ECMO consideration recommended for myositis-associated rapidly progressive ILD

Among patients with myositis-associated rapidly progressive interstitial lung disease, extracorporeal membrane oxygenation was well tolerated with no deaths from direct complications, according to a case series published in Chest.

“Limited available evidence and expert opinion support early, aggressive, multidrug immunosuppressive treatment,” Jonah Rubin, MD, pulmonary and critical care medicine fellow in the division of pulmonary and critical care medicine at Massachusetts General Hospital and Harvard Medical School, and colleagues wrote. “Consensus recommendations include consideration of ECMO as a bridge to recovery or transplant. However, given the rarity of this condition, this has not been rigorously evaluated.”

Researchers reported data from a case series of nine patients (mean age, 52.2 years; five women) at Massachusetts General Hospital from 2016 to 2020 who underwent veno-venous ECMO in recovery from myositis-associated rapidly progressive ILD. Researchers defined rapidly progressive ILD as progressive respiratory symptoms with new or worsening infiltrates on CT scans over 3 months. Seven patients had ILD evidence before hospital admission.

Six patients died following discontinuation of elective ECMO due to failure to recover, another patient died following decannulation as a result of ventilator-associated pneumonia and another patient died from sepsis. One patient survived until hospital discharge without disability.

“Thus, most deaths in this series were more withdrawal of life support after a failure to improve, reflecting the need for improved recognition and treatment of myositis-associated rapidly progressive ILD,” the researchers wrote.

The most common chest CT scan findings were nonspecific interstitial pneumonia in three patients and organizing pneumonia in three patients. Researchers also observed other radiographic patterns including usual interstitial pneumonia in one patient, indeterminate usual vs. nonspecific interstitial pneumonia in one patient and diffuse alveolar damage in one patient.

Six patient were treatment naive, including the patient who survived; the other three patients were on steroids for 3 months or longer before admission, according to the results. In addition, seven patients received triple immunosuppressive therapy with pulse-dosed steroids, rituximab (Rituxan; Genentech, Biogen) and IV immunoglobins. Mycophenolate mofetil was administered to one patient, and two patients received cyclophosphamide.

None of the patients were considered candidates for lung transplantation due to critical illness and acute respiratory distress severity, inability to consent, inability to rehabilitate and/or underlying comorbidities or adverse prognostic factors.

“Although not performed in our cohort, ECMO has been successfully used in myositis-associated rapidly progressive ILD as a bridge to lung transplant,” the researchers wrote. “Therefore, candidacy for transplant should be evaluated early in the hospital course.”

The researchers noted limitations of this case series, including its small number of patients and single-center, retrospective design. Moreover, patients in this series did not receive now-recommended initial treatments for patients with MDA5 rapidly progressive ILD, including calcineurin inhibitors or mycophenolate mofetil.

They noted that “one cannot draw definitive conclusions from a small, observational study with a single surviving patient. Elements of the surviving patient’s history and course, including age, sex and timing of treatment are at best hypothesis generating. Acknowledging these limitations, given improving therapeutics for myositis-associated rapidly progressive ILD and the tolerance of ECMO, ECMO should continue to be considered in this patient population.”