系统性硬化症和抗PL12抗体患者发生严重指尖溃疡、肺动脉高压和硬皮病肾危象

IF 1.1 Q4 ALLERGY

Journal of Cutaneous Immunology and Allergy Pub Date : 2022-09-02 DOI:10.1002/cia2.12270

Akiko Kaneshima MD, Takuya Miyagi MD, Sayaka Yamaguchi MD, PhD, Yoshinao Muro MD, PhD, Kenzo Takahashi MD, PhD

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引用次数: 1

摘要

一名50岁的日本女性因系统性硬化症(SSc)和间质性肺炎接受强的松龙治疗(2.5 mg/2天)约15年。患者突然出现剧烈疼痛、冷感、手指发绀，给予前列地尔静脉注射(10 μg/d)。2天后出现肝(天冬氨酸转氨酶，4400 IU/L;丙氨酸转氨酶，1997国际单位/升)，肾脏(肌酐，2.14毫克/分升)和呼吸(血氧饱和度，77%的室内空气)功能。入院时，患者出现血压升高(160/80 mmHg)，并在一周内出现右手第二至第五指、左手第二和第四指严重坏疽(图1A)。改良罗德曼皮肤评分为2分，雷诺现象，甲襞出血。硬皮病仅发生在四肢，并通过前臂病变的组织病理学检查得到证实。患者抗核(40×，细胞质)、氨基酰基trna合成酶(ARS, 170指数)和PL12抗体检测呈阳性，1但抗拓扑异构酶1、着丝粒、RNA聚合酶III、核糖核蛋白、心磷脂、抗心磷脂β2糖蛋白I复合物、狼疮病抗凝血剂和髓过氧化物酶抗中性粒细胞细胞质抗体检测呈阴性。肺灌注显像检查结果正常。计算机断层扫描显示轻度间质性肺炎。超声心动图评估的心动过压梯度、平均肺动脉压和脑利钠肽n终端激素原水平均明显升高，分别为65 mmHg(正常，<35 mmHg)、50 mmHg (<25 mmHg)和62,991 pg/ml。患者被诊断为局限性皮肤型SSc，伴有抗pl12抗体，急性心力衰竭，充血性肝功能衰竭，原因是硬皮病肾危像(SRC)和肺动脉高压(PAH)。患者的心脏、肾脏和肝功能在持续血液滤过和联合使用血管紧张素转换酶抑制剂和cachanchannel拮抗剂5天后得到改善。全身给予内皮素受体拮抗剂、磷酸二酯酶5型抑制剂和选择性前列腺环素受体激动剂(图1B)，并在坏疽的指尖局部涂抹无水乙醇和蔗糖聚维腺苷软膏。7个月后，观察到指尖的再上皮化(图1C)。抗合成酶综合征通常与特定临床症状的发展有关，如雷诺现象、机械性手、多发性关节炎、间质性肺炎和肌炎抗ars抗体，包括抗pl12抗体，是典型的多肌炎/皮肌炎特异性抗体。抗ars抗体阳性患者发生SSc的频率相对较低(3.6%)少数SSc伴抗pl12抗体的病例已被报道。然而，没有报道这种情况下指坏疽，多环芳烃和SRC。通常，SRC或PAH分别发生在抗rna聚合酶III或抗着丝粒抗体的SSc患者中。3,4治疗多环芳烃的药物对于同时治疗ssc患者的手指溃疡和坏疽通常是有效的在这个病例中，我们通过多种抗多环芳烃药物的联合治疗，阻止了坏疽的进展，并实现了治愈。这种方法可能有助于治疗难治性指部溃疡和/或坏疽的SSc患者。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

Development of severe fingertip ulcers, pulmonary hypertension, and scleroderma renal crisis in a patient with systemic sclerosis and anti-PL12 antibodies

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Development of severe fingertip ulcers, pulmonary hypertension, and scleroderma renal crisis in a patient with systemic sclerosis and anti-PL12 antibodies

A 50-year-old Japanese woman with limited cutaneous-type SSc presented with severe gangrene in the fingertips of the hands and hypertension, tested positive for anti-PL-12 antibodies. She was diagnosed with acute heart failure owing to scleroderma renal crisis and pulmonary arterial hypertension. Therapeutic agents for pulmonary arterial hypertension were also effective for the digital gangrene.

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来源期刊

Journal of Cutaneous Immunology and Allergy Medicine-Dermatology

CiteScore

0.60

自引率

10.00%

发文量

审稿时长

12 weeks