title: Where are we going in the management of interstitial lung disease in patients with systemic sclerosis?
abstract: Interstitial lung disease (ILD) affects about 90% of patients with systemic sclerosis (SSc). It is associated with a restrictive lung disease in only 30% of patients and is progressive in an even lower percentage. A low forced vital capacity at presentation, an extent of lung fibrosis >20% as detected by lung high-resolution computed tomography, high serum interleukin-6 levels, anti-topoisomerase I antibody positivity and diffuse cutaneous SSc are each associated with SSc-ILD progression. However, no such association is absolute. Treating patients with a recent deterioration of lung function may allow to capture those with active disease. To date, cyclophosphamide (CYC) is the only drug found to stabilize or improve lung function in randomized clinical trials, but its small beneficial effect is short lived. Therefore, immunosuppressive maintenance therapy after CYC treatment is warranted. At present, however, the best therapeutical strategy after CYC therapy both in responders and in non-responders to CYC is still controversial. Based on a review of the literature, we suggest an approach to the management of SSc-ILD.
authors.affiliation: Department of Clinical and Experimental Medicine, Rheumatology Section, Second University of Naples, Naples, Italy. Electronic address: michele_iudici@hotmail.com.
authors.forename: Michele
authors.initials: M
authors.lastname: Iudici
authors.affiliation: Department of Clinical and Molecular Sciences, Università Politecnica delle Marche, Ancona, Italy. Electronic address: g.moroncini@univpm.it.
authors.forename: Gianluca
authors.initials: G
authors.lastname: Moroncini
authors.affiliation: Rheumatology Unit, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L'Aquila, L'Aquila, Italy. Electronic address: paola.cipriani@cc.univaq.it.
authors.forename: Paola
authors.initials: P
authors.lastname: Cipriani
authors.affiliation: Rheumatology Unit, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L'Aquila, L'Aquila, Italy. Electronic address: roberto.giacomelli@univaq.it.
authors.forename: Roberto
authors.initials: R
authors.lastname: Giacomelli
authors.affiliation: Department of Clinical and Molecular Sciences, Università Politecnica delle Marche, Ancona, Italy. Electronic address: a.gabrielli@univpm.it.
authors.forename: Armando
authors.initials: A
authors.lastname: Gabrielli
authors.affiliation: Department of Clinical and Experimental Medicine, Rheumatology Section, Second University of Naples, Naples, Italy. Electronic address: gabriele.valentini@unina2.it.
authors.forename: Gabriele
authors.initials: G
authors.lastname: Valentini
mesh_terms: D003520:Cyclophosphamide / Q000627:therapeutic use; D018450:Disease Progression; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017563:Lung Diseases, Interstitial / Q000209:etiology / Q000628:therapy*; D012595:Scleroderma, Systemic / Q000150:complications* / Q000175:diagnosis; D033581:Stem Cell Transplantation
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide
keywords: Active SSc-ILD; First-line treatment; Second-line treatment