A Diagnostic and Therapeutic Dilemma Concerning Exostosin 1/Exostosin 2-associated Lupus-like Membranous Nephropathy with Positive Antinuclear Antibody in an Elderly Man with Various Immune Abnormalities.

在患有各种免疫异常的老年人中 ， 与 Exostosin 1 / Exostosin 2 相关的狼疮样膜性肾病具有阳性抗核抗体。

• 影响指数: 1.282
• 发表时间: Nov 2023 1
• 来源期刊: Intern Med
• DOI: 10.2169/internalmedicine.1251-22
• PMID: 36948625
• 文章类型: Journal Article
• 中科院分区: Q3
• 方向: 医学
• 作者列表: Kawagoe M,Odajima K,Asakawa S,Nagura M,Arai S,Yamazaki O,Tamura Y,Ohashi R,Iwakura T,Yasuda H,Shibata S,Fujigaki Y

关键词: Sjögren's syndrome; exostosin; full house glomerular deposits; lupus nephritis; secondary membranous nephropathy

Mesh: Male; Humans; Aged; Antibodies, Antinuclear; Glomerulonephritis, Membranous / diagnosis drug therapy complications; Lupus Erythematosus, Systemic / complications diagnosis drug therapy; Autoimmune Diseases / complications; Kidney

Abstract

Exostosin 1 (EXT1) and exostosin 2 (EXT2)-associated membranous nephropathy (MN) may be associated with active autoimmune disease. We encountered an elderly man who presented with EXT1/EXT2-associated lupus-like MN with full house immune deposits, monoclonal gammopathy of uncertain significance and Sjögren\'s syndrome. The patient exhibited various other immune abnormalities. Although he did not fulfill the criteria of clinical systemic lupus erythematosus (SLE), he met a stand-alone renal criterion of the Systemic Lupus International Collaborating Clinics (SLICC) 2012. Whether or not a stand-alone renal criterion with EXT1/EXT2 positivity, as in the present patient, can efficiently guide decisions regarding the diagnosis and treatment of SLE remains a clinical dilemma.

摘要

外泌素1（EXT1）和外泌素2（EXT2）相关膜性肾病（MN）可能与活动性自身免疫性疾病有关。我们遇到了一位老人，他患有与EXT1/EXT2相关的狼疮样MN，并有满屋的免疫沉积物，意义不确定的单克隆丙种球蛋白病和干燥综合征。患者表现出各种其他免疫异常。虽然他没有达到临床系统性红斑狼疮（SLE）的标准，他符合SLICC2012的独立肾脏标准.无论是否具有EXT1/EXT2阳性的独立肾脏标准，就像现在的病人一样，能否有效地指导SLE诊断和治疗的决策仍然是一个临床难题。