Clinical features and outcomes of interstitial lung disease in anti-Jo-1 positive antisynthetase syndrome

Zamora, Ana; Hoskote, Sumedh; Abascal-Bolado, Beatriz; White, Darin; Cox, Christian W.; Ryu, Jay H.; Moua, Teng

doi:10.1016/j.rmed.2016.07.009

Cited by 80 publications

(80 citation statements)

References 27 publications

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“…In other words, even in ARS-ILD patients showing a good response to initial therapy, pulmonary function frequently deteriorated during long-term follow-up. In terms of survival, only 2 patients died within 5 years from the ARS-ILD diagnosis and the 5-year survival rate was 97.0%, which indicates relatively good survival of ARS-ILD patients, similar to that reported in previous studies [6, 7]. Importantly, some patients with ARS-ILD suffered from refractory ILD over the long run, which led to a number of adverse events such as the necessity of home oxygen therapy and side effects related to the inability to reduce the dose of corticosteroids (e.g., muscle weakness, opportunistic infection, and diabetes mellitus).…”

Section: Discussionsupporting

confidence: 87%

“…The HRCT patterns were classified into nonspecific interstitial pneumonia (NSIP), organizing pneumonia (OP), usual interstitial pneumonia, and NSIP with OP overlap according to previous reports [6, 15]. NSIP with OP overlap was identified when consolidations were superimposed on a background of ground-glass opacity (GGO) with or without reticulations or traction bronchiectasis [6].…”

Section: Methodsmentioning

confidence: 99%

“…Previous reports have indicated that the clinical characteristics are similar in PM/DM-associated ILD and in ARS-ILD without PM/DM [4, 5]. Although some patients with rapidly progressive ARS-ILD have a poor prognosis in the short run, their overall prognosis is relatively good [6, 7]. However, because of frequent recurrence and ARS-ILD refractory to therapy, some patients progress to pulmonary fibrosis over the long run despite initial improvement [8-10].…”

Section: Introductionmentioning

confidence: 99%

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Predictive Factors for the Long-Term Deterioration of Pulmonary Function in Interstitial Lung Disease Associated with Anti-Aminoacyl-tRNA Synthetase Antibodies

et al. 2018

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Background: Little has been reported on long-term pulmonary function trends among patients with interstitial lung disease associated with anti-aminoacyl-tRNA synthetase antibodies (ARS-ILD). Objectives: To clarify the factors predictive of progression in ARS-ILD based on patients’ initial clinical and radiological features. Methods: The clinical courses of 88 patients with > 1 year of follow-up data on pulmonary function tests (PFTs) were retrospectively analyzed. Disease behavior was categorized into three groups: (1) improved or (2) worsened (defined as increases or decreases, respectively, of > 10% in forced vital capacity and > 15% in %diffusing capacity of lung carbon monoxide) or (3) stable based on PFT changes compared between 1-year results as the initial data and results at 3 years to assess the long-term course. Results: In the initial course of 75 patients with ARS-ILD who received anti-inflammatory therapy within 6 months after diagnosis, 48 patients (64.0%) improved and 6 patients (8.0%) worsened. The radiological patterns in the patients with ARS-ILD included nonspecific interstitial pneumonia (NSIP) in 46.7% and NSIP with organizing pneumonia overlap in 52.0% of the cases. One-third of the initially improved patients who worsened over the long-term course were assigned to the unstable group. By multivariate logistic analysis, middle lobe traction bronchiectasis was a significant predictive factor for the patients in the unstable group. Conclusions: Most patients with ARS-ILD receiving anti-inflammatory therapy had improved or remained stable in the first year. However, over the long-term course, some patients worsened despite their initial improvement. Even though the extent of the disease is limited, middle lobe traction bronchiectasis in ARS-ILD may be a useful predictor of poor long-term disease behavior.

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Section: Discussionsupporting

confidence: 87%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Predictive Factors for the Long-Term Deterioration of Pulmonary Function in Interstitial Lung Disease Associated with Anti-Aminoacyl-tRNA Synthetase Antibodies

et al. 2018

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“…Interstitial lung disease is a common extra-muscular complication of idiopathic inflammatory myopathies, and low-diffusing capacity for carbon monoxide (DLCO) is known to increase the risk of mortality [1, 2]. A recent study demonstrated that recovery of skeletal muscle strength and reduced creatine kinase levels by medication for idiopathic inflammatory myopathies may improve forced vital capacity (FVC) due to muscular strength reinforcement; however, DLCO and interstitial infiltrates on CT were not ameliorated in most cases [3].…”

Section: Introductionmentioning

confidence: 99%

Cardiopulmonary factors affecting 6-min walk distance in patients with idiopathic inflammatory myopathies

Mugii

Someya

2018

Rheumatol Int

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Idiopathic inflammatory myopathies involve skeletal muscles and can be associated with interstitial lung disease and/or heart dysfunction, which may reduce exercise capacity. We aimed to clarify cardiopulmonary factors affecting the 6-min walk distance in patients who were able to walk without leg pain or fatigue. Twenty-three patients with inactive adult idiopathic inflammatory myopathies, and 18 age- and gender-matched healthy controls were evaluated for hemodynamic responses using noninvasive impedance cardiography during the 6-min walk test. The patients were also examined by the pulmonary function test for forced vital capacity and diffusing capacity for carbon monoxide (DLCO), and by echocardiography for left ventricular ejection fraction and right ventricular systolic pressure. Interstitial lung disease was diagnosed in 19 patients using high-resolution computed tomography. There was no difference in 6-min walk distance or cardiac output after walking between the patients and healthy controls. However, stroke volume during the 6-min walk test was significantly lower in the patients than in healthy controls, suggesting malfunction in the heart. Moreover, the increased heart rate matched the cardiac output. Spearman’s correlation analysis demonstrated a correlation between 6-min walk distance and stroke volume, cardiac output after walking and DLCO, but not left ventricular ejection fraction or right ventricular systolic pressure, as this study lacked the patients with pulmonary hypertension. In conclusion, impaired DLCO due to interstitial lung disease was suggested to be a fundamental parameter affecting exercise capacity, in addition to heart involvement, in patients with idiopathic inflammatory myopathies.

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“…Questionnaires for chronic lung disease have been developed to evaluate the symptoms and the Chronic obstructive pulmonary disease Assessment Test (CAT) [1] has been adapted for interstitial lung disease [2]. Cross-sectional studies reported the relationship between the CAT score and the results of pulmonary function tests for interstitial lung disease, and found that higher CAT scores and/ or lower diffusion capacity of the lungs for carbon monoxide (D LCO ) significantly predicted mortality [3,4].…”

Section: Introductionmentioning

confidence: 99%

Characteristics for Quality of Life during the Clinical Course of Interstitial Lung Disease

Someya¹,

Nakagawa²,

Mugii³

2017

J Pulm Respir Med

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Clinical features and outcomes of interstitial lung disease in anti-Jo-1 positive antisynthetase syndrome

Abstract: We present a large cohort of anti-Jo-1 positive AS syndrome with ILD and note good overall survival.

Cited by 80 publications

References 27 publications

Predictive Factors for the Long-Term Deterioration of Pulmonary Function in Interstitial Lung Disease Associated with Anti-Aminoacyl-tRNA Synthetase Antibodies

Predictive Factors for the Long-Term Deterioration of Pulmonary Function in Interstitial Lung Disease Associated with Anti-Aminoacyl-tRNA Synthetase Antibodies

Cardiopulmonary factors affecting 6-min walk distance in patients with idiopathic inflammatory myopathies

Characteristics for Quality of Life during the Clinical Course of Interstitial Lung Disease

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