Zhang, Fengxia; Xie, Zhiyong; Peng, Siqi; Jiang, Nan; Li, Bohou; Chen, Boxi; Deng, Shuting; Yuan, Ye; Wu, Qiong; Wen, Sichun; Tao, Yiming; Ma, Jianchao; Li, Sijia; Lin, Ting; Wen, Feng; Li, Zhuo; Huang, Renwei;
Li, Xingxing; Ge, Qiaoyue; Yu, Chuan; Zhao, Wenting; Wu, Chenxin; Liu, Zhenmi; Meng, Xiandong; Xiao, Chenghan
doi: 10.1111/nep.14371pmid: 39168961
Choi, Ji‐Young; Nam, Eon Jeong; Han, Man‐hoon; Kim, Yong‐Jin; Lim, Jeong‐Hoon; Jung, Hee‐Yeon; Cho, Jang‐Hee; Kim, Chan‐Duck; Kim, Yong‐Lim; Park, Sun‐Hee
doi: 10.1111/nep.14375pmid: 39082196
Anti‐phospholipid syndrome (APS) nephropathy is an autoimmune disease that is sometimes accompanied by systemic lupus erythematosus (SLE). Here, we report the use of rituximab to treat a case of APS nephropathy in a SLE patient with recurrent vascular thrombosis. A 52‐year‐old woman, who had been diagnosed with SLE 11 years earlier, was referred to a nephrology clinic for evaluation of azotaemia and proteinuria. She had experienced spontaneous abortion at 35 years of age. The patient had been diagnosed with right popliteal thrombosis at 39 years of age, and with left pulmonary artery thrombosis and SLE at 41 years of age. Before admission, she was undergoing anticoagulant and immunosuppressive therapies, with follow‐up in the rheumatology clinic. At her last outpatient clinic visit before admission, she exhibited mild bilateral lower‐limb pitting oedema, impaired renal function and proteinuria. Renal biopsy revealed arteriolar wall thickening, with thrombi in the capillary lumina and marked inflammatory cell infiltration in the interstitium. The patient was treated with warfarin and high‐dose corticosteroids. Intravenous rituximab (500 mg) was also administered twice at a 4‐week interval. Her renal function did not worsen any further, and her proteinuria decreased. Here we report the successful use of rituximab to treat APS nephropathy in a patient with SLE, who had progressive renal insufficiency.
Tanemoto, Fumiaki; Mimura, Imari; Nangaku, Masaomi
doi: 10.1111/nep.14391pmid: 39307972
Kimura disease (KD) is a rare chronic inflammatory disease that typically presents with soft subcutaneous granulomas in the head and neck regions characterized by elevated blood eosinophils and immunoglobulin E (IgE) level, whose aetiology remains poorly elucidated. Minimal change disease (MCD) has been reported as one of the renal manifestations that KD can present with, indicating that they may share a common pathology. Herein we describe a case of recurrent MCD associated with KD. During a follow‐up period of 15 years, MCD recurred three times with increased disease activity of KD as reflected by flares of skin lesions and elevated peripheral eosinophils, and responded well to increased doses of prednisolone and cyclosporin. Notably, visual field defects in his right monocular vision appeared at the time of third recurrence of MCD, leading to the diagnosis of optic neuritis (ON). Optic nerve involvement associated with KD is extremely rare, and this case is noteworthy in that inflammation in the optic nerve was observed at the time of MCD recurrence with increased disease activity of KD, suggesting the existence of a common pathology between KD, MCD, and ON. In patients with KD, an imbalance of T helper (Th) cells with Th2 cells predominating over Th1 cells is observed, which results in hyperIgEemia and eosinophilia. This Th2‐predominant immunological status in KD considered to predispose to MCD may also predispose to ON. MCD with a background of Th2‐predominant immune state may require attention to the possibility of complication of ON.
Au‐Yang, Wai; Cheung, Tai Yiu; Chan, Hui Yiu; Cheuk, Wah; Cheung, Chi Yuen
doi: 10.1111/nep.14379pmid: 39147389
Waldenstrom macroglobulinaemia (WM), the predominant subtype of lymphoplasmacytic lymphoma with bone marrow involvement and serum IgM paraprotein, is a haematological condition commonly associated with renal parenchymal involvement. However, antineutrophil cytoplasmic antibody (ANCA)‐negative pauci‐immune crescentic glomerulonephritis (PICGN) in kidney infiltrated by lymphoma is very rare, with only two cases described in chronic lymphocytic leukaemia in English literature so far. We herein report the first patient with WM developing ANCA‐negative PICGN. He was a 76‐year‐old male who presented with elevated serum globulin level and bilateral groin lymph node enlargement, subsequently diagnosed to have WM after pathologic examination of the bone marrow and groin lymph node. One month later, he was found to have acute kidney injury and proteinuria. Renal biopsy confirmed the presence of parenchymal involvement by WM accompanied by PICGN; while ANCA testing was negative. He was treated with pulse methylprednisolone followed by oral prednisolone. In addition, six courses of intravenous rituximab and oral cyclophosphamide were given. There was significant improvement in both his renal and haematological conditions. The clinical course of this case suggested that ANCA‐negative PICGN may represent a paraneoplastic syndrome and a rare manifestation of WM‐associated renal lesion. Early kidney biopsy and prompt treatment may improve the outcome of patients.
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doi: 10.1111/nep.14387pmid: 39254037