Progressive lung fibrosis and mortality can occur in early systemic sclerosis patients without pulmonary abnormalities at baseline assessment

Objectives In systemic sclerosis, baseline extent of radiological involvement is an important outcome predictor and baseline absence of radiological involvement suggests a more favourable prognosis. As current predictive models are based on cohorts with variable disease duration, we aim to assess disease dynamics in early disease. Methods Patients were included from the prospective longitudinal Belgian Systemic Sclerosis Cohort. We included patients with a disease duration < = 36 months at baseline with available baseline thoracic high-resolution computed tomography (HRCT) images and longitudinal pulmonary function test (PFT) results until 42 months of follow-up. Results Fifty-two patients were included; 50% were male and 44% suffered from diffuse cutaneous systemic sclerosis. A total of 46% carried anti-topoisomerase 1 antibodies. The mean disease duration at baseline visit was 11 months. At baseline visit, 40.4% (21/52) patients had HRCT abnormalities. Patients with abnormal HRCT findings more frequently suffered from diffuse cutaneous systemic sclerosis (p < 0.05) and less frequently carried anti-centromere antibodies (p < 0.05). Patients without CT abnormalities at baseline had a shorter disease duration (9 ± 7 months versus 14 ± 12 months). After 42 months, 8/52 patients, including 3 patients with normal HRCT findings at baseline, died due to SSc-related manifestations. Progression of lung fibrosis occurred in 16 patients at month 42, including 7 patients with normal CT at baseline. No clear predictors of progression could be identified. Conclusion In early SSc patients, the disease dynamics differ from the large published cohorts. Progressive lung fibrosis and mortality can also occur in patients without radiological abnormalities at baseline.Key Points• Disease dynamics in early SSc differ from more established SSc.• In early SSc, progressive pulmonary fibrosis can occur in patients without CT abnormalities at baseline.• In early SSc, more stringent pulmonary follow-up is warranted both in lcSSc and dcSSc. Ausführliche Beschreibung

Gespeichert in:

Autor*in:	Vanaken, Lize [verfasserIn] Landini, Nicholas [verfasserIn] Lenaerts, Joris [verfasserIn] Claeys, Eveline [verfasserIn] Lenaerts, Jan [verfasserIn] Wuyts, Wim A. [verfasserIn] Verschakelen, Johny [verfasserIn] De Langhe, Ellen [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2020

Schlagwörter:	High-resolution computed tomography Interstitial lung disease Mortality Pulmonary fibrosis Systemic sclerosis

Übergeordnetes Werk:	Enthalten in: Clinical rheumatology - London : Springer, 1982, 39(2020), 11 vom: 08. Mai, Seite 3393-3400
Übergeordnetes Werk:	volume:39 ; year:2020 ; number:11 ; day:08 ; month:05 ; pages:3393-3400

Links:	Volltext

DOI / URN:	10.1007/s10067-020-05105-4

Katalog-ID:	SPR041382544

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520			\|a Objectives In systemic sclerosis, baseline extent of radiological involvement is an important outcome predictor and baseline absence of radiological involvement suggests a more favourable prognosis. As current predictive models are based on cohorts with variable disease duration, we aim to assess disease dynamics in early disease. Methods Patients were included from the prospective longitudinal Belgian Systemic Sclerosis Cohort. We included patients with a disease duration < = 36 months at baseline with available baseline thoracic high-resolution computed tomography (HRCT) images and longitudinal pulmonary function test (PFT) results until 42 months of follow-up. Results Fifty-two patients were included; 50% were male and 44% suffered from diffuse cutaneous systemic sclerosis. A total of 46% carried anti-topoisomerase 1 antibodies. The mean disease duration at baseline visit was 11 months. At baseline visit, 40.4% (21/52) patients had HRCT abnormalities. Patients with abnormal HRCT findings more frequently suffered from diffuse cutaneous systemic sclerosis (p < 0.05) and less frequently carried anti-centromere antibodies (p < 0.05). Patients without CT abnormalities at baseline had a shorter disease duration (9 ± 7 months versus 14 ± 12 months). After 42 months, 8/52 patients, including 3 patients with normal HRCT findings at baseline, died due to SSc-related manifestations. Progression of lung fibrosis occurred in 16 patients at month 42, including 7 patients with normal CT at baseline. No clear predictors of progression could be identified. Conclusion In early SSc patients, the disease dynamics differ from the large published cohorts. Progressive lung fibrosis and mortality can also occur in patients without radiological abnormalities at baseline.Key Points• Disease dynamics in early SSc differ from more established SSc.• In early SSc, progressive pulmonary fibrosis can occur in patients without CT abnormalities at baseline.• In early SSc, more stringent pulmonary follow-up is warranted both in lcSSc and dcSSc.
