Skip to main content
SpringerLink
Log in

Interstitial Pneumonia with Autoimmune Features

  • Chapter
  • First Online:
Orphan Lung Diseases

  • 537 Accesses

Abstract

A 45-year-old woman, never smoker, presents with a slowly progressive dyspnea and also reports a dry cough that is worse with exertion. On symptom review, she reports several months of puffiness of the hands but denies any other features to suggest an autoimmune disease. On physical examination, she is noted to have digital edema and mild distal digital fissuring but no evidence of Raynaud phenomenon, sclerodactyly, or telangiectasia. Her musculoskeletal examination is otherwise normal; no synovitis or muscle weakness is detected. She has audible crackles on respiratory examination bilaterally. Her high-resolution computed tomography images reveal evidence of diffuse lung disease suggestive of nonspecific interstitial pneumonia (NSIP) pattern (Fig. 15.1). Laboratory testing is notable only for a positive anti-nuclear antibody at high titer (1:1280). All other serologies and lab tests are normal.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 189.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 169.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 249.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. ATS/ERS. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS Board of Directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 2002;165(2):277–304. Epub 2002/01/16. https://doi.org/10.1164/ajrccm.165.2.ats01.

    Article  Google Scholar 

  2. Fischer A, du Bois R. Interstitial lung disease in connective tissue disorders. Lancet. 2012;380(9842):689–98. Epub 2012/08/21. https://doi.org/10.1016/S0140-6736(12)61079-4.

    Article  PubMed  Google Scholar 

  3. Mittoo S, Gelber AC, Christopher-Stine L, Horton MR, Lechtzin N, Danoff SK. Ascertainment of collagen vascular disease in patients presenting with interstitial lung disease. Respir Med. 2009;103(8):1152–8. Epub 2009/03/24. https://doi.org/10.1016/j.rmed.2009.02.009.

    Article  PubMed  Google Scholar 

  4. Strange C, Highland KB. Interstitial lung disease in the patient who has connective tissue disease. Clin Chest Med. 2004;25(3):549–59.

    Article  PubMed  Google Scholar 

  5. Kinder BW, Collard HR, Koth L, Daikh DI, Wolters PJ, Elicker B, Jones KD, King TE Jr. Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease? Am J Respir Crit Care Med. 2007;176(7):691–7.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Fischer A, West SG, Swigris JJ, Brown KK, du Bois RM. Connective tissue disease-associated interstitial lung disease: a call for clarification. Chest. 2010;138(2):251–6. Epub 2010/08/05. https://doi.org/10.1378/chest.10-0194.

    Article  PubMed  PubMed Central  Google Scholar 

  7. Vij R, Noth I, Strek ME. Autoimmune-featured interstitial lung disease: a distinct entity. Chest. 2011;140(5):1292–9. https://doi.org/10.1378/chest.10-2662.

    Article  PubMed  PubMed Central  Google Scholar 

  8. Fischer A, Antoniou KM, Brown KK, Cadranel J, Corte TJ, du Bois RM, Lee JS, Leslie KO, Lynch DA, Matteson EL, Mosca M, Noth I, Richeldi L, Strek ME, Swigris JJ, Wells AU, West SG, Collard HR, Cottin V, CTD-ILD EATFoUFo. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J. 2015;46(4):976–87. Epub 2015/07/15. https://doi.org/10.1183/13993003.00150-2015.

    Article  CAS  PubMed  Google Scholar 

  9. LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA Jr, Rowell N, Wollheim F. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15(2):202–5.

    CAS  PubMed  Google Scholar 

  10. LeRoy EC, Medsger TA Jr. Criteria for the classification of early systemic sclerosis. J Rheumatol. 2001;28(7):1573–6. Epub 2001/07/27.

    CAS  PubMed  Google Scholar 

  11. Corte TJ, Copley SJ, Desai SR, Zappala CJ, Hansell DM, Nicholson AG, Colby TV, Renzoni E, Maher TM, Wells AU. Significance of connective tissue disease features in idiopathic interstitial pneumonia. Eur Respir J. 2012;39(3):661–8. Epub 2011/09/17. https://doi.org/10.1183/09031936.00174910.

