Interstitial Pneumonia with Autoimmune Features: 8 Years after Nomenclature and Classification—Where are We Not and Where Are We Headed?
Purpose of Review The term interstitial pneumonia with autoimmune features (IPAF) was proposed by an international task force as a research classification to standardize nomenclature for patients with idiopathic interstitial pneumonia (IIP) and features of connective tissue disease (CTD). This revie...
Ausführliche Beschreibung
Gespeichert in:
| Autor*in: |
Kulkarni, Tejaswini [verfasserIn] |
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| Format: |
E-Artikel |
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| Sprache: |
Englisch |
| Erschienen: |
2023 |
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| Schlagwörter: |
Interstitial pneumonia with autoimmune features |
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| Anmerkung: |
© The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2023. Springer Nature or its licensor (e.g. a society or other partner) 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, 12(2023), 3 vom: 16. Mai, Seite 144-150 |
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| Übergeordnetes Werk: |
volume:12 ; year:2023 ; number:3 ; day:16 ; month:05 ; pages:144-150 |
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| DOI / URN: |
10.1007/s13665-023-00312-1 |
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| Katalog-ID: |
SPR052580725 |
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| 520 | |a Purpose of Review The term interstitial pneumonia with autoimmune features (IPAF) was proposed by an international task force as a research classification to standardize nomenclature for patients with idiopathic interstitial pneumonia (IIP) and features of connective tissue disease (CTD). This review aims to discuss the advances made in understanding this research classification and the clinical implications of this term. Recent Findings Multiple cohort studies have described the clinical presentation of patients meeting criteria for IPAF with heterogeneity noted particularly in the morphologic domain as compared to clinical and serologic domains. Treating these patients according to their clinical presentation and features remains the key expert opinion given the paucity of data to inform therapeutic strategies in IPAF. Summary There are still several challenges and unresolved questions which preclude the application of IPAF as a clinical diagnosis. Whether IPAF represents a clinical diagnosis distinct from CTD-ILD or IIPs or precursor or an overlap of these conditions remains controversial and warrants further investigation. | ||
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10.1007/s13665-023-00312-1 doi (DE-627)SPR052580725 (SPR)s13665-023-00312-1-e DE-627 ger DE-627 rakwb eng Kulkarni, Tejaswini verfasserin (orcid)0000-0002-4251-4988 aut Interstitial Pneumonia with Autoimmune Features: 8 Years after Nomenclature and Classification—Where are We Not and Where Are We Headed? 2023 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 2023. Springer Nature or its licensor (e.g. a society or other partner) 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 The term interstitial pneumonia with autoimmune features (IPAF) was proposed by an international task force as a research classification to standardize nomenclature for patients with idiopathic interstitial pneumonia (IIP) and features of connective tissue disease (CTD). This review aims to discuss the advances made in understanding this research classification and the clinical implications of this term. Recent Findings Multiple cohort studies have described the clinical presentation of patients meeting criteria for IPAF with heterogeneity noted particularly in the morphologic domain as compared to clinical and serologic domains. Treating these patients according to their clinical presentation and features remains the key expert opinion given the paucity of data to inform therapeutic strategies in IPAF. Summary There are still several challenges and unresolved questions which preclude the application of IPAF as a clinical diagnosis. Whether IPAF represents a clinical diagnosis distinct from CTD-ILD or IIPs or precursor or an overlap of these conditions remains controversial and warrants further investigation. Interstitial pneumonia with autoimmune features (dpeaa)DE-He213 Antifibrotics (dpeaa)DE-He213 Immunosuppression (dpeaa)DE-He213 Idiopathic interstitial pneumonia (dpeaa)DE-He213 Connective tissue disease (dpeaa)DE-He213 Enthalten in Current respiratory care reports New York, NY : Springer, 2012 12(2023), 3 vom: 16. Mai, Seite 144-150 (DE-627)718610954 (DE-600)2660484-X 2161-332X nnns volume:12 year:2023 number:3 day:16 month:05 pages:144-150 https://dx.doi.org/10.1007/s13665-023-00312-1 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER 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 12 2023 3 16 05 144-150 |
