Verdesca Simone Vincenzo Saverio, Villani Chiara, Rossini Michele, Manno Carlo, Gesualdo Loreto, Montinaro Vincenzo
Scuola di specializzazione in Nefrologia, Università degli Studi Aldo Moro di Bari, Azienda Ospedaliero-Universitaria Policlinico-Bari.
U.O. Nefrologia Universitaria, Azienda Ospedaliero-Universitaria Policlinico e Università degli Studi Aldo Moro di Bari.
G Ital Nefrol. 2018 Jul;35(4).
Small and medium vessel vasculitides, either ANCA-associated or caused by anti-GBM antibodies, are multisystemic diseases with predominantly renal involvement that often require dialysis support; clinical remission can be induced with immunosuppressive therapies including apheretic treatments, high doses of steroids, and immune suppressants. In addition to the complications resulting from the primary pathological process, those associated with the immunosuppressive therapies are not negligible. Reversible Posterior Encephalopathy Syndrome (PRES) is a clinical condition with a hyperacute onset, which can complicate the evolution of vasculitides while treated by immunosuppressive therapy. Relevant pathogenic factors are represented by alterations of the cerebral blood-brain barrier or vasogenic and/or brain edema phenomena, also related to uncontrolled hypertension. We describe two cases of patients with systemic vasculitides, rapidly progressive renal failure (RPGN) requiring dialysis, and poor response to the initial immunosuppressive therapy who were treated subsequently with rituximab. PRES developed immediately after administration of the drug, which, however resulted effective on the course of the vasculitis in one case and not effective in the other. In both cases, the subsequent radiological controls showed a total resolution of the encephalic alterations observed during the acute phase.
中小血管血管炎,无论是抗中性粒细胞胞浆抗体相关性还是抗肾小球基底膜抗体所致,都是多系统疾病,主要累及肾脏,常需透析支持;免疫抑制治疗(包括血浆置换、大剂量类固醇和免疫抑制剂)可诱导临床缓解。除了原发性病理过程导致的并发症外,与免疫抑制治疗相关的并发症也不容忽视。可逆性后部脑病综合征(PRES)是一种起病极急的临床病症,在免疫抑制治疗血管炎过程中可能使其病情复杂化。相关致病因素表现为血脑屏障改变或血管源性和/或脑水肿现象,也与未控制的高血压有关。我们描述了两例系统性血管炎患者,他们患有快速进展性肾衰竭(RPGN)需要透析,对初始免疫抑制治疗反应不佳,随后接受了利妥昔单抗治疗。用药后立即出现PRES,然而,在其中一例中该药物对血管炎病程有效,在另一例中则无效。在两例中,随后的影像学检查均显示急性期观察到的脑部病变完全消退。