DERMATOMYOSITIS WITH RAPIDLY PROGRESSIVE INTERSTITIAL LUNG DISEASE COMPLICATED BY SPONTANEOUS PNEUMOMEDIASTINUM IN A PATIENT AFTER ASYMPTOMATIC COVID-19 INFECTION

ABSTRACT

SESSION TITLE Systemic Disease with Diffuse Lung Symptoms Case Posters SESSION TYPE Case Report Posters PRESENTED ON 10/17/2022 1215 pm - 0115 pm INTRODUCTION: Rapidly progressive interstitial lung disease (RP-ILD) is a rare and potentially fatal manifestation of dermatomyositis (DM) and has considerable impact in terms of the prognosis. CASE PRESENTATION A 52-year-old male demonstrated DM-typical rash, fever, mialgias, and mild muscle weakness 3 months after asymptomatic COVID-19 infection. Two weeks later dysphonia and progressive dyspnea appeared. Lung CT scan showed the picture of organizing pneumonia. His COVID-19 PCR test was negative multiple times. Laboratory tests revealed the following numbers ALT 210 IU/L, AST 748 IU/L, LDH 613 IU/L, CPK 1165 IU/L, ferritin 1145ϻg/l, CRB 11 mg/l. The patient was tested positive for anti-Ro52 antibodies, while anti-synthetase and scleroderma-associated antibodies were not discovered. Anti-melanoma differentiation-associated gene 5 (MDA5) test was not available due to the lack of the necessary test systems in the country. The patient was diagnosed with DM. Combined immunosuppressive therapy was administered, including oral prednisolone 60 mg per day and 720 mg intravenously, dexamethasone 64-24 mg intravenously per diem, ciclosporin 200 mg и cyclophosphamide 600 mg, and 3 plasmapheresis sessions followed by an intravenous immunoglobulin. As a result of the therapy, muscle weakness disappeared and CPK levels returned to normal limits, however dyspnea progressed and ferritin levels hit 3500ϻg/l. After the following 3 weeks of intensive combined immunosuppressive therapy, the patient demonstrated symptoms of severe respiratory failure (RF). CT scan showed multiple traction bronchiectasis, wide areas of ground glass opacity, pneumomediastinum and subcutaneous emphysema of a neck and supraclavicular regions. Ciclosporin was replaced with tofacitinib with the dose of 10 mg per diem, IL-6 inhibitor (olokizumab 256 mg) was injected intravenously, massive broad-spectrum antibiotic therapy was administered. RF progressed and the patient was put on mechanical ventilation. The patient died of acute RF and sepsis a week later. DISCUSSION: RP-ILD is a common manifestation of severe MDA5+ DM, which is also associated with necrotizing vasculitis and amyopathic/hypomyopathic muscle involvement. In this case acute ILD in a patient with typical DM could also have been provoked by previous COVID-19 infection. CONCLUSIONS: The courses of disease for COVID-19 and MDA5+ DM have several similarities, which means it can be the same for their pathogenesis and clinical manifestations. In spite of early screening and intensive immunosuppressive therapy in such cases, the prognosis of patients with DM and RP-ILD is still poor and is associated with high mortality. Reference #1 Wang G, Wang Q, Wang Y, et al. Presence of Anti-MDA5 Antibody and Its Value for the Clinical Assessment in Patients With COVID-19 A Retrospective Cohort Study. Front Immunol. 2021 Dec 20;12791348. doi 10.3389/fimmu.2021.791348. PMID 34987516;PMCID PMC8720853. DISCLOSURES No relevant relationships by Lidia Ananyeva No relevant relationships by Maria Aristova No relevant relationships by Liudmila Garzanova No relevant relationships by Anna Khelkovskaya-Sergeeva No relevant relationships by Dmitry Kulikovsky