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A case of familial Mediterranean fever-associated systemic amyloidosis

机译：家族性地中海热相关的系统性淀粉样变性病一例

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Familial Mediterranean fever (FMF) is a chronic inflammatory disease, characterized by recurrent fever and polyserositis (pleuritis and/or peritonitis). The most important complication of FMF is amyloidosis, which causes chronic renal failure. Colchicine is the most effective treatment in acute attacks and amyloidosis development. However, the majority of patients with amyloidosis have a relentless progression to end-stage renal disease despite initiation of colchicine treatment. We present the case of a 38-year-old man with FMF-associated chronic renal failure due to systemic amyloidosis. The patient suffered from periodic fever and renal insufficiency, and was admitted to our hospital. Laboratory examination revealed an inflammatory reaction, renal dysfunction (serum creatinine 2.5 mg/dl), and proteinuria. Renal biopsy revealed segmental mesangial AA amyloid deposits in several glomeruli and the walls of several vessels. Genetic analysis showed that the patient was heterozygous for the MEFV gene (E148Q/M694I). Thus, he was diagnosed with FMF, and colchicine treatment was initiated. He remained almost attack free, with decreasing serum creatinine levels (1.6 mg/dl) and diminishing urinary protein excretion. In conclusion, renal amyloidosis is the most important long-term complication of FMF, and treatment with colchicine is effective for preventing progression. Therefore, colchicine treatment should be initiated as early as possible after the diagnosis of FMF.
机译：家族性地中海热（FMF）是一种慢性炎症性疾病，其特征是反复发烧和多发性浆膜炎（胸膜炎和/或腹膜炎）。 FMF最重要的并发症是淀粉样变性，可引起慢性肾功能衰竭。秋水仙碱是急性发作和淀粉样变性发展中最有效的治疗方法。然而，尽管开始了秋水仙碱治疗，但大多数淀粉样变性病患者仍持续发展为终末期肾脏疾病。我们介绍了一个因系统性淀粉样变性而患有FMF相关的慢性肾功能衰竭的38岁男子的病例。该患者患有周期性发烧和肾功能不全，已入院。实验室检查发现有炎症反应，肾功能不全（血清肌酐2.5 mg / dl）和蛋白尿。肾活检显示部分肾小球和部分血管壁中有节段性肾小球系膜AA淀粉样蛋白沉积。遗传分析表明该患者的MEFV基因（E148Q / M694I）是杂合的。因此，他被诊断出患有FMF，并开始了秋水仙碱治疗。他几乎没有发作，血清肌酐水平降低（1.6 mg / dl），尿蛋白排泄减少。总之，肾淀粉样变性是FMF的最重要的长期并发症，秋水仙碱可以有效地预防进展。因此，应在FMF诊断后尽早开始秋水仙碱治疗。

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