Heterogeneity of determining disease severity, clinical course and outcomes in systemic sclerosis-associated interstitial lung disease: a systematic literature review
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
机构:
Univ Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, BrazilUniv Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, Brazil
Aguila, Lisbeth A.
da Silva, Henrique Carrico
论文数: 0引用数: 0
h-index: 0
机构:
Univ Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, BrazilUniv Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, Brazil
da Silva, Henrique Carrico
Medeiros-Ribeiro, Ana Cristina
论文数: 0引用数: 0
h-index: 0
机构:
Univ Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, BrazilUniv Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, Brazil
Medeiros-Ribeiro, Ana Cristina
Bunjes, Bruna Giusto
论文数: 0引用数: 0
h-index: 0
机构:
Univ Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, BrazilUniv Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, Brazil
Bunjes, Bruna Giusto
Luppino-Assad, Ana Paula
论文数: 0引用数: 0
h-index: 0
机构:
Univ Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, BrazilUniv Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, Brazil
Luppino-Assad, Ana Paula
Sampaio-Barros, Percival D.
论文数: 0引用数: 0
h-index: 0
机构:
Univ Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, BrazilUniv Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, Brazil
机构:
Univ Hlth Network, Div Respirol, Dept Med, Toronto, ON, Canada
Univ Hlth Network, Interstitial Lung Dis Program, Toronto, ON, CanadaUniv Hlth Network, Div Respirol, Dept Med, Toronto, ON, Canada
Ahmed, Samar Shadly
Johnson, Sindhu R.
论文数: 0引用数: 0
h-index: 0
机构:
Mt Sinai Hosp, Toronto Scleroderma Program, Div Rheumatol, Dept Med,Toronto Western Hosp, Toronto, ON, Canada
Univ Toronto, Fac Med, Toronto, ON M5S 1A1, CanadaUniv Hlth Network, Div Respirol, Dept Med, Toronto, ON, Canada
Johnson, Sindhu R.
Meaney, Christopher
论文数: 0引用数: 0
h-index: 0
机构:
Univ Toronto, Dept Family & Community Med, Toronto, ON M5S 1A1, CanadaUniv Hlth Network, Div Respirol, Dept Med, Toronto, ON, Canada
Meaney, Christopher
Chau, Cathy
论文数: 0引用数: 0
h-index: 0
机构:
Mt Sinai Hosp, Toronto Western Hosp, Toronto Scleroderma Program, Div Rheumatol,Dept Med, Toronto, ON, CanadaUniv Hlth Network, Div Respirol, Dept Med, Toronto, ON, Canada
Chau, Cathy
Marras, Theodore K.
论文数: 0引用数: 0
h-index: 0
机构:
Univ Hlth Network, Div Respirol, Dept Med, Toronto, ON, Canada
Mt Sinai Hosp, Toronto, ON, Canada
Univ Toronto, Fac Med, Toronto, ON M5S 1A1, Canada
Univ Hlth Network, Interstitial Lung Dis Program, Toronto, ON, CanadaUniv Hlth Network, Div Respirol, Dept Med, Toronto, ON, Canada
机构:
Univ Western Ontario, St Josephs Hlth Ctr London, Div Rheumatol, Dept Med, London, ON N6A 4V2, CanadaUniv Western Ontario, St Josephs Hlth Ctr London, Div Rheumatol, Dept Med, London, ON N6A 4V2, Canada
Al-Dhaher, Firas F.
Pope, Janet E.
论文数: 0引用数: 0
h-index: 0
机构:
Univ Western Ontario, St Josephs Hlth Ctr London, Div Rheumatol, Dept Med, London, ON N6A 4V2, CanadaUniv Western Ontario, St Josephs Hlth Ctr London, Div Rheumatol, Dept Med, London, ON N6A 4V2, Canada
Pope, Janet E.
Ouimet, Janine M.
