WEGENER GRANULOMATOSIS

Objective: To highlight the different clinical features, parameters of diagnosis and to see the effectof chemotherapy and steroids on this disease. Setting: Department of Otolaryngology Head & Neck Surgery JinnahHospital Lahore. Period: From Dec 1998 to July 2001.Methods: Five cases of Wegener Granulomatosis were studiedduring the study period. Three patients had limited disease while two patients had generalized disease with renalinvolvement. All patients were started on azathiopurine (2mg/Kg/day) or cyclophoshamide (1-2mg/km/day) along withsteroids. After a months time the dose of steroid was reduced to alternate day. Weekly monitoring of white blood cellcount with urine examination for RBCs or protein casts was done. Any patient with haematuria or TLC reduced to lessthan 3500 were immediately taken off from cyclophoshamide and azathiopurine was started. Patients having classicdisease , dose of cyclophoshamide had to be increased from two to four milligram / per Kilogram per day to achievea good response. The response to treatment was measured by noticing the resolution of pulmonary infiltrates reducedESR and negative C-ANCA. Results: We found nasal symptoms like nasal obstruction and epistaxis to be the mostcommon symptom followed by fever, cough and deafness. Crusting, granulations and occasional perforation wereusually found on nasal examination. Conductive deafness wasmost common ear finding. History, clinical examination,ESR, C-ANCA and a representative biopsy were all essential in diagnosing this condition. Azathiopurine andcyclophosphamidewere the main chemotherapeutic agents used with prednisone to cause remission in all our patientsexcept one who died two weeks after having been admitted in our institution. Surprisingly in contrast to the normalcause of high mortality reported in international literature in this disease to be renal failure, our patient died due torespiratory failure. Four out of five patients went into remission a year after regular treatment with this regime. Themajor complications occurring during hospital stay and their treatment were nausea, vomiting, sudden fall of TLC, andoral thrush. Conclusion: The common presentation of patient with Wegner’s Granulomatosis is of upper respiratorytract symptoms like nasal obstruction and epistaxis followed by lower respiratory symptoms like cough, fever andHaemoptysis. A representative biopsy with positive C-ANCA and high ESR are hallmarks of diagnosing this condition.The most effective medical regime till today is cyclophosphamide and azathiopurine with alternate day prednisone forinducing remission.