Journal Article Review Epublish
PubMed: 40282891 DOI: 10.3390/medicina61040599
Immunosuppressive Therapy for Usual Interstitial Pneumonia in Autoimmune Rheumatic Diseases: A Review.
Medicina (Kaunas) 61(4)
Authors: Domenico Sambataro, Giulia Morina, Alessandro Libra, Stefano Palmucci, Francesco Pallotti, Giulio Geraci, Gaetano La Rocca, Francesco Ferro, Michele Moretti, Chiara Baldini, Carlo Vancheri, Gianluca Sambataro
Affiliations
- 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.
- Department of Medical Surgical Sciences and Advanced Technologies "GF Ingrassia", University Hospital Policlinico "G.Rodolico-San Marco", Unità Operativa Semplice Dipartimentale di Imaging Polmonare e Tecniche Radiologiche Avanzate (UOSD IPTRA), University of Catania, 95123 Catania, Italy.
- Department of Medicine and Surgery, University of Enna "Kore", 94100 Enna, Italy.
- Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, 56126 Pisa, Italy.
Abstract
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.
MeSH
Idiopathic Pulmonary Fibrosis, Fibrosis, Pharmaceutical Preparations, Rheumatic Diseases, Pneumonia, Arthritis, Rheumatoid, Therapeutics, Immunosuppression Therapy, Scleroderma, Systemic, Lung Diseases, Interstitial