Journal ArticleDOI
Assessment of Mortality in Autoimmune Myositis With and Without Associated Interstitial Lung Disease.
Cheilonda Johnson,Iago Pinal-Fernandez,Radhika Parikh,Julie Paik,Jemima Albayda,Andrew L. Mammen,Andrew L. Mammen,Lisa Christopher-Stine,Sonye K. Danoff +8 more
TLDR
The results suggest that ILD remains an important and significant source of mortality in patients with inflammatory myopathies and as such should be screened for and treated aggressively.Abstract:
Among patients with autoimmune myositis, associated interstitial lung disease (MA-ILD) is a known contributor of excess morbidity and mortality. Recent data on survival in idiopathic inflammatory myopathies originate primarily in Asia and Europe and vary widely. We sought to examine mortality in a large U.S. myositis cohort focusing in particular on the impact of associated ILD. A cross-sectional analysis of participants from the Johns Hopkins Myositis Center with autoimmune myositis (polymyositis [PM], dermatomyositis [DM], or clinically amyopathic dermatomyositis [CADM]) was conducted. The primary outcome assessed was all-cause mortality. Cumulative mortality rates were estimated using the Kaplan–Meier test; the Cox proportional hazards model was used to compare group differences in survival. Eight hundred and thirty-one participants were included with a median follow-up time of 4.5 years. Four hundred thirty-eight (53 %) had PM, 362 (43 %) had DM, and 31 (4 %) had CADM. Ninety-four (11 %) participants had clinically evident ILD. Overall, 51 participants died (6 %). In those without ILD, the survival rates at 1, 5, and 10 years were 99, 95, and 90 %, respectively. In those with ILD, the survival rates at 1, 5, and 10 years were 97, 91, and 81 %, respectively. The risk of death was statistically significantly higher among participants with ILD compared to those without ILD (HR 2.13. 95 % CI 1.06–4.25; p = 0.03). We analyzed one of the largest known cohorts of patients with autoimmune myositis and found significantly higher mortality rates among those with clinically evident ILD compared to those without clinically evident ILD. Our results suggest that ILD remains an important and significant source of mortality in patients with inflammatory myopathies and as such should be screened for and treated aggressively.read more
Citations
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Journal ArticleDOI
Autoantibodies in myositis
Neil McHugh,Sarah L Tansley +1 more
TL;DR: Evidence suggests that the alteration of expression of a myositis-related autoantigen by certain environmental influences or oncogenesis could be a pivotal event linking autoantibody generation to the development of disease.
Journal ArticleDOI
A longitudinal cohort study of the anti-synthetase syndrome: increased severity of interstitial lung disease in black patients and patients with anti-PL7 and anti-PL12 autoantibodies.
Iago Pinal-Fernandez,Maria Casal-Dominguez,Julio Huapaya,Jemima Albayda,Julie J. Paik,Cheilonda Johnson,Leann Silhan,Lisa Christopher-Stine,Andrew L. Mammen,Sonye K. Danoff +9 more
TL;DR: Different AsyS autoantibodies are associated with phenotypically distinct subgroups within the ASyS spectrum, characterized by more severe lung involvement, whereas anti-Jo1 is associated with more severe muscle involvement.
Journal ArticleDOI
Initial predictors of poor survival in myositis-associated interstitial lung disease: a multicentre cohort of 497 patients
Shinji Sato,Kenichi Masui,Naoshi Nishina,Yasushi Kawaguchi,Atsushi Kawakami,Maasa Tamura,Kei Ikeda,Takahiro Nunokawa,Yoshinori Tanino,Katsuaki Asakawa,Yuko Kaneko,Takahisa Gono,Takahisa Gono,Taro Ukichi,Shinjiro Kaieda,Taio Naniwa,Masataka Kuwana,Jami investigators +17 more
TL;DR: A large cohort of incident cases of PM/DM-associated ILD was established, and independent predictors of short-term ILD mortality were identified by univariate and multivariate analyses.
Journal ArticleDOI
Mortality in idiopathic inflammatory myopathy: results from a Swedish nationwide population-based cohort study
TL;DR: Mortality is increased in patients with contemporary IIM, which calls for extra vigilance during the first year of IIM diagnosis, and increased in both IIM and the general population with time.
Journal ArticleDOI
Interstitial Lung Disease in Polymyositis and Dermatomyositis.
Kathryn Long,Sonye K. Danoff +1 more
TL;DR: The idiopathic inflammatory myopathies, including polymyositis and dermatomyositis, are autoimmune connective tissue diseases with variable degrees of muscle inflammation and systemic involvement and the addition of other immunosuppressive therapy is typically necessary to achieve disease control.
References
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Journal ArticleDOI
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General considerations for lung function testing.
Martin R. Miller,Robert O. Crapo,John L. Hankinson,Vito Brusasco,Felip Burgos,Richard Casaburi,Allan L. Coates,Paul L. Enright,C.P.M. van der Grinten,P. Gustafsson,R. Jensen,D.C. Johnson,Neil R. MacIntyre,Roy T. McKay,Daniel Navajas,O. F. Pedersen,Riccardo Pellegrino,Giovanni Viegi,J. Wanger +18 more
TL;DR: This statement contains details about procedures that are common for many methods of lung function testing and, hence, are presented on their own and represent a change towards bringing this document in line with the ISO.
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