Male, 41 years old, smoker.
Two months history of dorsal pain radiating to the left shoulder, intensity 8/10 without response to analgesics, cough with mucous expectoration, weight loss and night sweats.
Physical initial normal.
She presented mild anemia, ESR: 96 mm/h, urinalysis, renal function, liver tests and normal lipid profile.
Computed tomography of the dorsal spine showed no significant findings.
Chest radiography: subpleural nodules in pulmonary vertices and left upper lobe.
Blood cultures were negative.
A normal abdominal echo-tomography, thoracic aTAC segmental aortic atheromatosis in the descending aorta, with extra-intimal thickening, significant ostium stenosis in the celiac trunk.
Pulmonary nodules and bilateral apical condensations.
VDRL: Nonreactive, HIV negative, PPD: 10 mm. Quatiferon (-).
The patient had bilateral scleritis and fever up to 37.8 °C. Large vessel vasculitis was suspected.
Nine studies demonstrated cytoplasmic ANCA(C): positive, anti proteinase 3 (PR3) +6.5 (VN < 1.2), C3 and C4 normal, ANA, anti-DNA antibodies and ENA
Lung biopsy: Nodule with fibrous center, necrosis and histiocytic reaction, with giant cells.
Peripheral organizing pneumonia, signs of old bleeding, consistent with PG, is recognized.
She was treated with corticosteroids and oral cyclophosphamide for one year and later with azathioprine.
The patient showed good clinical response, normalization of inflammatory parameters, chest X-ray and CT scan.
Anti PR3 (-) at 6 months.
