Interstitial Pneumonia with Autoimmune Features: Implications for Clinical Practice
Purpose of Review Interstitial pneumonia with autoimmune features (IPAF) is a research term that was proposed in 2015 to uniformly define patients with an idiopathic interstitial lung disease with subtle features of underlying autoimmunity. In this article, we reviewed studies that utilized IPAF cri...
Ausführliche Beschreibung
Autor*in: |
Cleland, Derrick [verfasserIn] |
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E-Artikel |
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Englisch |
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2022 |
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Anmerkung: |
© The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2022. Springer Nature or its licensor holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. |
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Übergeordnetes Werk: |
Enthalten in: Current respiratory care reports - New York, NY : Springer, 2012, 11(2022), 4 vom: 22. Okt., Seite 132-150 |
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Übergeordnetes Werk: |
volume:11 ; year:2022 ; number:4 ; day:22 ; month:10 ; pages:132-150 |
Links: |
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DOI / URN: |
10.1007/s13665-022-00296-4 |
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Katalog-ID: |
SPR048812773 |
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520 | |a Purpose of Review Interstitial pneumonia with autoimmune features (IPAF) is a research term that was proposed in 2015 to uniformly define patients with an idiopathic interstitial lung disease with subtle features of underlying autoimmunity. In this article, we reviewed studies that utilized IPAF criteria and examined the implications of this classification in clinical practice. Recent Findings There were significant differences in the clinical characteristics and outcomes of the IPAF cohorts suggesting that the interpretation and application of the IPAF criteria are variable. Despite these differences, numerous studies noted that a substantial number of patients with IPAF developed a definite connective tissue disease, and the survival rate for IPAF is likely in-between that of idiopathic pulmonary fibrosis and connective tissue disease–related interstitial lung diseases. Summary Further refinement of the IPAF criteria and larger prospective studies are needed to better understand the implications of IPAF in clinical practice. | ||
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10.1007/s13665-022-00296-4 doi (DE-627)SPR048812773 (SPR)s13665-022-00296-4-e DE-627 ger DE-627 rakwb eng Cleland, Derrick verfasserin aut Interstitial Pneumonia with Autoimmune Features: Implications for Clinical Practice 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2022. Springer Nature or its licensor holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. Purpose of Review Interstitial pneumonia with autoimmune features (IPAF) is a research term that was proposed in 2015 to uniformly define patients with an idiopathic interstitial lung disease with subtle features of underlying autoimmunity. In this article, we reviewed studies that utilized IPAF criteria and examined the implications of this classification in clinical practice. Recent Findings There were significant differences in the clinical characteristics and outcomes of the IPAF cohorts suggesting that the interpretation and application of the IPAF criteria are variable. Despite these differences, numerous studies noted that a substantial number of patients with IPAF developed a definite connective tissue disease, and the survival rate for IPAF is likely in-between that of idiopathic pulmonary fibrosis and connective tissue disease–related interstitial lung diseases. Summary Further refinement of the IPAF criteria and larger prospective studies are needed to better understand the implications of IPAF in clinical practice. Interstitial pneumonia with autoimmune features (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Connective tissue disease (dpeaa)DE-He213 Idiopathic pulmonary fibrosis (dpeaa)DE-He213 Sathananthan, Matheni aut Jeganathan, Niranjan (orcid)0000-0001-5533-5105 aut Enthalten in Current respiratory care reports New York, NY : Springer, 2012 11(2022), 4 vom: 22. Okt., Seite 132-150 (DE-627)718610954 (DE-600)2660484-X 2161-332X nnns volume:11 year:2022 number:4 day:22 month:10 pages:132-150 https://dx.doi.org/10.1007/s13665-022-00296-4 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_105 GBV_ILN_120 GBV_ILN_152 GBV_ILN_161 GBV_ILN_171 GBV_ILN_187 GBV_ILN_224 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2007 GBV_ILN_2009 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2059 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2111 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 AR 11 2022 4 22 10 132-150 |
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10.1007/s13665-022-00296-4 doi (DE-627)SPR048812773 (SPR)s13665-022-00296-4-e DE-627 ger DE-627 rakwb eng Cleland, Derrick verfasserin aut Interstitial Pneumonia with Autoimmune Features: Implications for Clinical Practice 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2022. Springer Nature or its licensor holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. Purpose of Review Interstitial pneumonia with autoimmune features (IPAF) is a research term that was proposed in 2015 to uniformly define patients with an idiopathic interstitial lung disease with subtle features of underlying autoimmunity. In this article, we reviewed studies that utilized IPAF criteria and examined the implications of this classification in clinical practice. Recent Findings There were significant differences in the clinical characteristics and outcomes of the IPAF cohorts suggesting that the interpretation and application of the IPAF criteria are variable. Despite these differences, numerous studies noted that a substantial number of patients with IPAF developed a definite connective tissue disease, and the survival rate for IPAF is likely in-between that of idiopathic pulmonary fibrosis and connective tissue disease–related interstitial