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State of the art in interstitial pneumonia with autoimmune features: a systematic review on retrospective studies and suggestions for further advances

Gianluca Sambataro, Domenico Sambataro, Sebastiano Emanuele Torrisi, Ada Vancheri, Mauro Pavone, Roberta Rosso, Matteo Schisano, Claudia Crimi, Francesca Pignataro, Aryeh Fischer, Nicoletta Del Papa, Carlo Vancheri
European Respiratory Review 2018 27: 170139; DOI: 10.1183/16000617.0139-2017
Gianluca Sambataro
1Regional Referral Centre for Rare Lung Diseases, A.O.U. “Policlinico-Vittorio Emanuele” Dept of Clinical and Experimental Medicine, University of Catania, Catania, Italy
2Rheumatology Outpatients, accredited with the National Health System, Artroreuma srl, Mascalucia, CT, Italy
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Domenico Sambataro
2Rheumatology Outpatients, accredited with the National Health System, Artroreuma srl, Mascalucia, CT, Italy
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Sebastiano Emanuele Torrisi
1Regional Referral Centre for Rare Lung Diseases, A.O.U. “Policlinico-Vittorio Emanuele” Dept of Clinical and Experimental Medicine, University of Catania, Catania, Italy
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Ada Vancheri
1Regional Referral Centre for Rare Lung Diseases, A.O.U. “Policlinico-Vittorio Emanuele” Dept of Clinical and Experimental Medicine, University of Catania, Catania, Italy
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Mauro Pavone
1Regional Referral Centre for Rare Lung Diseases, A.O.U. “Policlinico-Vittorio Emanuele” Dept of Clinical and Experimental Medicine, University of Catania, Catania, Italy
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Roberta Rosso
1Regional Referral Centre for Rare Lung Diseases, A.O.U. “Policlinico-Vittorio Emanuele” Dept of Clinical and Experimental Medicine, University of Catania, Catania, Italy
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Matteo Schisano
1Regional Referral Centre for Rare Lung Diseases, A.O.U. “Policlinico-Vittorio Emanuele” Dept of Clinical and Experimental Medicine, University of Catania, Catania, Italy
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Claudia Crimi
1Regional Referral Centre for Rare Lung Diseases, A.O.U. “Policlinico-Vittorio Emanuele” Dept of Clinical and Experimental Medicine, University of Catania, Catania, Italy
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Francesca Pignataro
3Day Hospital of Rheumatology, Dept of Rheumatology, ASST G. Pini-CTO, Milan, Italy
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Aryeh Fischer
4Dept of Medicine, University of Colorado School of Medicine, Denver, CO, USA
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Nicoletta Del Papa
3Day Hospital of Rheumatology, Dept of Rheumatology, ASST G. Pini-CTO, Milan, Italy
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Carlo Vancheri
1Regional Referral Centre for Rare Lung Diseases, A.O.U. “Policlinico-Vittorio Emanuele” Dept of Clinical and Experimental Medicine, University of Catania, Catania, Italy
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Abstract

The term interstitial pneumonia with autoimmune features (IPAF) has been proposed to define patients with interstitial lung disease (ILD) associated with autoimmune signs not classifiable for connective tissue diseases (CTDs). This new definition overcomes previous nomenclatures and provides a uniform structure for prospective studies through specific classification criteria.

This work evaluates the characteristics of IPAF patients reported in the literature, to highlight potential limits through a comparative analysis and to suggest better performing classification criteria.

Four retrospective studies on the IPAF population have been considered. The study subjects differed in age, sex, smoking habit, ILD pattern and outcomes. Another important difference lies in the diverse items considered in the classification criteria. The retrospective design of the studies and the absence from some of them of a rheumatologist clearly involved in the diagnosis may have influenced the data, but current IPAF criteria seem to include a rather heterogeneous population. To overcome these discrepancies, this review suggests a limitation in the use of single items and the exclusion of extremely specific CTD criteria. This should avoid the definition of IPAF for those diseases at different stages or at early onset. The investigation of a functional or morphological cut-off of pulmonary involvement would be useful.

Abstract

IPAF retrospective cohorts show numerous differences between them. We propose some ideas to improve IPAF criteria http://ow.ly/eubC30jlGJO

Footnotes

  • Provenance: Submitted article, peer reviewed.

  • Conflict of interest: C. Vancheri reports grants and personal fees from Roche and Boehringer Ingelheim, outside the submitted work.

  • Conflict of interest: A. Fischer reports personal fees (consultancy and steering committee) from Boehringer Ingelheim and F Hoffman La Roche, during the conduct of the study.

  • Received December 22, 2017.
  • Accepted March 29, 2018.
  • Copyright ©ERS 2018.

ERR articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.

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Vol 27 Issue 148 Table of Contents
European Respiratory Review: 27 (148)
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State of the art in interstitial pneumonia with autoimmune features: a systematic review on retrospective studies and suggestions for further advances
Gianluca Sambataro, Domenico Sambataro, Sebastiano Emanuele Torrisi, Ada Vancheri, Mauro Pavone, Roberta Rosso, Matteo Schisano, Claudia Crimi, Francesca Pignataro, Aryeh Fischer, Nicoletta Del Papa, Carlo Vancheri
European Respiratory Review Jun 2018, 27 (148) 170139; DOI: 10.1183/16000617.0139-2017

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State of the art in interstitial pneumonia with autoimmune features: a systematic review on retrospective studies and suggestions for further advances
Gianluca Sambataro, Domenico Sambataro, Sebastiano Emanuele Torrisi, Ada Vancheri, Mauro Pavone, Roberta Rosso, Matteo Schisano, Claudia Crimi, Francesca Pignataro, Aryeh Fischer, Nicoletta Del Papa, Carlo Vancheri
European Respiratory Review Jun 2018, 27 (148) 170139; DOI: 10.1183/16000617.0139-2017
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