    Article  CAS  PubMed  Google Scholar 

  12. Fischer A, Swigris JJ, du Bois RM, Lynch DA, Downey GP, Cosgrove GP, Frankel SK, Fernandez-Perez ER, Gillis JZ, Brown KK. Anti-synthetase syndrome in ANA and anti-Jo-1 negative patients presenting with idiopathic interstitial pneumonia. Respir Med. 2009;103(11):1719–24. PubMed PMID: 19497723; PMCID: 2857337. https://doi.org/10.1016/j.rmed.2009.05.001.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, Behr J, Cottin V, Danoff SK, Morell F, Flaherty KR, Wells A, Martinez FJ, Azuma A, Bice TJ, Bouros D, Brown KK, Collard HR, Duggal A, Galvin L, Inoue Y, Jenkins RG, Johkoh T, Kazerooni EA, Kitaichi M, Knight SL, Mansour G, Nicholson AG, SNJ P, Buendia-Roldan I, Selman M, Travis WD, Walsh S, Wilson KC, American Thoracic Society ERSJRS, Latin American Thoracic S. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018;198(5):e44–68. Epub 2018/09/01. https://doi.org/10.1164/rccm.201807-1255ST.

    Article  PubMed  Google Scholar 

  14. Steen VD. Auto antibodies in systemic sclerosis. Semin Arthritis Rheum. 2005;35(1):35–42. Epub 2005/08/09. https://doi.org/10.1016/j.semarthrit.2005.03.005.

    Article  CAS  PubMed  Google Scholar 

  15. Reveille JD, Solomon DH, American College of Rheumatology ad hoc Committee of immunologic testing G. Evidence-based guidelines for the use of immunologic tests: anticentromere, Scl-70, and nucleolar antibodies. Arthritis Rheum. 2003;49(3):399–412. Epub 2003/06/10. https://doi.org/10.1002/art.11113.

    Article  PubMed  Google Scholar 

  16. Lynch DA. Quantitative CT of fibrotic interstitial lung disease. Chest. 2007;131(3):643–4. Epub 2007/03/16. https://doi.org/10.1378/chest.06-2955.

    Article  PubMed  Google Scholar 

  17. Lynch DA, Travis WD, Muller NL, Galvin JR, Hansell DM, Grenier PA, King TE Jr. Idiopathic interstitial pneumonias: CT features. Radiology. 2005;236(1):10–21. Epub 2005/07/01. https://doi.org/10.1148/radiol.2361031674.

    Article  PubMed  Google Scholar 

  18. Hwang JH, Misumi S, Sahin H, Brown KK, Newell JD, Lynch DA. Computed tomographic features of idiopathic fibrosing interstitial pneumonia: comparison with pulmonary fibrosis related to collagen vascular disease. J Comput Assist Tomogr. 2009;33(3):410–5. Epub 2009/05/30. https://doi.org/10.1097/RCT.0b013e318181d551.

    Article  PubMed  Google Scholar 

  19. Tansey D, Wells AU, Colby TV, Ip S, Nikolakoupolou A, du Bois RM, Hansell DM, Nicholson AG. Variations in histological patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis. Histopathology. 2004;44(6):585–96. Epub 2004/06/10. https://doi.org/10.1111/j.1365-2559.2004.01896.x.

    Article  CAS  PubMed  Google Scholar 

  20. Leslie KO, Trahan S, Gruden J. Pulmonary pathology of the rheumatic diseases. Semin Respir Crit Care Med. 2007;28(4):369–78.

    Article  PubMed  Google Scholar 

  21. Graney BA, Fischer A. Interstitial pneumonia with autoimmune features. Ann Am Thorac Soc. 2019;16(5):525–33. Epub 2019/01/30. https://doi.org/10.1513/AnnalsATS.201808-565CME.

    Article  PubMed  PubMed Central  Google Scholar 

  22. Lega JC, Reynaud Q, Belot A, Fabien N, Durieu I, Cottin V. Idiopathic inflammatory myopathies and the lung. Eur Respir Rev. 2015;24(136):216–38. Epub 2015/06/02. https://doi.org/10.1183/16000617.00002015.

    Article  PubMed  PubMed Central  Google Scholar 

  23. Connors GR, Christopher-Stine L, Oddis CV, Danoff SK. Interstitial lung disease associated with the idiopathic inflammatory myopathies: what progress has been made in the past 35 years? Chest. 2010;138(6):1464–74. Epub 2010/12/09. https://doi.org/10.1378/chest.10-0180.

    Article  PubMed  Google Scholar 

  24. Jee AS, Bleasel JF, Adelstein S, Keir GJ, Corte TJ. A call for uniformity in implementing the IPAF (interstitial pneumonia with autoimmune features) criteria. Eur Respir J. 2016;48(6):1811–3. Epub 2016/12/03. https://doi.org/10.1183/13993003.01259-2016.

