Nausheen Qureshi.
Wegener Granulomatosis; diagnosis & management.
Professional Med J Jan ;15(1):13-9.

Objective: To highlight the different clinical features, parameters of diagnosis and to see the effect of chemotherapy and steroids on this disease. Setting: Department of Otolaryngology Head & Neck Surgery Jinnah Hospital Lahore. Period: From Dec 1998 to July 2001. Methods: Five cases of Wegener Granulomatosis were studied during the study period. Three patients had limited disease while two patients had generalized disease with renal involvement. All patients were started on azathiopurine (2mg/Kg/day) or cyclophoshamide (1-2mg/km/day) along with steroids. After a months time the dose of steroid was reduced to alternate day. Weekly monitoring of white blood cell count with urine examination for RBCs or protein casts was done. Any patient with haematuria or TLC reduced to less than 3500 were immediately taken off from cyclophoshamide and azathiopurine was started. Patients having classic disease , dose of cyclophoshamide had to be increased from two to four milligram / per Kilogram per day to achieve a g ood response. The response to treatment was measured by noticing the resolution of pulmonary infiltrates reduced ESR and negative C-ANCA. Results: We found nasal symptoms like nasal obstruction and epistaxis to be the most common symptom followed by fever, cough and deafness. Crusting, granulations and occasional perforation were usually found on nasal examination. Conductive deafness was most common ear finding. History, clinical examination, ESR, C-ANCA and a representative biopsy were all essential in diagnosing this condition. Azathiopurine and cyclophosphamide were the main chemotherapeutic agents used with prednisone to cause remission in all our patients except one who died two weeks after having been admitted in our institution. Surprisingly in contrast to the normal cause of high mortality reported in international literature in this disease to be renal failure, our patient died due to respiratory failure. Four out of five patients went into remission a year after regular treatment with this regime. The major complications occurring during hospital stay and their treatment were nausea, vomiting, sudden fall of TLC, and oral thrush. Conclusion: The common presentation of patient with Wegner`s Granulomatosis is of upper respiratory tract symptoms like nasal obstruction and epistaxis followed by lower respiratory symptoms like cough, fever and Haemoptysis. 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 for inducing remission.

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