WEGENER GRANULOMATOSIS

DIAGNOSIS & MANAGEMENT

Abstract

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 good 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 wasmost common ear finding. History, clinical examination,
ESR, C-ANCA and a representative biopsy were all essential in diagnosing this condition. Azathiopurine and
cyclophosphamidewere 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.