Background: Interstitial pneumonia with autoimmune features (IPAF) identifies a recently recognized autoimmune syndrome characterized by interstitial lung disease and autoantibodies positivity, but absence of a specific connective tissue disease diagnosis or alternative etiology. We retrospectively reviewed the clinical presentation, diagnostic workup and management of seven critically ill patients who met diagnostic criteria for IPAF. We compared baseline characteristics and clinical outcome of IPAF patients with those of the population of ARDS patients admitted in the same period. Results: Seven consecutive patients with IPAF admitted to intensive care unit for acute respiratory distress syndrome (ARDS) were compared with 78 patients with ARDS secondary to a known risk factor and with eight ARDS patients without recognized risk factors. Five IPAF patients (71%) survived and were discharged alive from ICU: Their survival rate was equal to that of patients with a known risk factor (71%), while the subgroup of patients without risk factors had a markedly lower survival (38%). According to the Berlin definition criteria, ARDS was severe in four IPAF patients and moderate in the remaining three. All had multiple organ dysfunction at presentation. The most frequent autoantibody detected was anti-SSA/Ro52. All patients required prolonged mechanical ventilation (median duration 49Â days, range 10â88); four received extracorporeal membrane oxygenation and one received low-flow extracorporeal CO2removal. All patients received immunosuppressive therapy. Conclusions: This is the first description of a cohort of critical patients meeting the diagnostic criteria for IPAF presenting with ARDS. This diagnosis should be considered in any critically ill patient with interstitial lung disease of unknown origin. While management is challenging and level of support high, survival appears to be good and comparable to that of patients with ARDS associated with a known clinical insult.
Interstitial pneumonia with autoimmune features : An additional risk factor for ARDS? / G. Grasselli, B. Vergnano, M.R. Pozzi, V. Sala, G. D'Andrea, V. Scaravilli, M. Mantero, A. Pesci, A.M. Pesenti. - In: ANNALS OF INTENSIVE CARE. - ISSN 2110-5820. - 7:1(2017 Sep 18).
|Titolo:||Interstitial pneumonia with autoimmune features : An additional risk factor for ARDS?|
PESENTI, ANTONIO (Ultimo)
|Parole Chiave:||ARDS; ECMO; interstitial pneumonia with autoimmune features; lung-dominant connective tissue disease; critical care and intensive care medicine|
|Settore Scientifico Disciplinare:||Settore MED/41 - Anestesiologia|
|Data di pubblicazione:||18-set-2017|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.1186/s13613-017-0320-3|
|Appare nelle tipologie:||01 - Articolo su periodico|