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author_variant	l v lv n l nl j l jl e c ec j l jl w a w wa waw j v jv l e d le led
matchkey_str	article:14349949:2020----::rgesvlnfboiadotltcncuieryytmcceoiptetwtotumnra
hierarchy_sort_str	2020
bklnumber	44.00 44.83
publishDate	2020
allfields	10.1007/s10067-020-05105-4 doi (DE-627)SPR041382544 (SPR)s10067-020-05105-4-e DE-627 ger DE-627 rakwb eng 610 ASE 610 ASE 44.00 bkl 44.83 bkl Vanaken, Lize verfasserin aut Progressive lung fibrosis and mortality can occur in early systemic sclerosis patients without pulmonary abnormalities at baseline assessment 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Objectives In systemic sclerosis, baseline extent of radiological involvement is an important outcome predictor and baseline absence of radiological involvement suggests a more favourable prognosis. As current predictive models are based on cohorts with variable disease duration, we aim to assess disease dynamics in early disease. Methods Patients were included from the prospective longitudinal Belgian Systemic Sclerosis Cohort. We included patients with a disease duration < = 36 months at baseline with available baseline thoracic high-resolution computed tomography (HRCT) images and longitudinal pulmonary function test (PFT) results until 42 months of follow-up. Results Fifty-two patients were included; 50% were male and 44% suffered from diffuse cutaneous systemic sclerosis. A total of 46% carried anti-topoisomerase 1 antibodies. The mean disease duration at baseline visit was 11 months. At baseline visit, 40.4% (21/52) patients had HRCT abnormalities. Patients with abnormal HRCT findings more frequently suffered from diffuse cutaneous systemic sclerosis (p < 0.05) and less frequently carried anti-centromere antibodies (p < 0.05). Patients without CT abnormalities at baseline had a shorter disease duration (9 ± 7 months versus 14 ± 12 months). After 42 months, 8/52 patients, including 3 patients with normal HRCT findings at baseline, died due to SSc-related manifestations. Progression of lung fibrosis occurred in 16 patients at month 42, including 7 patients with normal CT at baseline. No clear predictors of progression could be identified. Conclusion In early SSc patients, the disease dynamics differ from the large published cohorts. Progressive lung fibrosis and mortality can also occur in patients without radiological abnormalities at baseline.Key Points• Disease dynamics in early SSc differ from more established SSc.• In early SSc, progressive pulmonary fibrosis can occur in patients without CT abnormalities at baseline.• In early SSc, more stringent pulmonary follow-up is warranted both in lcSSc and dcSSc. High-resolution computed tomography (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Mortality (dpeaa)DE-He213 Pulmonary fibrosis (dpeaa)DE-He213 Systemic sclerosis (dpeaa)DE-He213 Landini, Nicholas verfasserin aut Lenaerts, Joris verfasserin aut Claeys, Eveline verfasserin aut Lenaerts, Jan verfasserin aut Wuyts, Wim A. verfasserin aut Verschakelen, Johny verfasserin aut De Langhe, Ellen verfasserin aut Enthalten in Clinical rheumatology London : Springer, 1982 39(2020), 11 vom: 08. Mai, Seite 3393-3400 (DE-627)27159909X (DE-600)1480901-1 1434-9949 nnns volume:39 year:2020 number:11 day:08 month:05 pages:3393-3400 https://dx.doi.org/10.1007/s10067-020-05105-4 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.00 ASE 44.83 ASE AR 39 2020 11 08 05 3393-3400
spelling	10.1007/s10067-020-05105-4 doi (DE-627)SPR041382544 (SPR)s10067-020-05105-4-e DE-627 ger DE-627 rakwb eng 610 ASE 610 ASE 44.00 bkl 44.83 bkl Vanaken, Lize verfasserin aut Progressive lung fibrosis and mortality can occur in early systemic sclerosis patients without pulmonary abnormalities at baseline assessment 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Objectives In systemic sclerosis, baseline extent of radiological involvement is an important outcome predictor and baseline absence of radiological involvement suggests a more favourable prognosis. As current predictive models are based on cohorts with variable disease duration, we aim to assess disease dynamics in early disease. Methods Patients were included from the prospective longitudinal Belgian Systemic Sclerosis Cohort. We included patients with a disease duration < = 36 months at baseline with available baseline thoracic high-resolution computed tomography (HRCT) images and longitudinal pulmonary function test (PFT) results until 42 months of follow-up. Results Fifty-two patients were included; 