lung diseases. Summary Further refinement of the IPAF criteria and larger prospective studies are needed to better understand the implications of IPAF in clinical practice. Interstitial pneumonia with autoimmune features (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Connective tissue disease (dpeaa)DE-He213 Idiopathic pulmonary fibrosis (dpeaa)DE-He213 Sathananthan, Matheni aut Jeganathan, Niranjan (orcid)0000-0001-5533-5105 aut Enthalten in Current respiratory care reports New York, NY : Springer, 2012 11(2022), 4 vom: 22. Okt., Seite 132-150 (DE-627)718610954 (DE-600)2660484-X 2161-332X nnns volume:11 year:2022 number:4 day:22 month:10 pages:132-150 https://dx.doi.org/10.1007/s13665-022-00296-4 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_105 GBV_ILN_120 GBV_ILN_152 GBV_ILN_161 GBV_ILN_171 GBV_ILN_187 GBV_ILN_224 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2007 GBV_ILN_2009 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2059 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2111 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 AR 11 2022 4 22 10 132-150 |
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10.1007/s13665-022-00296-4 doi (DE-627)SPR048812773 (SPR)s13665-022-00296-4-e DE-627 ger DE-627 rakwb eng Cleland, Derrick verfasserin aut Interstitial Pneumonia with Autoimmune Features: Implications for Clinical Practice 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2022. Springer Nature or its licensor holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. Purpose of Review Interstitial pneumonia with autoimmune features (IPAF) is a research term that was proposed in 2015 to uniformly define patients with an idiopathic interstitial lung disease with subtle features of underlying autoimmunity. In this article, we reviewed studies that utilized IPAF criteria and examined the implications of this classification in clinical practice. Recent Findings There were significant differences in the clinical characteristics and outcomes of the IPAF cohorts suggesting that the interpretation and application of the IPAF criteria are variable. Despite these differences, numerous studies noted that a substantial number of patients with IPAF developed a definite connective tissue disease, and the survival rate for IPAF is likely in-between that of idiopathic pulmonary fibrosis and connective tissue disease–related interstitial lung diseases. Summary Further refinement of the IPAF criteria and larger prospective studies are needed to better understand the implications of IPAF in clinical practice. Interstitial pneumonia with autoimmune features (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Connective tissue disease (dpeaa)DE-He213 Idiopathic pulmonary fibrosis (dpeaa)DE-He213 Sathananthan, Matheni aut Jeganathan, Niranjan (orcid)0000-0001-5533-5105 aut Enthalten in Current respiratory care reports New York, NY : Springer, 2012 11(2022), 4 vom: 22. Okt., Seite 132-150 (DE-627)718610954 (DE-600)2660484-X 2161-332X nnns volume:11 year:2022 number:4 day:22 month:10 pages:132-150 https://dx.doi.org/10.1007/s13665-022-00296-4 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_105 GBV_ILN_120 GBV_ILN_152 GBV_ILN_161 GBV_ILN_171 GBV_ILN_187 GBV_ILN_224 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2007 GBV_ILN_2009 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2059 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2111 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 AR 11 2022 4 22 10 132-150 |
allfieldsGer |
10.1007/s13665-022-00296-4 doi (DE-627)SPR048812773 (SPR)s13665-022-00296-4-e DE-627 ger DE-627 rakwb eng Cleland, Derrick verfasserin aut Interstitial Pneumonia with Autoimmune Features: Implications for Clinical Practice 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2022. Springer Nature or its licensor holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. Purpose of Review Interstitial pneumonia with autoimmune features (IPAF) is a research term that was proposed in 2015 to uniformly define patients with an idiopathic interstitial lung disease with subtle features of underlying autoimmunity. In this article, we reviewed studies that utilized IPAF criteria and examined the implications of this classification in clinical practice. Recent Findings There were significant differences in the clinical characteristics and outcomes of the IPAF cohorts suggesting that the interpretation and application of the IPAF criteria are variable. Despite these differences, numerous studies noted that a substantial number of patients with IPAF developed a definite connective tissue disease, and the survival rate for IPAF is likely in-between that of idiopathic pulmonary fibrosis and connective tissue disease–related interstitial lung diseases. Summary Further refinement of the IPAF criteria and larger prospective studies are needed to better understand the implications of IPAF in clinical practice. Interstitial pneumonia with autoimmune features (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Connective tissue disease (dpeaa)DE-He213 Idiopathic pulmonary fibrosis (dpeaa)DE-He213 Sathananthan, Matheni aut Jeganathan, Niranjan (orcid)0000-0001-5533-5105 aut Enthalten in Current respiratory care reports New York, NY : Springer, 2012 11(2022), 4 vom: 22. Okt., Seite 132-150 (DE-627)718610954 (DE-600)2660484-X 2161-332X nnns volume:11 year:2022 number:4 day:22 month:10 pages:132-150 https://dx.doi.org/10.1007/s13665-022-00296-4 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_105 GBV_ILN_120 GBV_ILN_152 GBV_ILN_161 GBV_ILN_171 GBV_ILN_187 GBV_ILN_224 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2007 GBV_ILN_2009 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2059 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2111 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 AR 11 2022 4 22 10 132-150 |
