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一例侵袭性冷球蛋白血症合并膜增生性肾小球肾炎病例报告

An Aggressive Case of Cryoglobulinemia and Membranoproliferative Glomerulonephritis: A Case Report.

作者信息

Ferdman Leonard, Jensen Hannah, Hazaa Alshaimaa, Donnell Robert W

机构信息

Internal Medicine, University of Arkansas for Medical Sciences, Fayetteville, USA.

Department of Surgery, University of Arkansas for Medical Sciences, Fayetteville, USA.

出版信息

Cureus. 2024 Jun 11;16(6):e62193. doi: 10.7759/cureus.62193. eCollection 2024 Jun.

DOI:10.7759/cureus.62193
PMID:39006560
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11239600/
Abstract

This case report describes a 66-year-old female with membranoproliferative glomerulonephritis (MPGN) with pulmonary involvement presumed secondary to Hepatitis C virus (HCV)-associated with mixed cryoglobulinemia. In this condition, pulmonary involvement is uncommon, and aggressive lung involvement can be associated with poor outcomes. Within eight weeks, the patient was hospitalized twice with acute pulmonary presentations and presented at a third hospitalization with dyspnea, chest pain, abdominal pain, and edema. Imaging revealed persistent and historically evolving lung consolidation, as well as a renal biopsy showing MPGN associated with mixed cryoglobulinemia. A lung biopsy revealed inflammation. Bronchoalveolar lavage did not show hemosiderin-laden macrophages and did not grow infectious agents. Serology revealed negative ANCAs and rheumatoid factor positive at 476 IU/ml (upper limit normal 14 IU/ml). Qualitative cryoglobulins were positive at 2 %ppt (reference range: negative %ppt) and Type II mixed cryoglobulinemia with IgM kappa plus polyclonal IgG. The treatment involved steroids and rituximab. The patient's clinical status deteriorated, and she elected to change her resuscitation status to comfort care measures. This case emphasizes that cryoglobulinemia can present with aggressive manifestations on a wide spectrum. Pulmonary manifestations are rare and were evident in this case (although without clear evidence of diffuse alveolar hemorrhage) and led to a complicated disease course and an unfavorable outcome. Overall, this case underscores the complexity of mixed cryoglobulinemia presentations and the challenges of managing severe cases with multi-organ involvement.

摘要

本病例报告描述了一名66岁女性,患有膜增生性肾小球肾炎(MPGN),伴有肺部受累,推测继发于丙型肝炎病毒(HCV)相关的混合性冷球蛋白血症。在这种情况下,肺部受累并不常见,严重的肺部受累可能与不良预后相关。在八周内,该患者因急性肺部症状两次住院,并在第三次住院时出现呼吸困难、胸痛、腹痛和水肿。影像学检查显示肺部持续存在且有历史演变的实变,肾活检显示MPGN与混合性冷球蛋白血症相关。肺活检显示有炎症。支气管肺泡灌洗未发现含铁血黄素巨噬细胞,也未培养出感染病原体。血清学检查显示抗中性粒细胞胞浆抗体阴性,类风湿因子阳性,为476 IU/ml(正常上限为14 IU/ml)。定性冷球蛋白为阳性,2%ppt(参考范围:阴性%ppt),为II型混合性冷球蛋白血症,伴有IgM κ加多克隆IgG。治疗包括使用类固醇和利妥昔单抗。患者的临床状况恶化,她选择将复苏状态改为舒适护理措施。本病例强调冷球蛋白血症可表现出广泛的侵袭性表现。肺部表现罕见,在本病例中很明显(尽管没有弥漫性肺泡出血的确切证据),并导致了复杂的病程和不良结局。总体而言,本病例强调了混合性冷球蛋白血症表现的复杂性以及管理多器官受累严重病例的挑战。

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本文引用的文献

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HCV-Associated Nephropathies in the Era of Direct Acting Antiviral Agents.直接抗病毒药物时代的丙型肝炎病毒相关肾病
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