50% were male and 44% suffered from diffuse cutaneous systemic sclerosis. A total of 46% carried anti-topoisomerase 1 antibodies. The mean disease duration at baseline visit was 11 months. At baseline visit, 40.4% (21/52) patients had HRCT abnormalities. Patients with abnormal HRCT findings more frequently suffered from diffuse cutaneous systemic sclerosis (p < 0.05) and less frequently carried anti-centromere antibodies (p < 0.05). Patients without CT abnormalities at baseline had a shorter disease duration (9 ± 7 months versus 14 ± 12 months). After 42 months, 8/52 patients, including 3 patients with normal HRCT findings at baseline, died due to SSc-related manifestations. Progression of lung fibrosis occurred in 16 patients at month 42, including 7 patients with normal CT at baseline. No clear predictors of progression could be identified. Conclusion In early SSc patients, the disease dynamics differ from the large published cohorts. Progressive lung fibrosis and mortality can also occur in patients without radiological abnormalities at baseline.Key Points• Disease dynamics in early SSc differ from more established SSc.• In early SSc, progressive pulmonary fibrosis can occur in patients without CT abnormalities at baseline.• In early SSc, more stringent pulmonary follow-up is warranted both in lcSSc and dcSSc. High-resolution computed tomography (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Mortality (dpeaa)DE-He213 Pulmonary fibrosis (dpeaa)DE-He213 Systemic sclerosis (dpeaa)DE-He213 Landini, Nicholas verfasserin aut Lenaerts, Joris verfasserin aut Claeys, Eveline verfasserin aut Lenaerts, Jan verfasserin aut Wuyts, Wim A. verfasserin aut Verschakelen, Johny verfasserin aut De Langhe, Ellen verfasserin aut Enthalten in Clinical rheumatology London : Springer, 1982 39(2020), 11 vom: 08. Mai, Seite 3393-3400 (DE-627)27159909X (DE-600)1480901-1 1434-9949 nnns volume:39 year:2020 number:11 day:08 month:05 pages:3393-3400 https://dx.doi.org/10.1007/s10067-020-05105-4 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.00 ASE 44.83 ASE AR 39 2020 11 08 05 3393-3400
allfields_unstemmed	10.1007/s10067-020-05105-4 doi (DE-627)SPR041382544 (SPR)s10067-020-05105-4-e DE-627 ger DE-627 rakwb eng 610 ASE 610 ASE 44.00 bkl 44.83 bkl Vanaken, Lize verfasserin aut Progressive lung fibrosis and mortality can occur in early systemic sclerosis patients without pulmonary abnormalities at baseline assessment 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Objectives In systemic sclerosis, baseline extent of radiological involvement is an important outcome predictor and baseline absence of radiological involvement suggests a more favourable prognosis. As current predictive models are based on cohorts with variable disease duration, we aim to assess disease dynamics in early disease. Methods Patients were included from the prospective longitudinal Belgian Systemic Sclerosis Cohort. We included patients with a disease duration < = 36 months at baseline with available baseline thoracic high-resolution computed tomography (HRCT) images and longitudinal pulmonary function test (PFT) results until 42 months of follow-up. Results Fifty-two patients were included; 50% were male and 44% suffered from diffuse cutaneous systemic sclerosis. A total of 46% carried anti-topoisomerase 1 antibodies. The mean disease duration at baseline visit was 11 months. At baseline visit, 40.4% (21/52) patients had HRCT abnormalities. Patients with abnormal HRCT findings more frequently suffered from diffuse cutaneous systemic sclerosis (p < 0.05) and less frequently carried anti-centromere antibodies (p < 0.05). Patients without CT abnormalities at baseline had a shorter disease duration (9 ± 7 months versus 14 ± 12 months). After 42 months, 8/52 patients, including 3 patients with normal HRCT findings at baseline, died due to SSc-related manifestations. Progression of lung fibrosis occurred in 16 patients at month 42, including 7 patients with normal CT at baseline. No clear predictors of progression could be identified. Conclusion In early SSc patients, the disease dynamics differ from the large published cohorts. Progressive lung fibrosis and mortality can also occur in patients without radiological abnormalities at baseline.Key Points• Disease dynamics in early SSc differ from more established SSc.• In early SSc, progressive pulmonary fibrosis can occur in patients without CT abnormalities at baseline.