Wegener’s granulomatosis, a diagnosis that must not be forgotten in emergency

A 32 year old patient, with eutocic birth two weeks before, goes to emergency department because of intermittent dyspnoea for two weeks, which, in the last 24 hours is at rest. It is accompanied by dry cough and persistent fever of 38ºC for 4 days. She refers to tachycardia, orthopnea, oropharyngeal discomfort and general discomfort. She has been diagnosed with rhinosinusual polyposis. Her basal oxygen saturation: 93-94%. Emergency analysis showed leukocytosis (22,370) (Neutrophils 90.4%), PCR 326 mg/l, troponin 654.7, ProBNP 7367. Thoracic TC shows multiple rounded bilateral pulmonary nodules with well-defined edges, the largest being 3 cm. It also associates peribronchial thickening.

On admission, myocarditis data were found in cardiac MRI with severe ventricular dysfunction (LVEF 36%). A diagnosis of granulomatosis with polyangiitis with multisystemic affectation is obtained by nasal polyposis biopsy where granulomas with small vessel vasculitis are observed. It presents systemic involvement with an active sediment with proteinuria, microhematuria and leukocyturia, pulmonary nodules in pulmonary CT, positive rheumatoid factor, fever and joint pain. Granulomatosis with poliangeitis (Wegener's granulomatosis) is a systemic necrotizing vasculitis characterized by upper respiratory disease, thoracic lesions and nephropathy. The lungs are involved in 90% of patients typically after the involvement of the upper respiratory tract, such as sinusitis or otitis. Although final diagnosis requires a biopsy, it is possible to approximate the diagnosis based on the clinical course and the imaging findings. The thoracic CT is particularly important, the most commonly observed finding are the masses or nodules and have usually subpleural location. The consolidation or opacity in ground glass and the thickening of the bronchial wall are other common manifestations.

Wegener's granulomatosis is a systemic disease consisting of necrotizing granulomatous vasculitis of respiratory tracts (90-95%), focal necrotizing glomerulonephritis, and generalized small vessels vasculitis which could affect any organ. It shows various CT manifestations, the most common abnormalities are masses or nodules. Although there are no specific radiological findings, it is important to know the different appearences in order to, among with clinical orientation, make us suspect the final diagnosis.

- Martinez F, Chung JH, Digumarthy SR, Kanne JP, Abbott GF, Shepard JO, et al. Common and Uncommon Manifestations of Wegener Granulomatosis at Chest CT: Radiologic-Pathologic Correlation. RadioGraphics, 2011, Vol. 32, No. 1. - Amanuma M, Taketomi-Takah