Granulomatosis with polyangiitis in Tunisia

Submitted: 5 October 2016
Accepted: 27 April 2017
Published: 22 May 2017
Abstract Views: 1546
PDF: 740
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Granulomatosis with polyangiitis (GPA) is more frequent in Northern rather than Southern countries. Very few studies have been conducted in Africa. We have performed a retrospective descriptive study including clinical and laboratory profiles of 30 Tunisian GPA patients seen at the department of Internal Medicine of the University Hospital of la Rabta from 2000 to 2014. Mean age at initial GPA diagnosis was 46±12 years, and the average number of months between the onset of symptoms and diagnosis was 25. Seventeen (56%) were male, and 13 (44%) were female. Ear/nose/throat involvement occurred in 83%. Lung and renal involvement were observed in respectively 70% and 56% followed by mucocutaneous (50%), neurological (50%), ocular (33%), vascular (20%), ureteral (16%), and cardiac involvement in 10%. Cytoplasmic pattern-antineutrophil cytoplasmic antibodies (ANCA) was detected in 27 (90%) patients. Induction therapy consisted of intravenous cyclophosphamide pulses in 27 patients (90%) and oral methotrexate in 3 patients (10%). Trimethoprime-sulfamethoxazole was used in 26 patients (86%). Maintenance therapy consisted of azathioprine in 17 cases and methotrexate in 13 cases. Relapses occurred in 36%. Eighteen patients had favorable outcome and 12 died. Our patients had a distinct phenotype with high prevalence of pleural involvement, lymph node enlargement, sensorimotor neuropathy and ureter stenosis. ENT symptoms were less frequent as inaugural presentation. Overall 2-year survival was 60%.

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I. Ben Ghorbel, Department of Internal Medicine, Medical Faculty of Tunis
Department of Internal Medicine
N. Belfeki, Department of Internal Medicine, Medical Faculty of Tunis
Department of Internal Medicine
N. Baouendi, Department of Internal Medicine, Medical Faculty of Tunis
Department of Internal Medicine
T. Ben Salem, Department of Internal Medicine, Medical Faculty of Tunis
Department of Internal Medicine
M.H. Houman, Department of Internal Medicine, Medical Faculty of Tunis
Department of Internal Medicine

How to Cite

Ben Ghorbel, I., Belfeki, N., Baouendi, N., Ben Salem, T., & Houman, M. (2017). Granulomatosis with polyangiitis in Tunisia. Reumatismo, 69(1), 23–29. https://doi.org/10.4081/reumatismo.2017.935

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