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.

Keywords: Egypt; Egyptian guidelines IgA vasculitis; Henoch-Schönlein purpura; IgA vasculitis; guidelines; vasculitis algorithm.

Figure 1.

Flow chart for the study selection process.

Figure 2.

Monitoring of immunoglobulin A vasculitis. AM, morning; BP, blood pressure.

Figure 3.

Systemic treatment plan for immunoglobulin A vasculitis. ACE-I, angiotensin-converting enzyme (ACE) inhibitors; ARBs, angiotensin receptor blockers; Aza, azathioprine; Cyc, cyclophosphamide; MMF, mycophenolate mofetil; NSAID, non-steroidal anti-inflammatory drugs.