Granulomatose avec polyangéite du sujet âgé: à propos de deux cas et revue de la literature

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Abstract

La granulomatose avec polyangéite (GPA) est une vascularite nécrosante des vaisseaux de petit calibre. L’âge moyen d'entrée dans la GPA est entre 35 et 55 ans, les formes gériatriques sont cependant rares, Nous rapportons deux cas de GPA révélés après 60 ans, le mode de révélation était inhabituel, ophtalmologique dans le premier cas et cutané dans le deuxième cas.

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Most cited references 12

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Wegener granulomatosis: an analysis of 158 patients.

Gary Hoffman (1992)

To prospectively study the clinical features, pathophysiology, treatment and prognosis of Wegener granulomatosis. Of the 180 patients with Wegener granulomatosis referred to the National Institute of Allergy and Infectious Diseases during the past 24 years, 158 have been followed for 6 months to 24 years (a total of 1229 patient-years). Characteristics of clinical presentation, surgical pathology, course of illness, laboratory and radiographic findings, and the results of medical and surgical treatment have been recorded in a computer-based information retrieval system. The Warren Magnuson Clinical Center of the National Institutes of Health. Men and women were equally represented; 97% of patients were white, and 85% were more than 19 years of age. The mean period of follow-up was 8 years. One hundred and thirty-three patients (84%) received "standard" therapy with daily low-dose cyclophosphamide and glucocorticoids. Eight (5.0%) received only low-dose cyclophosphamide. Six (4.0%) never received cyclophosphamide and were treated with other cytotoxic agents and glucocorticoids. Ten patients (6.0%) were treated with only glucocorticoids. Ninety-one percent of patients experienced marked improvement, and 75% achieved complete remission. Fifty percent of remissions were associated with one or more relapses. Of 99 patients followed for greater than 5 years, 44% had remissions of greater than 5 years duration. Thirteen percent of patients died of Wegener granulomatosis, treatment-related causes, or both. Almost all patients had serious morbidity from irreversible features of their disease (86%) or side effects of treatment (42%). The course of Wegener granulomatosis has been dramatically improved by daily treatment with cyclophosphamide and glucocorticoids. Nonetheless, disease- and treatment-related morbidity is often profound. Alternative forms of therapy have not yet achieved the high rates of remission induction and successful maintenance that have been reported with daily cyclophosphamide treatment. Despite continued therapeutic success with cyclophosphamide, our long-term follow-up of patients with Wegener granulomatosis has led to increasing concerns about toxicity resulting from prolonged cyclophosphamide therapy and has encouraged investigation of other therapeutic regimens.

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Wegener's granulomatosis of the orbit: a clinicopathological study of 15 patients.

William Faquin, P U Fechner, B Z Pilch (2002)

Wegener's granulomatosis is a granulomatous and necrotizing vasculitis that classically involves the respiratory and renal systems. The goal of the study was to define clinical and pathological characteristics in a subgroup of patients with the changes of Wegener's granulomatosis involving the orbit. Retrospective study. A database search identified 15 patients with the histological changes of Wegener's granulomatosis of the orbit presenting over a 23-year period. A review of the histological specimens, radiological studies, and patient charts was performed. Additional follow-up data were obtained through patient interviews. Of 15 patients (median age, 54 y) with Wegener's granulomatosis of the orbit identified, the disease was limited to the orbit in 12 patients; 3 patients had additional sinonasal involvement. All patients underwent various surgical procedures followed by medical treatment (cyclophosphamide and prednisone). Specimens showed characteristic histopathological features of Wegener's granulomatosis. Follow-up data were available for 12 patients with a median period of 5 years. In the group with only orbital involvement, none of the patients developed systemic progression of Wegener's granulomatosis. Only one patient had multiple local recurrences and later developed contralateral orbital Wegener's granulomatosis. Wegener's granulomatosis limited to the orbit is a localized form of the disease without systemic progression. Diagnostic surgical procedures followed by aggressive medical treatment results in good outcome, although local recurrence may occur.

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Wegener's granulomatosis in the elderly.

S R Weiner, R Weisbart, H E Paulus (1986)

Wegener's granulomatosis is a systemic necrotizing granulomatous vasculitis in which the average age at onset is in the forties. The disease has an excellent response to treatment with cyclophosphamide. We proposed that the tendency to accept multisystem disease in elderly patients (greater than or equal to 60 years old) and a reluctance to administer aggressive therapy would be reflected in the diagnosis and treatment of Wegener's granulomatosis in the elderly, and that as a consequence, the delay in diagnosis and implementation of therapy could result in a needlessly higher morbidity and mortality rate for this population of patients.

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Journal

Journal ID (nlm-ta): Pan Afr Med J

Journal ID (iso-abbrev): Pan Afr Med J

Journal ID (publisher-id): PAMJ

Title: The Pan African Medical Journal

Publisher: The African Field Epidemiology Network

ISSN (Electronic): 1937-8688

Publication date (Electronic): 10 April 2015

Publication date Collection: 2015

Volume: 20

Electronic Location Identifier: 341

Affiliations

[1 ]Service de Médecine Interne, Hopital Taher Sfar, Mahdia, Tunisie

[2 ]Service de Médecine Interne, Hopital Fattouma Bourguiba, Monastir, Tunisie

[3 ]Unité De Médecine Physique, Hopital Taher Sfar, Mahdia, Tunisie

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[& ]Corresponding author: Olfa Berriche, Service de Médecine Interne, Hopital Taher Sfar, Mahdia, Tunisie

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Publisher ID: PAMJ-20-341

DOI: 10.11604/pamj.2015.20.341.6618

PMC ID: 4491465

SO-VID: a42d31d2-ca9d-425b-88ce-10bca53f1672

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The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Granulomatose avec polyangéite du sujet âgé: à propos de deux cas et revue de la literature Translated title: Granulomatosis with polyangiitis in the elderly: report of two cases and review of literature

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Abstract

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Pan African Medical Journal

Most cited references 12

Wegener granulomatosis: an analysis of 158 patients.

Wegener's granulomatosis of the orbit: a clinicopathological study of 15 patients.

Wegener's granulomatosis in the elderly.

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