A RAPIDLY PROGRESSIVE AND RARE ILLNESS: AUTOANTIBODIES AGAINST MELANOMA DIFFERENTIATION-ASSOCIATED PROTEIN 5 (ANTI-MDA5): AMYOPATHIC DERMATOMYOSITIS WITH PROGRESSIVE INTERSTITIAL LUNG DISEASE THAT DEVELOPED AFTER COVID-19 VACCINE

Abstract

TOPIC: Critical Care TYPE: Global Case Reports INTRODUCTION: Anti-MDA5 dermatomyositis is a subtype of dermatomyositis characterized by a high risk of rapidly progressive interstitial lung disease. It is characterized by fever, progressive shortness of breath, joint swelling and skin ulcerations. (1) There is invariable hyper-ferritinemia and radiographically there is a pattern of diffuse bilateral ground glass opacities. Complications with spontaneous pneumothorax and pneumomediastinum are not uncommon. Diagnosis is confirmed by biopsy as well as serum antibodies against MDA5. A known trigger is viral infection. (2) CASE PRESENTATION: We present the case of a 58-year-old man who was admitted to the Intensive Care Unit on account of respiratory failure and shock. He had a progressive Illness that started 4 days after receiving a novel mRNA COVID-19 Vaccine. He developed bilateral extremities edema, oral sores, worsening fatigue and dyspnea on exertion. He failed outpatient treatment for empiric pneumonia as well as prednisone for vaccine reaction. He presented to the hospital with fever, progressive dyspnea and fatigue. On physical exam he had oral blisters, digital tip ischemia and ulceration. His vital signs were pertinent for a SpO2 of 90% on ventilator support. A chest CT revealed diffuse and sub-pleural ground-glass opacities.There was an extensive negative infectious work up from serum, urine and BAL samples. These included negative SARS COV2 Antigen and SARS-Cov2 IgG. His inflammatory markers were pertinent for a positive ANA with titer of 1:320, Ferritin of 2,143 mcg/L, LDH of 1,105 U/L and CRP of 2.7mg/dL. Based on this information the differential for amyopathic dermatomyositis with progressive interstitial lung disease was considered. A skin biopsy of his index finger confirmed the diagnosis. His therapeutic interventions through his hospitalization included a trial of stress dose steroids, cyclophosphamide, IVIG, colchicine and tacrolimus. His ICU course was complicated by pneumomediastinum, pneumothorax and pericardial effusion. These were managed by placing a chest tube thoracotomy and pericardial drain respectively. His plan of care was changed to comfort measures after he had a drastic and prolonged desaturation episode without recovery. DISCUSSION: For conditions that lead to cytokine storm with subsequent development of ARDS or multi-organ dysfunction;there is a progressive clinical deterioration and high risk of death. We had insufficient evidence to definitively demonstrate a direct correlation between the vaccination and the patient's illness. CONCLUSIONS: In conditions that lead to Cytokine storm with multi-organ dysfunction targeting timely control of this inflammatory cascade via immunomodulation, cytokine antagonist and reduction of inflammatory cell infiltration in particular early in the process could lead to a reduction in mortality. REFERENCE #1: Fiorentino D, Chung L, Zwerner J, Rosen A, Casciola-Rosen L. The mucocutaneous and systemic phenotype of dermatomyositis patients with antibodies to MDA5 (CADM-140): a retrospective study. J Am Acad Dermatol. 2011;65(1):25-34. doi:10.1016/j.jaad.2010.09.016 REFERENCE #2: Sakamoto N., Ishimoto H., Nakashima S. Clinical features of anti-MDA5 antibody-positive rapidly progressive interstitial lung disease without signs of dermatomyositis. Intern Med. 2019;58(6):837–841. doi: 10.2169/internalmedicine. 1516-18. REFERENCE #3: Margherita Giannini,1 Mickael Ohana,2 Benoit Nespola,3 Giovanni Zanframundo,4,5 Bernard Geny,1,6 and Alain Meyer1,6,7 Similarities between COVID-19 and anti-MDA5 syndrome: what can we learn for better care? Eur Respir J. 2020 Sep;56(3): 2001618. Published online 2020 Sep DISCLOSURES: No relevant relationships by Ashlie Arthur, source=Web Response No relevant relationships by Leonel Carrasco, source=Web Response no disclosure on file for Chad Case;No relevant relationships by Juan Gaitan Rueda, source=Web Response