[ANCA-Associated Vasculitides]

Area of Science:

• Rheumatology
• Immunology
• Internal Medicine

Background:

• ANCA-associated vasculitides (AAV) are rare autoimmune diseases classified by immunoserological markers (anti-neutrophil cytoplasmic antibodies - ANCA).
• The main types include microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA), distinguished by clinical, histological, and imaging findings.
• GPA and EGPA are characterized by granulomatous inflammation in the airways, differentiating them from MPA.

Purpose of the Study:

• To highlight the importance of recognizing typical symptoms of AAV.
• To emphasize the critical need for timely diagnosis and immunosuppressive treatment.
• To discuss recent advancements in AAV therapeutic approaches.

Main Methods:

• Classification based on Chapel Hill nomenclature (2012).
• Diagnosis relies on clinical, histological, and imaging findings.
• Review of current and emerging treatment strategies.

Main Results:

• AAV are rare diseases with geographical prevalence variations.
• Untreated AAV have a poor prognosis regarding organ function and survival.
• Rituximab has become a key treatment, largely replacing cyclophosphamide.
• Current therapeutic goals focus on minimizing steroid exposure due to significant side effects.

Conclusions:

• Timely diagnosis and appropriate immunosuppressive therapy are crucial for managing AAV.
• Novel treatments like Rituximab offer improved efficacy and potentially fewer side effects.
• Reducing steroid dependency is a primary objective in AAV treatment to mitigate long-term morbidity.