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Résumé Les vascularites systémiques avec atteinte rénale sont une urgence en néphrologie. Devenue exceptionnelle en France, la périartérite noueuse reste un diagnostic à savoir évoquer. Un patient âgé de 70 ans, sans antécédent notable, présente une insuffisance rénale oligurique avec hypertension artérielle maligne et protéinurie glomérulaire abondante. Un nodule sous-cutané, une orchite et une mononévrite apparaissent secondairement. Le bilan immunologique et les sérologies virales sont négatifs. Des stigmates de microangiopathie thrombotique présents initialement s’amendent avec le contrôle tensionnel. Devant la normalité d’un angioscanner rénal, une biopsie rénale est réalisée. Elle montre des lésions vasculaires et ischémique, et se complique d’un choc hémorragique à 36 heures. C’est l’artériographie thérapeutique (embolisation) qui permet finalement le diagnostic de périartérite noueuse, devant la présence de micro-anévrysmes rénaux. Face à une vascularite systémique avec atteinte rénale, l’hypertension artérielle maligne, les nodules sous-cutanés et l’orchite doivent faire évoquer une périartérite noueuse, même en cas de protéinurie glomérulaire. Évoquer le diagnostic implique la réalisation d’une artériographie rénale première à la recherche de micro-anévrysmes. Confirmant le diagnostic, ces derniers contre-indiquent formellement la biopsie rénale, à haut risque de complication hémorragique. La périartérite noueuse existe encore en néphrologie, même sans hépatite B. Une insuffisance rénale aiguë associée à une hypertension artérielle maligne et une vascularite des moyens vaisseaux, sans anticorps anticytoplasme des polynucléaires neutrophiles (ANCA), doivent faire évoquer le diagnostic et faire discuter une artériographie et non une biopsie rénale. Renal involvement of systemic vasculitides is an emergency in nephrology. Although it has become very rare, the diagnosis of polyarteritis nodosa must be considered in some patients. A 70-year-old patient, previously healthy, presented with acute renal failure and malignant hypertension and abundant albuminuria. Subcutaneous nodule, orchitis and mononeuritis appeared subsequently. The search for auto-immunity or viral infection was negative. Markers of thrombotic microangiopathy, present initially, resolved after blood pressure control. After a renal computed tomography with contrast medium injection was considered normal, without any micro-aneurysm, a renal biopsy was performed. It showed vascular lesions and glomerular ischemia. It was complicated by hemorragic shock after 36 hours. The diagnosis of periarteritis nodosa was finally made after arterial angiography showed millimetric renal micro-aneurysms. In case of systemic vasculitis with renal involvement, periarteritis nodosa must be part of differential diagnosis, especially in case of malignant hypertension, subcutaneous nodosa and orchitis, and despite albuminuria. This implies the search for micro-aneurysms with arterial angiography, and the contraindication of renal biopsy, associated with a high risk of severe hemorrhage. Periarteritis nodosa still exists in nephrology, even without hepatitis B infection. The association of acute renal failure with medium vessel vasculitis, with negative ANCA, must alert the nephrologist and lead to arterial angiography not to renal biopsy. [ABSTRACT FROM AUTHOR]

Renal involvement of systemic vasculitides is an emergency in nephrology. Although it has become very rare, the diagnosis of polyarteritis nodosa must be considered in some patients. A 70-year-old patient, previously healthy, presented with acute renal failure and malignant hypertension and abundant albuminuria. Subcutaneous nodule, orchitis and mononeuritis appeared subsequently. The search for auto-immunity or viral infection was negative. Markers of thrombotic microangiopathy, present initially, resolved after blood pressure control. After a renal computed tomography with contrast medium injection was considered normal, without any micro-aneurysm, a renal biopsy was performed. It showed vascular lesions and glomerular ischemia. It was complicated by hemorragic shock after 36hours. The diagnosis of periarteritis nodosa was finally made after arterial angiography showed millimetric renal micro-aneurysms. In case of systemic vasculitis with renal involvement, periarteritis nodosa must be part of differential diagnosis, especially in case of malignant hypertension, subcutaneous nodosa and orchitis, and despite albuminuria. This implies the search for micro-aneurysms with arterial angiography, and the contraindication of renal biopsy, associated with a high risk of severe hemorrhage. Periarteritis nodosa still exists in nephrology, even without hepatitis B infection. The association of acute renal failure with medium vessel vasculitis, with negative ANCA, must alert the nephrologist and lead to arterial angiography not to renal biopsy., (Copyright © 2016 Association Société de néphrologie. Published by Elsevier SAS. All rights reserved.)