Interstitial Lung Disease: How Should Therapeutics Be Implemented?

Cosimo Bruni; Corrado Campochiaro; Jeska K de Vries-Bouwstra

doi:10.1016/j.rdc.2023.01.005

Interstitial Lung Disease: How Should Therapeutics Be Implemented?

Rheum Dis Clin North Am. 2023 May;49(2):279-293. doi: 10.1016/j.rdc.2023.01.005.

Authors

Cosimo Bruni¹, Corrado Campochiaro², Jeska K de Vries-Bouwstra³

Affiliations

¹ Department of Rheumatology, University Hospital Zurich, University of Zurich, Schmelzbergstrasse 24, Zurich 8006, Switzerland; Division of Rheumatology, Department of Experimental Medicine, Careggi University Hospital - University of Florence, Florence, Italy. Electronic address: cosimo.bruni@usz.ch.
² Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, Vita-Salute San Raffaele University, Via Olgettina 60, Milan, Italy. Electronic address: https://twitter.com/CampochiaroCor.
³ Department of Rheumatology, Leiden University Hospital, Postal Zone C1-R, PO Box 9600, Leiden 2300 RC, the Netherlands.

PMID: 37028835
DOI: 10.1016/j.rdc.2023.01.005

Abstract

Systemic sclerosis-interstitial lung disease (SSc-ILD) is a major complication of SSc resulting in important morbidity and mortality. Next to cyclophosphamide and mycophenolate mofetil, tocilizumab and nintedanib have proven efficacy in the treatment of SSc-ILD. The highly variable course of SSc-ILD, the complexity in determining and predicting the progression of SSc-ILD, and the diversity of treatment options for SSc-ILD, pose many challenges for everyday clinical practice. In this review, currently available evidence for monitoring and treatment of SSc-ILD is summarized and areas where additional evidence is highly desirable are discussed.

Keywords: Combination therapy; Interstitial lung disease; Systemic sclerosis; Targeted therapies; Treatment.

Publication types

Review

MeSH terms

Cyclophosphamide
Humans
Immunosuppressive Agents / therapeutic use
Lung
Lung Diseases, Interstitial* / complications
Lung Diseases, Interstitial* / etiology
Mycophenolic Acid / therapeutic use
Scleroderma, Systemic* / complications
Scleroderma, Systemic* / drug therapy

Substances

Immunosuppressive Agents
Cyclophosphamide
Mycophenolic Acid