• In early SSc, more stringent pulmonary follow-up is warranted both in lcSSc and dcSSc. High-resolution computed tomography (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Mortality (dpeaa)DE-He213 Pulmonary fibrosis (dpeaa)DE-He213 Systemic sclerosis (dpeaa)DE-He213 Landini, Nicholas verfasserin aut Lenaerts, Joris verfasserin aut Claeys, Eveline verfasserin aut Lenaerts, Jan verfasserin aut Wuyts, Wim A. verfasserin aut Verschakelen, Johny verfasserin aut De Langhe, Ellen verfasserin aut Enthalten in Clinical rheumatology London : Springer, 1982 39(2020), 11 vom: 08. Mai, Seite 3393-3400 (DE-627)27159909X (DE-600)1480901-1 1434-9949 nnns volume:39 year:2020 number:11 day:08 month:05 pages:3393-3400 https://dx.doi.org/10.1007/s10067-020-05105-4 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.00 ASE 44.83 ASE AR 39 2020 11 08 05 3393-3400
allfieldsGer	10.1007/s10067-020-05105-4 doi (DE-627)SPR041382544 (SPR)s10067-020-05105-4-e DE-627 ger DE-627 rakwb eng 610 ASE 610 ASE 44.00 bkl 44.83 bkl Vanaken, Lize verfasserin aut Progressive lung fibrosis and mortality can occur in early systemic sclerosis patients without pulmonary abnormalities at baseline assessment 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Objectives In systemic sclerosis, baseline extent of radiological involvement is an important outcome predictor and baseline absence of radiological involvement suggests a more favourable prognosis. As current predictive models are based on cohorts with variable disease duration, we aim to assess disease dynamics in early disease. Methods Patients were included from the prospective longitudinal Belgian Systemic Sclerosis Cohort. We included patients with a disease duration < = 36 months at baseline with available baseline thoracic high-resolution computed tomography (HRCT) images and longitudinal pulmonary function test (PFT) results until 42 months of follow-up. Results Fifty-two patients were included; 50% were male and 44% suffered from diffuse cutaneous systemic sclerosis. A total of 46% carried anti-topoisomerase 1 antibodies. The mean disease duration at baseline visit was 11 months. At baseline visit, 40.4% (21/52) patients had HRCT abnormalities. Patients with abnormal HRCT findings more frequently suffered from diffuse cutaneous systemic sclerosis (p < 0.05) and less frequently carried anti-centromere antibodies (p < 0.05). Patients without CT abnormalities at baseline had a shorter disease duration (9 ± 7 months versus 14 ± 12 months). After 42 months, 8/52 patients, including 3 patients with normal HRCT findings at baseline, died due to SSc-related manifestations. Progression of lung fibrosis occurred in 16 patients at month 42, including 7 patients with normal CT at baseline. No clear predictors of progression could be identified. Conclusion In early SSc patients, the disease dynamics differ from the large published cohorts. Progressive lung fibrosis and mortality can also occur in patients without radiological abnormalities at baseline.Key Points• Disease dynamics in early SSc differ from more established SSc.• In early SSc, progressive pulmonary fibrosis can occur in patients without CT abnormalities at baseline.• In early SSc, more stringent pulmonary follow-up is warranted both in lcSSc and dcSSc. High-resolution computed tomography (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Mortality (dpeaa)DE-He213 Pulmonary fibrosis (dpeaa)DE-He213 Systemic sclerosis (dpeaa)DE-He213 Landini, Nicholas verfasserin aut Lenaerts, Joris verfasserin aut Claeys, Eveline verfasserin aut Lenaerts, Jan verfasserin aut Wuyts, Wim A. verfasserin aut Verschakelen, Johny verfasserin aut De Langhe, Ellen verfasserin aut Enthalten in Clinical rheumatology London : Springer, 1982 39(2020), 11 vom: 08. Mai, Seite 3393-3400 (DE-627)27159909X (DE-600)1480901-1 1434-9949 nnns volume:39 year:2020 number:11 day:08 month:05 pages:3393-3400 https://dx.doi.org/10.1007/s10067-020-05105-4 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.00 ASE 44.83 ASE AR 39 2020 11 08 05 3393-3400
