title: Interstitial lung disease in systemic sclerosis: current and future treatment.
abstract: Systemic sclerosis (SSc) has the highest fatality rate among connective tissue diseases and is characterized by vascular damage, inflammation and fibrosis of the skin and various internal organs. Interstitial lung disease (ILD) frequently complicates SSc and can be a debilitating disorder with a poor prognosis. ILD is the most frequent cause of death in SSc, and the management of SSc-ILD patients is a great challenge. Early detection of pulmonary involvement based on a recent decline of lung function tests and on the extent of lung involvement at high-resolution computed tomography is critical for the best management of these patients. This article summarizes classification, pathogenesis, diagnosis, prognosis, survival and finally current and future treatment options in SSc-ILD.
authors.affiliation: Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy. roberto.giacomelli@cc.univaq.it.
authors.forename: Roberto
authors.initials: R
authors.lastname: Giacomelli
authors.affiliation: Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy.
authors.forename: Vasiliki
authors.initials: V
authors.lastname: Liakouli
authors.affiliation: Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy.
authors.forename: Onorina
authors.initials: O
authors.lastname: Berardicurti
authors.affiliation: Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy.
authors.forename: Piero
authors.initials: P
authors.lastname: Ruscitti
authors.affiliation: Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Di Benedetto
authors.affiliation: Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Carubbi
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, University of Palermo, Palermo, Italy.
authors.forename: Giuliana
authors.initials: G
authors.lastname: Guggino
authors.affiliation: Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy.
authors.forename: Salvatore
authors.initials: S
authors.lastname: Di Bartolomeo
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, University of Palermo, Palermo, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Ciccia
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, University of Palermo, Palermo, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Triolo
authors.affiliation: Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L'Aquila, Delta 6 Building, Via dell'Ospedale, 67100, L'Aquila, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Cipriani
mesh_terms: D000818:Animals; D042241:Early Diagnosis; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D008168:Lung / Q000187:drug effects* / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D016040:Lung Transplantation; D058990:Molecular Targeted Therapy; D011237:Predictive Value of Tests; D012129:Respiratory Function Tests; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents
keywords: Fibrosis; Interstitial lung disease; Scleroderma; Systemic sclerosis; Treatment