Bookmarks
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Abbreviations
- Interstitial pneumonia with autoimmune features (IPAF)
Synonyms
- Undifferentiated connective tissue disease-associated interstitial lung disease (CTD-ILD)
- Lung-dominant connective tissue disease (CTD)
- Autoimmune-featured interstitial lung disease (ILD)
Definitions
- Interstitial pneumonia demonstrated by histology or HRCT/CT without complete rheumatologic criteria for specific CTD diagnosis, but with clinical, serological, and morphologic criteria that suggest autoimmunity
Associated Syndromes
- Usual interstitial pneumonia (UIP)
- Nonspecific interstitial pneumonia (NSIP)
- Organizing pneumonia (OP)
- Lymphoid interstitial pneumonia (LIP)
Historic Perspective
- IPAF diagnosis proposed in 2015 by European Respiratory Society and American Thoracic Society task force in response to absence of consensus regarding terminology
- Term considered work in progress that requires further scientific testing and validation
- Revisions of criteria will likely occur as more data becomes available
- Guidelines or recommendations for clinical care, diagnostic testing, or management not included in task force proposal
- IPAF diagnosis proposed in 2015 by European Respiratory Society and American Thoracic Society task force in response to absence of consensus regarding terminology
Diagnostic Criteria
- Presence of interstitial pneumonia by HRCT/CT or surgical lung biopsy and
- Exclusion of alternative etiologies and
- Failure to meet criteria of defined CTD and
- At least 1 feature from at least 2 of following 3 domains
- Clinical domain
- Distal digital fissuring (i.e., "mechanic hands")
- Distal digital tip ulceration
- Inflammatory arthritis or polyarticular morning joint stiffness ≥ 60 minutes
- Palmar telangiectasia
- Raynaud phenomenon
- Unexplained digital edema
- Unexplained fixed rash on digital extensor surfaces (Gottron sign)
- Serologic domain
- Antinuclear antibodies (ANA) ≥ 1:320 titer, diffuse speckled homogeneous patterns or ANA nucleolar pattern (any titer), or ANA centromere pattern (any titer)
- Rheumatoid factor ≥ 2x upper limit of normal
- Anti-CCP (anticyclic citrullinated peptide)
- Anti-dsDNA (antidouble stranded DNA)
- Anti-Ro (SS-A): Anti-Sjögren-syndrome-related antigen A, also called anti-Ro
- Anti-La (SS-B): Anti-Sjögren-syndrome-related antigen B, also called anti-La
- Antiribonucleoprotein
- Anti-Smith
- Antitopoisomerase (Scl-70)
- Anti-tRSNA synthetase
- Jo-1 (antihistidyl)
- PL-7 (antithreonyl)
- PL-12 (antialanyl)
- Others: EJ (antiglycyl), OJ (antiisoleucyl), KS (antiasparaginyl), Zo (antiphenylalanyl), tRS
- Anti-PM-Scl (antiexosome)
- Anti-MDA-5 (melanoma-differentiation-associated gene 5)
- Morphologic domain
- Suggestive patterns on HRCT/CT
- NSIP
- OP
- LIP
- NSIP with OP overlap
- Histopathology patterns or features by surgical lung biopsy
- NSIP
- OP
- LIP
- NSIP with OP overlap
- Interstitial lymphoid aggregates with germinal centers
- Diffuse lymphoplasmacytic infiltration (± lymphoid follicles)
- Multicompartmental involvement (in addition to interstitial pneumonia)
- Unexplained pleural effusion or thickening
- Unexplained pericardial effusion or thickening
- Unexplained intrinsic airways disease (by pulmonary function tests, imaging, or pathology)
- Unexplained pulmonary vasculopathy
- Suggestive patterns on HRCT/CT
- Clinical domain
IMAGING
CT Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
- Karampeli M et al: Interstitial pneumonia with autoimmune features (IPAF): a single-centre, prospective study. Mediterr J Rheumatol. 31(3):330-6, 2020
- Sebastiani M et al: Interstitial pneumonia with autoimmune features: a single center prospective follow-up study. Autoimmun Rev. 19(2):102451, 2020
- Fernandes L et al: Interstitial pneumonia with autoimmune features (IPAF). Front Med (Lausanne). 6:209, 2019
- Oldham JM et al: Counterpoint: does interstitial pneumonia with autoimmune features represent a distinct class of patients with idiopathic interstitial pneumonia? No. Chest. 155(2):260-3, 2019
- Chung JH et al: CT findings, radiologic-pathologic correlation, and imaging predictors of survival for patients with interstitial pneumonia with autoimmune features. AJR Am J Roentgenol. 208(6):1229-36, 2017
