Sekine Akinari, Hasegawa Eiko, Hiramatsu Rikako, Mise Koki, Sumida Keiichi, Ueno Toshiharu, Yamanouchi Masayuki, Hayami Noriko, Suwabe Tatsuya, Hoshino Junichi, Sawa Naoki, Takaichi Kenmei, Ohashi Kenichi, Fujii Takeshi, Ubara Yoshifumi
Nephrology Center, Toranomon Hospital, Tokyo, Japan.
Nephrology Center, Toranomon Hospital, Tokyo, Japan ; Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Tokyo, Japan.
Case Rep Nephrol Dial. 2015 Oct 9;5(3):192-9. doi: 10.1159/000441107. eCollection 2015 Sep-Dec.
Renovascular lesions of lupus nephritis (LN) were classified into five categories by D'Agati in Heptinstall's Pathology of the Kidney, with thrombotic microangiopathy (TMA) and clinical thrombotic thrombocytopenic purpura (TTP) being combined. We encountered 2 cases with histological LN (class III and lass V), and they presented with clinical features of TTP, such as acute renal failure, microangiopathic hemolytic anemia, thrombocytopenia, fever, and central neurologic symptoms. Immunosuppressive therapy with plasmapheresis was performed in both patients. Case 1 progressed to end-stage renal failure requiring dialysis and died, while case 2 responded to treatment. In case 1, small renal arteries showed positive mural staining for IgG and C3, while intraluminal material was negative for IgG and C3 [although it was positive for phosphotungstic acid-hematoxylin (PTAH), indicating fibrin deposition]. In case 2, small renal arteries showed mural staining for IgG, C1q, and C3, with the intraluminal material also being positive for these immunoglobulins, but negative for PTAH. These cases suggest that immunosuppressive therapy with plasmapheresis can control LN when intravascular thrombosis is related to immune complexes associated with activation of the early complement components C1q and C3. In contrast, immunosuppressive therapy with plasmapheresis may not be effective when intravascular thrombosis is unrelated to these factors and involves fibrin deposition. Accordingly, in LN patients with clinical features of TTP, we report two types of renovascular lesions, in addition to typical vascular change of TMA with no immune deposits seen in nonlupus patients.
狼疮性肾炎（LN）的肾血管病变在《Heptinstall肾脏病理学》中被达加蒂分为五类，其中血栓性微血管病（TMA）和临床血栓性血小板减少性紫癜（TTP）合并为一类。我们遇到2例组织学诊断为LN（Ⅲ类和Ⅴ类）的患者，他们表现出TTP的临床特征，如急性肾衰竭、微血管病性溶血性贫血、血小板减少、发热和中枢神经系统症状。两名患者均接受了血浆置换免疫抑制治疗。病例1进展为终末期肾衰竭，需要透析，最终死亡，而病例2对治疗有反应。在病例1中，肾小动脉壁IgG和C3染色呈阳性，而管腔内物质IgG和C3染色呈阴性[尽管其磷钨酸苏木精（PTAH）染色呈阳性，表明有纤维蛋白沉积]。在病例2中，肾小动脉壁IgG、C1q和C3染色呈阳性，管腔内物质这些免疫球蛋白染色也呈阳性，但PTAH染色呈阴性。这些病例表明，当血管内血栓形成与早期补体成分C1q和C3激活相关的免疫复合物有关时，血浆置换免疫抑制治疗可以控制LN。相反，当血管内血栓形成与这些因素无关且涉及纤维蛋白沉积时，血浆置换免疫抑制治疗可能无效。因此，在具有TTP临床特征的LN患者中，我们报告了两种肾血管病变类型，此外还有非狼疮患者中所见的无免疫沉积物的典型TMA血管变化。
