143 Wegener Granulomatosis or ANCA-Associated Granulomatous Vasculitis

Clinical Presentation

72-year-old woman with cough, sinusitis, and renal insufficiency

Radiologic Findings

PA chest radiograph (Fig. 143.1) demonstrates a right upper lobe mass-like consolidation with associated volume loss. Unenhanced chest CT (lung window) (Figs. 143.2, 143.3, 143.4) shows a peripheral wedge-shaped right upper lobe consolidation and multi-focal peripheral subpleural and angiocentric pulmonary masses and nodules.

Diagnosis

Wegener Granulomatosis or ANCA-Associated Granulomatous Vasculitis

Differential Diagnosis

• Multicentric Primary or Secondary Neoplasia

• Multifocal Pneumonia

• Bland or Septic Emboli, Pulmonary Infarcts

• Other Pulmonary Vasculitis

• Cryptogenic Organizing Pneumonia

Fig. 143.1

Fig. 143.2

Fig. 143.3

Fig. 143.4

Discussion

Background

The pulmonary vasculitides include several disorders characterized by inflammation of the pulmonary blood vessel walls. The term ANCA-associated granulomatous vasculitis has been proposed to replace the eponymous term Wegener granulomatosis, the most common pulmonary vasculitis. The diagnosis of pulmonary vasculitis requires careful correlation of clinical and imaging findings with the underlying histologic features and the exclusion of infectious granulomatous diseases, many of which often exhibit histologic features of vasculitis.

Etiology

The etiology of pulmonary vasculitis is unknown.

Clinical Findings

ANCA-associated granulomatous vasculitis (Wegener granulomatosis) is a systemic necrotizing vasculitis that commonly affects the lung, with an annual incidence of one case per 100,000 population. Although many organs are affected, classical Wegener granulomatosis

Only gold members can continue reading. Log In or Register to continue