| spelling |
10.1007/s13665-023-00312-1 doi (DE-627)SPR052580725 (SPR)s13665-023-00312-1-e DE-627 ger DE-627 rakwb eng Kulkarni, Tejaswini verfasserin (orcid)0000-0002-4251-4988 aut Interstitial Pneumonia with Autoimmune Features: 8 Years after Nomenclature and Classification—Where are We Not and Where Are We Headed? 2023 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 2023. Springer Nature or its licensor (e.g. a society or other partner) 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 The term interstitial pneumonia with autoimmune features (IPAF) was proposed by an international task force as a research classification to standardize nomenclature for patients with idiopathic interstitial pneumonia (IIP) and features of connective tissue disease (CTD). This review aims to discuss the advances made in understanding this research classification and the clinical implications of this term. Recent Findings Multiple cohort studies have described the clinical presentation of patients meeting criteria for IPAF with heterogeneity noted particularly in the morphologic domain as compared to clinical and serologic domains. Treating these patients according to their clinical presentation and features remains the key expert opinion given the paucity of data to inform therapeutic strategies in IPAF. Summary There are still several challenges and unresolved questions which preclude the application of IPAF as a clinical diagnosis. Whether IPAF represents a clinical diagnosis distinct from CTD-ILD or IIPs or precursor or an overlap of these conditions remains controversial and warrants further investigation. Interstitial pneumonia with autoimmune features (dpeaa)DE-He213 Antifibrotics (dpeaa)DE-He213 Immunosuppression (dpeaa)DE-He213 Idiopathic interstitial pneumonia (dpeaa)DE-He213 Connective tissue disease (dpeaa)DE-He213 Enthalten in Current respiratory care reports New York, NY : Springer, 2012 12(2023), 3 vom: 16. Mai, Seite 144-150 (DE-627)718610954 (DE-600)2660484-X 2161-332X nnns volume:12 year:2023 number:3 day:16 month:05 pages:144-150 https://dx.doi.org/10.1007/s13665-023-00312-1 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER 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 12 2023 3 16 05 144-150 |
| allfields_unstemmed |
10.1007/s13665-023-00312-1 doi (DE-627)SPR052580725 (SPR)s13665-023-00312-1-e DE-627 ger DE-627 rakwb eng Kulkarni, Tejaswini verfasserin (orcid)0000-0002-4251-4988 aut Interstitial Pneumonia with Autoimmune Features: 8 Years after Nomenclature and Classification—Where are We Not and Where Are We Headed? 2023 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 2023. Springer Nature or its licensor (e.g. a society or other partner) 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 The term interstitial pneumonia with autoimmune features (IPAF) was proposed by an international task force as a research classification to standardize nomenclature for patients with idiopathic interstitial pneumonia (IIP) and features of connective tissue disease (CTD). This review aims to discuss the advances made in understanding this research classification and the clinical implications of this term. Recent Findings Multiple cohort studies have described the clinical presentation of patients meeting criteria for IPAF with heterogeneity noted particularly in the morphologic domain as compared to clinical and serologic domains. Treating these patients according to their clinical presentation and features remains the key expert opinion given the paucity of data to inform therapeutic strategies in IPAF. Summary There are still several challenges and unresolved questions which preclude the application of IPAF as a clinical diagnosis. Whether IPAF represents a clinical diagnosis distinct from CTD-ILD or IIPs or precursor or an overlap of these conditions remains controversial and warrants further investigation. Interstitial pneumonia with autoimmune features (dpeaa)DE-He213 Antifibrotics (dpeaa)DE-He213 Immunosuppression (dpeaa)DE-He213 Idiopathic interstitial pneumonia (dpeaa)DE-He213 Connective tissue disease (dpeaa)DE-He213 Enthalten in Current respiratory care reports New York, NY : Springer, 2012 12(2023), 3 vom: 16. Mai, Seite 144-150 (DE-627)718610954 (DE-600)2660484-X 2161-332X nnns volume:12 year:2023 number:3 day:16 month:05 pages:144-150 https://dx.doi.org/10.1007/s13665-023-00312-1 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER 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 12 2023 3 16 05 144-150 |
