Design: a cross-sectional study.

Participants: One hundred and ninety-nine patients with Wegener's granulomatosis belonging to a patient self-help group.

Main outcome measures: Mode of initial presentation; diagnosis timescales and delay; rhinologic involvement; and treatment.

Results: Sixty-three per cent of patients initially presented with ENT-related symptoms. Ninety-two per cent faced a delay in their diagnosis of more than one month, a pattern which has not improved over the last few decades and which compares unfavourably with North American figures. Rhinologic and otologic symptoms are a common occurrence in up to 85 and 50 per cent of Wegener's granulomatosis patients, respectively, attracting varying degrees of targeted treatment.

Conclusions: As sinonasal and other ENT symptoms are so common at the presentation of Wegener's granulomatosis, it is clear that the otolaryngologist has an important role in its diagnosis and treatment. There are long delays in the diagnosis and possible under-treatment of the ENT symptoms of this condition, highlighting the need for greater awareness, particularly in the ENT community.

Topical cocaine is used by many otorhinolaryngologists because of its good local anaesthetic and vasoconstrictive properties. A review of the available literature suggests a risk/benefit analysis would suggest that in the out-patient setting local anaesthetic agents which do not contain cocaine should be used. For rhinosurgical procedures, preparations containing cocaine and adrenaline in the appropriate concentrations and doseage are safe in the vast majority of patients without previous cardiac disease. The relative benefit of using cocaine in relation to other topical analgesics and vasoconstrictors in surgery remains unproven and alternative preparations should be used wherever there is concern over the cardiac status of the patient.

It is important to be alert to the possibility that patients presenting with a septal perforation or destructive midfacial lesions may be suffering from an addiction to cocaine. It is important to arrive at the correct diagnosis in a destructive process and even in the presence of raised antineutrophilic cytoplasmic antibodies (ANCA) a diagnosis of cocaine abuse should actively be excluded.

Wegener's granulomatosis (WG) is a complex autoimmune syndrome that is characterised by upper/lower respiratory necrotising granulomatosis, glomerulonephritis and small-vessel vasculitis. Since Wegener's 1936 description, considerable advances in recognition and treatment have changed this disease from a rapidly and uniformly fatal illness to a chronic disease characterised by remissions and relapses. The serendipitous discovery of anti-neutrophil cytoplasmic antibodies (ANCAs) as a marker associated with WG focused attention on the potential pathogenic role of these antibodies and has recently led to the development of novel animal models that might facilitate our understanding of the disease pathogenesis. Future animal models of this disease will have to account for the role of both ANCA-mediated pathology and granulomatous inflammation to enable us to understand the chronic and persistent features of WG in humans.

Formerly a fatal condition, Wegener's granulomatosis is now treated with good results. Although the annual incidence of Wegener's granulomatosis in the general population is not known, most European and American studies revealed an incidence in the range of one for every 250,000 of the population. The estimated total population of Bahrain as of June 1997 is 586,110; here we report the first diagnosed case of Wegener's granulomatosis in Bahrain.

Our patient presented with epistaxis, and from the clinical features, the very high ESR, the operative, histopathological and the radiological findings, and a high index of suspicion we could confirm the diagnosis along with the appropriate immunological test (positive cANCA). Standard treatment with cytotoxic agents and corticosteroids has been used. On follow-up the patient has shown a progressive improvement in her clinical and laboratory parameters.

The number and distribution pattern of silver staining nucleolar organizer regions (AgNORs) were thought to reflect the cellular proliferative activity and the malignancy of tumours. Using a silver-staining method, the variations of AgNORs have been studied in patients with atypical inflammatory lesion (n = 5), malignant reticulosis (n = 5) and Wegener's granulomatosis (n = 6). Our results reveal that there was a statistically significant difference (p<0.01), highly suggestive of a difference in AgNOR counts between the atypical inflammatory lesion and Wegener's granulomatosis, with the Wegener's granulomatosis specimens having the higher irregular AgNORs, but the difference between Wegener's granulomatosis and malignant reticulosis is probably not clinically important. It is concluded that AgNORs may be useful in differentiating Wegener's granulomatosis from an atypical inflammatory lesion, and the simplified counting technique is adequate for the purpose.

Wegener's granulomatosis is a systemic vasculitis that may present with a Variety of findings and be difficult to diagnose. We report a case of a patient who presented with serous otitis media and subsequently developed a suspected primary lung tumour. Thoracotomy and pulmonary mass excision were required to establish the diagnosis. Otological manifestations of Wegener's granulomatosis, differential diagnosis, pathological findings and c-ANCA test role are discussed.

Cochlear implantation is now a routine clinical procedure for deaf patients in many countries (Gibson, 1987). It replaces the function of damaged cochlear hair cells and, therefore, detects sound and by electrical stimulation produces an appropriate signal in the remaining cochlear nerve fibres (House and Berliner, 1991). Wegener's granulomatosis is an uncommon auto-immune disease. It has a peak incidence at the fifth decade with slight male predominance. Nasal problems are the predominant presentation with otological manifestations presenting rarely. We report a case of Wegener's granulomatosis presenting with total hearing loss and after right cochlear implantation a free field threshold of 40 dB and 20 per cent Bamford-Kowal-Bench (BKB) speech test.

