Differential Diagnosis of Wegener’s Granulomatosis from Other Small Vessel Vasculitides

Abstract

Wegener’s granulomatosis (WG) is an uncommon autoimmune disease characterized by small vessel inflammation, most often involving the respiratory or renal systems. Although the etiology is currently unknown, evidence suggests that environmental factors and a genetic predisposition interact to create an inappropriate autoimmune response. Wegener’s granulomatosis is characterized by the presence of anti-neutrophil cytoplasmic antibodies (ANCA), which have long been suspected of involvement in disease pathology. This suspicion is supported by ongoing research, although much remains to be done in identifying risk factors for developing WG and elucidating all factors contributing to the disease pathophysiology. This manuscript will review basic information about WG, ANCA production, and their role in WG pathology, and discuss differential diagnosis based on common presentation symptoms and clinical laboratory testing results.