- Płóciniczak A et al: Interstitial pneumonia with autoimmune features (IPAF) and radiological findings suggestive of lymphocytic interstitial pneumonia (LIP) - case report. Adv Respir Med. 85(1):46-50, 2017
- Collins B et al: Interstitial pneumonia with autoimmune features: the new consensus-based definition for this cohort of patients should be broadened. Eur Respir J. 47(4):1293-5, 2016
- Ferri C et al: Interstitial pneumonia with autoimmune features and undifferentiated connective tissue disease: our interdisciplinary rheumatology-pneumology experience, and review of the literature. Autoimmun Rev. 15(1):61-70, 2016
- Jee AS et al: A call for uniformity in implementing the IPAF (interstitial pneumonia with autoimmune features) criteria. Eur Respir J. 48(6):1811-3, 2016
- Luppi F et al: Interstitial pneumonitis with autoimmune features (IPAF): a work in progress. Eur Respir J. 47(6):1622-4, 2016
- Oldham JM et al: Characterisation of patients with interstitial pneumonia with autoimmune features. Eur Respir J. 47(6):1767-75, 2016
- Strek ME et al: A call for uniformity in implementing the IPAF (interstitial pneumonia with autoimmune features) criteria. Eur Respir J. 48(6):1813-4, 2016
- Assayag D et al: Survival in interstitial pneumonia with features of autoimmune disease: a comparison of proposed criteria. Respir Med. 109(10):1326-31, 2015
- Fischer A et al: An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J. 46(4):976-87, 2015
- Omote N et al: Lung-dominant connective tissue disease: clinical, radiologic, and histologic features. Chest. 148(6):1438-46, 2015
- de Lauretis A et al: Review series: aspects of interstitial lung disease: connective tissue disease-associated interstitial lung disease: how does it differ from IPF? How should the clinical approach differ? Chron Respir Dis. 8(1):53-82, 2011
- Vij R et al: Autoimmune-featured interstitial lung disease: a distinct entity. Chest. 140(5):1292-9, 2011
- Fischer A et al: Connective tissue disease-associated interstitial lung disease: a call for clarification. Chest. 138(2):251-6, 2010
- Kinder BW et al: Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease? Am J Respir Crit Care Med. 176(7):691-7, 2007
Related Anatomy
Loading...
Related Differential Diagnoses
Loading...
References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Abbreviations
- Interstitial pneumonia with autoimmune features (IPAF)
Synonyms
- Undifferentiated connective tissue disease-associated interstitial lung disease (CTD-ILD)
- Lung-dominant connective tissue disease (CTD)
- Autoimmune-featured interstitial lung disease (ILD)
Definitions
- Interstitial pneumonia demonstrated by histology or HRCT/CT without complete rheumatologic criteria for specific CTD diagnosis, but with clinical, serological, and morphologic criteria that suggest autoimmunity
Associated Syndromes
- Usual interstitial pneumonia (UIP)
- Nonspecific interstitial pneumonia (NSIP)
- Organizing pneumonia (OP)
- Lymphoid interstitial pneumonia (LIP)
Historic Perspective
- IPAF diagnosis proposed in 2015 by European Respiratory Society and American Thoracic Society task force in response to absence of consensus regarding terminology
- Term considered work in progress that requires further scientific testing and validation
- Revisions of criteria will likely occur as more data becomes available
- Guidelines or recommendations for clinical care, diagnostic testing, or management not included in task force proposal
- IPAF diagnosis proposed in 2015 by European Respiratory Society and American Thoracic Society task force in response to absence of consensus regarding terminology
Diagnostic Criteria
- Presence of interstitial pneumonia by HRCT/CT or surgical lung biopsy and
- Exclusion of alternative etiologies and
- Failure to meet criteria of defined CTD and
- At least 1 feature from at least 2 of following 3 domains
- Clinical domain
- Distal digital fissuring (i.e., "mechanic hands")
- Distal digital tip ulceration
- Inflammatory arthritis or polyarticular morning joint stiffness ≥ 60 minutes
- Palmar telangiectasia
- Raynaud phenomenon
- Unexplained digital edema
- Unexplained fixed rash on digital extensor surfaces (Gottron sign)
- Serologic domain
- Antinuclear antibodies (ANA) ≥ 1:320 titer, diffuse speckled homogeneous patterns or ANA nucleolar pattern (any titer), or ANA centromere pattern (any titer)
- Rheumatoid factor ≥ 2x upper limit of normal
