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Interstitial Pneumonia With Autoimmune Features (IPAF)
Takeshi Johkoh, MD, PhD
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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 CTD
            • Autoimmune-featured ILD
          • Definitions

            • Interstitial pneumonia demonstrated by histology or HRCT/CT without complete rheumatologic criteria for specific CTD, 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 was proposed in 2015 by task force of European Respiratory Society and American Thoracic Society in response to absence of consensus regarding terminology
              • Term is 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 are 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
                • 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

          IMAGING

          • CT Findings

            DIFFERENTIAL DIAGNOSIS

              PATHOLOGY

              • General Features

                • Staging, Grading, & Classification

                  CLINICAL ISSUES

                  • Presentation

                    • Demographics

                      • Natural History & Prognosis

                        • Treatment

                          Selected References

                          1. 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
                          2. 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
                          3. Jee AS et al: A call for uniformity in implementing the IPAF (interstitial pneumonia with autoimmune features) criteria. Eur Respir J. 48(6):1811-1813, 2016
                          4. Luppi F et al: Interstitial pneumonitis with autoimmune features (IPAF): a work in progress. Eur Respir J. 47(6):1622-4, 2016
                          5. Oldham JM et al: Characterisation of patients with interstitial pneumonia with autoimmune features. Eur Respir J. 47(6):1767-75, 2016
                          6. Strek ME et al: A call for uniformity in implementing the IPAF (interstitial pneumonia with autoimmune features) criteria. Eur Respir J. 48(6):1813-1814, 2016
                          7. 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
                          8. 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
                          9. Omote N et al: Lung-dominant connective tissue disease: clinical, radiologic, and histologic features. Chest. 148(6):1438-46, 2015
                          10. 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
                          11. Vij R et al: Autoimmune-featured interstitial lung disease: a distinct entity. Chest. 140(5):1292-9, 2011
                          12. Fischer A et al: Connective tissue disease-associated interstitial lung disease: a call for clarification. Chest. 138(2):251-6, 2010
                          13. 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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                          Related Differential Diagnoses
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                          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 CTD
                                    • Autoimmune-featured ILD
                                  • Definitions

                                    • Interstitial pneumonia demonstrated by histology or HRCT/CT without complete rheumatologic criteria for specific CTD, 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 was proposed in 2015 by task force of European Respiratory Society and American Thoracic Society in response to absence of consensus regarding terminology
                                      • Term is 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 are 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
                                        • 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

                                  IMAGING

                                  • CT Findings

                                    DIFFERENTIAL DIAGNOSIS

                                      PATHOLOGY

                                      • General Features

                                        • Staging, Grading, & Classification

                                          CLINICAL ISSUES

                                          • Presentation

                                            • Demographics

                                              • Natural History & Prognosis

                                                • Treatment

                                                  Selected References

                                                  1. 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
                                                  2. 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
                                                  3. Jee AS et al: A call for uniformity in implementing the IPAF (interstitial pneumonia with autoimmune features) criteria. Eur Respir J. 48(6):1811-1813, 2016
                                                  4. Luppi F et al: Interstitial pneumonitis with autoimmune features (IPAF): a work in progress. Eur Respir J. 47(6):1622-4, 2016
                                                  5. Oldham JM et al: Characterisation of patients with interstitial pneumonia with autoimmune features. Eur Respir J. 47(6):1767-75, 2016
                                                  6. Strek ME et al: A call for uniformity in implementing the IPAF (interstitial pneumonia with autoimmune features) criteria. Eur Respir J. 48(6):1813-1814, 2016
                                                  7. 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
                                                  8. 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
                                                  9. Omote N et al: Lung-dominant connective tissue disease: clinical, radiologic, and histologic features. Chest. 148(6):1438-46, 2015
                                                  10. 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
                                                  11. Vij R et al: Autoimmune-featured interstitial lung disease: a distinct entity. Chest. 140(5):1292-9, 2011
                                                  12. Fischer A et al: Connective tissue disease-associated interstitial lung disease: a call for clarification. Chest. 138(2):251-6, 2010
                                                  13. 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