritis, based on best available evidence and expert opinion. The guideline presented a strategy of care with a pathway to achieve a state of remission as early as possible.Plain Language SummaryGiven its potential life-threatening systemic complications, early and accurate diagnosis of immunoglobulin A vasculitis represents a major challenge for health care professionals. This work provided cornerstone principles for the management of the condition. Adopting PICO approach and implementing Delphi process a consensus was reached on evidence-based treat-to-target treatment recommendations. This will endorse enhancement and consistency of care of this cohort of patients in standard practice.

Global Glomerulosclerosis and Segmental Glomerulosclerosis Could Serve as Effective Markers for Prognosis and Treatment of IgA Vasculitis With Nephritis.

Background: This study was aimed at investigating the clinical significance and curative effect of global glomerulosclerosis (GS) and segmental glomerulosclerosis (S) in adult-onset IgA vasculitis with nephritis (IgAV-N) patients since there was no consensus pathological grading method for adult IgAV-N.Methods: A total of 188 biopsy-proven IgAV-N patients were prospectively identified. Patients were separately assigned to GS0/GS1/GS2 group and S0/S1/S2 based on the scores of global glomerulosclerosis and segmental glomerulosclerosis (0% /0–15% />15%, respectively).Results: GS0, GS1, and GS2 occurred in 56.4, 29.2, and 14.4% of the adult-onset IgAV-N, respectively. Patients in GS2 group tended to have the most serious renal deterioration and the highest levels of blood pressure. IgAV-N patients were also divided into S0 group (64.4%), S1 group (20.7%), and S2 group (14.9%), where no obvious differences in baseline data were noted. K–M curves indicated that GS2 group had the worst renal outcome (P = 0.05) while there seemed to be no significant differences between GS0 group and GS1 group. In addition, no remarkable differences in primary outcome were found among S0 group, S1 group, and S2 group though the prognosis of S2 group tended to be the worst. However, the prognosis of S0/S1 group was markedly better than that of S2 (P = 0.04). The discrimination of poor prognosis could be improved by adding the pathological indicators of global glomerulosclerosis and segmental glomerulosclerosis. Most importantly, immunosuppressive treatment might be a superior alternative in IgAV-N patients without sclerosis scores or with lower level of sclerosis scores. But addition of immunosuppression was not recommended in patients with higher sclerosis scores.Conclusions: Global glomerulosclerosis and segmental sclerosis might be used for management and treatment of adult-onset IgAV-N.

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

BackgroundThe 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.ObjectivesTo 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.MethodsClinical 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.ResultsFrom 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.ConclusionAzathioprine may be beneficial in the treatment of IgA vasculitis with renal involvement.

Current status and prospects of the immune pathogenesis and treatment of IgA vasculitis

IgA vasculitis (IgAV) is a common systemic small-sized vessel vasculitis.It′s characterized by non-thrombocytopenic palpable purpura, arthralgia/arthritis, bowel angina, and nephritis, but some details of etiology and pathogenesis is not very clear.The disease course is usually benign and self-limited, however, it is necessary to discuss the therapy of severe or chronic cases, especially there is not enough evidence-based basis for using key drugs to IgAV.Excellent clinical trails is expected to establish scientific system of prediction and evaluation of disease course, which will be helpful to individualize treatment of IgAV. Key words: Immunoglobulin A vasculitis; Pathogenesis; Therapy; Glucocorticoid