Heterogeneity of determining disease severity, clinical course and outcomes in systemic sclerosis-associated interstitial lung disease: a systematic literature review

被引:5
作者
Petelytska, Liubov [1 ,2 ]
Bonomi, Francesco [3 ]
Cannistra, Carlo [3 ]
Fiorentini, Elisa [3 ]
Peretti, Silvia [3 ]
Torracchi, Sara [3 ]
Bernardini, Pamela [3 ]
Coccia, Carmela [3 ]
De Luca, Riccardo [3 ]
Economou, Alessio [3 ]
Levani, Juela [3 ]
Matucci-Cerinic, Marco [3 ,4 ]
Distler, Oliver [1 ]
Bruni, Cosimo [1 ,3 ]
机构
[1] Univ Zurich, Univ Hosp Zurich, Rheumatol, Zurich, Switzerland
[2] Bogomolets Natl Med Univ, Dept Internal Med 3, Kiiv, Ukraine
[3] Univ Florence, Careggi Univ Hosp, Dept Expt & Clin Med, Div Rheumatol, Florence, Italy
[4] Osped San Raffaele, Unit Immunol Rheumatol Allergy & Rare Dis, Milan, Italy
来源
RMD OPEN | 2023年 / 9卷 / 04期
关键词
Systemic Sclerosis; Pulmonary Fibrosis; Outcome Assessment; Health Care; Epidemiology; RESOLUTION COMPUTED-TOMOGRAPHY; PULMONARY-FUNCTION TESTS; VON DEN LUNGEN-6; CONNECTIVE-TISSUE DISEASES; STEM-CELL TRANSPLANTATION; EULAR SCLERODERMA TRIALS; PLACEBO-CONTROLLED TRIAL; HIGH-DOSE PREDNISOLONE; ALVEOLAR NITRIC-OXIDE; LONG-TERM PROGRESSION;
D O I
10.1136/rmdopen-2023-003426
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Objective The course of systemic sclerosis-associated interstitial lung disease (SSc-ILD) is highly variable and different from continuously progressive idiopathic pulmonary fibrosis (IPF). Most proposed definitions of progressive pulmonary fibrosis or SSc-ILD severity are based on the research data from patients with IPF and are not validated for patients with SSc-ILD. Our study aimed to gather the current evidence for severity, progression and outcomes of SSc-ILD.Methods A systematic literature review to search for definitions of severity, progression and outcomes recorded for SSc-ILD was performed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines in Medline, Embase, Web of Science and Cochrane Library up to 1 August 2023.Results A total of 9054 papers were reviewed and 342 were finally included. The most frequent tools used for the definition of SSc-ILD progression and severity were combined changes of carbon monoxide diffusing capacity (DLCO) and forced vital capacity (FVC), isolated FVC or DLCO changes, high-resolution CT (HRCT) extension and composite algorithms including pulmonary function test, clinical signs and HRCT data. Mortality was the most frequently reported long-term event, both from all causes or ILD related.Conclusions The studies presenting definitions of SSc-ILD 'progression', 'severity' and 'outcome' show a large heterogeneity. These results emphasise the need for developing a standardised, consensus definition of severe SSc-ILD, to link a disease specific definition of progression as a surrogate outcome for clinical trials and clinical practice.PROSPERO registration number CRD42022379254.Cite Now
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页数:19
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