allfieldsSound	10.1007/s10067-020-05105-4 doi (DE-627)SPR041382544 (SPR)s10067-020-05105-4-e DE-627 ger DE-627 rakwb eng 610 ASE 610 ASE 44.00 bkl 44.83 bkl Vanaken, Lize verfasserin aut Progressive lung fibrosis and mortality can occur in early systemic sclerosis patients without pulmonary abnormalities at baseline assessment 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Objectives In systemic sclerosis, baseline extent of radiological involvement is an important outcome predictor and baseline absence of radiological involvement suggests a more favourable prognosis. As current predictive models are based on cohorts with variable disease duration, we aim to assess disease dynamics in early disease. Methods Patients were included from the prospective longitudinal Belgian Systemic Sclerosis Cohort. We included patients with a disease duration < = 36 months at baseline with available baseline thoracic high-resolution computed tomography (HRCT) images and longitudinal pulmonary function test (PFT) results until 42 months of follow-up. Results Fifty-two patients were included; 50% were male and 44% suffered from diffuse cutaneous systemic sclerosis. A total of 46% carried anti-topoisomerase 1 antibodies. The mean disease duration at baseline visit was 11 months. At baseline visit, 40.4% (21/52) patients had HRCT abnormalities. Patients with abnormal HRCT findings more frequently suffered from diffuse cutaneous systemic sclerosis (p < 0.05) and less frequently carried anti-centromere antibodies (p < 0.05). Patients without CT abnormalities at baseline had a shorter disease duration (9 ± 7 months versus 14 ± 12 months). After 42 months, 8/52 patients, including 3 patients with normal HRCT findings at baseline, died due to SSc-related manifestations. Progression of lung fibrosis occurred in 16 patients at month 42, including 7 patients with normal CT at baseline. No clear predictors of progression could be identified. Conclusion In early SSc patients, the disease dynamics differ from the large published cohorts. Progressive lung fibrosis and mortality can also occur in patients without radiological abnormalities at baseline.Key Points• Disease dynamics in early SSc differ from more established SSc.• In early SSc, progressive pulmonary fibrosis can occur in patients without CT abnormalities at baseline.• In early SSc, more stringent pulmonary follow-up is warranted both in lcSSc and dcSSc. High-resolution computed tomography (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Mortality (dpeaa)DE-He213 Pulmonary fibrosis (dpeaa)DE-He213 Systemic sclerosis (dpeaa)DE-He213 Landini, Nicholas verfasserin aut Lenaerts, Joris verfasserin aut Claeys, Eveline verfasserin aut Lenaerts, Jan verfasserin aut Wuyts, Wim A. verfasserin aut Verschakelen, Johny verfasserin aut De Langhe, Ellen verfasserin aut Enthalten in Clinical rheumatology London : Springer, 1982 39(2020), 11 vom: 08. Mai, Seite 3393-3400 (DE-627)27159909X (DE-600)1480901-1 1434-9949 nnns volume:39 year:2020 number:11 day:08 month:05 pages:3393-3400 https://dx.doi.org/10.1007/s10067-020-05105-4 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_267 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_711 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 44.00 ASE 44.83 ASE AR 39 2020 11 08 05 3393-3400
language	English
source	Enthalten in Clinical rheumatology 39(2020), 11 vom: 08. Mai, Seite 3393-3400 volume:39 year:2020 number:11 day:08 month:05 pages:3393-3400
sourceStr	Enthalten in Clinical rheumatology 39(2020), 11 vom: 08. Mai, Seite 3393-3400 volume:39 year:2020 number:11 day:08 month:05 pages:3393-3400
format_phy_str_mv	Article
institution	findex.gbv.de
topic_facet	High-resolution computed tomography Interstitial lung disease Mortality Pulmonary fibrosis Systemic sclerosis
dewey-raw	610
isfreeaccess_bool	false
container_title	Clinical rheumatology
authorswithroles_txt_mv	Vanaken, Lize @@aut@@ Landini, Nicholas @@aut@@ Lenaerts, Joris @@aut@@ Claeys, Eveline @@aut@@ Lenaerts, Jan @@aut@@ Wuyts, Wim A. @@aut@@ Verschakelen, Johny @@aut@@ De Langhe, Ellen @@aut@@
publishDateDaySort_date	2020-05-08T00:00:00Z
hierarchy_top_id	27159909X
dewey-sort	3610
id	SPR041382544
language_de	englisch
fullrecord	<?xml version="1.0" encoding="UTF-8"?><collection xmlns="http://www.loc.gov/MARC21/slim"><record><leader>01000caa a22002652 4500</leader><controlfield tag="001">SPR041382544</controlfield><controlfield tag="003">DE-627</controlfield><controlfield tag="005">20230520005739.0</controlfield><controlfield tag="007">cr uuu---uuuuu</controlfield><controlfield tag="008">201102s2020 xx \|\|\|\|\|o 00\| \|\|eng c</controlfield><datafield tag="024" ind1="7" ind2=" "><subfield code="a">10.1007/s10067-020-05105-4</subfield><subfield code="2">doi</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(DE-627)SPR041382544</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(SPR)s10067-020-05105-4-e</subfield></datafield><datafield tag="040" ind1=" " ind2=" "><subfield code="a">DE-627</subfield><subfield code="b">ger</subfield><subfield code="c">DE-627</subfield><subfield code="e">rakwb</subfield></datafield><datafield tag="041" ind1=" " ind2=" "><subfield