论文数: 0引用数: 0
h-index: 0
机构:
Univ Western Ontario, St Josephs Hlth Ctr London, Div Rheumatol, Dept Med, London, ON N6A 4V2, CanadaUniv Western Ontario, St Josephs Hlth Ctr London, Div Rheumatol, Dept Med, London, ON N6A 4V2, Canada
机构:
Paris Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, FranceParis Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, France
Allanore, Yannick
Khanna, Dinesh
论文数: 0引用数: 0
h-index: 0
机构:
Univ Michigan, Dept Med, Ann Arbor, MI USAParis Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, France
Khanna, Dinesh
论文数: 引用数:
h-index:
机构:
Smith, Vanessa
Aringer, Martin
论文数: 0引用数: 0
h-index: 0
机构:
Dresden TU, Univ Med Ctr, Dept Med 3, Div Rheumatol, Dresden, Germany
Dresden TU, Fac Med Carl Gustav Carus, Dresden, GermanyParis Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, France
Aringer, Martin
Hoffmann-Vold, Anna-Maria
论文数: 0引用数: 0
h-index: 0
机构:
Oslo Univ Hosp, Dept Rheumatol, Oslo, NorwayParis Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, France
Hoffmann-Vold, Anna-Maria
Kuwana, MasatakaKuwana
论文数: 0引用数: 0
h-index: 0
机构:
Nippon Med Sch, Grad Sch Med, Dept Allergy & Rheumatol, Tokyo, JapanParis Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, France
Kuwana, MasatakaKuwana
Merkel, Peter A.
论文数: 0引用数: 0
h-index: 0
机构:
Univ Penn, Dept Med, Dept Biostat Epidemiol & Informat, Div Rheumatol, Philadelphia, PA USAParis Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, France
Merkel, Peter A.
Stock, Christian
论文数: 0引用数: 0
h-index: 0
机构:Paris Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, France
Stock, Christian
Sambevsk, Steven
论文数: 0引用数: 0
h-index: 0
机构:Paris Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, France
Sambevsk, Steven
Denton, Christopher P.
论文数: 0引用数: 0
h-index: 0
机构:
Boehringer Ingelheim Pharm GmbH & Co KG, Biberach, Germany
Boehringer Ingelheim Int GmbH, Ingelheim, Germany
UCL, Ctr Rheumatol & Connect Tissue Dis, Div Med, London, EnglandParis Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, France
机构:
Univ Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, BrazilUniv Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, Brazil
Aguila, Lisbeth A.
da Silva, Henrique Carrico
论文数: 0引用数: 0
h-index: 0
机构:
Univ Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, BrazilUniv Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, Brazil
da Silva, Henrique Carrico
Medeiros-Ribeiro, Ana Cristina
论文数: 0引用数: 0
h-index: 0
机构:
Univ Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, BrazilUniv Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, Brazil
Medeiros-Ribeiro, Ana Cristina
Bunjes, Bruna Giusto
论文数: 0引用数: 0
h-index: 0
机构:
Univ Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, BrazilUniv Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, Brazil
Bunjes, Bruna Giusto
Luppino-Assad, Ana Paula
论文数: 0引用数: 0
h-index: 0
机构:
Univ Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, BrazilUniv Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, Brazil
Luppino-Assad, Ana Paula
Sampaio-Barros, Percival D.
论文数: 0引用数: 0
h-index: 0
机构:
Univ Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, BrazilUniv Sao Paulo, Hosp Clin HCFMUSP, Fac Med, Div Rheumatol, Av Dr Arnaldo 455,3o Andar, BR-01246903 Sao Paulo, SP, Brazil
机构:
Univ Hlth Network, Div Respirol, Dept Med, Toronto, ON, Canada
Univ Hlth Network, Interstitial Lung Dis Program, Toronto, ON, CanadaUniv Hlth Network, Div Respirol, Dept Med, Toronto, ON, Canada
Ahmed, Samar Shadly
Johnson, Sindhu R.
论文数: 0引用数: 0
h-index: 0
机构:
Mt Sinai Hosp, Toronto Scleroderma Program, Div Rheumatol, Dept Med,Toronto Western Hosp, Toronto, ON, Canada
Univ Toronto, Fac Med, Toronto, ON M5S 1A1, CanadaUniv Hlth Network, Div Respirol, Dept Med, Toronto, ON, Canada
Johnson, Sindhu R.
