Diffuse Lung Disease: Fellow Case Report Slide: Diffuse Lung Disease |

Rapidly Progressive Hypoxic Respiratory Failure After a Rash: A Case of Clinically Amyopathic Dermatomyositis (CADM)-Associated ILD FREE TO VIEW

Daniel Hershberger, MD
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Creighton University Medical Center, Bellevue, NE

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):509A. doi:10.1016/j.chest.2016.08.523
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SESSION TITLE: Fellow Case Report Slide: Diffuse Lung Disease

SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Wednesday, October 26, 2016 at 11:00 AM - 12:15 PM

INTRODUCTION: Dermatomyositis (DM) & Polymyositis (PM) are associated with interstitial lung disease (ILD). Myositis ILD is tightly linked to anti-Jo1 antibody. Other antibodies can be seen including: Anti- PL-7, PL-12, OJ and EJ. A subset of DM patients present with skin manifestations but without muscular involvement. They are described as having Clinically Amyopathic DM (CADM). CADM is associated with ILD at a similar rate to DM & PM.

CASE PRESENTATION: A 46 year-old African American female with no significant PMH presented with a pruritic, papular rash on her arms and chest. The rash resolved with steroid cream. Over the next 6 weeks she developed dyspnea, fevers, dry cough and hypoxia. Chest radiograph revealed bilateral interstitial infiltrate. Patient failed to respond to antibiotics and diuretics. CT scan showed diffuse ground-glass and consolidative opacities. A bronchoscopy with BAL revealed a lymphocytosis of 36%. Cultures were negative. Labs revealed ANA and dsDNA positivity. Patient denied all rheumatologic symptoms. SS-A, SS-B, Scl-70, Smith, RNP, ANCA, Anti-Jo1, RF, Anti-CCP and HIV were negative. CK and aldolase were normal. Patient was placed on Prednisone 1 mg/kg. She clinically deteriorated. Open lung biopsy showed organizing pneumonia with diffuse alveolar damage. Based on her 100% oxygen requirement and failure to respond to corticosteroids, she was sent for lung transplant evaluation. At the transplant center an extended myositis panel revealed Anti-MDA5 antibody. Patient was diagnosed with CADM-ILD, started on mycophenolate and given rituxamab. She responded well and is now 1 year removed from her initial presentation on minimal oxygen.

DISCUSSION: Despite CADM patients having comparable rates of ILD, when Anti-MDA-5 is found there is an increased incidence and severity of ILD. This antibody has been mostly described in Asia. A variety of treatments have been described, including high dose prednisone, steroid sparing agents and rituximab.

CONCLUSIONS: This case highlights that in the setting of recent onset rash and new ILD, CADM-ILD should be considered as a diagnosis. This case report adds to the increasing recognition of Anti-MDA-5 outside of Asian populations. This report demonstrates that African American patients can develop Anti-MDA-5 disease. This case also provides further evidence that rituximab is a viable option for refractory hypoxia in CADM-ILD.

Reference #1: Sato S, et al. Autoantibodies to a 140-kd polypeptide, CADM-140, in Japanese patients with CADM. Arthritis Rheum. 2005; 52:1571-6.

DISCLOSURE: The following authors have nothing to disclose: Daniel Hershberger

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