PMID- 28921478
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20201001
IS  - 2110-5820 (Print)
IS  - 2110-5820 (Electronic)
IS  - 2110-5820 (Linking)
VI  - 7
IP  - 1
DP  - 2017 Sep 18
TI  - Interstitial pneumonia with autoimmune features: an additional risk factor for 
      ARDS?
PG  - 98
LID - 10.1186/s13613-017-0320-3 [doi]
LID - 98
AB  - 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 49 days, range 10-88); four received extracorporeal membrane oxygenation 
      and one received low-flow extracorporeal CO(2) removal. 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.
FAU - Grasselli, Giacomo
AU  - Grasselli G
AUID- ORCID: 0000-0002-1735-1400
AD  - Dipartimento di Anestesia, Rianimazione ed Emergenza-Urgenza, Fondazione IRCCS 
      Ca' Granda, Ospedale Maggiore Policlinico, Via Francesco Sforza 35, 20122, Milan, 
      Italy. giacomo.grasselli@unimi.it.
FAU - Vergnano, Beatrice
AU  - Vergnano B
AD  - Dipartimento di Emergenza-Urgenza, Ospedale San Gerardo, Monza, Italy.
FAU - Pozzi, Maria Rosa
AU  - Pozzi MR
AD  - Dipartimento di Medicina, Unita Operativa di Reumatologia, Ospedale San Gerardo, 
      Monza, Italy.
FAU - Sala, Vittoria
AU  - Sala V
AD  - Dipartimento di Emergenza-Urgenza, Ospedale San Gerardo, Monza, Italy.
FAU - D'Andrea, Gabriele
AU  - D'Andrea G
AD  - Unita Operativa di Radiodiagnostica, Ospedale San Gerardo, Monza, Italy.
FAU - Scaravilli, Vittorio
AU  - Scaravilli V
AD  - Dipartimento di Anestesia, Rianimazione ed Emergenza-Urgenza, Fondazione IRCCS 
      Ca' Granda, Ospedale Maggiore Policlinico, Via Francesco Sforza 35, 20122, Milan, 
      Italy.
FAU - Mantero, Marco
AU  - Mantero M
AD  - Dipartimento di Fisiopatologia Medico Chirurgica e dei Trapianti, Universita 
      degli Studi di Milano, Milan, Italy.
FAU - Pesci, Alberto
AU  - Pesci A
AD  - Dipartimento di Medicina e Chirurgia, Universita Milano Bicocca, Monza, Italy.
AD  - Clinica Pneumologica, Ospedale San Gerardo, Monza, Italy.
FAU - Pesenti, Antonio
AU  - Pesenti A
AD  - Dipartimento di Anestesia, Rianimazione ed Emergenza-Urgenza, Fondazione IRCCS 
      Ca' Granda, Ospedale Maggiore Policlinico, Via Francesco Sforza 35, 20122, Milan, 
      Italy.
AD  - Dipartimento di Fisiopatologia Medico Chirurgica e dei Trapianti, Universita 
      degli Studi di Milano, Milan, Italy.
LA  - eng
PT  - Journal Article
DEP - 20170918
PL  - Germany
TA  - Ann Intensive Care
JT  - Annals of intensive care
JID - 101562873
PMC - PMC5603462
OTO - NOTNLM
OT  - ARDS
OT  - ECMO
OT  - Interstitial pneumonia with autoimmune features
OT  - Lung-dominant connective tissue disease
EDAT- 2017/09/19 06:00
MHDA- 2017/09/19 06:01
PMCR- 2017/09/18
CRDT- 2017/09/19 06:00
PHST- 2017/04/20 00:00 [received]
PHST- 2017/09/07 00:00 [accepted]
PHST- 2017/09/19 06:00 [entrez]
PHST- 2017/09/19 06:00 [pubmed]
PHST- 2017/09/19 06:01 [medline]
PHST- 2017/09/18 00:00 [pmc-release]
AID - 10.1186/s13613-017-0320-3 [pii]
AID - 320 [pii]
AID - 10.1186/s13613-017-0320-3 [doi]
PST - epublish
SO  - Ann Intensive Care. 2017 Sep 18;7(1):98. doi: 10.1186/s13613-017-0320-3.