| allfieldsGer |
10.1007/s13665-023-00312-1 doi (DE-627)SPR052580725 (SPR)s13665-023-00312-1-e DE-627 ger DE-627 rakwb eng Kulkarni, Tejaswini verfasserin (orcid)0000-0002-4251-4988 aut Interstitial Pneumonia with Autoimmune Features: 8 Years after Nomenclature and Classification—Where are We Not and Where Are We Headed? 2023 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 2023. Springer Nature or its licensor (e.g. a society or other partner) 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 The term interstitial pneumonia with autoimmune features (IPAF) was proposed by an international task force as a research classification to standardize nomenclature for patients with idiopathic interstitial pneumonia (IIP) and features of connective tissue disease (CTD). This review aims to discuss the advances made in understanding this research classification and the clinical implications of this term. Recent Findings Multiple cohort studies have described the clinical presentation of patients meeting criteria for IPAF with heterogeneity noted particularly in the morphologic domain as compared to clinical and serologic domains. Treating these patients according to their clinical presentation and features remains the key expert opinion given the paucity of data to inform therapeutic strategies in IPAF. Summary There are still several challenges and unresolved questions which preclude the application of IPAF as a clinical diagnosis. Whether IPAF represents a clinical diagnosis distinct from CTD-ILD or IIPs or precursor or an overlap of these conditions remains controversial and warrants further investigation. Interstitial pneumonia with autoimmune features (dpeaa)DE-He213 Antifibrotics (dpeaa)DE-He213 Immunosuppression (dpeaa)DE-He213 Idiopathic interstitial pneumonia (dpeaa)DE-He213 Connective tissue disease (dpeaa)DE-He213 Enthalten in Current respiratory care reports New York, NY : Springer, 2012 12(2023), 3 vom: 16. Mai, Seite 144-150 (DE-627)718610954 (DE-600)2660484-X 2161-332X nnns volume:12 year:2023 number:3 day:16 month:05 pages:144-150 https://dx.doi.org/10.1007/s13665-023-00312-1 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER 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 12 2023 3 16 05 144-150 |
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10.1007/s13665-023-00312-1 doi (DE-627)SPR052580725 (SPR)s13665-023-00312-1-e DE-627 ger DE-627 rakwb eng Kulkarni, Tejaswini verfasserin (orcid)0000-0002-4251-4988 aut Interstitial Pneumonia with Autoimmune Features: 8 Years after Nomenclature and Classification—Where are We Not and Where Are We Headed? 2023 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 2023. Springer Nature or its licensor (e.g. a society or other partner) 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 The term interstitial pneumonia with autoimmune features (IPAF) was proposed by an international task force as a research classification to standardize nomenclature for patients with idiopathic interstitial pneumonia (IIP) and features of connective tissue disease (CTD). This review aims to discuss the advances made in understanding this research classification and the clinical implications of this term. Recent Findings Multiple cohort studies have described the clinical presentation of patients meeting criteria for IPAF with heterogeneity noted particularly in the morphologic domain as compared to clinical and serologic domains. Treating these patients according to their clinical presentation and features remains the key expert opinion given the paucity of data to inform therapeutic strategies in IPAF. Summary There are still several challenges and unresolved questions which preclude the application of IPAF as a clinical diagnosis. Whether IPAF represents a clinical diagnosis distinct from CTD-ILD or IIPs or precursor or an overlap of these conditions remains controversial and warrants further investigation. Interstitial pneumonia with autoimmune features (dpeaa)DE-He213 Antifibrotics (dpeaa)DE-He213 Immunosuppression (dpeaa)DE-He213 Idiopathic interstitial pneumonia (dpeaa)DE-He213 Connective tissue disease (dpeaa)DE-He213 Enthalten in Current respiratory care reports New York, NY : Springer, 2012 12(2023), 3 vom: 16. Mai, Seite 144-150 (DE-627)718610954 (DE-600)2660484-X 2161-332X nnns volume:12 year:2023 number:3 day:16 month:05 pages:144-150 https://dx.doi.org/10.1007/s13665-023-00312-1 lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER 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 12 2023 3 16 05 144-150 |
