Pulmonary Manifestations of Systemic Disease |

Two Distinct Pulmonary Manifestations of an Inflammatory Myopathy FREE TO VIEW

Shilpi Ahuja, MD; Benjamin Wu, MD; William Bender, MD; Jennifer Zeng, MD; Paru Patrawalla, MD
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NYU Langone Medical Center, New York, NY

Chest. 2015;148(4_MeetingAbstracts):861A. doi:10.1378/chest.2259470
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SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case Report Posters

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Dermatomyositis is a systemic inflammatory condition characterized by skin findings, myopathy, and rarely pulmonary findings. We present a case of a patient who developed two distinct pulmonary manifestations: organizing pneumonia as the initial manifestation of systemic disease and pulmonary vasculitis a year later.

CASE PRESENTATION: Our patient is a 44-year-old woman with a history of moderate persistent asthma who presented to Asthma Clinic to establish care. Recent periorbital edema and unilateral arm pain suggested an underlying systemic disease. Rheumatologic panel was positive for RF, CCP, and Jo-1 with a normal Creatine Kinase (CK). CXR revealed hazy nodular opacities at the bases, as well as bronchial wall thickening. CT revealed multi-lobar peripheral nodular densities (Figure 1). Trans-bronchial biopsy (TBBX) showed organizing pneumonia without vasculitis. The patient developed synovitis and was started on steroids for treatment of organizing pneumonia and an undifferentiated rheumatic disease. Two months later, she developed diffuse myalgias and weakness and an elevated CK and Aldolase. Pathologic exam of a right forearm muscle biopsy was consistent with dermatomyositis. Over the next several months, she developed refractory dermatomyositis, despite improvement of the CT findings on steroids. One year later, she presented with digital ischemia and lower extremity purpura. CT chest revealed new ground glass opacities (Figure 2). Skin biopsy showed leukocytoclastic vasculitis and TBBX revealed alveolar hemorrhage with clusters of neutrophils consistent with small vessel capillaritis. She was treated with pulse dose steroids and Cytoxan with improvement of pulmonary findings.

DISCUSSION: Interstitial lung disease has been described in association with inflammatory myositis and presents amidst other rheumatologic complaints. The most common pulmonary histopathological findings identified in retrospective studies include diffuse alveolar damage and nonspecific interstitial pneumonia, but only rarely are organizing pneumonia or pulmonary capillaritis identified as the predominant pathology.

CONCLUSIONS: While pulmonary capillaritis and organizing pneumonia remain uncommon pulmonary histopathologic findings in patients with dermatomyositis, it is exceedingly rare to have both diagnosed in the same patient.

Reference #1: Douglas WW, Tazelaar HD, Hartman TE, Hartman RP, Decker PA, Schroeder DR, Ryu JH. Polymyositis-dermatomyositis-associated interstitial lung disease. Am J Respir Crit Care Med 2001;164:1182-1185.

DISCLOSURE: The following authors have nothing to disclose: Shilpi Ahuja, Benjamin Wu, William Bender, Jennifer Zeng, Paru Patrawalla

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