Elisabeth Bendstrup

Clinical characteristics and outcome in patients with antisynthetase syndrome associated interstitial lung disease: a retrospective cohort study

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Clinical characteristics and outcome in patients with antisynthetase syndrome associated interstitial lung disease : a retrospective cohort study. / Jensen, Mads Lynge; Løkke, Anders; Hilberg, Ole; Hyldgaard, Charlotte; Bendstrup, Elisabeth; Tran, Dan.

In: European Clinical Respiratory Journal, Vol. 6, No. 1, 1583516, 2019.

Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaperJournal articleResearchpeer-review

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Jensen, Mads Lynge ; Løkke, Anders ; Hilberg, Ole ; Hyldgaard, Charlotte ; Bendstrup, Elisabeth ; Tran, Dan. / Clinical characteristics and outcome in patients with antisynthetase syndrome associated interstitial lung disease : a retrospective cohort study. In: European Clinical Respiratory Journal. 2019 ; Vol. 6, No. 1.

Bibtex

@article{0b6793b7080c4593b3b37e93211e71fc,
title = "Clinical characteristics and outcome in patients with antisynthetase syndrome associated interstitial lung disease: a retrospective cohort study",
abstract = "Background and objective: To describe the clinical characteristics including the bronchoalveolar lavage fluid (BALF) characteristics of patients with antisynthetase syndrome (AS) associated interstitial lung disease (ILD) in a tertiary ILD outpatient clinic, their medical therapy and outcome. Methods: Retrospective cohort study of patients with AS-ILD. All available data of clinical characteristics, pulmonary function tests, laboratory parameters, BALF analysis, histology, high-resolution computed tomography (HRCT) and treatment were collected from the patient files. Results and conclusions: Twelve patients with AS-ILD were identified. Mean age at diagnosis was 55 years (range 45-69), 67% were female. Mean follow-up time was 7 years. The anti-aminoacyl tRNA-synthetase antibodies presented were anti-Jo1 (n = 6), anti-PL7 (n = 3), anti-PL12 (n = 2) and anti-EJ (n = 1). HRCT patterns were mainly non-specific interstitial pneumonia (75%). Four patients had BALF-eosinophilia (two of four anti-Jo1 patients) and two anti-PL12 positive patients had BALF-neutrophilia. Two AS-ILD patients improved on rituximab (RTX) as induction treatment and three out of four patients were stabilised on RTX as maintenance treatment. Two patient obtained remission by cyclophosphamide. Four patients were stabilised on azathioprine alone or in combination with oral corticosteroids. Our cohort of AS-ILD patients showed clinical characteristics in accordance with previous reports at baseline and was comparable to other cohorts. Most patients can be stabilised with immunosuppressive treatment.",
keywords = "ANTIBODIES, ARTHRITIS, Antisynthetase syndrome, DERMATOMYOSITIS, POLYMYOSITIS, RITUXIMAB, SEVERITY, bronchoalveolar lavage fluid, interstitial lung diseases, polymyositis, treatments",
author = "Jensen, {Mads Lynge} and Anders L{\o}kke and Ole Hilberg and Charlotte Hyldgaard and Elisabeth Bendstrup and Dan Tran",
year = "2019",
doi = "10.1080/20018525.2019.1583516",
language = "English",
volume = "6",
journal = "European Clinical Respiratory Journal",
issn = "2001-8525",
publisher = "Taylor & Francis",
number = "1",

}

RIS

TY - JOUR

T1 - Clinical characteristics and outcome in patients with antisynthetase syndrome associated interstitial lung disease

T2 - a retrospective cohort study

AU - Jensen, Mads Lynge

AU - Løkke, Anders

AU - Hilberg, Ole

AU - Hyldgaard, Charlotte

AU - Bendstrup, Elisabeth

AU - Tran, Dan

PY - 2019

Y1 - 2019

N2 - Background and objective: To describe the clinical characteristics including the bronchoalveolar lavage fluid (BALF) characteristics of patients with antisynthetase syndrome (AS) associated interstitial lung disease (ILD) in a tertiary ILD outpatient clinic, their medical therapy and outcome. Methods: Retrospective cohort study of patients with AS-ILD. All available data of clinical characteristics, pulmonary function tests, laboratory parameters, BALF analysis, histology, high-resolution computed tomography (HRCT) and treatment were collected from the patient files. Results and conclusions: Twelve patients with AS-ILD were identified. Mean age at diagnosis was 55 years (range 45-69), 67% were female. Mean follow-up time was 7 years. The anti-aminoacyl tRNA-synthetase antibodies presented were anti-Jo1 (n = 6), anti-PL7 (n = 3), anti-PL12 (n = 2) and anti-EJ (n = 1). HRCT patterns were mainly non-specific interstitial pneumonia (75%). Four patients had BALF-eosinophilia (two of four anti-Jo1 patients) and two anti-PL12 positive patients had BALF-neutrophilia. Two AS-ILD patients improved on rituximab (RTX) as induction treatment and three out of four patients were stabilised on RTX as maintenance treatment. Two patient obtained remission by cyclophosphamide. Four patients were stabilised on azathioprine alone or in combination with oral corticosteroids. Our cohort of AS-ILD patients showed clinical characteristics in accordance with previous reports at baseline and was comparable to other cohorts. Most patients can be stabilised with immunosuppressive treatment.

AB - Background and objective: To describe the clinical characteristics including the bronchoalveolar lavage fluid (BALF) characteristics of patients with antisynthetase syndrome (AS) associated interstitial lung disease (ILD) in a tertiary ILD outpatient clinic, their medical therapy and outcome. Methods: Retrospective cohort study of patients with AS-ILD. All available data of clinical characteristics, pulmonary function tests, laboratory parameters, BALF analysis, histology, high-resolution computed tomography (HRCT) and treatment were collected from the patient files. Results and conclusions: Twelve patients with AS-ILD were identified. Mean age at diagnosis was 55 years (range 45-69), 67% were female. Mean follow-up time was 7 years. The anti-aminoacyl tRNA-synthetase antibodies presented were anti-Jo1 (n = 6), anti-PL7 (n = 3), anti-PL12 (n = 2) and anti-EJ (n = 1). HRCT patterns were mainly non-specific interstitial pneumonia (75%). Four patients had BALF-eosinophilia (two of four anti-Jo1 patients) and two anti-PL12 positive patients had BALF-neutrophilia. Two AS-ILD patients improved on rituximab (RTX) as induction treatment and three out of four patients were stabilised on RTX as maintenance treatment. Two patient obtained remission by cyclophosphamide. Four patients were stabilised on azathioprine alone or in combination with oral corticosteroids. Our cohort of AS-ILD patients showed clinical characteristics in accordance with previous reports at baseline and was comparable to other cohorts. Most patients can be stabilised with immunosuppressive treatment.

KW - ANTIBODIES

KW - ARTHRITIS

KW - Antisynthetase syndrome

KW - DERMATOMYOSITIS

KW - POLYMYOSITIS

KW - RITUXIMAB

KW - SEVERITY

KW - bronchoalveolar lavage fluid

KW - interstitial lung diseases

KW - polymyositis

KW - treatments

U2 - 10.1080/20018525.2019.1583516

DO - 10.1080/20018525.2019.1583516

M3 - Journal article

C2 - 30834073

VL - 6

JO - European Clinical Respiratory Journal

JF - European Clinical Respiratory Journal

SN - 2001-8525

IS - 1

M1 - 1583516

ER -