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Interstitial Pneumonia with Autoimmune Features: 8 Years after Nomenclature and Classification—Where are We Not and Where Are We Headed? Interstitial pneumonia with autoimmune features (dpeaa)DE-He213 Antifibrotics (dpeaa)DE-He213 Immunosuppression (dpeaa)DE-He213 Idiopathic interstitial pneumonia (dpeaa)DE-He213 Connective tissue disease (dpeaa)DE-He213 |
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Purpose of Review The term interstitial pneumonia with autoimmune features (IPAF) was proposed by an international task force as a research classification to standardize nomenclature for patients with idiopathic interstitial pneumonia (IIP) and features of connective tissue disease (CTD). This review aims to discuss the advances made in understanding this research classification and the clinical implications of this term. Recent Findings Multiple cohort studies have described the clinical presentation of patients meeting criteria for IPAF with heterogeneity noted particularly in the morphologic domain as compared to clinical and serologic domains. Treating these patients according to their clinical presentation and features remains the key expert opinion given the paucity of data to inform therapeutic strategies in IPAF. Summary There are still several challenges and unresolved questions which preclude the application of IPAF as a clinical diagnosis. Whether IPAF represents a clinical diagnosis distinct from CTD-ILD or IIPs or precursor or an overlap of these conditions remains controversial and warrants further investigation. © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2023. Springer Nature or its licensor (e.g. a society or other partner) 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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Purpose of Review The term interstitial pneumonia with autoimmune features (IPAF) was proposed by an international task force as a research classification to standardize nomenclature for patients with idiopathic interstitial pneumonia (IIP) and features of connective tissue disease (CTD). This review aims to discuss the advances made in understanding this research classification and the clinical implications of this term. Recent Findings Multiple cohort studies have described the clinical presentation of patients meeting criteria for IPAF with heterogeneity noted particularly in the morphologic domain as compared to clinical and serologic domains. Treating these patients according to their clinical presentation and features remains the key expert opinion given the paucity of data to inform therapeutic strategies in IPAF. Summary There are still several challenges and unresolved questions which preclude the application of IPAF as a clinical diagnosis. Whether IPAF represents a clinical diagnosis distinct from CTD-ILD or IIPs or precursor or an overlap of these conditions remains controversial and warrants further investigation. © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2023. Springer Nature or its licensor (e.g. a society or other partner) 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 The term interstitial pneumonia with autoimmune features (IPAF) was proposed by an international task force as a research classification to standardize nomenclature for patients with idiopathic interstitial pneumonia (IIP) and features of connective tissue disease (CTD). This review aims to discuss the advances made in understanding this research classification and the clinical implications of this term. Recent Findings Multiple cohort studies have described the clinical presentation of patients meeting criteria for IPAF with heterogeneity noted particularly in the morphologic domain as compared to clinical and serologic domains. Treating these patients according to their clinical presentation and features remains the key expert opinion given the paucity of data to inform therapeutic strategies in IPAF. Summary There are still several challenges and unresolved questions which preclude the application of IPAF as a clinical diagnosis. Whether IPAF represents a clinical diagnosis distinct from CTD-ILD or IIPs or precursor or an overlap of these conditions remains controversial and warrants further investigation. © The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2023. Springer Nature or its licensor (e.g. a society or other partner) 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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Interstitial Pneumonia with Autoimmune Features: 8 Years after Nomenclature and Classification—Where are We Not and Where Are We Headed? |
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