Rashes to Recognition: A Case of Rapidly Progressive Interstitial Lung Disease (ILD) in Dermatomyositis
Recommended Citation
Merah A, Esteitie R, Abdul Hameed AM. Rashes to Recognition: A Case of Rapidly Progressive Interstitial Lung Disease (ILD) in Dermatomyositis. Am J Respir Crit Care Med 2025; 211:A5968.
Document Type
Conference Proceeding
Publication Date
5-20-2025
Publication Title
Am J Respir Crit Care Med
Abstract
Introduction: In idiopathic inflammatory myopathies such as dermatomyositis (DM), Interstitial Lung Disease is a common extra-muscular manifestation associated with poor outcomes. Rapidly progressive ILD (RP-ILD) is a life-threatening subtype that requires prompt recognition due to its high mortality rate. Case: A previously healthy 57-year-old female presented to the hospital with abdominal pain, nausea, vomiting, and weakness. She had been evaluated at another hospital where she also reported difficulty walking, and violaceous rashes on her eyelids, hands, and chest. Workup there was only significant for elevated liver enzymes and positive ANA. On presentation, she was febrile, tachypneic, tachycardic, saturating 90% on room air. Labs showed elevated D-dimer and inflammatory markers, and computed tomography (CT scan) showed patchy bilateral infiltrates. She remained febrile despite broad spectrum antibiotics. Oxygen requirements also increased, and she was moved to the ICU for worsening hypoxemia. High resolution CT showed extensive ground glass opacities with worsening infiltrates. Based on her overall presentation, there was high suspicion for RP-ILD secondary to DM. She was transferred to another facility for extracorporeal membrane oxygenation (ECMO) evaluation due to worsening respiratory status. She was also started on pulse-dose steroids and intravenous immunoglobulin (IVIG). Unfortunately, she did not qualify for ECMO, and she passed away from complications secondary to septic shock. Myositis serology obtained shortly after her transfer resulted 3 weeks later with positive anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibodies. Discussion: RP-ILD is defined as worsening dyspnea, hypoxemia, and radiological interstitial changes within 3 months of symptom onset. This phenotype of myositis-associated ILD tends to be refractory to glucocorticoid and immunosuppressive therapy, and often requires non-pharmacological therapies such as plasmapheresis, IVIG, ECMO, or lung transplantation. Overall mortality rate is at least 30%, and in some studies, RP-ILD carried a 9.7-fold risk of death. RP-ILD is strongly associated with anti-MDA5 dermatomyositis, and the latter should be considered in patients with pathognomonic skin findings and worsening respiratory status. Positive autoantibodies are useful in confirming the disease but can take time to result. The diagnosis of DM based on criteria from the American College of Rheumatology utilizes five categories (objective muscle weakness, skin findings, esophageal dysmotility, positive anti-synthetase autoantibodies, and muscle biopsy) to generate an aggregate score classifying DM into probable and definite . Skin findings alone provide a score of 8.5 in the absence of muscle biopsy, which corresponds to a definite diagnosis. This is useful in making a clinical diagnosis and initiating prompt management.
Volume
211
First Page
A5968