Meaney, Christopher
论文数: 0引用数: 0
h-index: 0
机构:
Univ Toronto, Dept Family & Community Med, Toronto, ON M5S 1A1, CanadaUniv Hlth Network, Div Respirol, Dept Med, Toronto, ON, Canada
Meaney, Christopher
Chau, Cathy
论文数: 0引用数: 0
h-index: 0
机构:
Mt Sinai Hosp, Toronto Western Hosp, Toronto Scleroderma Program, Div Rheumatol,Dept Med, Toronto, ON, CanadaUniv Hlth Network, Div Respirol, Dept Med, Toronto, ON, Canada
Chau, Cathy
Marras, Theodore K.
论文数: 0引用数: 0
h-index: 0
机构:
Univ Hlth Network, Div Respirol, Dept Med, Toronto, ON, Canada
Mt Sinai Hosp, Toronto, ON, Canada
Univ Toronto, Fac Med, Toronto, ON M5S 1A1, Canada
Univ Hlth Network, Interstitial Lung Dis Program, Toronto, ON, CanadaUniv Hlth Network, Div Respirol, Dept Med, Toronto, ON, Canada
机构:
Univ Western Ontario, St Josephs Hlth Ctr London, Div Rheumatol, Dept Med, London, ON N6A 4V2, CanadaUniv Western Ontario, St Josephs Hlth Ctr London, Div Rheumatol, Dept Med, London, ON N6A 4V2, Canada
Al-Dhaher, Firas F.
Pope, Janet E.
论文数: 0引用数: 0
h-index: 0
机构:
Univ Western Ontario, St Josephs Hlth Ctr London, Div Rheumatol, Dept Med, London, ON N6A 4V2, CanadaUniv Western Ontario, St Josephs Hlth Ctr London, Div Rheumatol, Dept Med, London, ON N6A 4V2, Canada
Pope, Janet E.
Ouimet, Janine M.
论文数: 0引用数: 0
h-index: 0
机构:
Univ Western Ontario, St Josephs Hlth Ctr London, Div Rheumatol, Dept Med, London, ON N6A 4V2, CanadaUniv Western Ontario, St Josephs Hlth Ctr London, Div Rheumatol, Dept Med, London, ON N6A 4V2, Canada
机构:
Paris Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, FranceParis Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, France
Allanore, Yannick
Khanna, Dinesh
论文数: 0引用数: 0
h-index: 0
机构:
Univ Michigan, Dept Med, Ann Arbor, MI USAParis Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, France
Khanna, Dinesh
论文数: 引用数:
h-index:
机构:
Smith, Vanessa
Aringer, Martin
论文数: 0引用数: 0
h-index: 0
机构:
Dresden TU, Univ Med Ctr, Dept Med 3, Div Rheumatol, Dresden, Germany
Dresden TU, Fac Med Carl Gustav Carus, Dresden, GermanyParis Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, France
Aringer, Martin
Hoffmann-Vold, Anna-Maria
论文数: 0引用数: 0
h-index: 0
机构:
Oslo Univ Hosp, Dept Rheumatol, Oslo, NorwayParis Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, France
Hoffmann-Vold, Anna-Maria
Kuwana, MasatakaKuwana
论文数: 0引用数: 0
h-index: 0
机构:
Nippon Med Sch, Grad Sch Med, Dept Allergy & Rheumatol, Tokyo, JapanParis Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, France
Kuwana, MasatakaKuwana
Merkel, Peter A.
论文数: 0引用数: 0
h-index: 0
机构:
Univ Penn, Dept Med, Dept Biostat Epidemiol & Informat, Div Rheumatol, Philadelphia, PA USAParis Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, France
Merkel, Peter A.
Stock, Christian
论文数: 0引用数: 0
h-index: 0
机构:Paris Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, France
Stock, Christian
Sambevsk, Steven
论文数: 0引用数: 0
h-index: 0
机构:Paris Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, France
Sambevsk, Steven
Denton, Christopher P.
论文数: 0引用数: 0
h-index: 0
机构:
Boehringer Ingelheim Pharm GmbH & Co KG, Biberach, Germany
Boehringer Ingelheim Int GmbH, Ingelheim, Germany
UCL, Ctr Rheumatol & Connect Tissue Dis, Div Med, London, EnglandParis Cite Univ, Cochin Hosp, APHP, Dept Rheumatol, Paris, France