Wegener Granulomatosis presented with epistaxis, hemoptysis and polyarthralgia: A case report

Abstract

Wegener granulomatosis (WG) is a rare, multisystem, autoimmune disease with necrotizing granulomatous inflammation, tissue necrosis, vasculitis in small and medium-sized blood-vessels. The classic clinical pattern is a triad involving upper airways, lungs and kidneys. A 33-year-old woman was admitted to hospital because of dry cough, shortness of breath, polyarthralgia, intermittent fever, epistaxis, hemoptysis. Chest X-ray presented multiple, small infiltrates bilaterally. Laboratory results: Hgb 90g/L, hematocrit 30%, erythrocytes 3,6×1012/L, leucocytes 13,8×109/L, platelet 4,45×109/L, CRP 110mg/L, sedimentation 70mm/h. Urine sediment: erythrocytes(16-18), proteins(+), epithelial cells(++), 24hour-proteinuria 0,5g/L. ECG: sinus tachycardia 120 beats/min. Gas analyses: partial respiratory failure, hypoxemia 7,5kPa, hypocapnia 3,6kPa, saturation 91%. Lung-CT revealed multiple bilateral infiltrates. Chest-ultrasound with bilateral, subpleural, hypoechogenic changes with central necrosis. Because of intermittent fever, polyarthralgia, chest X-ray changes, elevated sedimentation, rheumatologist was consulted and tests for autoimmune disease were performed (positive c-ANCA 95U/ml, RF 158IU/ml, ASO 88U/ml). Ophthalmologist revealed punctiform conjunctival bleeding. Bronchoscopy: intranasal coagulum without changes of nasal mucosa, transoral intubation presented diffuse erythema and edema of the vulnerable tracheobronchial mucosa without ulcerous lesions or infiltrative changes. Bronchial alveolar lavage detected small increase of neutrophils (total cell counts 320/ L, neutrophils 19,2%, macrophages 85,0%, lymphocytes 7,4%, eosinophils 0,0%), no growth of bacterial culture, negative Gene X-pert. Transbronchial biopsy was performed, histologic analysis detected necrotic granulomas with multinucleated giant cells accompanied by inflammatory cells. According to all investigations, the diagnosis was WG. The patient was successfully treated multidisciplinary with high-dose steroids and cyclophosphamide. Recognition of multisystem disease involving joints, kidney, eye and lung is critical for diagnosis.