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Diffuse Lung Disease |

An Interesting Case of Interstitial Lung Disease and Myositis

Syed Amin, MD; Nausheen Afroz, BS; David Kaufman, MD; Assad Oskuei, MD
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Bridgeport Hospital/Yale University School of Medicine, Bridgeport, CT


Chest. 2014;146(4_MeetingAbstracts):410A. doi:10.1378/chest.1974676
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Abstract

SESSION TITLE: ILD Student/Resident Case Report Posters

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Inflammatory myopathies are a group of rare systemic disorders affecting skeletal muscles and other organs, notably the lungs. We present a case highlighting the early diagnosis and management of interstitial lung disease (ILD) associated with an inflammatory myopathy.

CASE PRESENTATION: A 38-year-old man presented to another hospital with a 2-month history of difficulty breathing on exertion, dry cough, weakness, night sweats and fever. On admission, he was treated for community acquired pneumonia, without improvement. Chest high-resolution CT (HRCT) revealed patchy areas of bilateral airspace and interstitial lung opacities (Figure 1A). Transbronchial biopsy of RLL revealed fragments of lung parenchyma with interstitial and organizing pneumonia pattern (Figure 1B). He was discharged on inhaled bronchodilators and tapering dose of prednisone with a diagnosis of ILD. Two months after discharge, he presented to our hospital with lower extremity swelling, diffuse myalgia, polyarthralgia and profound proximal muscle weakness 4 weeks after completing prednisone. Pertinent laboratory findings are shown in Table 1. A right quadriceps muscle biopsy demonstrated necrotizing inflammatory changes within myofibers, most consistent with polymyositis (PM). He was prescribed high-dose prednisone and azathioprine, and eventually treated with rituximab infusions.

DISCUSSION: The presence of organizing pneumonia on transbronchial biopsy with symptoms of myalgia, prompted the muscle biopsy and led to the diagnosis of PM. The uniqueness of this case is highlighted by the diagnosis of ILD preceding that of myositis by a few months, whereas in majority of cases the myositis precedes the lung involvement by several months to years (2, 3). In addition, our patient met criteria for Anti-synthetase (AS) Syndrome given the presence of anti-AS Ab (Anti-Jo-1 Ab -most common), accompanied by evidence of myositis, and ILD (1). Anti-Jo-1 Ab (AJ1 Ab) remains the strongest predictive factor for ILD in myositis as about 70% of AJ1 Ab positive patients have ILD (1, 2). Pulmonary complications respond well to high dose steroids along with immunosuppressive therapy (3).

CONCLUSIONS: Despite ILD being a frequent manifestation of PM and associated with increased morbidity and mortality, it is often underdiagnosed as respiratory symptoms are gradual and can occur over months (2). Thus, investigations (HRCT and PFTs) should be performed during initial evaluation as well as during follow up on patients with an inflammatory myopathy (2, 3).

Reference #1: Solomon, J. et al. Myositis-related interstitial lung disease and antisynthetase syndrome. J Bras Pneumol. 2011;37(1):100-109.

Reference #2: Fathi, M. et al. Interstitial lung disease, a common manifestation of newly diagnosed polymyositis and dermatomyositis. Ann Rheum Dis.2004;63(3):297-301.

Reference #3: Fathi, M. et al. Interstitial lung disease in polymyositis and dermatomyositis:longitudinal evaluation by pulmonary function and radiology. Arthritis Rheum.2008;59(5):677-85.

DISCLOSURE: The following authors have nothing to disclose: Syed Amin, Nausheen Afroz, David Kaufman, Assad Oskuei

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