Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2019 Aug 4;13(1):241.
doi: 10.1186/s13256-019-2146-0.

Antisynthetase syndrome presenting as interstitial lung disease: a case report

Aliena Badshah  1 , Iqbal Haider  2 , Shayan Pervez  2 , Mohammad Humayun  2
Affiliations
Case Reports

Antisynthetase syndrome presenting as interstitial lung disease: a case report

Aliena Badshah et al. J Med Case Rep. .

Abstract

Background: Antisynthetase syndrome is a relatively uncommon entity, and can be easily missed if not specifically looked for in adults whose initial presentation is with interstitial lung disease. Its presentation with interstitial lung disease alters its prognosis.

Case presentation: This case report describes a 27-year-old Pakistani, Asian man, a medical student, with no previous comorbidities or significant family history who presented with a 3 months' history of low grade fever and lower respiratory tract infections, associated with exertional dyspnea, arthralgias, and gradual weight loss. During these 3 months, he had received multiple orally administered antibiotics for suspected community-acquired pneumonia. When he presented to us, he was pale and febrile. A chest examination was significant for bi-basal end-inspiratory crackles. Preliminary investigations revealed raised erythrocyte sedimentation rate. High resolution computed tomography of his chest showed fine ground-glass attenuation in posterior basal segments of both lower lobes suggestive of interstitial lung disease. He was started on dexamethasone, to which he responded and showed improvement. However, during the course of events, he developed progressive proximal muscle weakness. Further investigations revealed raised creatinine phosphokinase and lactate dehydrogenase. A thorough autoimmune profile was carried out which showed positive anti-Jo-1 antibodies in high titers. A muscle biopsy was consistent with inflammatory myopathy. Clinical, radiological, serological, and histopathological markers aided in making the definitive diagnosis of antisynthetase syndrome. Antisynthetase syndrome is a variant of polymyositis but with visceral involvement, that is, interstitial lung disease and positive anti-Jo-1 antibodies. Our patient responded very well to glucocorticoids and azathioprine.

Conclusion: Antisynthetase syndrome is a rare clinical entity which apart from clinical presentation requires specific serological investigations for diagnosis. Concomitant association of interstitial lung disease gives it a guarded prognosis.

Keywords: Anti-Jo-1 antibodies; Antisynthetase syndrome; Arthralgia; Interstitial lung disease; Myopathy; Polymyositis.

PubMed Disclaimer

Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
CT scan showing features suggestive of Interstitial Lumg Disease (ILD)
Fig. 2
Fig. 2
CT scan showing areas of ground glass haze and traction bronchiectasis
See this image and copyright information in PMC

References

    1. Trallero-Araguás E et al. Clinical manifestations and long-term outcome of anti Jo1 antisynthetase patients in a large cohort of Spanish patients from the GEAS-IIM group. Semin Arthritis Rheum. 2016;16(30):1–4. - PubMed
    1. Fathi M, Lundberg IE, Tornling G. Pulmonary complications of polymyositis and dermatomyositis. Semin Respir Crit Care Med. 2007;28(4):451–458. doi: 10.1055/s-2007-985666. - DOI - PubMed
    1. Mirrakhimov AE. Antisynthetase syndrome: a review of etiopathogenesis, diagnosis and management. Curr Med Chem. 2015;22(16):1963–1975. doi: 10.2174/0929867322666150514094935. - DOI - PubMed
    1. Chatterjee S, Prayson R, Farver C. Antisynthetase syndrome: not just an inflammatory myopathy. Cleve Clin J Med. 2013;80(10):655–666. doi: 10.3949/ccjm.80a.12171. - DOI - PubMed
    1. Mahler M, Miller FW, Fritzler MJ. Idiopathic inflammatory myopathies and the anti-synthetase syndrome: a comprehensive review. Autoimmun Rev. 2014;13:367–371. doi: 10.1016/j.autrev.2014.01.022. - DOI - PMC - PubMed

Publication types

Supplementary concepts