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10.1007/s13665-022-00296-4 doi (DE-627)SPR048812773 (SPR)s13665-022-00296-4-e DE-627 ger DE-627 rakwb eng Cleland, Derrick verfasserin aut Interstitial Pneumonia with Autoimmune Features: Implications for Clinical Practice 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2022. Springer Nature or its licensor holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. Purpose of Review Interstitial pneumonia with autoimmune features (IPAF) is a research term that was proposed in 2015 to uniformly define patients with an idiopathic interstitial lung disease with subtle features of underlying autoimmunity. In this article, we reviewed studies that utilized IPAF criteria and examined the implications of this classification in clinical practice. Recent Findings There were significant differences in the clinical characteristics and outcomes of the IPAF cohorts suggesting that the interpretation and application of the IPAF criteria are variable. Despite these differences, numerous studies noted that a substantial number of patients with IPAF developed a definite connective tissue disease, and the survival rate for IPAF is likely in-between that of idiopathic pulmonary fibrosis and connective tissue disease–related interstitial lung diseases. Summary Further refinement of the IPAF criteria and larger prospective studies are needed to better understand the implications of IPAF in clinical practice. Interstitial pneumonia with autoimmune features (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Connective tissue disease (dpeaa)DE-He213 Idiopathic pulmonary fibrosis (dpeaa)DE-He213 Sathananthan, Matheni aut Jeganathan, Niranjan (orcid)0000-0001-5533-5105 aut Enthalten in Current respiratory care reports New York, NY : Springer, 2012 11(2022), 4 vom: 22. Okt., Seite 132-150 (DE-627)718610954 (DE-600)2660484-X 2161-332X nnns volume:11 year:2022 number:4 day:22 month:10 pages:132-150 https://dx.doi.org/10.1007/s13665-022-00296-4 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_105 GBV_ILN_120 GBV_ILN_152 GBV_ILN_161 GBV_ILN_171 GBV_ILN_187 GBV_ILN_224 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2007 GBV_ILN_2009 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2059 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2111 GBV_ILN_2116 GBV_ILN_2118 GBV_ILN_2119 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 AR 11 2022 4 22 10 132-150 |
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Purpose of Review Interstitial pneumonia with autoimmune features (IPAF) is a research term that was proposed in 2015 to uniformly define patients with an idiopathic interstitial lung disease with subtle features of underlying autoimmunity. In this article, we reviewed studies that utilized IPAF criteria and examined the implications of this classification in clinical practice. Recent Findings There were significant differences in the clinical characteristics and outcomes of the IPAF cohorts suggesting that the interpretation and application of the IPAF criteria are variable. Despite these differences, numerous studies noted that a substantial number of patients with IPAF developed a definite connective tissue disease, and the survival rate for IPAF is likely in-between that of idiopathic pulmonary fibrosis and connective tissue disease–related interstitial lung diseases. Summary Further refinement of the IPAF criteria and larger prospective studies are needed to better understand the implications of IPAF in clinical practice. © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2022. Springer Nature or its licensor holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. |
abstractGer |
Purpose of Review Interstitial pneumonia with autoimmune features (IPAF) is a research term that was proposed in 2015 to uniformly define patients with an idiopathic interstitial lung disease with subtle features of underlying autoimmunity. In this article, we reviewed studies that utilized IPAF criteria and examined the implications of this classification in clinical practice. Recent Findings There were significant differences in the clinical characteristics and outcomes of the IPAF cohorts suggesting that the interpretation and application of the IPAF criteria are variable. Despite these differences, numerous studies noted that a substantial number of patients with IPAF developed a definite connective tissue disease, and the survival rate for IPAF is likely in-between that of idiopathic pulmonary fibrosis and connective tissue disease–related interstitial lung diseases. Summary Further refinement of the IPAF criteria and larger prospective studies are needed to better understand the implications of IPAF in clinical practice. © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2022. Springer Nature or its licensor holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. |
abstract_unstemmed |
Purpose of Review Interstitial pneumonia with autoimmune features (IPAF) is a research term that was proposed in 2015 to uniformly define patients with an idiopathic interstitial lung disease with subtle features of underlying autoimmunity. In this article, we reviewed studies that utilized IPAF criteria and examined the implications of this classification in clinical practice. Recent Findings There were significant differences in the clinical characteristics and outcomes of the IPAF cohorts suggesting that the interpretation and application of the IPAF criteria are variable. Despite these differences, numerous studies noted that a substantial number of patients with IPAF developed a definite connective tissue disease, and the survival rate for IPAF is likely in-between that of idiopathic pulmonary fibrosis and connective tissue disease–related interstitial lung diseases. Summary Further refinement of the IPAF criteria and larger prospective studies are needed to better understand the implications of IPAF in clinical practice. © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2022. Springer Nature or its licensor holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. |
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Despite these differences, numerous studies noted that a substantial number of patients with IPAF developed a definite connective tissue disease, and the survival rate for IPAF is likely in-between that of idiopathic pulmonary fibrosis and connective tissue disease–related interstitial lung diseases. 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7.398575 |