Pulmonary manifestations of wegener granulomatosis: CT findings in 57 patients and a review of the literature

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Abstract

Wegener granulomatosis is a multisystem disease of unknown cause characterized by a necrotizing granulomatous vasculitis. In comparison to other vasculitides, the lung is the most common organ involved in wegener granulomatosis presenting with a very aggressive airways pathology and chronic relapsing course. Chest radiographs fail to describe the pattern and distribution of thoracic pathology sufficiently, and CT has shown to be more sensitive for detecting lung involvement. We present the CT findings of 57 patients with wegener granulomatosis and a review of the literature.

Introduction

Wegener granulomatosis (WG) is a multisystem disease of unknown cause characterized by a necrotizing granulomatous vasculitis affecting predominantly the upper and lower respiratory tract, lung, and kidneys [1], [2], [3], [4]. Absence of necrotizing glomerulonephritis as renal involvement is defined the “limited form”, considered as early-stage disease [5]. In comparison to other vasculitides, the lung is the most common organ involved in WG presenting with a very aggressive airways pathology and chronic relapsing course [4]. In addition to clinical and radiographic findings, the diagnosis of WG is confirmed by anti-neutrophil cytoplasmatic antibodies as serological marker and biopsy [6]. Combined treatment with cytotoxic and corticosteroid agents has improved prognosis and long-term survival in patients with WG [3], [4], [7]. Radiographic and CT findings of pulmonary pathologies in WG have been described [8], [9], [10], [11], [12], [13]. Chest radiographs fail to describe the exact pattern and distribution of thoracic pathology, and the superior sensitivity of CT for detecting lung involvement has been documented. We present the CT findings of 57 patients with WG and a review of the literature.

Section snippets

Patients

Medical records and CT scans of 57 patients with WG from 1993 to 2003 were reviewed. WG was diagnosed in accordance with the 1990 American College of Rheumatology [14] and the Chapel Hill criteria [15]. Pathohistologic verification was achieved in 50 patients. Biopsy specimens were obtained in the lung (n = 20), paranasal sinuses and nasal cavity (n = 24) and kidneys (n = 6). Anti-neutrophil cytoplasmatic antibodies were elevated in the serum at the time of the first CT scanning in 48 patients.

Results

Abnormal findings were depicted on CT scans of 52 patients (92%) (Table 1).

In 51 patients (89%), the most commonly observed CT pathologies were nodules or masses (Fig. 1, Fig. 2). Fifty-three patients (93%), each showing 1–22 nodules or masses (mean: 5), had a total number of 165 nodules or masses ranging from 4 to 65 mm (mean: 29 mm). They were bilateral in 37 patients (70%) and predominantly subpleural and diffuse. They represented isolated findings in 12 patients (23%) and observed with other

Discussion

Pathologically, pulmonary WG is characterized by a necrotizing vasculitis that affects blood vessels of all sizes-arteries, veins, arterioles, venules, and capillaries. It is postulated that the necrotic pulmonary granulomas are related to ischemia from vasculitis with thrombotic occlusion, hemorrhage, and subsequent lung infarction [3], [4], [6].

Pathohistologic confirmation can be achieved by open or thoracoscopic lung biopsy, CT-guided transthoracic biopsy, and bronchoscopic biopsy [6], [9]

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    Influence of disease manifestation and antineutrophil cytoplasmic antibody titer on the response to pulse cyclophosphamide therapy in patients with Wegener’s granulomatosis

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