Abstract
Anti-melanoma differentiation-associated gene 5 juvenile dermatomyositis (anti-MDA5 JDM) is associated with high risk of developing rapidly progressive interstitial lung disease (RP-ILD). Here we report an 11-year-old girl with anti-MDA5 JDM and RP-ILD which led to a fatal outcome, further aggravated by SARS-CoV-2 infection. She was referred to our hospital after being diagnosed with anti-MDA5 JDM and respiratory failure due to RP-ILD. On admission, fibrobronchoscopy with bronchoalveolar lavage (BAL) revealed Pneumocystis jirovecii infection so treatment with intravenous trimethoprim-sulfamethoxazole was initiated. Due to RP-ILD worsening, immunosuppressive therapy was intensified using methylprednisolone pulses, cyclophosphamide, tofacitinib and intravenous immunoglobulin without response. She developed severe hypoxemic respiratory failure, pneumomediastinum and pneumothorax, further complicated with severe RP-ILD and cervical subcutaneous emphysema. Three real-time RT-PCR for SARS-CoV-2 were made with a negative result. In addition, she was complicated with a secondary hemophagocytic lymphohistiocytosis and a fourth real-time PCR for SARS-CoV-2 performed in BAS sample was positive. Despite aggressive treatment of RP-ILD due to anti-MDA5 JDM, there was no improvement of respiratory failure in the following days and patient developed refractory septic shock and died. Anti-MDA5 JDM patients with RP-ILD have a poor prognosis with a high mortality rate. For this reason, intensive immunosuppressive therapy is essential including the use of promising drugs such as tofacitinib. COVID-19 in children with underlying health conditions like anti-MDA5 JDM may still be at risk for disease and severe complications.
Keywords: Anti-MDA5; COVID-19; interstitial lung disease (ILD); juvenile dermatomyositis; tofacitinib.
【저자키워드】 COVID-19, Anti-MDA5, interstitial lung disease (ILD), juvenile dermatomyositis, tofacitinib., 【초록키워드】 Methylprednisolone, Treatment, SARS-CoV-2, Intravenous immunoglobulin, therapy, Respiratory failure, SARS-COV-2 infection, children, hospital, Infection, Lung disease, risk, Interstitial lung disease, drug, outcome, BAL, Immunoglobulin, Real-time PCR, MDA5, Patient, Septic shock, hemophagocytic lymphohistiocytosis, Pneumothorax, real-time RT-PCR, secondary hemophagocytic lymphohistiocytosis, disease, Admission, Intensive, immunosuppressive therapy, juvenile dermatomyositis, Hypoxemic respiratory failure, Tofacitinib, Bronchoalveolar lavage, immunosuppressive, Pneumomediastinum, dermatomyositis, high risk, intravenous, Cyclophosphamide, referred to, risk for disease, subcutaneous emphysema, sulfamethoxazole, worsening, poor prognosis, severe complications, high mortality rate, positive, emphysema, health condition, hypoxemic, performed, died, addition, diagnosed, initiated, BAS, 【제목키워드】 outcome, Case report, juvenile dermatomyositis,