A 16 years old female, with a history of chronic rhinitis, had presented, for the past 5 months, a dry cough with two episodes of low-abundance hemoptysis, a progressive worsening dyspnea and epistaxis. She had fever, fatigue and weight loss without other extra pulmonary manifestations (Figure 1). On examination, her pulse was 124 beats/min, blood pressure was 120/60mmHg, the temperature was 37 C and the oxygen saturation was 91% while she was breathing ambient air. The urine dipstick found hematuria (+) without proteinuria. Pulmonary examination revealed bilateral sibilant rales without crackling. Other systems examination revealed no significant abnormalities.
Chest X-ray showed bilateral excavated opacities in the upper lungs (Figure 2). The chest computed tomography (CT) found multiple bilateral excavated nodules with the reduction of the right main bronchus caliber and mediastinal lymphadenopathy (Figure 3). The CT-scan of the sinus showed filled maxillary and sphenoid sinuses with wall thickening, a polypoid mass of the right middle meatus and nasal septal erosion (Figure 4). The blood tests showed microcytic hypochromic anemia 11.1 g/dl, elevated CRP 109 mg/l, erythrocyte sedimentation rate (ESR) 68mm, normal urine proteinuria excretion 7mg/24 hours, urea 0,32g/l,serum creatinine 7.5mg/l and glomerular filtration rate GFR at 133 ml/min/1.73m2. Cyto-bacteriological examination of urine was sterile with microscopic hematuria 17x104 red blood cell (RBC)/ml. The tests for viral hepatitis B, C and HIV were negative. The electrocardiography (ECG) and cardiac ultrasound were normal. The bronchoscopy revealed an infiltration of the entire bronchial tree which was covered by whitish granulations, with narrowing of the orifice of the intermediate trunk, as well as the bronchi of the upper right lobe (Figure 5).
The research of acid-fast bacilli (AFB) and other bacteria like actinomyces and fungus in bronchial aspiration culture were negative. The biopsies of the bronchus and the granulations showed tuberculoid granuloma inflammation without caseous necrosis. The rhinocavoscopy showed a significant inflammation of the turbinates with the presence of whitish granulations and synechiae. The biopsy of the middle turbinate and the cavum revealed a granulomatous inflammation without caseous necrosis and a dense peri-vascular infiltrate, destroying the vessel wall, composed of neutrophils with fibrinoid deposits. The anti-proteinase 3 anti-neutrophil cytoplasmic antibodies (ANCA-Pr3) was positive. The anti-myeloperoxidase anti-neutrophil cytoplasmic antibodies (ANCA-MPO) was negative. Granulomatosis with polyangiitis also called Wegener s granulomatosis was diagnosed on the basis of pulmonary, nasal and renal involvement, the positivity of anti-neutrophil cytoplasmic antibody directed against proteinase 3 (ANCA-Pr3 or C-ANCA), the bronchial and nasal biopsies that confirm the granulomatosis and after eliminating infectious diseases such as tuberculosis.
Regarding the diffuse bilateral pulmonary involvement with significant inflammation and narrowing of the bronchial orifices, as well as the Ear-Nose-Throat (ENT) involvement, and the hematuria considered as incipient renal lesion, the bolus of methyl prednisolone were started (15mg/kg/day) for 3 successive days followed by corticosteroid therapy prednisone (1mg/kg/day). Adjuvant treatment including calcium and vitamin D was prescribed. Due to the risk of infertility after exposure to cyclophosphamide (CYC) and the high price of rituximab (RTX), we opted for azathioprine bolus. The symptoms reversed after the second bolus of corticosteroids by decreasing of dyspnea and regaining appetite. Currently, the patient is still in remission phase.
CT scan with multiple bilateral excavated nodules, reduction in the right main bronchus caliber with mediastinal lymphadenopathy.