Presenting features in Pakistani patients suffering from the antineutrophil cytoplasmic antibody - classical subtype [c-ANCA] associated vasculitis

ABSTRACT

To study the clinicopathological features in c-ANCA positive patients suffering from vasculitis with a view to find out the most common mode of presentation. STUDY DESIGN: Retrospective. SETTINGS Department of Immunology, AFIP, Rawalpindi, MH Rawalpindi, CMH Rawalpindi, Department of Rheumatology, PIMS, Islamabad, RGH Rawalpindi, FFH, Rawalpindi. SUBJECTS: Seventeen patients suffering from vasculitis and found to be positive for c-ANCA. MAIN OUTCOME MEASURES: Clinico-pathological features at presentation. There were 9 males in age range 11-60 years [mean age 32.5 years] and 8 females in age range 26-42 years [mean age 32.3 years]. Common presenting features were a combination of cough, blocked nose and post nasal drip 14/17 [82%] followed by nose bleed and haematuria 11/17 [65%]. Six patients were demonstrated to be suffering from Wegener's granulomatosis after biopsy. C-ANCA was detected by indirect immunofluorescence in the titre range of 8-640. The auto antibody levels related to disease activity. c-ANCA associated vasculitis is a rare [17 patients in five years] but aggressive form of vasculitis. It must be suspected in patients with persistent respiratory tract related symptoms associated with fever and joint pains which fail to respond to adequate treatment for infections. The c-ANCA estimations can be utilised as sensitive and specific diagnostic and prognostic marker in this form of vasculitis