Department of Nephrology, Erciyes University Medical School, Dede Efendi Sreet, Köşk District, 38030, Melikgazi, Kayseri, Turkey.
Department of Internal Medicine, Kayseri State Hospital, Kayseri, Turkey.
BMC Nephrol. 2023 Mar 31;24(1):84. doi: 10.1186/s12882-023-03134-0.
Microscopic polyangiitis (MPA), a kind of antineutrophil cytoplasmic autoantibody associated vasculitis (AAV), predominantly affects small-sized vessels. MPA is a significant cause of the pulmonary-renal syndrome. Pauci-immune necrotizing and crescentic glomerulonephritis is the typical renal histological feature of AAV. Tubulointerstitial lesions may occur and mostly form with inflammatory cell infiltration in the interstitium. However, a few cases reported only tubulointerstitial involvement without glomerular lesions in patients with MPA.
We present an MPA case, a 70-year-old male patient diagnosed with acute kidney injury accompanying the dialysis requirement. Only acute tubulointerstitial nephritis was revealed in kidney biopsy without evidence of glomerular injury. Also, interstitial pulmonary fibrosis was determined on computerized tomography, and myeloperoxidase antineutrophil cytoplasmic autoantibody was positive. Consequently, we have considered the main diagnosis as MPA. We did not prefer a standard tubulointerstitial nephritis treatment regimen due to the presence of life-threatening systemic vasculitis. Treatment was established like crescentic glomerulonephritis. Induction therapy consisted of pulse steroid, cyclophosphamide, and plasmapheresis. Unfortunately, severe SARS-CoV-2 infection caused death during induction therapy in this case.
The lack of glomerular injury and solely interstitial inflammation is atypical regarding AAV involvement in the kidney. This diversity might be initially considered as only a simple histological elaboration. However, it is a significant entity for guiding the treatment of AAV.
显微镜下多血管炎(MPA)是一种与中性粒细胞胞浆抗体相关的血管炎(AAV),主要影响小血管。MPA 是肺-肾综合征的重要原因。少免疫性坏死性和新月体肾小球肾炎是 AAV 的典型肾组织学特征。肾小管间质性病变可能发生,主要表现为间质中炎症细胞浸润。然而,有少数病例报告称,在 MPA 患者中,仅出现肾小管间质性受累而无肾小球病变。
我们报告了一例 MPA 病例,一名 70 岁男性患者因急性肾损伤需要透析而被诊断。肾活检仅显示急性肾小管间质性肾炎,无肾小球损伤证据。此外,计算机断层扫描显示间质性肺纤维化,髓过氧化物酶抗中性粒细胞胞浆抗体阳性。因此,我们认为主要诊断为 MPA。由于存在威胁生命的全身血管炎,我们不倾向于采用标准的肾小管间质性肾炎治疗方案。治疗方案与新月体肾小球肾炎相同。诱导治疗包括脉冲类固醇、环磷酰胺和血浆置换。不幸的是,在本例中,严重的 SARS-CoV-2 感染导致诱导治疗期间死亡。
肾脏 AAV 受累时缺乏肾小球损伤和仅间质炎症是非典型的。这种多样性最初可能被认为只是一种简单的组织学表现。然而,它对于指导 AAV 的治疗具有重要意义。