PMID- 28137497 OWN - NLM STAT- MEDLINE DCOM- 20180108 LR - 20220321 IS - 1532-3064 (Electronic) IS - 0954-6111 (Linking) VI - 123 DP - 2017 Feb TI - Interstitial pneumonia with autoimmune features: Clinical, radiologic, and histological characteristics and outcome in a series of 57 patients. PG - 56-62 LID - S0954-6111(16)30280-3 [pii] LID - 10.1016/j.rmed.2016.10.017 [doi] AB - BACKGROUND: Interstitial pneumonia with autoimmune features (IPAF) has recently been defined by an international Taskforce to characterize interstitial lung disease associated with systemic manifestations limited to subtle serological and clinical autoimmune abnormalities and not fulfilling the international criteria for the diagnosis of a given connective tissue disease. OBJECTIVE: to report on a series of patients with IPAF, and to compare their outcome to that of a cohort of patients with idiopathic pulmonary fibrosis (IPF). METHODS: Retrospective analysis of consecutive patients in a single institution over a 3-year period. RESULTS: Out of 778 consecutive patients with interstitial lung disease, 55% had idiopathic interstitial pneumonia (including 20.1% with IPF), 21.5% had connective tissue disease, and 7.3% had IPAF. Patients (49% of females) had a mean FVC of 64% and a mean DLco of 49%. Serologic criteria for IPAF were the most frequent (93%), followed by "morphologic" criteria (79%), and clinical criteria (47%). Fifty three percent of patients had a NSIP pattern on CT. Nailfold capillaroscopy found giant capillaries in 13/30 patients tested (23%). No significant was found in overall survival between patients with IPAF and those with IPF. CONCLUSION: The recently defined criteria for IPAF are fulfilled by a significant proportion of patients referred for interstitial lung disease. As compared to those with IPF, patients with IPAF are more frequently females, have distinctive characteristics, have relatively frequent abnormalities at nailfold capillaroscopy, with no difference in age or in overall survival. Prospective studies are needed to guide the management of IPAF. CI - Copyright (c) 2016 Elsevier Ltd. All rights reserved. FAU - Ahmad, Kais AU - Ahmad K AD - Hospices Civils de Lyon, Groupe Hospitalier Est, Service de Pneumologie - Centre national de Reference des Maladies Pulmonaires Rares, Universite de Lyon, Universite Lyon I, UCBL-INRA, UMR754, Lyon, France. FAU - Barba, Thomas AU - Barba T AD - Hospices Civils de Lyon, Groupe Hospitalier Est, Service de Pneumologie - Centre national de Reference des Maladies Pulmonaires Rares, Universite de Lyon, Universite Lyon I, UCBL-INRA, UMR754, Lyon, France. FAU - Gamondes, Delphine AU - Gamondes D AD - Hospices Civils de Lyon, Groupe Hospitalier Est, Service de Radiologie, Lyon, France. FAU - Ginoux, Marylise AU - Ginoux M AD - Hospices Civils de Lyon, Groupe Hospitalier Est, Service de Pneumologie - Centre national de Reference des Maladies Pulmonaires Rares, Universite de Lyon, Universite Lyon I, UCBL-INRA, UMR754, Lyon, France. FAU - Khouatra, Chahera AU - Khouatra C AD - Hospices Civils de Lyon, Groupe Hospitalier Est, Service de Pneumologie - Centre national de Reference des Maladies Pulmonaires Rares, Universite de Lyon, Universite Lyon I, UCBL-INRA, UMR754, Lyon, France. FAU - Spagnolo, Paolo AU - Spagnolo P AD - Section of Respiratory Diseases, Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padua, Italy. FAU - Strek, Mary AU - Strek M AD - Section of Pulmonary and Critical Care Medicine, Department of Medicine, University of Chicago, Chicago, IL, USA. FAU - Thivolet-Bejui, Francoise AU - Thivolet-Bejui F AD - Hospices Civils de Lyon, Groupe Hospitalier Est, Centre de Pathologie Est, Universite de Lyon, Universite Lyon I, Lyon, France. FAU - Traclet, Julie AU - Traclet J AD - Hospices Civils de Lyon, Groupe Hospitalier Est, Service de Pneumologie - Centre national de Reference des Maladies Pulmonaires Rares, Universite de Lyon, Universite Lyon I, UCBL-INRA, UMR754, Lyon, France. FAU - Cottin, Vincent AU - Cottin V AD - Hospices Civils de Lyon, Groupe Hospitalier Est, Service de Pneumologie - Centre national de Reference des Maladies Pulmonaires Rares, Universite de Lyon, Universite Lyon I, UCBL-INRA, UMR754, Lyon, France. Electronic address: vincent.cottin@chu-lyon.fr. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20161031 PL - England TA - Respir Med JT - Respiratory medicine JID - 8908438 SB - IM CIN - Respir Med. 2017 Jun;127:65-66. PMID: 28285991 CIN - Respir Med. 2017 Jun;127:67. PMID: 28416271 MH - Aged MH - Autoimmune Diseases/*diagnosis/pathology MH - Diagnosis, Differential MH - Female MH - Humans MH - Idiopathic Pulmonary Fibrosis/diagnosis MH - Kaplan-Meier Estimate MH - Lung Diseases, Interstitial/*diagnosis/pathology MH - Male MH - Microscopic Angioscopy MH - Middle Aged MH - Prognosis MH - Retrospective Studies MH - Severity of Illness Index MH - Tomography, X-Ray Computed OTO - NOTNLM OT - Connected tissue disease OT - Idiopathic pulmonary fibrosis OT - Interstitial lung disease OT - Interstitial pneumonia EDAT- 2017/02/01 06:00 MHDA- 2018/01/09 06:00 CRDT- 2017/02/01 06:00 PHST- 2016/06/22 00:00 [received] PHST- 2016/09/23 00:00 [revised] PHST- 2016/10/24 00:00 [accepted] PHST- 2017/02/01 06:00 [entrez] PHST- 2017/02/01 06:00 [pubmed] PHST- 2018/01/09 06:00 [medline] AID - S0954-6111(16)30280-3 [pii] AID - 10.1016/j.rmed.2016.10.017 [doi] PST - ppublish SO - Respir Med. 2017 Feb;123:56-62. doi: 10.1016/j.rmed.2016.10.017. Epub 2016 Oct 31.