IMMUNOGLOBULIN A VASCULITIS: DIAGNOSIS, PATHOPHYSIOLOGY, AND TREATMENT

Abstract

Immunoglobulin A vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is a non-thrombocytopenic vasculitis predominantly affecting children, with an annual incidence ranging from 3 to 26.7 per 100,000 inhabitants. Clinically, it is characterized by palpable purpuric rash, abdominal pain, renal involvement, and arthralgia. The pathophysiology involves a complex interplay of genetic, environmental, and immunological factors, with viral infections, such as Epstein-Barr virus, suggested as potential triggers. Management of IgAV is primarily symptomatic, and nephritis serves as the main prognostic factor. Although the disease is often self-limiting, severe complications may arise, necessitating long-term follow-up. This study reviews the pathophysiology, diagnosis, and therapeutic approaches for IgAV, aiming to enhance the understanding of this condition.