Immunoglobulin A vasculitis: The clinical features and pathophysiology

Area of Science:

• Rheumatology and Nephrology
• Immunology
• Pathophysiology

Background:

• Immunoglobulin A vasculitis (IgAV) is a systemic vasculitis characterized by palpable purpura, gastrointestinal symptoms, arthritis, and renal disease.
• Renal involvement in IgAV spans from mild proteinuria to severe nephritic or nephrotic syndromes, necessitating vigilant monitoring of kidney function.
• The precise pathogenesis of IgAV is not fully elucidated, but involves complex interactions between immune components and external triggers.

Purpose of the Study:

• To review the key clinical features of IgAV, emphasizing the spectrum of renal involvement.
• To explore the current understanding of IgAV pathogenesis, including the roles of aberrant IgA, anti-endothelial cell antibodies, and neutrophils.
• To discuss the influence of infection triggers, such as Coronavirus disease 2019, on IgAV development.

Main Methods:

• Literature review of clinical presentations and pathogenetic mechanisms of IgAV.
• Analysis of factors contributing to IgAV, including immune dysregulation and infectious triggers.
• Synthesis of current research on IgAV, incorporating recent findings related to viral infections.

Main Results:

• IgAV diagnosis relies on recognizing characteristic clinical manifestations, particularly the involvement of the kidneys.
• Key pathogenetic mechanisms involve aberrant glycosylation of IgA, anti-endothelial cell antibodies, and neutrophil activation.
• Infections, including COVID-19, are increasingly recognized as significant triggers for IgAV.

Conclusions:

• Early recognition of IgAV's clinical features is vital for timely diagnosis and management to prevent renal sequelae.
• A comprehensive understanding of IgAV pathogenesis is crucial for developing targeted therapies.
• This review consolidates current knowledge on IgAV, serving as a resource for clinicians and researchers.