    Article  PubMed  Google Scholar 

  25. Jee AS, Adelstein S, Bleasel J, Keir GJ, Nguyen M, Sahhar J, Youssef P, Corte TJ. Role of autoantibodies in the diagnosis of connective-tissue disease ILD (CTD-ILD) and interstitial pneumonia with autoimmune features (IPAF). J Clin Med. 2017;6(5):51. PubMed PMID: 28471413; PMCID: PMC5447942. Epub 2017/05/05. https://doi.org/10.3390/jcm6050051.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Scire CA, Gonzalez-Gay MA, Selva-O'Callaghan A, Cavagna L. Clinical spectrum time course of interstitial pneumonia with autoimmune features in patients positive for antisynthetase antibodies. Respir Med. 2017;(132):265–6. Epub 2017/04/08. https://doi.org/10.1016/j.rmed.2017.03.028.

  27. Cavagna L, Nuno L, Scire CA, Govoni M, Longo FJ, Franceschini F, Neri R, Castaneda S, Sifuentes Giraldo WA, Caporali R, Iannone F, Fusaro E, Paolazzi G, Pellerito R, Schwarting A, Saketkoo LA, Ortego-Centeno N, Quartuccio L, Bartoloni E, Specker C, Murcia TP, La Corte R, Furini F, Foschi V, Corral JB, Airo P, Cavazzana I, Martinez-Barrio J, Hinojosa M, Giannini M, Barsotti S, Menke J, Triantafyllias K, Vitetta R, Russo A, Bajocchi G, Bravi E, Barausse G, Bortolotti R, Selmi C, Parisi S, Montecucco C, Gonzalez-Gay MA, Group Ac. Clinical spectrum time course in anti Jo-1 positive antisynthetase syndrome: results from an international retrospective multicenter study. Medicine (Baltimore). 2015;94(32):e1144. Epub 2015/08/13. PubMed PMID: 26266346; PMCID: PMC4616698. https://doi.org/10.1097/MD.0000000000001144.

    Article  CAS  PubMed  Google Scholar 

  28. Graham J, Bauer Ventura I, Newton CA, Lee C, Boctor N, Pugashetti JV, Cutting C, Joerns E, Sandhu H, Chung JH, Garcia CK, Kadoch M, Noth I, Adegunsoye A, Strek ME, Oldham JM. Myositis-specific antibodies identify a distinct interstitial pneumonia with autoimmune features phenotype. Eur Respir J. 2020:2001205. Epub 2020/07/18. https://doi.org/10.1183/13993003.01205-2020.

  29. Yamada H. ANCA: associated lung fibrosis. Semin Respir Crit Care Med. 2011;32(3):322–7. Epub 2011/06/16. https://doi.org/10.1055/s-0031-1279828.

    Article  PubMed  Google Scholar 

  30. Hervier B, Pagnoux C, Agard C, Haroche J, Amoura Z, Guillevin L, Hamidou MA, French Vasculitis study G. Pulmonary fibrosis associated with ANCA-positive vasculitides. Retrospective study of 12 cases and review of the literature. Ann Rheum Dis. 2009;68(3):404–7. Epub 2008/10/30. https://doi.org/10.1136/ard.2008.096131.

    Article  CAS  PubMed  Google Scholar 

  31. Comarmond C, Crestani B, Tazi A, Hervier B, Adam-Marchand S, Nunes H, Cohen-Aubart F, Wislez M, Cadranel J, Housset B, Lloret-Linares C, Seve P, Pagnoux C, Abad S, Camuset J, Bienvenu B, Duruisseaux M, Hachulla E, Arlet JB, Hamidou M, Mahr A, Resche-Rigon M, Brun AL, Grenier P, Cacoub P, Saadoun D. Pulmonary fibrosis in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: a series of 49 patients and review of the literature. Medicine (Baltimore). 2014;93(24):340–9. Epub 2014/12/17. https://doi.org/10.1097/MD.0000000000000217.

    Article  CAS  PubMed  Google Scholar 

  32. Moiseev S, Cohen Tervaert JW, Arimura Y, Bogdanos DP, Csernok E, Damoiseaux J, Ferrante M, Flores-Suarez LF, Fritzler MJ, Invernizzi P, Jayne D, Jennette JC, Little MA, SP MA, Novikov P, Pusey CD, Radice A, Salama AD, Savige JA, Segelmark M, Shoenfeld Y, Sinico RA, Sousa MJ, Specks U, Terrier B, Tzioufas AG, Vermeire S, Zhao MH, Bossuyt X. 2020 international consensus on ANCA testing beyond systemic vasculitis. Autoimmun Rev. 2020;19(9):102618. Epub 2020/07/15. https://doi.org/10.1016/j.autrev.2020.102618.

