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Published on: April 10, 2019
Efficacy of plasma exchange in patients with anti-MDA5 rapidly progressive interstitial lung disease
Pierre Bay1, Marc Pineton de Chambrun2, Vincent Rothstein3
1Université Sorbonne Paris Nord, Assistance Publique-Hôpitaux de Paris, Hôpital Avicenne, Service de Pneumologie, Bobigny, France; Service de Médecine Intensive Réanimation, Hôpitaux Universitaires Henri Mondor - Albert Chenevier, Assistance Publique - Hôpitaux de Paris (AP-HP), Créteil, France.
Background:
Rapidly progressive interstitial lung disease (RP-ILD) is a frequent and severe manifestation of anti-MDA5 dermatomyositis (MDA5-DM) associated with poor outcome. The optimal treatment regimen for MDA5-DM RP-ILD is yet to be determined. Specifically, the value of adding plasma exchange (PLEX) to corticosteroids and immunosuppressants remains unclear. We aimed to evaluate the effect of PLEX on the outcome of patients with MDA5-DM RP-ILD.
Methods:
This French nationwide multicentre retrospective study included all MDA5-DM RP-ILD patients from 2012 to 2021 admitted to 18 centres. The primary endpoint was one-year transplant-free survival.
Results:
51 patients with MDA5-DM RP-ILD (female 67%; mean age at disease onset: 51 ± 11.6 years) were included. Thirty-two (63%) patients required mechanical ventilation and twenty-five (49%) received PLEX. One-year mortality or lung transplant occurred in 63% cases after a median follow-up of 77 [38-264] days. The Cox proportional hazards multivariable model only retained mechanical ventilation but not PLEX (p = 0.7) as independent predictor of the primary endpoint. One-year transplant-free survival rates in PLEX + vs. PLEX-were 20% vs. 54% (p = 0.01), respectively. The Kaplan-Meier estimated probabilities of one-year transplant-free survival was statistically higher in PLEX-compared to PLEX + patients (p = 0.05). PLEX + compared to PLEX-patients more frequently received mechanical ventilation and immunosuppressants suggesting PLEX + patients had a more severe disease.
Conclusion:
MDA5-DM RP-ILD is associated with poor rate of one-year transplant-free survival. The use of PLEX was not associated with a better outcome albeit they were mainly given to more severe patients. While our study reports the largest series of MDA5-DM RP-ILD given PLEX, these results needs to be interpreted with caution owing the numerous selection, indication and interpretation bias. Further studies are needed to evaluate their efficacy in this setting.
Insights
Plasma exchange (PLEX) did not improve one-year survival for rapidly progressive interstitial lung disease (RP-ILD) in anti-MDA5 dermatomyositis patients. Patients receiving PLEX had worse outcomes, suggesting it may be used in more severe cases.
Area of Science:
- Rheumatology
- Pulmonology
- Critical Care Medicine
Background:
- Rapidly progressive interstitial lung disease (RP-ILD) is a severe complication of anti-MDA5 dermatomyositis (MDA5-DM).
- Optimal treatment for MDA5-DM RP-ILD is unclear, particularly the role of plasma exchange (PLEX).
Purpose of the Study:
- To evaluate the effect of plasma exchange (PLEX) on outcomes in patients with anti-MDA5 dermatomyositis-associated RP-ILD.
- To determine if PLEX improves one-year transplant-free survival in this patient population.
Main Methods:
- A French nationwide retrospective study included 51 patients with MDA5-DM RP-ILD from 2012 to 2021.
- The primary endpoint was one-year transplant-free survival.
- Cox proportional hazards models and Kaplan-Meier survival analysis were used to assess the impact of PLEX.
Main Results:
- One-year transplant-free survival was 20% for patients receiving PLEX versus 54% for those not receiving PLEX (p=0.01).
- Mechanical ventilation, but not PLEX, was an independent predictor of mortality.
- Patients receiving PLEX were more likely to require mechanical ventilation, indicating more severe disease.
Conclusions:
- Plasma exchange (PLEX) was not associated with improved one-year transplant-free survival in MDA5-DM RP-ILD patients.
- PLEX was administered to more severely ill patients, complicating the interpretation of its efficacy.
- Further research is necessary to clarify the role and efficacy of PLEX in managing MDA5-DM RP-ILD.
