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Characterisation of patients with interstitial pneumonia with autoimmune features

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image:thewellnessolutions.com

Justin M. Oldham1,8, Ayodeji Adegunsoye2,8, Eleanor Valenzi3, Cathryn Lee3, Leah Witt2, Lena Chen2, Aliya N. Husain4, Steven Montner5, Jonathan H. Chung5, Vincent Cottin6, Aryeh Fischer7, Imre Noth2, Rekha Vij2,9 and Mary E. Strek2,9
1Dept of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, The University of California at Davis 2Dept of Medicine, Section of Pulmonary and Critical Care Medicine, The University of Chicago, Chicago, IL, USA 3Dept of Medicine, The University of Chicago, Chicago, IL, USA4Dept of Pathology, The University of Chicago, Chicago, IL, USA5Dept of Radiology, The University of Chicago, Chicago, IL, USA6Dept of Respiratory Medicine, Louis Pradel Hospital, Hospices Civils de Lyon, University Claude Bernard Lyon, Lyon, France7Dept of Medicine, Division of Rheumatology, University of Colorado, Denver, CO, USA8These authors contributed equally 9These authors contributed equally


Patients with interstitial lung disease (ILD) may have features of connective tissue disease (CTD), but lack findings diagnostic of a specific CTD. A recent European Respiratory Society/American Thoracic Society research statement proposed criteria for patients with interstitial pneumonia with autoimmune features (IPAF).

We applied IPAF criteria to patients with idiopathic interstitial pneumonia and undifferentiated CTD-ILD (UCTD). We then characterised the clinical, serological and morphological features of the IPAF cohort, compared outcomes to other ILD cohorts and validated individual IPAF domains using survival as an endpoint.

Of 422 patients, 144 met IPAF criteria. Mean age was 63.2 years with a slight female predominance. IPAF cohort survival was marginally better than patients with idiopathic pulmonary fibrosis, but worse than CTD-ILD. A non-usual interstitial pneumonia pattern was associated with improved survival, as was presence of the clinical domain. A modified IPAF cohort of those meeting the clinical domain and a radiographic or histological feature within the morphological domain displayed survival similar to those with CTD-ILD.

IPAF is common among patients with idiopathic interstitial pneumonia and UCTD. Specific IPAF features can identify subgroups with differential survival. Further research is needed to replicate these findings and determine whether patients meeting IPAF criteria benefit from immunosuppressive therapy.



Source : http://erj.ersjournals.com/content/47/6/1767
Image : http://www.thewellnessolutions.com/wp-content/uploads/2016/05/2015_prot_autoimmune_hero.jpg


PDPI Sulawesi Selatan & Utara. 11/08/17.



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