MD Baylor College of Medicine, Texas Heart Institute
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Introduction: In patients with acute, progressive hypoxemic respiratory failure (AHRF) due to interstitial lung disease (ILD), dual lumen VV-ECMO cannulation can be a bridge to transplant (BTT). Patients with rare autoimmune etiologies of rheumatoid arthritis (RA) require special considerations, especially given the lack of established literature for management and prognosis.
Description:
Methods: A 37-year-old female with 7-month history of RA presented from an outside hospital (OSH) for worsening AHRF of unclear etiology. The OSH’s extensive infectious and autoimmune investigations were negative. Despite immunosuppression, antimicrobials, and intubation with optimal ventilation, her pulmonary status declined as indicated by escalating airway pressures, worsening PaO2 to FiO2 (P:F) ratio, hypoxemia, hypercapnia, and respiratory acidosis. Four days after transfer, she underwent emergent VV-ECMO cannulation via left subclavian crescent cannula under fluoroscopy. Repeat serologies remained pending; however, patient age, lack of comorbidities, and unilluminating infectious disease workup prompted cannulation as BTT for bilateral orthotopic lung transplantation (BOLT). Tracheostomy was performed on hospital day thirteen.
Results: Five days after VV-ECMO cannulation, serologies identified strong positivity for anti-MDA5 consistent with MDA-5 dermatomyositis. Her hospital course has been complicated by disseminated intravascular coagulopathy (DIC), gastrointestinal bleeding, and transaminitis. At time of abstract submission, hospital length of stay (LOS) totals 37 days, while VV-ECMO support totals 33 days. The patient is reconditioning with physical therapy and VV-ECMO in anticipation of BOLT with marginal improvements with optimal rheumatologic interventions.
Discussion: MDA-5 dermatomyositis is associated with rapidly progressive ILD and high mortality. Despite sparse literature regarding MDA-5 ILD prognosis, early suspicion and proactive pulmonary interventions are crucial due to swift disease progression and rapid pulmonary decline. If refractory to immunosuppressive therapy, BOLT is a patient’s definitive destination therapy. This case highlights the importance of early ECMO consideration and transplant planning in younger patients with otherwise unexplained, rapidly worsening ILD.
