Refractory Wegener's granulomatosis: effect of rituximab on granulomatous bilateral orbital involvement.

case cOmmunicatiOns suppressive treatment, progressing to bone destruction and development of bilateral orbital masses. Rituximab treatment induced significant clinical and radiological improvement. Patient descriPtiOn A 50 year old man was initially admitted to another hospital in October 2002 because of right-sided headache and dyspnea of 1 month duration and development of right-side deafness and facial nerve palsy 2 days before admission. The patient had a prior history of chronic obstructive pulmonary disease, diabetes mellitus, and morbid obesity treated only with diet and broncho-dilator inhalations. Emergency room evaluation had included sinus X-rays, which revealed right maxillary sinus opacification. Laboratory investigations were significant for normocytic nor-mochromic anemia (hemoglobin 11.38 g/L), leukocytosis (15,000/ml), throm-bocytosis (600,000/ml), erythrocyte sedimentation rate 100 mm/hour and C-reactive protein 215 mg/L (normal 0–6). On nasopharyngeal examination, a right inferior nasal conchial mass with bloody crusts and evidence of external and otitis media were found. Computed tomography scan of the sinuses demonstrated bilateral maxillary, ethmoid, sphenoid and frontal sinus and right mastoid air cell opacification. Chest X-ray and CT showed small intersti-tial infiltrates and nodular shadows bilaterally, which were consistent with Wegener's granulomatosis. w egener's granulomatosis is a multi-organ systemic disease characterized by granulomatous inflammation, tissue necrosis and variable degrees of vasculitis in small and medium-sized blood vessels. Individual cases present with variable histopathology, with vasculitis and granulomatous disease representing opposite ends of the spectrum. Two patterns of disease involvement were recently described. The classic type is generally characterized by severe widespread disease including renal involvement, requires aggressive treatment and is associated with a high relapse rate. The second type is a limited disease usually associated with a good prognosis, does not require aggressive treatment and is characterized by a high complete remission rate. Differences in outcome between those WG patients with a predominance of granulomatous versus vasculitic lesions were recently reported [1]. While WG may affect the eyes in a variety of ways, orbital masses with granulomatous expression are often the WG lesions most refractory to treatment [2]. We describe a case of limited WG with severe paranasal and nasal involvement resistant to significant immuno-WG = Wegener's granulomatosis Biopsy of the nasal mass showed necrotizing granulomatous vasculitis with Langhans giant cells consistent with WG. Further blood tests revealed positive cytoplasmic anti-neutrophil cytoplasmic antibodies by direct immu-nofluorescence. There was no evidence of renal involvement. The patient responded rapidly to daily oral cyclophosphamide (2 mg/kg/day) and prednisone (80 mg) with trimetho-prim/sulfamethoxazole …