CHEST Guidelines-Response_chest

Response_chest_13
Pdf Summary
The document is a collaborative medical correspondence discussing the treatment and outcomes of patients with rapidly progressive interstitial lung disease (RP-ILD), emphasizing the role of veno-venous extracorporeal membrane oxygenation (VV-ECMO). It begins by highlighting cases where viral superinfection exacerbated ILD in patients who were successfully weaned from VV-ECMO, suggesting the strategy could bridge recovery. The therapeutic regimens by Rubin et al. for RP-ILD patients have been deemed unusual, diverging from international guidelines, and relying on rituximab and IV immunoglobulins instead of the traditional cyclophosphamide. Novel treatments, including calcineurin inhibitors and Janus kinase inhibitors, show promise, especially for myositis-associated ILD linked to anti-MDA5 disease.<br /><br />The document suggests considering early lung transplantation in young patients not weanable from ECMO, although recognizing current treatment outcomes as unsatisfactory. It underscores that RP-ILD should not contraindicate VV-ECMO, advocating for immediate data to identify any need for early transplantation strategies in anti-MDA5 positive patients.<br /><br />Responding to feedback, Rubin et al. clarify several points from their studies, acknowledging the challenges associated with diagnosing RP-ILD and the absence of standard definitions and treatment protocols for myositis-associated ILD. They point out that the prognosis can vary based on myositis subtypes, and recognize the outdated nature of their treatment regimens. They advocate that, with improved diagnostics and treatments, ECMO should be considered for patients as a bridge to recovery or transplantation.<br /><br />Additionally, concerns regarding the management of infections in RP-ILD patients are raised, particularly the lack of emphasis on antibiotic treatments in existing protocols. Ensuring the utilization of prophylactic measures against infections is critical for managing patients with conditions like microscopic polyangiitis (MPA), who are at heightened risk for infections.
Keywords
RP-ILD
VV-ECMO
viral superinfection
rituximab
calcineurin inhibitors
Janus kinase inhibitors
lung transplantation
anti-MDA5
myositis-associated ILD
microscopic polyangiitis