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

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

Antibodies to the extractable nuclear antigens [ENA] in Pakistani patients with systemic rheumatic disease

Autoantibodies to the saline extracts of cells are being used as diagnostic and prognostic markers in the investigation of patients with suspected systemic rheumatic disease. These are also called antibodies to the extractable nuclear antigens or anti ENA antibodies. We used saline extracts of human spleen cells and rabbit thymus extract to detect six subtypes of anti ENA antibodies, namely anti SSA [anti Sjogren's syndrome A], anti SSB [anti Sjogren's syndrome B], anti RNP [anti Ribonucleoprotein], anti Sm [anti Smith], anti Jo-1 [anti Jo-1] and anti Sc1 70 [anti Systemic sclerosis 70] antibodies. Two hundred and thirty-seven patients' sera were screened in the department of Immunology, Armed Forces Institute of Pathology [AFIP], Rawalpindi, over a period of 16 months. Fifty-seven were positive for auto antibodies, of which 26 had anti SSA antibodies, 12 anti SSA+ anti RNP+ anti Sm antibodies, 7 anti RNP+ anti Sm antibodies, 6 anti SSA+ anti SSB antibodies, 5 anti SSA+ anti RNP antibodies while one patient showed only anti RNP antibody. The detection of the anti ENA antibodies was specially helpful in diagnosing patients with systemic lupus erythematosis presenting with atypical features and with mixed connective tissue disease

Anti-neutrophil cytoplasmic auto antibodies[ANCA] in Pakistani patients with systemic vasculitides

Anti-neutrophil cytoplasmic auto antibodies are directed against antigens in the neutrophil granules. Their detection by the indirect immunofluorescence clearly divides them into two distinct types, namely c-ANCA [Classical antineutrophil cytoplasmic antibody] and the p-ANCA [Perinuclear antineutrophil cytoplasmic antibody]. These antibodies have been found to be useful as non-invasive markers to help establish the diagnosis in patients with systemic vasculitides. The antibodies also help in monitoring disease activity in some patients with systemic necrotising vasculitides. This study was aimed at demonstrating the utility of these auto-antibodies in the management of our patients presenting with systemic vasculitides. Fifty-six patients presenting with features of systemic vasculitides were examined over a six month period out of whom eight were found positive for these antibodies. The detection of these antibodies helped in early diagnosis and the institution of specific treatment. Six months follow-up in one patient with Wegener's granulomatosis, the disease activity related closely with the ANCA levels