- Anti-CCP (anticyclic citrullinated peptide)
- Anti-dsDNA (antidouble stranded DNA)
- Anti-Ro (SS-A): Anti-Sjögren-syndrome-related antigen A, also called anti-Ro
- Anti-La (SS-B): Anti-Sjögren-syndrome-related antigen B, also called anti-La
- Antiribonucleoprotein
- Anti-Smith
- Antitopoisomerase (Scl-70)
- Anti-tRSNA synthetase
- Jo-1 (antihistidyl)
- PL-7 (antithreonyl)
- PL-12 (antialanyl)
- Others: EJ (antiglycyl), OJ (antiisoleucyl), KS (antiasparaginyl), Zo (antiphenylalanyl), tRS
- Anti-PM-Scl (antiexosome)
- Anti-MDA-5 (melanoma-differentiation-associated gene 5)
- Morphologic domain
- Suggestive patterns on HRCT/CT
- NSIP
- OP
- LIP
- NSIP with OP overlap
- Histopathology patterns or features by surgical lung biopsy
- NSIP
- OP
- LIP
- NSIP with OP overlap
- Interstitial lymphoid aggregates with germinal centers
- Diffuse lymphoplasmacytic infiltration (± lymphoid follicles)
- Multicompartmental involvement (in addition to interstitial pneumonia)
- Unexplained pleural effusion or thickening
- Unexplained pericardial effusion or thickening
- Unexplained intrinsic airways disease (by pulmonary function tests, imaging, or pathology)
- Unexplained pulmonary vasculopathy
- Suggestive patterns on HRCT/CT
- Clinical domain
IMAGING
CT Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
- Karampeli M et al: Interstitial pneumonia with autoimmune features (IPAF): a single-centre, prospective study. Mediterr J Rheumatol. 31(3):330-6, 2020
- Sebastiani M et al: Interstitial pneumonia with autoimmune features: a single center prospective follow-up study. Autoimmun Rev. 19(2):102451, 2020
- Fernandes L et al: Interstitial pneumonia with autoimmune features (IPAF). Front Med (Lausanne). 6:209, 2019
- Oldham JM et al: Counterpoint: does interstitial pneumonia with autoimmune features represent a distinct class of patients with idiopathic interstitial pneumonia? No. Chest. 155(2):260-3, 2019
- Chung JH et al: CT findings, radiologic-pathologic correlation, and imaging predictors of survival for patients with interstitial pneumonia with autoimmune features. AJR Am J Roentgenol. 208(6):1229-36, 2017
- Płóciniczak A et al: Interstitial pneumonia with autoimmune features (IPAF) and radiological findings suggestive of lymphocytic interstitial pneumonia (LIP) - case report. Adv Respir Med. 85(1):46-50, 2017
- Collins B et al: Interstitial pneumonia with autoimmune features: the new consensus-based definition for this cohort of patients should be broadened. Eur Respir J. 47(4):1293-5, 2016
- Ferri C et al: Interstitial pneumonia with autoimmune features and undifferentiated connective tissue disease: our interdisciplinary rheumatology-pneumology experience, and review of the literature. Autoimmun Rev. 15(1):61-70, 2016
- Jee AS et al: A call for uniformity in implementing the IPAF (interstitial pneumonia with autoimmune features) criteria. Eur Respir J. 48(6):1811-3, 2016
- Luppi F et al: Interstitial pneumonitis with autoimmune features (IPAF): a work in progress. Eur Respir J. 47(6):1622-4, 2016
- Oldham JM et al: Characterisation of patients with interstitial pneumonia with autoimmune features. Eur Respir J. 47(6):1767-75, 2016
- Strek ME et al: A call for uniformity in implementing the IPAF (interstitial pneumonia with autoimmune features) criteria. Eur Respir J. 48(6):1813-4, 2016
- Assayag D et al: Survival in interstitial pneumonia with features of autoimmune disease: a comparison of proposed criteria. Respir Med. 109(10):1326-31, 2015
- Fischer A et al: An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J. 46(4):976-87, 2015
- Omote N et al: Lung-dominant connective tissue disease: clinical, radiologic, and histologic features. Chest. 148(6):1438-46, 2015
- de Lauretis A et al: Review series: aspects of interstitial lung disease: connective tissue disease-associated interstitial lung disease: how does it differ from IPF? How should the clinical approach differ? Chron Respir Dis. 8(1):53-82, 2011
- Vij R et al: Autoimmune-featured interstitial lung disease: a distinct entity. Chest. 140(5):1292-9, 2011
- Fischer A et al: Connective tissue disease-associated interstitial lung disease: a call for clarification. Chest. 138(2):251-6, 2010
- Kinder BW et al: Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease? Am J Respir Crit Care Med. 176(7):691-7, 2007
