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静脉注射免疫球蛋白治疗坏疽性脓皮病:81例患者的多中心回顾性分析

Intravenous Immunoglobulin Therapy for Pyoderma Gangrenosum: A Multicenter Retrospective Analysis in 81 Patients.

作者信息

Ronicke Moritz, Sollfrank Lukas, Vitus Martin V, Walter Lukas J, Krieter Manuel, Moelleken Maurice, Dissemond Joachim, Schultz Erwin, Lauffer Felix, von den Driesch Peter, Erfurt-Berge Cornelia

机构信息

Department of Dermatology, Uniklinikum Erlangen, Friedrich-Alexander University Erlangen-Nürnberg, Ulmenweg 18, 91054, Erlangen, Germany.

Deutsches Zentrum Immuntherapie (DZI), Uniklinikum, Erlangen, Germany.

出版信息

Am J Clin Dermatol. 2025 Jan;26(1):139-146. doi: 10.1007/s40257-024-00904-w. Epub 2024 Nov 14.

Abstract

BACKGROUND

Pyoderma gangrenosum (PG) is rare neutrophil skin disease causing painful, progressively enlarging ulcers. Among the treatment options, intravenous immunoglobulin (IVIG) is a therapy of first choice for paraneoplastic PG. Otherwise, it is used in therapy-refractory courses.

OBJECTIVE

To assess the efficacy and safety of IVIG therapy in patients with PG.

METHODS

A retrospective chart review for patients in five dermatologic wound centres in Germany was performed.

RESULTS

Overall, 81 patients were included. IVIG was used as adjunct therapy with (methyl-) prednisolone and/or a steroid sparing therapy in 77 (95.1%) cases. Response to treatment (combined complete and partial, defined as tendency to heal and cessation of lesion progression, respectively) was 49.3% 1 month after initiation of IVIG. In total 18.8% had a complete response after 6 months. Statistically significantly higher response rates were observed in patients with diabetes mellitus and thyroid disease [odds ratio (OR) 3.49, confidence interval (CI) 1.13-10.80 and OR 6.64, CI 1.01-43.57, respectively]. Patients with solid malignancy tended to have better response (OR 4.36, CI 0.79-23.91). A higher IVIG dose was also associated with a tendency towards better response rates (OR 2.70, CI 0.84-8.63). In total, 1 (1.2%) severe adverse event (myocardial infarction with consequent death) was observed as well as three moderate adverse events, with two thromboembolic events (2.5%) and one acute kidney injury (1.2%). Other adverse events were mild or unlikely to be associated with IVIG therapy, with 14 events in 10 patients overall (12.3%).

CONCLUSIONS

This multicentre retrospective study shows the important role of adjunctive IVIG therapy in patients with PG with recalcitrant courses. Identifying subgroups with a higher probability of response could improve future response rates and save patients from ineffective treatment and potential adverse events.

摘要

背景

坏疽性脓皮病(PG)是一种罕见的中性粒细胞性皮肤病,可导致疼痛且逐渐扩大的溃疡。在治疗方案中,静脉注射免疫球蛋白(IVIG)是副肿瘤性PG的首选治疗方法。否则,它用于治疗难治性病程。

目的

评估IVIG治疗PG患者的疗效和安全性。

方法

对德国五个皮肤科伤口中心的患者进行回顾性病历审查。

结果

总共纳入了81例患者。在77例(95.1%)病例中,IVIG与(甲基)泼尼松龙和/或类固醇节省疗法联合用作辅助治疗。IVIG开始治疗1个月后,治疗反应(联合完全缓解和部分缓解,分别定义为愈合趋势和病变进展停止)为49.3%。6个月后,共有18.8%的患者完全缓解。糖尿病和甲状腺疾病患者的反应率在统计学上显著更高[优势比(OR)分别为3.49,置信区间(CI)为1.13 - 10.80;OR为6.64,CI为1.01 - 43.57]。实体恶性肿瘤患者往往有更好的反应(OR为4.36,CI为0.79 - 23.91)。较高的IVIG剂量也与更好的反应率趋势相关(OR为2.70,CI为0.84 - 8.63)。总共观察到1例(1.2%)严重不良事件(心肌梗死并导致死亡)以及3例中度不良事件,其中2例血栓栓塞事件(2.5%)和1例急性肾损伤(1.2%)。其他不良事件为轻度或不太可能与IVIG治疗相关,总共10例患者出现14例事件(12.3%)。

结论

这项多中心回顾性研究表明辅助IVIG治疗在病程顽固的PG患者中的重要作用。识别反应可能性较高的亚组可以提高未来的反应率,并使患者避免无效治疗和潜在的不良事件。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2f55/11741994/4cbf0822e8ee/40257_2024_904_Fig1_HTML.jpg

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