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Infectious agents, genetic background, and environmental exposure as observed in this case report, are thought to play a part in Wegener's granulomatosis (WG).1
A 54 year old man consulted in 1999 for an eight year history of bilateral crusty and bloody rhinosinusitis. He had worked from 1965 to 1980 as a ship's carpenter. During this period he had constantly had nose pain, sore mucosal discomfort, frequent nasal bloody discharge, and chronic nasal obstruction when planing woods (iroko, oak, and ash), removing paint, and undertaking activities with dust exposure. He did not wear a mask. He had been a heavy smoker until the age of 40 and had never used cocaine. No nasal deformity was noted. Nasal fibroscopy disclosed erythematous and inflamed mucosa of the nasal septum and inferior turbinate in the left nasal cavity. There was biological inflammation with normal kidney function. A search for antinuclear and antiphospholipid antibodies was negative and serum angiotensin converting enzyme was normal. A high titre of antineutrophil cytoplasmic antibodies (ANCA) with cytoplasmic pattern on immunofluorescence and positivity of an enzyme linked immunosorbent assay (ELISA) for antiproteinase 3 antibodies (153 IU/ml, normal <2) was noted. Blood lymphocyte immunophenotyping was normal. A thoracoabdominal computed tomographic (CT) scan was normal and a sinus CT scan showed evidence of thickening of the mucous membranes of the maxillar and frontal sinuses, associated with a bilateral ethmoiditis. Endonasal biopsy showed important granulomatous inflammation in the mucosa and around the vessel walls with necrosis compatible with WG.
The patient received oral steroids (40 mg/day) and trimethoprim-sulfamethoxazole in association with four intravenous cyclophosphamide pulses (0.6 g/m2 every four weeks). One year later, the nasosinusal symptoms had almost completely regressed. The oral steroid dose was 18 mg/day in association with trimethoprim-sulfamethoxazole. Control of the nasal fibroscopy and sinus CT scan were normal. A search for ANCA was negative.
In our patient, WG was diagnosed with chronic bilateral crusty and bloody rhinosinusitis, strong positivity of cANCA, and histopathological inflammatory infiltrate with necrosis. Moreover, the patient had been a ship carpenter with occupational exposure to irritating dusts and a long history of severe and bloody rhinitis at work.
Carpenters usually have an increased risk of chronic sinusitis, extrinsic allergic alveolitis, asthma, and sinus cancers.2,3 Neoplasms may occur up to 30 years after the end of exposure. Our patient had presented chronic rhinosinusitis when he was a ship's carpenter and 10 years later he presented recurrence of upper respiratory tract symptoms which led to the diagnosis of WG. A study of environmental exposure clearly showed that patients with WG had a significantly high exposure to fumes or particulate materials (chimney cleaning, carpet cleaning, plastering, and pesticides).4 Moreover, cases of WG in woodworkers were reported in the 1970s.5,6 The case described by Fombeur et al concerned a 23 year old man who had been a woodworker since the age of 18.5 WG affected the sinuses, lungs, and kidney.5 Grimaud et al reported another case of sinonasal WG in a 34 year old carpenter.6 He presented necrotic pseudotumours in both sinuses associated with nasal septum perforation and ulceration of the soft palate.6 The fatal outcome was due to a pneumorenal syndrome. In their experience with 12 cases of WG, Israel and Patchefsky observed one case in a black woodworker.7
Neutrophils and cANCA seem to have an active role in the pathogenesis of WG.8 In our case, inflammatory response to irritative dusts might have induced a chronic rhinosinusitis and later favoured a cANCA related neutrophilic activation with a granulomatous reaction. Our observation reminds us that environmental exposure may constitute an underestimated triggering factor for WG. However, as woodworkers are rare nowadays, recent publications do not mention environmental exposure, which should nevertheless be taken into account.