The Respiratory System in Autoimmune Vascular Diseases

Area of Science:

• Pulmonology
• Rheumatology
• Internal Medicine

Background:

• Systemic vasculitis encompasses a group of rare diseases characterized by inflammation of blood vessels.
• The respiratory system is frequently involved in various systemic vasculitides, presenting diverse clinical manifestations and outcomes.

Purpose of the Study:

• To review the spectrum of respiratory system involvement in different types of systemic vasculitis.
• To highlight the distinct clinical presentations, diagnostic features, and management strategies for respiratory manifestations in vasculitis.

Main Methods:

• Literature review of systemic vasculitis with respiratory involvement.
• Analysis of clinical presentations, diagnostic criteria, and treatment outcomes.

Main Results:

• Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis) commonly affect the respiratory tract.
• Granulomatosis with polyangiitis characteristically involves the upper/lower respiratory tract (90%) and ENT (80%).
• Eosinophilic granulomatosis with polyangiitis is linked to asthma and hypereosinophilia; microscopic polyangiitis presents with diffuse alveolar hemorrhage and renal failure. Takayasu arteritis and Behçet disease also have distinct pulmonary involvements.

Conclusions:

• Systemic vasculitis is a significant cause of respiratory pathology, ranging from airway inflammation to diffuse alveolar hemorrhage and pulmonary hypertension.
• Early recognition and appropriate treatment with corticosteroids and immunosuppressants are crucial for managing respiratory complications in vasculitis.