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
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
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
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
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Abbreviations
Interstitial pneumonia with autoimmune features (IPAF)
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
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
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
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
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