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. 2019 Feb 27;6(1):1583516.
doi: 10.1080/20018525.2019.1583516. eCollection 2019.

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

Mads Lynge Jensen  1 , Anders Løkke  1 , Ole Hilberg  2 , Charlotte Hyldgaard  3 , Elisabeth Bendstrup  1 , Dan Tran  4
Affiliations

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

Mads Lynge Jensen et al. Eur Clin Respir J. .

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: Antisynthetase syndrome; bronchoalveolar lavage fluid; interstitial lung diseases; polymyositis; treatments.

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Figures

Figure 1.
Figure 1.
HRCT at debut. A NSIP pattern with consolidations, sub-pleural sparing and ground glass opacities in a perilobular localisation are seen in the basal lung zones.
Figure 2.
Figure 2.
HRCT 3 years later. Distributions of the interstitial features are unchanged but the pattern has turned more fibrotic with the development of traction bronchiectasis and increased reticulation.
Figure 3.
Figure 3.
Left: Gottron’s papules; right: mechanic’s hands.
See this image and copyright information in PMC

References

    1. Cavagna L, Nuño L, Scirè CA, et al. AENEAS (American and European Network of Antisynthetase Syndrome) collaborative group. Serum Jo-1 autoantibody and isolated arthritis in the antisynthetase syndrome: review of the literature and report of the experience of AENEAS collaborative group. Clin Rev Allerg Immunol. 2017;52:71–7. - PubMed
    1. Satoh M, Tanaka S, Ceribelli A, et al. A comprehensive overview on myositis-specific antibodies: new and old biomarkers in idiopathic inflammatory myopathy. Clin Rev Allerg Immunol. 2017February;52(1):1–19. - PMC - PubMed
    1. Marie I, Josse S, Hatron PY, et al. Interstitial lung Disease in anti-Jo-1 patients with antisynthetase syndrome. Arthritis Res Ther. 2013May;65(5):800–808. - PubMed
    1. Marie I, Josse S, Decaux O, et al. Comparison of long-term outcome between anti-Jo-1 and anti-PL7/PL12 positive patients with antisynthetase syndrome. Autoimmun Rev. 2012;11:739–745. - PubMed
    1. Dobloug C, Garen T, Bitter H, et al. Prevalence and clinical characteristics of adult polymyositis and dermatomyositis; data from a large and unselected Norwegian cohort. Ann Rheum Dis. 2015;74:1551–1556. - PubMed