Guidelines for the diagnosis and management of childhood immunoglobulin A vasculitis

Immunoglobulin A vasculitis (IgAV) is the most common cause of systemic vasculitis in childhood. Due to the continued use of the disease name “anaphylactoid purpura” in China, several misunderstandings have arisen in clinical practice and treatment regimens differ widely. In addition, new research and evidence-based data have grown. The Subspecialty Group of Immunology, Society of Pediatrics, Chinese Medical Association and the Chinese Alliance of Pediatric Rheumatic and Immunologic Diseases initiated an update of guidelines for the diagnosis and management of childhood IgAV. The aim therefore was to provide agreed consensus recommendations for diagnosis and treatment for children with IgAV.

This study utilized the Delphi technique to develop an evidence-based expert consensus for childhood IgAV. We conducted a systematic literature review to retrieve evidence, which was graded using GRADE (Grading of Recommendations Assessment, Development, and Evaluation) criteria. Two rounds of Delphi voting and a consensus meeting involving 23 experts were conducted. Recommendations were accepted when ≥ 75% of experts agreed.

In total, five recommendations for diagnosis, six for treatment, one for prognosis, and two for health education for pediatric IgAV were accepted. Diagnostic recommendations included the use of the European Alliance of Associations for Rheumatology/Pediatric Rheumatology International Trials Organization/Pediatric Rheumatology European Society-endorsed Ankara 2008 classification criteria for IgAV diagnosis. In addition, appropriate use of imaging, gastrointestinal endoscopy, skin biopsy, and kidney biopsy are recommended. Kidney biopsy is recommended for children with IgAV presenting with nephrotic syndrome/nephrotic-range proteinuria, reduced estimated glomerular filtration rate, acute nephritis syndrome, or persistent moderated/mild proteinuria. Treatment recommendations involved indications for glucocorticoid use, immunosuppressant use, and intravenous immunoglobulin use. It also addressed the inappropriate use of prophylactic glucocorticoid treatment and recommended against routine employment of plasmapheresis. Health education placed emphasis on the inappropriate use of anti-anaphylactic treatment and proffers dietary suggestions.

This guideline provides evidence-based recommendations for the diagnosis and management of IgAV in children. This will facilitate improved and standardized care.

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