早期活动性皮肌炎患者肾小球病变:临床、病理和毛细血管镜表现及对治疗的反应

IF 1.4 Q3 RHEUMATOLOGY
Reumatologia Pub Date : 2022-01-01 Epub Date: 2022-07-13 DOI:10.5114/reum.2022.117840
Saeedeh Shenavandeh, Mahsa TorabiJahromi, Sahand Mohammadzadeh
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引用次数: 0

摘要

特发性炎症性肌病(IIMs)是一组系统性结缔组织疾病,表现为肌肉和肌肉外表现。很少有关于肾脏受累的报道,特别是皮肌炎(DM)患者。我们评估了第一年出现蛋白尿的糖尿病患者的临床、实验室、毛细血管镜检查和肾脏病理,并随访他们对治疗的反应。材料和方法:我们评估了2010年4月至2021年10月在甲襞毛细血管镜诊所就诊的205例近端肌无力或高肌酶患者。74例患者符合新的2017年EULAR/ACR成人和青少年IM分类标准,DM的概率≥90%,持续时间≤12个月,蛋白尿> 350mg /24小时。回顾肾小球病变患者的所有表现和肾活检,并随访他们的治疗反应。结果:74例糖尿病患者中,女性52例,男性22例,中位年龄37(19-65)岁,病程中位4.5(1-12)个月,2例(2.7%)患者(25- 28岁男性)有蛋白尿。肾活检显示系血管增殖性肾小球肾炎(GN)。无急性或慢性肾损害或横纹肌溶解病例。两例患者均有高活动性、高红细胞沉降率(ESR)、高c反应蛋白(CRP)、毛细血管镜检查异常、高抗- ro阳性,经免疫抑制治疗3-6个月后肾功能、肌无力、实验室检查早期反应良好。1例患者行毛细血管镜随访,8根手指均无异常。1例患者随访不良,8个月后复发。结论:我们发现在糖尿病患者的活跃期和早期,血管增生性GN是一种罕见的肌肉外表现。全面免疫抑制治疗显示这些患者早期完全康复。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Glomerulopathy in patients with dermatomyositis in early active disease: clinical, pathological and capillaroscopic manifestations, and response to treatment.

Glomerulopathy in patients with dermatomyositis in early active disease: clinical, pathological and capillaroscopic manifestations, and response to treatment.

Glomerulopathy in patients with dermatomyositis in early active disease: clinical, pathological and capillaroscopic manifestations, and response to treatment.

Glomerulopathy in patients with dermatomyositis in early active disease: clinical, pathological and capillaroscopic manifestations, and response to treatment.

Introduction: Idiopathic inflammatory myopathies (IIMs) are a group of systemic connective tissue diseases that present with muscular and extra-muscular manifestations. There are few reports on kidney involvement, especially in dermatomyositis (DM) patients. We evaluated the clinical, laboratory, capillaroscopy, and kidney pathology of patients with DM, who presented with proteinuria during the first year, and followed them for response to treatment.

Material and methods: We evaluated 205 patients with proximal muscle weakness or high muscle enzymes, who referred to the nailfold capillaroscopy clinic from April 2010 to October 2021. Seventy-four patients fulfilled the New 2017 EULAR/ACR Classification Criteria for adult and juvenile IM with probability of ≥ 90% for DM with duration of ≤ 12 months and proteinuria > 350 mg/24 hours. All manifestations of patients with glomerulopathy and their kidney biopsies were reviewed, and they were followed for their treatment response.

Results: From 74 patients with DM, 52 female and 22 male, median age 37 (19-65) years, and disease duration of median 4.5 (1-12) months, 2 (2.7%) patients (25- and 28-year-old male) had proteinuria. Their kidney biopsy showed mesangioproliferative glomerulonephritis (GN). There was no case of acute or chronic kidney damage or rhabdomyolysis. Both had high disease activity, high erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), abnormal capillaroscopy, and high anti-Ro positivity with good early response of their kidney function, muscle weakness, and laboratory tests after immunosuppressive treatment for 3-6 months. One patient had capillaroscopy follow-up, and all abnormalities were resolved in 8 fingers. One patient, due to poor follow-up, after 8 months had recurrence of his disease.

Conclusions: We found mesangioproliferative GN as a rare extra-muscular manifestation in patients with DM in the active and early phase of the disease. Full immunosuppressive treatment showed early complete recovery in these patients.

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来源期刊
Reumatologia
Reumatologia Medicine-Rheumatology
CiteScore
2.70
自引率
0.00%
发文量
44
审稿时长
10 weeks
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