code="a">eng</subfield></datafield><datafield tag="082" ind1="0" ind2="4"><subfield code="a">610</subfield><subfield code="q">ASE</subfield></datafield><datafield tag="082" ind1="0" ind2="4"><subfield code="a">610</subfield><subfield code="q">ASE</subfield></datafield><datafield tag="084" ind1=" " ind2=" "><subfield code="a">44.00</subfield><subfield code="2">bkl</subfield></datafield><datafield tag="084" ind1=" " ind2=" "><subfield code="a">44.83</subfield><subfield code="2">bkl</subfield></datafield><datafield tag="100" ind1="1" ind2=" "><subfield code="a">Vanaken, Lize</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="245" ind1="1" ind2="0"><subfield code="a">Progressive lung fibrosis and mortality can occur in early systemic sclerosis patients without pulmonary abnormalities at baseline assessment</subfield></datafield><datafield tag="264" ind1=" " ind2="1"><subfield code="c">2020</subfield></datafield><datafield tag="336" ind1=" " ind2=" "><subfield code="a">Text</subfield><subfield code="b">txt</subfield><subfield code="2">rdacontent</subfield></datafield><datafield tag="337" ind1=" " ind2=" "><subfield code="a">Computermedien</subfield><subfield code="b">c</subfield><subfield code="2">rdamedia</subfield></datafield><datafield tag="338" ind1=" " ind2=" "><subfield code="a">Online-Ressource</subfield><subfield code="b">cr</subfield><subfield code="2">rdacarrier</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">Objectives In systemic sclerosis, baseline extent of radiological involvement is an important outcome predictor and baseline absence of radiological involvement suggests a more favourable prognosis. As current predictive models are based on cohorts with variable disease duration, we aim to assess disease dynamics in early disease. Methods Patients were included from the prospective longitudinal Belgian Systemic Sclerosis Cohort. We included patients with a disease duration < = 36 months at baseline with available baseline thoracic high-resolution computed tomography (HRCT) images and longitudinal pulmonary function test (PFT) results until 42 months of follow-up. Results Fifty-two patients were included; 50% were male and 44% suffered from diffuse cutaneous systemic sclerosis. A total of 46% carried anti-topoisomerase 1 antibodies. The mean disease duration at baseline visit was 11 months. At baseline visit, 40.4% (21/52) patients had HRCT abnormalities. Patients with abnormal HRCT findings more frequently suffered from diffuse cutaneous systemic sclerosis (p < 0.05) and less frequently carried anti-centromere antibodies (p < 0.05). Patients without CT abnormalities at baseline had a shorter disease duration (9 ± 7 months versus 14 ± 12 months). After 42 months, 8/52 patients, including 3 patients with normal HRCT findings at baseline, died due to SSc-related manifestations. Progression of lung fibrosis occurred in 16 patients at month 42, including 7 patients with normal CT at baseline. No clear predictors of progression could be identified. Conclusion In early SSc patients, the disease dynamics differ from the large published cohorts. Progressive lung fibrosis and mortality can also occur in patients without radiological abnormalities at baseline.Key Points• Disease dynamics in early SSc differ from more established SSc.• In early SSc, progressive pulmonary fibrosis can occur in patients without CT abnormalities at baseline.• In early SSc, more stringent pulmonary follow-up is warranted both in lcSSc and dcSSc.</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">High-resolution computed tomography</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Interstitial lung disease</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Mortality</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Pulmonary fibrosis</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">Systemic sclerosis</subfield><subfield code="7">(dpeaa)DE-He213</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Landini, Nicholas</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Lenaerts, Joris</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Claeys, Eveline</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Lenaerts, Jan</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Wuyts, Wim A.</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Verschakelen, Johny</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">De Langhe, Ellen</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="773" ind1="0" ind2="8"><subfield code="i">Enthalten in</subfield><subfield code="t">Clinical rheumatology</subfield><subfield code="d">London : Springer, 1982</subfield><subfield code="g">39(2020), 11 vom: 08. 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author	Vanaken, Lize