We conclude that Wegener's granulomatosis is not an absolute contraindication for cochlear implantation.

Formerly a fatal condition, Wegener's granulomatosis is now treated with good results. Clinical morbidity is often due to failure by clinicians to make the diagnosis. Many patients (including our cases reported here) present with atypical symptoms, and only a high index of suspicion will ensure early diagnosis. Classical chest and renal symptoms often indicate late stage disease. We present two cases that underline the limitations of current ‘diagnostic’ immunological tests whilst emphasising the importance of clinical features in diagnosis.

Standard treatment with Cytotoxic agents and corticosteroids are effective but carry considerable morbidity. We have followed the current trend of incorporating a less toxic antibiotic agent in the management of this enigmatic condition.

Salivary gland involvement is rare in Wegener's granulomatosis. We report the first case of widespread major salivary gland enlargement as part of the presentation of this disease. A review of the few reported cases in the literature suggests that salivary gland involvement may be associated with a limited form of the disease and an improved prognosis. The anti-neutrophil cytoplasmic antibody (c-ANCA) assay remains the gold standard of diagnosis but care should be exercised in the interpretation of results. This patient responded well to current immunosuppressive therapy.

Wegener's granulomatosis is a multi-system disease characterized by granuloma formation and a necrotizing vasculitis. It classically presents with involvement of the upper and lower respiratory and renal systems. However locoregional disease is common and may include otological manifestations. Facial nerve palsy has been reported during the course of the disease process but it is extremely rare for it to be the presenting feature. Previously reported cases have involved a protracted diagnostic process including exploratory tympanotomy, mastoidectomy and facial nerve decompression. We report a case of Wegener's granulomatosis which presented with a facial nerve palsy. An early diagnosis was achieved by measurement of the erythrocyte sedimentation rate (ESR), followed by serological assay of cytoplasmic anti-neutrophil cytoplasmic antibody (cANCA). Histological examination of nasal mucosal biopsies confirmed granuloma formation consistent with a diagnosis of Wegener's granulomatosis. This allowed early treatment with immunosuppressive therapy and avoided unnecessary and potentially hazardous middle ear surgery.

Wegener's granulomatosis is a systemic vasculitis characterized by necrotizing granulomata and without treatment is associated with a poor prognosis. Antineutrophil cytoplasmic antibodies (ANCA) have proved to be a useful serological marker allowing for early diagnosis and treatment of this condition. We report two cases of patients who were ANCA-negative on presentation despite clinical suspicion of Wegener's granulomatosis. In both cases the patients developed a positive ANCA titre as the disease progressed.

Wegener's granuloma is a systemic necrotizing vasculitis which predominantly affects the upper and lower respiratory tracts and kidneys. The nose and paranasal sinuses are often the first or main sites to be affected. Equally ear involvement is common and a recognized early manifestation of the disease. Salivary gland involvement is rare and is usually part of more widespread systemic involvement.

We present a 34-year-old female with a six-week history of hearing loss and a unilateral parotid swelling. One year previously she was diagnosed as having myalgic encephalomyelitis following a long period of post-viral malaise. The case points to the difficulties encountered when Wegener's granuloma presents in an unusual and varied way. A high index of suspicion coupled with early diagnosis and treatment is essential. The patient made a good recovery.

Cordyceps ophioglossoides is one of the Japanese old ’Kanpoh‘ drugs used for metrorrhagia as a decoction. We found that CO-N, a galactosaminoglycan from Cordyceps ophioglossoides, reacted with sera from patients with some collagen diseases. By using CO-N, we made a new serological activity index (CO—N reac tion). In the present study, we investigated CO-N reaction on patients with Wegener's granulomatosis (WG). The aggregation titres of CO-N (CO-N numbers) displayed a possible correlation with their clinical activity. CO-N reaction might also serve an important role in supporting the diagnosis of active WG and in helping to assess the degree of disease activity. The purpose of this report is to introduce this new serological activity index for Wegener's granulomatosis.

Fifteen Japanese patients with Wegener's granulomatosis (WG) were evaluated according to their lymphocyte subset abnormalities. Two colour immunofluorescent flow cytometry was used to distinguish the lymphocyte subset alterations. WG group showed a decrease in the percentage of CD4+ cells and the increase of CD8 + cells. Within the NK cell family, the functionally unidentified CD8 +57 + cells were markedly elevated. The disproportion of thelymphocyte subsets (CD4 +↓ CD8 +57 +↑) were similar to those of Acquired Immunodeficiency Syndrome (AIDS) and AIDS relating complexes (ARC). To assesssilent infection of Human immunodeficiency virus (HIV), the polymerase chain reaction (PCR) wasdone for all patients to selectively amplify specific HIV proviral DNA sequences in peripheral blood mononuclear cells, HIV-1 proviral DNA was not found in any patients but these changes of WG might suggest a possible adaptive response to unknown viral infection.