PMID- 31838159
OWN - NLM
STAT- MEDLINE
DCOM- 20200228
LR  - 20200228
IS  - 1873-0183 (Electronic)
IS  - 1568-9972 (Linking)
VI  - 19
IP  - 2
DP  - 2020 Feb
TI  - Interstitial pneumonia with autoimmune features: A single center prospective 
      follow-up study.
PG  - 102451
LID - S1568-9972(19)30264-2 [pii]
LID - 10.1016/j.autrev.2019.102451 [doi]
AB  - BACKGROUND AND OBJECTIVE: Recently the term "interstitial pneumonia with 
      autoimmune features" (IPAF) has been proposed to identify patients with 
      interstitial lung disease and autoimmune characteristics, not fulfilling the 
      criteria for specific connective tissue diseases (CTD). Only few data are 
      available about the clinical and serological features of IPAF patients, their 
      survival and the possible evolution in a CTD. The aims of the study were to 
      investigate the demographic and clinico-serologic features of patients with IPAF, 
      their relationship to survival, and the possible evolution in a definite CTD. 
      PATIENTS AND METHODS: Fifty-two patients were consecutively enrolled and 
      prospectively followed for 45 +/- 31.6 months. Data about disease onset, 
      serological, clinical and therapeutic features, pulmonary function tests and 
      high-resolution computed tomography were periodically repeated. The survival of 
      patients with IPAF was compared with that of 104 patients with idiopathic 
      pulmonary fibrosis (IPF). RESULTS: The clinical domain for IPAF was satisfied in 
      44 patients, serological domain in 49 and the morphological domain in 29 
      patients. During the follow-up, a definite CTD was diagnosed in 7 patients, in 
      particular Sjogren's syndrome in 4 patients, rheumatoid arthritis in 2, and 
      polymyositis in the last. The estimated 5-year survival of IPAF patients 
      69.5 +/- 7.8%, significantly higher than survival observed in IPF patients, and the 
      baseline value of FVC and DLCO were the only factors associated to death. 
      CONCLUSIONS: IPAF seems to a distinct entity, with a low tendency to evolve in a 
      definite CTD. Nevertheless, further studies are needed to better define the 
      clinical evolution and the outcome of IPAF.
CI  - Copyright (c) 2019. Published by Elsevier B.V.
FAU - Sebastiani, Marco
AU  - Sebastiani M
AD  - Chair and Rheumatology Unit, University of Modena and Reggio Emilia, Azienda 
      Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy.
FAU - Cassone, Giulia
AU  - Cassone G
AD  - Chair and Rheumatology Unit, University of Modena and Reggio Emilia, Azienda 
      Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy; Clinical and 
      Experimental Medicine PhD Program, University of Modena and Reggio Emilia, 
      Modena, Italy; Rheumatology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio 
      Emilia, Italy.
FAU - De Pasquale, Lisa
AU  - De Pasquale L
AD  - Chair and Rheumatology Unit, University of Modena and Reggio Emilia, Azienda 
      Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy.
FAU - Cerri, Stefania
AU  - Cerri S
AD  - Respiratory Disease Unit, University of Modena and Reggio Emilia, Azienda 
      Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy.
FAU - Della Casa, Giovanni
AU  - Della Casa G
AD  - Radiology Unit, University of Modena and Reggio Emilia, Azienda 
      Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy.
FAU - Vacchi, Caterina
AU  - Vacchi C
AD  - Chair and Rheumatology Unit, University of Modena and Reggio Emilia, Azienda 
      Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy.
FAU - Luppi, Fabrizio
AU  - Luppi F
AD  - Respiratory Disease Unit, University of Modena and Reggio Emilia, Azienda 
      Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy.
FAU - Salvarani, Carlo
AU  - Salvarani C
AD  - Chair and Rheumatology Unit, University of Modena and Reggio Emilia, Azienda 
      Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy; Rheumatology 
      Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy. Electronic 
      address: carlo.salvarani@unimore.it.
FAU - Manfredi, Andreina
AU  - Manfredi A
AD  - Chair and Rheumatology Unit, University of Modena and Reggio Emilia, Azienda 
      Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy.
LA  - eng
PT  - Journal Article
DEP - 20191212
PL  - Netherlands
TA  - Autoimmun Rev
JT  - Autoimmunity reviews
JID - 101128967
SB  - IM
MH  - Aged
MH  - Autoimmune Diseases/*complications/pathology
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Idiopathic Pulmonary Fibrosis/*complications/pathology
MH  - Lung Diseases, Interstitial/*complications/pathology
MH  - Male
MH  - Prospective Studies
OTO - NOTNLM
OT  - Connective tissue diseases (CTD)
OT  - Idiopathic pulmonary fibrosis (IPF)
OT  - Interstitial lung disease (ILD)
OT  - Interstitial pneumonia with autoimmune features (IPAF)
OT  - Survival
EDAT- 2019/12/16 06:00
MHDA- 2020/02/29 06:00
CRDT- 2019/12/16 06:00
PHST- 2019/08/17 00:00 [received]
PHST- 2019/08/22 00:00 [accepted]
PHST- 2019/12/16 06:00 [pubmed]
PHST- 2020/02/29 06:00 [medline]
PHST- 2019/12/16 06:00 [entrez]
AID - S1568-9972(19)30264-2 [pii]
AID - 10.1016/j.autrev.2019.102451 [doi]
PST - ppublish
SO  - Autoimmun Rev. 2020 Feb;19(2):102451. doi: 10.1016/j.autrev.2019.102451. Epub 
      2019 Dec 12.