Tuberc Respir Dis.  2016 Jul;79(3):188-192. 10.4046/trd.2016.79.3.188.

Acute Respiratory Distress Syndrome as the Initial Clinical Manifestation of an Antisynthetase Syndrome

• Kim SH
• Park IN

• KMID: 2326688
• DOI: http://doi.org/10.4046/trd.2016.79.3.188

Abstract

Antisynthetase syndrome has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with polymyositis and dermatomyositis. It is associated with serum antibody to aminoacyl-transfer RNA synthetases and is characterized by a constellation of manifestations, including fever, myositis, interstitial lung disease, mechanic's hand-like cutaneous involvement, Raynaud phenomenon, and polyarthritis. Lung disease is the presenting feature in 50% of the cases. We report a case of a 60-year-old female with acute respiratory distress syndrome (ARDS), which later proved to be an unexpected and initial manifestation of anti-Jo-1 antibody-positive antisynthetase syndrome. The present case showed resolution of ARDS after treatment with high-dose corticosteroids. Given that steroids are not greatly beneficial in the treatment of ARDS, it is likely that the improvement of the respiratory symptoms in this patient also resulted from the prompt suppression of the inflammatory systemic response by corticosteroids.

Keyword

Respiratory Distress Syndrome, Adult; Immunoglobulins; Antisynthetase Syndrome

MeSH Terms

Adrenal Cortex Hormones
Arthritis
Dermatomyositis
Female
Fever
Humans
Immunoglobulins
Ligases
Lung Diseases
Lung Diseases, Interstitial
Middle Aged
Myositis
Polymyositis
Raynaud Disease
Respiratory Distress Syndrome, Adult*
RNA
Steroids
Adrenal Cortex Hormones
Immunoglobulins
Ligases
RNA
Steroids

Figure

• 

  Figure 1 Chest X-ray film at 2 months prior to admission (A), on admission (B), before the first intubation (C), after the first extubation (D), after the second intubation (E), and at discharge (F).

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  Figure 2 High-resolution computed tomography (HRCT) after the second extubation when the patient was on prednisolone. On HRCT, there were still irregular diffuse ground glass opacities in both upper lobes (A) and irregular patchy consolidation in both lower lobes without honeycombing (B).

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  Figure 3 The time course of laboratory values including creatine kinase (CK), lactate dehydrogenase (LDH), and aspartate aminotransferase (AST), and history of ventilator use and medications. Intravenous methylprednisolone was administered at a dose of 125 mg every 6 hours for 1 day, followed by oral prednisolone at a dose of 60 mg/day.

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