Refractory cutaneous ulcers in anti-MDA5 dermatomyositis were not associated with rapidly progressive interstitial lung disease, and responded to tadalafil

IF 4.6 2区医学 Q1 RHEUMATOLOGY

Seminars in arthritis and rheumatism Pub Date : 2025-01-31 DOI:10.1016/j.semarthrit.2025.152651

Yongpeng Ge, Wei Jiang, Xin Lu, Guochun Wang , Xiaoming Shu

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引用次数: 0

Abstract

Objectives

The purpose of this study was to analyze the characteristics of an ulcerative rash in dermatomyositis (DM) patients with anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody and to assess the efficacy of tadalafil in the management of refractory cutaneous ulcers.

Methods

Medical records of DM patients with anti-MDA5 antibody were reviewed. Skin rash data, radiologic characteristic and serum markers were collected. Patients with refractory cutaneous ulcers treated with tadalafil were reviewed retrospectively.

Results

Eighty-two consecutive cases with anti-MDA5-antibody positive DM (anti-MDA5 DM) were identified, including 62 (75.6 %) female patients. Approximately 95.0 % of the patients had interstitial lung disease (ILD) and 45.1 % had rapidly progressive ILD (RP-ILD). All patients (100 %) had typical skin rashes, such as Gottron's sign (87.8 %), heliotrope sign (69.5 %), and the holster sign (24.4 %). Twenty-six patients (31.7 %) had cutaneous ulcers. Univariate analysis showed that ulcer patients were prone to Gottron's sign (p = 0.026), perlungual erythematosus (p = 0.007), and arthritis (p = 0.031), while shortness of breath after exercise (p = 0.027) and RP-ILD (p = 0.024) are more likely to appear in patients with non-skin ulcers. Among the anti-MDA5 DM patients with skin ulcers, 10 (38.5 %) were refractory cutaneous ulcers, including five male patients. After tadalafil was added, eight (80 %) patients showed improvement in the physician global assessment (PGA), Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) score and the patient's pain score using the visual analog score (p-VAS), within one to two months. In addition, the refractory cutaneous ulcers of five patients completely resolved after treatment.

Conclusions

This study found that ulcerative rash in anti-MDA5 DM may not be associated with RP-ILD. The results also suggest that tadalafil may be a useful therapy for refractory cutaneous ulcers.

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来源期刊

Seminars in arthritis and rheumatism 医学-风湿病学

CiteScore

9.20

自引率

4.00%

发文量

176

审稿时长

46 days

期刊介绍： Seminars in Arthritis and Rheumatism provides access to the highest-quality clinical, therapeutic and translational research about arthritis, rheumatology and musculoskeletal disorders that affect the joints and connective tissue. Each bimonthly issue includes articles giving you the latest diagnostic criteria, consensus statements, systematic reviews and meta-analyses as well as clinical and translational research studies. Read this journal for the latest groundbreaking research and to gain insights from scientists and clinicians on the management and treatment of musculoskeletal and autoimmune rheumatologic diseases. The journal is of interest to rheumatologists, orthopedic surgeons, internal medicine physicians, immunologists and specialists in bone and mineral metabolism.