Sambataro Domenico, Morina Giulia, Libra Alessandro, Palmucci Stefano, Pallotti Francesco, Geraci Giulio, La Rocca Gaetano, Ferro Francesco, Moretti Michele, Baldini Chiara, Vancheri Carlo, Sambataro Gianluca
Outpatient Clinic Associated with the Regional Health System, Artroreuma s.r.l., 95030 Mascalucia (CT), Italy.
Department of Clinical and Experimental Medicine, Regional Referral Center for Rare Lung Diseases, Policlinico "G.Rodolico-San Marco", University of Catania, 95123 Catania, Italy.
Medicina (Kaunas). 2025 Mar 26;61(4):599. doi: 10.3390/medicina61040599.
Usual Interstitial Pneumonia (UIP) is the most severe radiological/histological pattern of Interstitial Lung Disease (ILD). It is typical of Idiopathic Pulmonary Fibrosis (IPF), but is also frequently described in Autoimmune Rheumatic Diseases (ARDs), sharing with IPF common risk factors, genetic backgrounds, and in some cases, disease progression and prognosis. Following the results of the PANTHER study, immunosuppressive drugs are now not recommended for the treatment of IPF; however, their use for the treatment of UIP secondary to ARDs is still under debate. The aim of this review is to summarize existing knowledge on the clinical presentation of autoimmune UIP and its treatment with immunosuppressive drugs. We searched PubMed for English language clinical trials and studies on treatment of ARDs-ILD, looking for specific treatments of UIP-ARDs. The available clinical trials rarely stratify patients by ILD pattern, and clinical studies generally lack a comparison with a placebo group. In Systemic Sclerosis, UIP patients showed a non-significant trend of worsening under immunosuppression. On the contrary, in Interstitial Pneumonia with Autoimmune Features and, above all, Rheumatoid Arthritis, immunosuppressive treatment produced promising results in the management of UIP patients. In conclusion, the current evidence about the immunosuppressive treatment of UIP-ARDs is limited and conflicting. There is an urgent need to adequately assess this topic with specific clinical trials, as has already been performed for IPF. The possibility should be considered that different ARDs can respond differently to immunosuppression. Finally, a wider use of histological samples could produce valuable information from a diagnostic, therapeutic, and research point of view.
寻常型间质性肺炎（UIP）是间质性肺疾病（ILD）最严重的放射学/组织学类型。它是特发性肺纤维化（IPF）的典型表现，但在自身免疫性风湿性疾病（ARDs）中也经常被描述，与IPF具有共同的危险因素、遗传背景，在某些情况下，还有疾病进展和预后情况。根据PANTHER研究结果，目前不推荐使用免疫抑制药物治疗IPF；然而，其用于治疗继发于ARDs的UIP仍存在争议。本综述的目的是总结关于自身免疫性UIP临床表现及其免疫抑制药物治疗的现有知识。我们在PubMed上搜索了关于ARDs-ILD治疗的英文临床试验和研究，以寻找UIP-ARDs的特定治疗方法。现有的临床试验很少按ILD类型对患者进行分层，临床研究通常缺乏与安慰剂组的比较。在系统性硬化症中，UIP患者在免疫抑制下有病情恶化的非显著趋势。相反，在具有自身免疫特征的间质性肺炎，尤其是类风湿关节炎中，免疫抑制治疗在UIP患者的管理中取得了有希望的结果。总之，目前关于UIP-ARDs免疫抑制治疗的证据有限且相互矛盾。迫切需要通过特定的临床试验对这一主题进行充分评估，就像已经对IPF所做的那样。应该考虑到不同的ARDs对免疫抑制的反应可能不同。最后，更广泛地使用组织学样本可能会从诊断、治疗和研究的角度产生有价值的信息。
