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

Abstract

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 Summary Given 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.

Individualized medication based on pharmacogenomics and treatment progress in children with IgAV nephritis

“…Patients can use it for 3 days, up to 1 g daily. Subsequently, in the latest evidence-based consensus in Egypt, oral prednisolone or pulsed MP is recommended for mild, moderate, and severe nephritis (see Table 1: Comparison of guidelines for the severity of the condition) (Abu-Zaid et al, 2021). The publication of these guidelines and consensus has given the international community a clearer understanding of the treatment of IgAV in children.…”

“…The latest KDIGO guideline published in 2021 suggests that CYC should be used for IgAV nephritis with nephrotic syndrome or rapid deterioration of renal function (Rovin et al, 2021). IV CYC can be taken into account as second-line therapy for moderate IgAV nephritis and induction therapy (6-9 months) for severe IgAV nephritis (Ozen et al, 2019;Abu-Zaid et al, 2021), and second-line therapy for mild IgAV nephritis (Ozen et al, 2019). Considering the adverse reactions (ADR) of CYC, sequential maintenance therapy with other immunosuppressants after induction therapy is recommended.…”

“…AZA and MMF can be taken into account as secondline therapy for mild IgAV nephritis and the first-line or second-line therapy for moderate patients. Considering the ADR of CYC, it is suggested that MMF or AZA should be the maintenance therapy after CYC induction treatment for the severe IgAV nephritis group (Ozen et al, 2019;Abu-Zaid et al, 2021). Besides CYC, KDIGO was skeptical about immunosuppressants, so no other immunosuppressants were recommended.…”

“…Besides CYC, KDIGO was skeptical about immunosuppressants, so no other immunosuppressants were recommended. The SHARE initiative and the consensus of Egyptian experts agreed that effective immunosuppressants could prevent/minimize the risk of renal kidney disease or renal failure, especially in rapidly progressive acute vasculitis (Ozen et al, 2019;Abu-Zaid et al, 2021), so the recommended intensity of immunosuppressants was also stronger. However, for MMF or AZA, which is more suitable for priority recommendation, there is a lack of corresponding research, and high-quality evidence is needed to distinguish the advantages and disadvantages.…”

“…Although RTX should not deplete terminally differentiated, immunoglobulinsecreting plasma cells, the benefit of depleting B cells by RTX in other B cell-mediated diseases guarantees its use in refractory IgAV (Donnithorne et al, 2009). RTX has therefore been suggested as an alternative therapy for refractory or recurrent IgAV (Abu-Zaid et al, 2021) and is a potential therapeutic tool for IgAV nephritis that is attractive in refractory IgAV nephritis.…”