在2024 EULAR年会期间，来自英国利兹大学的Francesco Del Galdo教授汇报了系统性硬化症（SSc）管理指南的更新结果。 该共识包括了八项临床领域，分别为雷诺现象（Raynaud Phenomenon，RP），指端溃疡（Digital Ulcers），肺动脉高压（Pulmonary Hypertension, PAH），皮肤纤维化（Skin Fibrosis），间质性肺病（Interstitial Lung Disease, ILD），皮肤纤维化，肌肉骨骼受累，胃肠道表现和肾危象（Renal Crisis）；涵盖一线和二线治疗。
The recommendation strength of consensus is divided into four levels, from strong to weak, which are "should be considered", "can be considered" (B), "may be considered" (C), and "expert opinion" (D). In this new version of the recommendation, the expert group introduced Raynaud's phenomenon, digital ulcers, and pulmonary hypertension as vascular lineages, and introduced and updated skin fibrosis and pulmonary interstitial lesions as a set of clinical manifestations. The authors emphasize that this update of the recommendation is not intended as an absolute guideline, given the complexity of the SSc disease.
Fig.1 Summary of systemic sclerosis treatment drugs and their recommended intensities
注：CCB，钙离子拮抗剂；PED5i，磷酸二酯酶5抑制剂；MTX，甲氨蝶呤；CYC；环磷酰胺；TCZ，托珠单抗；ACEI，血管紧张素转化酶抑制剂；MMF，吗替麦考酚酯；ERAs，内皮素受体拮抗剂
Table 1 Recommendations for Raynaud's phenomenon, digital ulcers, and pulmonary hypertension in systemic sclerosis
Fig.2 Flow chart of Raynaud's phenomenon and digital ulcer treatment of systemic sclerosis
Fig.3 Flow chart of the treatment of pulmonary hypertension in systemic sclerosis
Table 2 Recommendations for systemic sclerosis, skin fibrosis, and interstitial lung disease
Figure 4: Flow chart of the treatment of interstitial lung disease in integrated sclerosis
Table 3 Recommendations for renal crisis, gastrointestinal involvement, and musculoskeletal involvement in systemic sclerosis
Table 4 Recommendations for poor prognosis of systemic sclerosis
After introducing the recommendations, Professor Francesco Del Galdo also elaborated on the content of the study, which mainly includes the following five points:
1) To evaluate the efficacy of immunosuppressants and/or other immune-targeted disease-modifying antirheumatic drugs (DMARDs) in SSc vascular lesions and gastrointestinal lesions;
2) to evaluate the treatment of non-pharmacological interventions for digital ulcers;
3) to evaluate the efficacy of biologics in the intervention of SSc cardiovascular lesions;
4) to assess the efficacy of pharmacological and non-pharmacological interventions for calcinosis;
5) To evaluate the efficacy of novel immunosuppressants to expand the combination of immunosuppressants and anti-fibrotic drugs and improve clinical outcomes for patients.
Information sources:
Yimaitong is a professional online doctor platform, and the mission of the platform is to "sense the pulse of the world's medicine and help China's clinical decision-making". Yimaitong has a series of products such as "Clinical Guidelines", "Medication Reference", "Medical Literature King", "Yizhiyuan", "eYantong" and "ePulse", which fully meet the needs of medical workers in clinical decision-making, obtaining new knowledge and improving scientific research efficiency.