Case Series: ECMO for Myositis-Associated Rapidly Progressive Interstitial Lung Disease

Myositis-associated rapidly progressive interstitial lung disease (RP-ILD) is a rare, often fatal condition that can present similarly to acute respiratory distress syndrome (ARDS). For patients without known myositis, a high index of clinical suspicion is required for correct diagnosis.

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Early, aggressive, multidrug immunosuppressive treatment is recommended based on limited available evidence and expert opinion. Consensus guidance also recommends the consideration of extra-corporeal membrane oxygenation (ECMO) as a bridge to recovery or lung transplantation, but supportive data are lacking.

Massachusetts General Hospital physicians recently reported in Chest a nine-case series of patients who underwent veno-venous ECMO between 2016 and 2020 as an attempted bridge to recovery from myositis-associated RP-ILD. This is the largest series to evaluate ECMO for that indication and the first to share details on the hospital course of patients with myositis-associated IP-ILD.

The authors from Massachusetts General Hospital are Jonah Rubin, MD, Pulmonary and Critical Care Medicine fellow, Yuval Raz, MD, physician in the Division of Pulmonary and Critical Care Medicine, and colleagues.

Diagnosis

Diagnosis of myositis was made via multidisciplinary discussions with the Rheumatology Division at Mass General. Pathologic specimens were previously available or obtained in three cases and supported the diagnosis. Six patients were positive for the anti-melanoma differentiation–associated protein 5 antibody.

The most common findings on chest computed tomography (CT) were non-specific interstitial pneumonia (n=3) and organizing pneumonia (n=3). Other radiographic patterns were usual interstitial pneumonia, diffuse alveolar damage and indeterminate usual vs. nonspecific interstitial pneumonia.

Pharmacotherapy

Six patients were treatment-naïve; the others had been on steroids for at least three months before hospital admission.

During admission, seven patients received triple immunosuppressive therapy (pulse-dosed steroids, rituximab and intravenous immunoglobulins). In the other two cases, therapeutic care was withdrawn after steroid administration and before additional immunosuppressive therapy. Advances in therapeutic options for myositis associated RP-ILD have developed since the time period under investigation.

Survival

• One patient survived to discharge without disability
• One patient survived to ECMO decannulation but subsequently died from ventilator-associated pneumonia
• One patient died from sepsis while on ECMO
• Six patients died after elective ECMO discontinuation given failure to recover

Commentary

The surviving patient in this series had no discernable lung disease before admission. Triple immunosuppressive therapy was initiated within five days of ICU admission (within two weeks of hospital admission). ECMO itself is not therapeutic, but in this case, it provided time for the early, aggressive treatment to be effective.

One cannot draw definitive conclusions from a small, single-center observational study with a single surviving patient. However, given the tolerability of ECMO in this series, along with the advances in diagnostic algorithms and treatment regimens for myositis-associated RP-ILD, ECMO should continue to be considered in this patient population.

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