BACKGROUND AND OBJECTIVE: Interstitial lung disease (ILD) is a common pulmonary manifestation of systemic sclerosis (SSc). It is unknown whether radiographic fibrosis score predicts mortality in SSc-associated ILD (SSc-ILD). We retrospectively analysed patients with SSc-ILD to evaluate whether radiographic fibrosis score was a useful predictor of mortality. METHODS: We identified SSc-ILD patients evaluated at Kurashiki Central Hospital (Japan) from 2006 to 2016, and radiographic fibrosis scores based on the extent of reticulation and honeycombing on high-resolution computed tomography (HRCT) scanning were calculated by manually tracing around each fibrotic area. Independent predictors of overall survival were determined using the Cox proportional hazards model. RESULTS: The study included 48 patients, of whom 19 had usual interstitial pneumonia on HRCT. The median follow-up period was 56.6 months, and over the follow-up period 15 patients died. The 5-year survival was 72.4%. In the multivariate analysis, radiographic fibrosis score, age, being male and forced vital capacity were independently associated with an increased risk of death, while HRCT pattern was not. CONCLUSION: A high radiographic fibrosis score was a poor prognostic factor in SSc-ILD. More widespread fibrosis was associated with an increased risk of death, independent of HRCT pattern.
BACKGROUND AND OBJECTIVE:Interstitial lung disease (ILD) is a common pulmonary manifestation of systemic sclerosis (SSc). It is unknown whether radiographic fibrosis score predicts mortality in SSc-associated ILD (SSc-ILD). We retrospectively analysed patients with SSc-ILD to evaluate whether radiographic fibrosis score was a useful predictor of mortality. METHODS: We identified SSc-ILD patients evaluated at Kurashiki Central Hospital (Japan) from 2006 to 2016, and radiographic fibrosis scores based on the extent of reticulation and honeycombing on high-resolution computed tomography (HRCT) scanning were calculated by manually tracing around each fibrotic area. Independent predictors of overall survival were determined using the Cox proportional hazards model. RESULTS: The study included 48 patients, of whom 19 had usual interstitial pneumonia on HRCT. The median follow-up period was 56.6 months, and over the follow-up period 15 patients died. The 5-year survival was 72.4%. In the multivariate analysis, radiographic fibrosis score, age, being male and forced vital capacity were independently associated with an increased risk of death, while HRCT pattern was not. CONCLUSION: A high radiographic fibrosis score was a poor prognostic factor in SSc-ILD. More widespread fibrosis was associated with an increased risk of death, independent of HRCT pattern.
Authors: Nicholas Landini; Martina Orlandi; Cosimo Bruni; Edoardo Carlesi; Cosimo Nardi; Linda Calistri; Giovanni Morana; Sara Tomassetti; Stefano Colagrande; Marco Matucci-Cerinic Journal: Front Med (Lausanne) Date: 2022-01-27
Authors: Minna Mononen; Eeva Saari; Hannele Hasala; Hannu-Pekka Kettunen; Sanna Suoranta; Hanna Nurmi; Miia Kärkkäinen; Tuomas Selander; Jukka Randell; Jari Laurikka; Toomas Uibu; Heikki Koskela; Riitta Kaarteenaho; Minna Purokivi Journal: BMC Pulm Med Date: 2022-08-14 Impact factor: 3.320
Authors: Dinesh Khanna; Alain Lescoat; David Roofeh; Elana J Bernstein; Ella A Kazerooni; Michael D Roth; Fernando Martinez; Kevin R Flaherty; Christopher P Denton Journal: Arthritis Rheumatol Date: 2021-11-10 Impact factor: 15.483