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.
MeSH terms
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Adenosine Monophosphate / analogs & derivatives
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Adenosine Monophosphate / therapeutic use
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Alanine / analogs & derivatives
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Alanine / therapeutic use
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Anti-Bacterial Agents / therapeutic use
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Antibodies, Monoclonal, Humanized / therapeutic use
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Antiviral Agents / therapeutic use
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Autoantibodies / immunology
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Bronchoscopy
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COVID-19 / complications*
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COVID-19 / therapy
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COVID-19 Nucleic Acid Testing
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Child
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Cyclophosphamide / therapeutic use
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Dermatomyositis / complications*
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Dermatomyositis / drug therapy
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Dermatomyositis / immunology
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Disease Progression
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Fatal Outcome
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Female
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Humans
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Hydroxychloroquine / therapeutic use
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Immunocompromised Host
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Immunoglobulins, Intravenous / therapeutic use
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Immunologic Factors / therapeutic use
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Immunosuppressive Agents / therapeutic use*
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Interferon-Induced Helicase, IFIH1 / immunology
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Lung / diagnostic imaging
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Lung Diseases, Interstitial / complications*
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Lung Diseases, Interstitial / diagnostic imaging
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Lung Diseases, Interstitial / immunology
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Lung Diseases, Interstitial / therapy
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Lymphohistiocytosis, Hemophagocytic / etiology*
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Lymphohistiocytosis, Hemophagocytic / immunology
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Mediastinal Emphysema / etiology
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Methylprednisolone / therapeutic use
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Piperidines / therapeutic use
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Pneumonia, Pneumocystis / complications*
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Pneumonia, Pneumocystis / immunology
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Pneumothorax / etiology
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Pyrimidines / therapeutic use
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Respiratory Insufficiency / etiology
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Respiratory Insufficiency / therapy
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Shock, Septic / etiology
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Subcutaneous Emphysema / etiology
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Tomography, X-Ray Computed
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Trimethoprim, Sulfamethoxazole Drug Combination / therapeutic use
Substances
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Anti-Bacterial Agents
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Antibodies, Monoclonal, Humanized
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Antiviral Agents
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Autoantibodies
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Immunoglobulins, Intravenous
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Immunologic Factors
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Immunosuppressive Agents
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Piperidines
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Pyrimidines
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remdesivir
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Adenosine Monophosphate
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Hydroxychloroquine
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Trimethoprim, Sulfamethoxazole Drug Combination
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tofacitinib
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Cyclophosphamide
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IFIH1 protein, human
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Interferon-Induced Helicase, IFIH1
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tocilizumab
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Alanine
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Methylprednisolone