PMID- 27692137
OWN - NLM
STAT- MEDLINE
DCOM- 20180116
LR  - 20220330
IS  - 1532-3064 (Electronic)
IS  - 0954-6111 (Linking)
VI  - 119
DP  - 2016 Oct
TI  - Clinical features and natural history of interstitial pneumonia with autoimmune 
      features: A single center experience.
PG  - 150-154
LID - S0954-6111(16)30225-6 [pii]
LID - 10.1016/j.rmed.2016.09.002 [doi]
AB  - OBJECTIVE: To describe the clinical phenotype and natural history of a cohort of 
      patients with interstitial pneumonia with autoimmune features (IPAF). METHODS: A 
      retrospective, single center study of 56 patients with IPAF evaluated between 
      February 2008 and August 2014. All clinical data were extracted from the 
      electronic medical record and longitudinal changes in forced vital capacity (FVC) 
      were analyzed with mixed-effects, piecewise linear regression models that 
      considered time as a continuous factor. RESULTS: All patients fulfilled 
      classification criteria for IPAF. The majority were women (71%) and never smokers 
      (68%). The most frequently identified clinical features were Raynaud's phenomenon 
      (39%), distal digital fissuring (29%), Gottron's sign (18%) and inflammatory 
      arthropathy (16%). The most frequently identified serologies were antinuclear 
      antibody (ANA) (48%), anti-Ro (SSA) (43%) and anti-tRNA-synthetase antibodies 
      (36%). Nonspecific interstitial pneumonia (NSIP) (57.1%) followed by NSIP with 
      organizing pneumonia (18%) were the most common radiologic patterns, while usual 
      interstitial pneumonia was identified in only 9%. All but one patient was treated 
      with immunosuppression: prednisone (82%) and mycophenolate mofetil (76%) were the 
      most frequently used agents. During a follow-up period of 284.9 +/- 141.3 days, 
      modeled longitudinal FVC% was stable (slope = 0.69/year) and no deaths were 
      observed in the cohort. CONCLUSIONS: In this single center study, patients with 
      IPAF were predominately non-smoking women with high-resolution computed 
      tomography scans that suggested NSIP. Their pulmonary physiology was stable, and 
      during limited follow-up, no deaths were observed. Prospective and multi-center 
      studies are needed to better inform our understanding of IPAF.
CI  - Copyright (c) 2016 Elsevier Ltd. All rights reserved.
FAU - Chartrand, Sandra
AU  - Chartrand S
AD  - Department of Medicine, Hopital Maisonneuve-Rosemont affiliated to Universite de 
      Montreal, Montreal, Quebec, Canada.
FAU - Swigris, Jeffrey J
AU  - Swigris JJ
AD  - Department of Medicine, National Jewish Health, Denver, CO, USA; Department of 
      Medicine, University of Colorado School of Medicine, Aurora, CO, USA.
FAU - Stanchev, Lina
AU  - Stanchev L
AD  - Department of Medicine, University of Colorado School of Medicine, Aurora, CO, 
      USA.
FAU - Lee, Joyce S
AU  - Lee JS
AD  - Department of Medicine, University of Colorado School of Medicine, Aurora, CO, 
      USA.
FAU - Brown, Kevin K
AU  - Brown KK
AD  - Department of Medicine, National Jewish Health, Denver, CO, USA; Department of 
      Medicine, University of Colorado School of Medicine, Aurora, CO, USA.
FAU - Fischer, Aryeh
AU  - Fischer A
AD  - Department of Medicine, University of Colorado School of Medicine, Aurora, CO, 
      USA. Electronic address: aryeh.fischer@ucdenver.edu.
LA  - eng
PT  - Journal Article
DEP - 20160903
PL  - England
TA  - Respir Med
JT  - Respiratory medicine
JID - 8908438
RN  - 0 (Adrenal Cortex Hormones)
RN  - 0 (Antibodies, Antinuclear)
RN  - 0 (Enzyme Inhibitors)
RN  - 0 (Immunosuppressive Agents)
RN  - 7U1EE4V452 (Carbon Monoxide)
RN  - EC 6.1.1.- (Amino Acyl-tRNA Synthetases)
RN  - HU9DX48N0T (Mycophenolic Acid)
SB  - IM
CIN - Respir Med. 2017 Nov;132:265-266. PMID: 28385573
CIN - Respir Med. 2017 Nov;132:267. PMID: 28760577
MH  - Adrenal Cortex Hormones/therapeutic use
MH  - Adult
MH  - Amino Acyl-tRNA Synthetases/*antagonists & inhibitors/immunology
MH  - Antibodies, Antinuclear
MH  - Autoimmune Diseases/diagnosis/immunology
MH  - Biopsy
MH  - Carbon Monoxide/metabolism
MH  - Connective Tissue Diseases/diagnosis/pathology
MH  - Diagnosis, Differential
MH  - Enzyme Inhibitors/therapeutic use
MH  - Female
MH  - Humans
MH  - Idiopathic Interstitial Pneumonias/diagnosis/pathology
MH  - Immunosuppressive Agents/therapeutic use
MH  - Lung/*diagnostic imaging/pathology/physiopathology
MH  - Lung Diseases, Interstitial/*diagnosis/drug therapy/pathology/physiopathology
MH  - Male
MH  - Middle Aged
MH  - Mycophenolic Acid/therapeutic use
MH  - Phenotype
MH  - Pulmonary Diffusing Capacity/physiology
MH  - Respiratory Function Tests/methods
MH  - Retrospective Studies
MH  - Tomography, X-Ray Computed
MH  - Vital Capacity/physiology
OTO - NOTNLM
OT  - Connective tissue disease
OT  - Idiopathic interstitial pneumonia
OT  - Interstitial lung disease
OT  - Interstitial pneumonia with autoimmune features
EDAT- 2016/10/04 06:00
MHDA- 2018/01/18 06:00
CRDT- 2016/10/04 06:00
PHST- 2016/05/24 00:00 [received]
PHST- 2016/09/01 00:00 [accepted]
PHST- 2016/10/04 06:00 [entrez]
PHST- 2016/10/04 06:00 [pubmed]
PHST- 2018/01/18 06:00 [medline]
AID - S0954-6111(16)30225-6 [pii]
AID - 10.1016/j.rmed.2016.09.002 [doi]
PST - ppublish
SO  - Respir Med. 2016 Oct;119:150-154. doi: 10.1016/j.rmed.2016.09.002. Epub 2016 Sep 
      3.