Ammayappan Shiva Kumar, Rajagopalan Arul, Rajendran Manorajan, Arunachalam Jegan, Prasath Arun, Durai Rakesh, Kurien Anila A
Department of Nephrology, Government Rajaji Hospital, Madurai Medical College, Madurai, Tamil Nadu, India.
Renopath, Center for Renal and Urological Pathology, Chennai, Tamil Nadu, India.
Indian J Nephrol. 2023 May-Jun;33(3):206-208. doi: 10.4103/ijn.ijn_187_21. Epub 2023 Feb 20.
Fibrillary glomerulonephritis (FGN) is a rare form of glomerulonephritis, usually occurring in concurrence with other conditions such as hepatitis C, dysproteinemia, autoimmune conditions, diabetes mellitus, and malignancy. The diagnosis is made by the presence of randomly oriented fibrillar deposits with a mean diameter of 20 nm, which stain positive for IgG and C3 and are negative for congo red and thioflavin T stains. Staining for DNAJB9 (DnaJ homolog subfamily B member 9) is a recently discovered mode of diagnosis of FGN without electron microscopy. The prognosis is poor and optimal treatment is yet not clearly defined, though rituximab may be useful in FGN patients with relatively preserved renal functions. In this case report, we discuss a case of post-renal transplant patient with occurrence of fibrillary glomerulonephritis.
纤维性肾小球肾炎(FGN)是一种罕见的肾小球肾炎形式,通常与其他疾病同时发生,如丙型肝炎、蛋白血症、自身免疫性疾病、糖尿病和恶性肿瘤。诊断依据是存在平均直径为20纳米的随机排列的纤维状沉积物,其IgG和C3染色呈阳性,刚果红和硫黄素T染色呈阴性。DNAJB9(DnaJ同源亚家族B成员9)染色是一种最近发现的无需电子显微镜即可诊断FGN的方法。预后较差,最佳治疗方法尚未明确界定,尽管利妥昔单抗可能对肾功能相对保留的FGN患者有用。在本病例报告中,我们讨论了一例肾移植后发生纤维性肾小球肾炎的患者。