title: Interstitial pneumonia with autoimmune features: Clinical, radiologic, and histological characteristics and outcome in a series of 57 patients.
abstract: 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.
  
  
  
  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).
  
  
  
  Retrospective analysis of consecutive patients in a single institution over a 3-year period.
  
  
  
  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.
  
  
  
  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.
authors.affiliation: Hospices Civils de Lyon, Groupe Hospitalier Est, Service de Pneumologie - Centre national de Référence des Maladies Pulmonaires Rares, Université de Lyon, Université Lyon I, UCBL-INRA, UMR754, Lyon, France.
authors.forename: Kais
authors.initials: K
authors.lastname: Ahmad
authors.affiliation: Hospices Civils de Lyon, Groupe Hospitalier Est, Service de Pneumologie - Centre national de Référence des Maladies Pulmonaires Rares, Université de Lyon, Université Lyon I, UCBL-INRA, UMR754, Lyon, France.
authors.forename: Thomas
authors.initials: T
authors.lastname: Barba
authors.affiliation: Hospices Civils de Lyon, Groupe Hospitalier Est, Service de Radiologie, Lyon, France.
authors.forename: Delphine
authors.initials: D
authors.lastname: Gamondes
authors.affiliation: Hospices Civils de Lyon, Groupe Hospitalier Est, Service de Pneumologie - Centre national de Référence des Maladies Pulmonaires Rares, Université de Lyon, Université Lyon I, UCBL-INRA, UMR754, Lyon, France.
authors.forename: Marylise
authors.initials: M
authors.lastname: Ginoux
authors.affiliation: Hospices Civils de Lyon, Groupe Hospitalier Est, Service de Pneumologie - Centre national de Référence des Maladies Pulmonaires Rares, Université de Lyon, Université Lyon I, UCBL-INRA, UMR754, Lyon, France.
authors.forename: Chahera
authors.initials: C
authors.lastname: Khouatra
authors.affiliation: Section of Respiratory Diseases, Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padua, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Spagnolo
authors.affiliation: Section of Pulmonary and Critical Care Medicine, Department of Medicine, University of Chicago, Chicago, IL, USA.
authors.forename: Mary
authors.initials: M
authors.lastname: Strek
authors.affiliation: Hospices Civils de Lyon, Groupe Hospitalier Est, Centre de Pathologie Est, Université de Lyon, Université Lyon I, Lyon, France.
authors.forename: Françoise
authors.initials: F
authors.lastname: Thivolet-Béjui
authors.affiliation: Hospices Civils de Lyon, Groupe Hospitalier Est, Service de Pneumologie - Centre national de Référence des Maladies Pulmonaires Rares, Université de Lyon, Université Lyon I, UCBL-INRA, UMR754, Lyon, France.
authors.forename: Julie
authors.initials: J
authors.lastname: Traclet
authors.affiliation: Hospices Civils de Lyon, Groupe Hospitalier Est, Service de Pneumologie - Centre national de Référence des Maladies Pulmonaires Rares, Université de Lyon, Université Lyon I, UCBL-INRA, UMR754, Lyon, France. Electronic address: vincent.cottin@chu-lyon.fr.
authors.forename: Vincent
authors.initials: V
authors.lastname: Cottin
mesh_terms: D000368:Aged; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis; D053208:Kaplan-Meier Estimate; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000473:pathology; D008297:Male; D020678:Microscopic Angioscopy; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed
keywords: Connected tissue disease; Idiopathic pulmonary fibrosis; Interstitial lung disease; Interstitial pneumonia