A CASE OF FATAL INVASIVE PULMONARY ASPERGILLOSIS IN A PATIENT WITH ANTI-MDA5 DERMATOMYOSITIS

Abstract

TOPIC: Chest Infections TYPE: Medical Student/Resident Case Reports INTRODUCTION: Invasive pulmonary aspergillosis (IPA) is classically associated with solid/hematologic organ transplant, hematologic malignancy, and severe neutropenia. The presence of anti-melanoma differentiation-associated gene-5 (MDA-5) autoantibodies in dermatomyositis confers a higher risk of rapidly progressing interstitial lung disease (RP-ILD) resulting in high mortality. We present a case of a patient with a history of diagnosed anti-MDA5 dermatomyositis who developed acute fulminant IPA. CASE PRESENTATION: We report a case of a 65-year-old female with a history of MDA-5 positive dermatomyositis who presented to the hospital with symptoms of dyspnea, fever, and hypoxemia. A CT scan demonstrated bilateral peripheral patchy ground-glass opacities (image 1). The differential diagnosis was broad and included bacterial vs viral vs pneumocystis pneumonia. Upon presentation, she had been on 24 days of prednisone on a dose of 60mg for the first 14 days and 40mg daily for the last 10 for her recent diagnosis of dermatomyositis. Also on the differential was the possibility of RP-ILD. She was started on high-dose steroids at 60mg prednisone daily and broad-spectrum antibiotics. Given high clinical suspicion for RP-ILD, she was started on IVIG therapy. Bronchoscopy with bronchoalveolar lavage grew mold. A repeat CT days later demonstrated dramatically worsening bilateral pulmonary infiltrates with a consolidative pattern (image 2). The patient's clinical status worsened and required intubation for worsening respiratory failure. Eventually, galactomannan resulted positive on the initial and repeat BAL specimens (Ag Index 5.75, 3.62 respectively) with positive serum galactomannan (Ag index 3.33) making IPA the most likely diagnosis. She was started on voriconazole and micafungin with little clinical response and suffered from progressive respiratory decline leading to death. DISCUSSION: Our patient had anti-MDA-5 dermatomyositis with likely RP-ILD who experienced a rapid respiratory decline in the span of weeks. What makes this case interesting is the fulminant lung injury that occurred without a clear and early known diagnosis. Most notably the highest possibilities were RP-ILD and infection, now known to be from Aspergillus spp. The relationship between IPA in chronic respiratory diseases (CRD) particularly interstitial lung diseases is still unclear. There is some evidence to suggest even a short course of steroids may lead to substantial risk in those with underlying CRD such as dermatomyositis-related ILD. This may be the case in our patient who had been on high-dose prednisone before her presentation. CONCLUSIONS: There is the possibility that anti-MDA-5 positive dermatomyositis increases susceptibility to IPA, possibly through a mechanism related to RP-ILD. If characterized further, it may warrant more aggressive surveillance and treatment for invasive pulmonary fungal infections in these patients. REFERENCE #1: Naaraayan, A., Kavian, R., Lederman, J., Basak, P., & Jesmajian, S. (2015). Invasive pulmonary aspergillosis - case report and review of literature. Journal of community hospital internal medicine perspectives, 5(1), 26322. https://doi.org/10.3402/jchimp.v5.26322 REFERENCE #2: Sontheimer R. D. (2017). MDA5 autoantibody-another indicator of clinical diversity in dermatomyositis. Annals of translational medicine, 5(7), 160. https://doi.org/10.21037/atm.2017.03.94 DISCLOSURES: No relevant relationships by Merwise Baray, source=Web Response No relevant relationships by Deepa Lazarous, source=Web Response No relevant relationships by Robert Varipapa, source=Web Response No relevant relationships by Samuel Wopperer, source=Web Response