ANCA-Associated Vasculitis: Practical Issues in Management

Abstract

ANCA associated vasculitides are multi-system autoimmune diseases which are increasing in prevalence. In this review we will discuss the clinical manifestations and review the management options. We highlight the various trials of induction and maintenance therapy and discuss the areas of unmet need. These include understanding which patients are at highest risk of relapse, clinical adaptation of improved biomarkers of disease activity and tools to discuss long term prognosis.

Keywords: Anti neutrophil cytoplasm antibody; glucocorticoids; granulomatosis with polyangiitis; microscopic polyangiitis; pathogenesis; treatment; vasculitis.

Figure 1:

Cavitating granulomatous lung lesions in a GPA patient observed on plain X-ray (highlighted by an Asterix) and on an FDG-PET.

Figure 2:

(a) High power of a single glomerulus showing a crescentic change from a kidney biopsy of a patient with microscopic polyangiitis (H and E ×400), (b) extraglomerular vessel fibrinoid necrosis (Asterix) and interstitial hemorrhage (large arrow) in a kidney biopsy from a patient with granulomatosis with polyangiitis.

Figure 3:

Granulomatous retro-orbital pseudotumor behind the eye, restricting eye movement and causing diplopia as well as compressing the optic nerve, which untreated can lead to blindness.

Figure 4:

Pulmonary fibrosis in a patient with microscopic polyangiitis on (a) plain chest X-ray and (b) CT scan.