Department of Nephrology, Faculty of Medicine, Eskisehir Osmangazi University, Eskisehir, Turkey.
Department of Pathology, Faculty of Medicine, Eskisehir Osmangazi University, Eskisehir, Turkey.
Saudi J Kidney Dis Transpl. 2020 Nov-Dec;31(6):1420-1426. doi: 10.4103/1319-2442.308362.
A 69-year old male patient attended our clinic with fatigue, fever, anuria, nephritic syndrome and severe renal failure. Kidney biopsy showed pauci-immune crescentic glomerulonephritis with an unusual association of suppurative interstitial nephritis. Though most patients with renal involvement linked to antineutrophil cytoplasmic antibodies associated vasculitis (AAV) have pauci-immune glomerulonephritis, only a few patients were identified to have atypical renal pathology. In most cases, mononuclear tubulointerstitial infiltrate may be a feature of AAV, suppurative interstitial nephritis is very rare. In the literature, we found only one case reported associated with suppurative interstitial nephritis without glomerulonephritis who later developed classic pauci-immune necrotizing glomerulonephritis. Here, we report a case diagnosed as AAV, presenting with pauci-immune crescentic glomerulonephritis and suppurative interstitial nephritis. It is not clear whether suppurative interstitial nephritis is a severe disease variant in AAV-associated renal disease. As described in the first case the lack of improvement in renal functions in spite of intense immunosuppressive treatment leads to the conclusion that suppurative interstitial nephritis is a marker of poor prognosis.
一位 69 岁男性患者因疲劳、发热、无尿、肾病综合征和严重肾衰竭到我院就诊。肾活检显示为少免疫性新月体肾小球肾炎,并伴有化脓性间质肾炎的不常见关联。尽管大多数与抗中性粒细胞胞质抗体相关血管炎(AAV)相关的肾受累患者都有少免疫性肾小球肾炎,但只有少数患者被确定为具有非典型肾病理。在大多数情况下,单核性肾小管间质浸润可能是 AAV 的特征,化脓性间质肾炎非常罕见。在文献中,我们仅发现一例报告与化脓性间质肾炎而无肾小球肾炎相关的病例,随后发展为典型的少免疫性坏死性肾小球肾炎。在这里,我们报告一例诊断为 AAV 的病例,表现为少免疫性新月体肾小球肾炎和化脓性间质肾炎。目前尚不清楚化脓性间质肾炎是否是 AAV 相关肾脏疾病的严重疾病变异。正如第一个病例所描述的,尽管进行了强烈的免疫抑制治疗,但肾功能仍未改善,这导致化脓性间质肾炎是预后不良的标志的结论。