Ayaash Ali, Maan Dipesh, Kapetanos Anastasios, Bunker Mark, Wasko Mary Chester, Clark Barbara
Allegheny General Hospital, Allegheny Health Network, Drexel University School of Medicine, Pittsburgh, Pennsylvania, USA.
Division of Nephrology, Allegheny General Hospital, Allegheny Health Network, Drexel University School of Medicine, Pittsburgh, Pennsylvania, USA.
Case Rep Nephrol Dial. 2019 Apr 30;9(1):42-48. doi: 10.1159/000500105. eCollection 2019 Jan-Apr.
Crescentic glomerulonephritis (GN) without immune reactants or deposits (referred to as pauci-immune) is typically characterized by the presence of anti-neutrophilic cytoplasmic antibodies (ANCA). While ANCA-negative patients might be expected to have a more benign course, they often have poor renal outcomes, especially without treatment with steroids and immune-modulating therapy. Pauci-immune crescentic GN can also co-exist with other autoimmune conditions, including rheumatoid arthritis (RA). Here, we describe an ANCA-negative patient with RA who developed dialysis-requiring acute kidney injury (AKI) with findings consistent with focal pauci-immune crescentic GN (i.e., no IgG or immune complex on kidney biopsy). Coexistent conditions included Klebsiella sepsis attributed to pneumonia, rhabdomyolysis, leukocytoclastic immune-mediated skin vasculitis, and positive ANA. He had spontaneous improvement in renal function without immunosuppressive therapy. This crescentic GN was not associated with poor renal outcome as AKI resolved with supportive care and treatment of his infection. The AKI was likely multifactorial with co-existing acute tubular necrosis in the setting of Kebsiella sepsis and rhabdomyolysis, and the crescentic GN was felt more likely to be related to the infection rather than having a primary role. This case highlights the importance of viewing crescentic GN in the context of the clinical picture, as it may not always lead to the need of aggressive immune suppression and is not a universally poor prognostic kidney finding. However, these cases do warrant close follow-up as our patient had recurrent RA disease manifestations over the next 2 years that eventually led to his death from severe pulmonary hypertension.
无免疫反应物或沉积物的新月体性肾小球肾炎(GN)(称为少免疫性)通常以抗中性粒细胞胞浆抗体(ANCA)的存在为特征。虽然预计ANCA阴性患者的病程可能更良性,但他们往往肾脏预后较差,尤其是在未接受类固醇和免疫调节治疗的情况下。少免疫性新月体性GN也可与其他自身免疫性疾病共存,包括类风湿性关节炎(RA)。在此,我们描述了一名患有RA的ANCA阴性患者,该患者发生了需要透析的急性肾损伤(AKI),其表现与局灶性少免疫性新月体性GN一致(即肾活检未见IgG或免疫复合物)。共存情况包括因肺炎导致的克雷伯菌败血症、横纹肌溶解、白细胞破碎性免疫介导的皮肤血管炎和ANA阳性。他在未接受免疫抑制治疗的情况下肾功能自发改善。由于AKI通过支持治疗和感染治疗得以缓解,这种新月体性GN并未导致不良肾脏预后。AKI可能是多因素的,在克雷伯菌败血症和横纹肌溶解的情况下并存急性肾小管坏死,并且新月体性GN更可能与感染有关而非起主要作用。该病例强调了结合临床情况看待新月体性GN的重要性,因为它不一定总是需要积极的免疫抑制,也并非普遍预示着不良的肾脏预后。然而,这些病例确实需要密切随访,因为我们的患者在接下来的2年中出现了复发性RA疾病表现,最终因严重肺动脉高压死亡。