spellingShingle	Vanaken, Lize ddc 610 bkl 44.00 bkl 44.83 misc High-resolution computed tomography misc Interstitial lung disease misc Mortality misc Pulmonary fibrosis misc Systemic sclerosis Progressive lung fibrosis and mortality can occur in early systemic sclerosis patients without pulmonary abnormalities at baseline assessment
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topic_title	610 ASE 44.00 bkl 44.83 bkl Progressive lung fibrosis and mortality can occur in early systemic sclerosis patients without pulmonary abnormalities at baseline assessment High-resolution computed tomography (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Mortality (dpeaa)DE-He213 Pulmonary fibrosis (dpeaa)DE-He213 Systemic sclerosis (dpeaa)DE-He213
topic	ddc 610 bkl 44.00 bkl 44.83 misc High-resolution computed tomography misc Interstitial lung disease misc Mortality misc Pulmonary fibrosis misc Systemic sclerosis
topic_unstemmed	ddc 610 bkl 44.00 bkl 44.83 misc High-resolution computed tomography misc Interstitial lung disease misc Mortality misc Pulmonary fibrosis misc Systemic sclerosis
topic_browse	ddc 610 bkl 44.00 bkl 44.83 misc High-resolution computed tomography misc Interstitial lung disease misc Mortality misc Pulmonary fibrosis misc Systemic sclerosis
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title	Progressive lung fibrosis and mortality can occur in early systemic sclerosis patients without pulmonary abnormalities at baseline assessment
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title_full	Progressive lung fibrosis and mortality can occur in early systemic sclerosis patients without pulmonary abnormalities at baseline assessment
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journal	Clinical rheumatology
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author_browse	Vanaken, Lize Landini, Nicholas Lenaerts, Joris Claeys, Eveline Lenaerts, Jan Wuyts, Wim A. Verschakelen, Johny De Langhe, Ellen
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title_sort	progressive lung fibrosis and mortality can occur in early systemic sclerosis patients without pulmonary abnormalities at baseline assessment
title_auth	Progressive lung fibrosis and mortality can occur in early systemic sclerosis patients without pulmonary abnormalities at baseline assessment
abstract	Objectives In systemic sclerosis, baseline extent of radiological involvement is an important outcome predictor and baseline absence of radiological involvement suggests a more favourable prognosis. As current predictive models are based on cohorts with variable disease duration, we aim to assess disease dynamics in early disease. Methods Patients were included from the prospective longitudinal Belgian Systemic Sclerosis Cohort. We included patients with a disease duration < = 36 months at baseline with available baseline thoracic high-resolution computed tomography (HRCT) images and longitudinal pulmonary function test (PFT) results until 42 months of follow-up. Results Fifty-two patients were included; 50% were male and 44% suffered from diffuse cutaneous systemic sclerosis. A total of 46% carried anti-topoisomerase 1 antibodies. The mean disease duration at baseline visit was 11 months. At baseline visit, 40.4% (21/52) patients had HRCT abnormalities. Patients with abnormal HRCT findings more frequently suffered from diffuse cutaneous systemic sclerosis (p < 0.05) and less frequently carried anti-centromere antibodies (p < 0.05). Patients without CT abnormalities at baseline had a shorter disease duration (9 ± 7 months versus 14 ± 12 months). After 42 months, 8/52 patients, including 3 patients with normal HRCT findings at baseline, died due to SSc-related manifestations. Progression of lung fibrosis occurred in 16 patients at month 42, including 7 patients with normal CT at baseline. No clear predictors of progression could be identified. Conclusion In early SSc patients, the disease dynamics differ from the large published cohorts. Progressive lung fibrosis and mortality can also occur in patients without radiological abnormalities at baseline.Key Points• Disease dynamics in early SSc differ from more established SSc.• In early SSc, progressive pulmonary fibrosis can occur in patients without CT abnormalities at baseline.• In early SSc, more stringent pulmonary follow-up is warranted both in lcSSc and dcSSc.