    Article  CAS  PubMed  Google Scholar 

  33. Hozumi H, Oyama Y, Yasui H, Suzuki Y, Kono M, Karayama M, Furuhashi K, Enomoto N, Fujisawa T, Inui N, Nakamura Y, Suda T. Clinical significance of myeloperoxidase-anti-neutrophil cytoplasmic antibody in idiopathic interstitial pneumonias. PLoS One. 2018;13(6):e0199659. Epub 2018/06/22. PubMed PMID: 29928060; PMCID: PMC6013167. https://doi.org/10.1371/journal.pone.0199659.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  34. Hozumi H, Enomoto N, Oyama Y, Kono M, Fujisawa T, Inui N, Nakamura Y, Suda T. Clinical implication of proteinase-3-antineutrophil cytoplasmic antibody in patients with idiopathic interstitial pneumonias. Lung. 2016;194(2):235–42. Epub 2016/02/14. https://doi.org/10.1007/s00408-016-9851-x.

    Article  CAS  PubMed  Google Scholar 

  35. Oldham JM, Adegunsoye A, Valenzi E, Lee C, Witt L, Chen L, Husain AN, Montner S, Chung JH, Cottin V, Fischer A, Noth I, Vij R, Strek ME. Characterisation of patients with interstitial pneumonia with autoimmune features. Eur Respir J. 2016;47(6):1767–75. Epub 2016/04/23. https://doi.org/10.1183/13993003.01565-2015.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  36. Oldham JM, Danoff SK. COUNTERPOINT: does interstitial pneumonia with autoimmune features represent a distinct class of patients with idiopathic interstitial pneumonia? No. Chest. 2019;155(2):260–3. Epub 2019/02/09. https://doi.org/10.1016/j.chest.2018.08.1073.

    Article  PubMed  Google Scholar 

  37. Kim HC, Lee JH, Chae EJ, Song JS, Song JW. Long-term clinical course and outcome of interstitial pneumonia with autoimmune features. Respirology. 2019;25(6):636–43. Epub 2019/08/07. https://doi.org/10.1111/resp.13665.

    Article  PubMed  Google Scholar 

  38. Newton CA, Oldham JM, Ley B, Anand V, Adegunsoye A, Liu G, Batra K, Torrealba J, Kozlitina J, Glazer C, Strek ME, Wolters PJ, Noth I, Garcia CK. Telomere length and genetic variant associations with interstitial lung disease progression and survival. Eur Respir J. 2019;53(4):1801641. Epub 2019/01/13. PubMed PMID: 30635297; PMCID: PMC6612265. https://doi.org/10.1183/13993003.01641-2018.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  39. Chartrand S, Swigris JJ, Stanchev L, Lee JS, Brown KK, Fischer A. Clinical features and natural history of interstitial pneumonia with autoimmune features: a single center experience. Respir Med. 2016;119:150–4. Epub 2016/10/04. https://doi.org/10.1016/j.rmed.2016.09.002.

    Article  PubMed  Google Scholar 

  40. Ito Y, Arita M, Kumagai S, Takei R, Noyama M, Tokioka F, Nishimura K, Koyama T, Notohara K, Ishida T. Serological and morphological prognostic factors in patients with interstitial pneumonia with autoimmune features. BMC Pulm Med. 2017;17(1):111. Epub 2017/08/16. PubMed PMID: 28807021; PMCID: 5554971. https://doi.org/10.1186/s12890-017-0453-z.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  41. Dai J, Wang L, Yan X, Li H, Zhou K, He J, Meng F, Xu S, Liang G, Cai H. Clinical features, risk factors, and outcomes of patients with interstitial pneumonia with autoimmune features: a population-based study. Clin Rheumatol. 2018;37(8):2125–32. Epub 2018/04/19. https://doi.org/10.1007/s10067-018-4111-5.

    Article  PubMed  Google Scholar 

  42. Ferri C, Manfredi A, Sebastiani M, Colaci M, Giuggioli D, Vacchi C, Della Casa G, Cerri S, Torricelli P, Luppi F. Interstitial pneumonia with autoimmune features and undifferentiated connective tissue disease: our interdisciplinary rheumatology-pneumology experience, and review of the literature. Autoimmun Rev. 2016;15(1):61–70. Epub 2015/09/20. https://doi.org/10.1016/j.autrev.2015.09.003.

    Article  CAS  PubMed  Google Scholar 

  43. Biffi A, Dei G, De Giacomi F, Stainer A, Parma LO, Pozzi MR, Faverio P, Pesci A. Non-specific interstitial pneumonia and features of connective tissue disease: what are the consequences of a different point of view? Monaldi Arch Chest Dis. 2018;88(3):970. Epub 2018/09/06. https://doi.org/10.4081/monaldi.2018.970.

    Article  PubMed  Google Scholar 

  44. Ahmad K, Barba T, Gamondes D, Ginoux M, