JMIR Publications

ABSTRACT

Background:

Henoch-Schönlein purpura is a leukocytoclastic small vessel vasculitis with both cutaneous and systemic manifestations. Although Henoch-Schönlein purpura is most common in childhood it may manifest in adults with more extensive systemic manifestations. Additionally, the disease is present worldwide with differences in epidemiologic and systemic characteristics.

Objective:

The main objective of this study was to examine the features of extracutaneous manifestations in adults with Henoch-Schönlein purpura as distinguished by geographic region worldwide.

Methods:

A search of Pubmed, Embase, Cochrane Library, and Web of Science was performed for articles published January 1, 1970 through December 1, 2019. Keywords included “Henoch-Schönlein purpura" OR "henoch schonlein purpura + adult”, “IgA vasculitis + adult”, “HSP + adult”, and “IgAV”. A total of 995 publications were found. 42 studies, comprised of 3388 patients were included with the vast majority of reported cases being from continents Asia, Europe, and the Americas.

Results:

Of adults with Henoch-Schönlein purpura, European patients were more likely to be male (P<0.001), have gastrointestinal involvement (P<0.001), and have musculoskeletal involvement (P<0.001). Patients from America were the least likely to have genitourinary involvement (P<0.001).

Conclusions:

The distribution and extracutaneous manifestations of Henoch- Schönlein purpura vary between geographical boundaries. Among adult patients with Henoch-Schönlein purpura, Europeans affected were more likely to be males, and were more likely to experience gastrointestinal and musculoskeletal involvement. Asian patients were more likely to experience genitourinary involvement than those in America. Prospective studies utilizing standardized reporting measures are needed to confirm the relationships identified in this study.