abstractGer	Objectives In systemic sclerosis, baseline extent of radiological involvement is an important outcome predictor and baseline absence of radiological involvement suggests a more favourable prognosis. As current predictive models are based on cohorts with variable disease duration, we aim to assess disease dynamics in early disease. Methods Patients were included from the prospective longitudinal Belgian Systemic Sclerosis Cohort. We included patients with a disease duration < = 36 months at baseline with available baseline thoracic high-resolution computed tomography (HRCT) images and longitudinal pulmonary function test (PFT) results until 42 months of follow-up. Results Fifty-two patients were included; 50% were male and 44% suffered from diffuse cutaneous systemic sclerosis. A total of 46% carried anti-topoisomerase 1 antibodies. The mean disease duration at baseline visit was 11 months. At baseline visit, 40.4% (21/52) patients had HRCT abnormalities. Patients with abnormal HRCT findings more frequently suffered from diffuse cutaneous systemic sclerosis (p < 0.05) and less frequently carried anti-centromere antibodies (p < 0.05). Patients without CT abnormalities at baseline had a shorter disease duration (9 ± 7 months versus 14 ± 12 months). After 42 months, 8/52 patients, including 3 patients with normal HRCT findings at baseline, died due to SSc-related manifestations. Progression of lung fibrosis occurred in 16 patients at month 42, including 7 patients with normal CT at baseline. No clear predictors of progression could be identified. Conclusion In early SSc patients, the disease dynamics differ from the large published cohorts. Progressive lung fibrosis and mortality can also occur in patients without radiological abnormalities at baseline.Key Points• Disease dynamics in early SSc differ from more established SSc.• In early SSc, progressive pulmonary fibrosis can occur in patients without CT abnormalities at baseline.• In early SSc, more stringent pulmonary follow-up is warranted both in lcSSc and dcSSc.
abstract_unstemmed	Objectives In systemic sclerosis, baseline extent of radiological involvement is an important outcome predictor and baseline absence of radiological involvement suggests a more favourable prognosis. As current predictive models are based on cohorts with variable disease duration, we aim to assess disease dynamics in early disease. Methods Patients were included from the prospective longitudinal Belgian Systemic Sclerosis Cohort. We included patients with a disease duration < = 36 months at baseline with available baseline thoracic high-resolution computed tomography (HRCT) images and longitudinal pulmonary function test (PFT) results until 42 months of follow-up. Results Fifty-two patients were included; 50% were male and 44% suffered from diffuse cutaneous systemic sclerosis. A total of 46% carried anti-topoisomerase 1 antibodies. The mean disease duration at baseline visit was 11 months. At baseline visit, 40.4% (21/52) patients had HRCT abnormalities. Patients with abnormal HRCT findings more frequently suffered from diffuse cutaneous systemic sclerosis (p < 0.05) and less frequently carried anti-centromere antibodies (p < 0.05). Patients without CT abnormalities at baseline had a shorter disease duration (9 ± 7 months versus 14 ± 12 months). After 42 months, 8/52 patients, including 3 patients with normal HRCT findings at baseline, died due to SSc-related manifestations. Progression of lung fibrosis occurred in 16 patients at month 42, including 7 patients with normal CT at baseline. No clear predictors of progression could be identified. Conclusion In early SSc patients, the disease dynamics differ from the large published cohorts. Progressive lung fibrosis and mortality can also occur in patients without radiological abnormalities at baseline.Key Points• Disease dynamics in early SSc differ from more established SSc.• In early SSc, progressive pulmonary fibrosis can occur in patients without CT abnormalities at baseline.• In early SSc, more stringent pulmonary follow-up is warranted both in lcSSc and dcSSc.
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container_issue	11
title_short	Progressive lung fibrosis and mortality can occur in early systemic sclerosis patients without pulmonary abnormalities at baseline assessment
url	https://dx.doi.org/10.1007/s10067-020-05105-4
remote_bool	true
author2	Landini, Nicholas Lenaerts, Joris Claeys, Eveline Lenaerts, Jan Wuyts, Wim A. Verschakelen, Johny De Langhe, Ellen
author2Str	Landini, Nicholas Lenaerts, Joris Claeys, Eveline Lenaerts, Jan Wuyts, Wim A. Verschakelen, Johny De Langhe, Ellen
ppnlink	27159909X
mediatype_str_mv	c
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hochschulschrift_bool	false
doi_str	10.1007/s10067-020-05105-4
up_date	2024-07-03T21:50:53.779Z
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Progressive lung fibrosis and mortality can occur in early systemic sclerosis patients without pulmonary abnormalities at baseline assessment

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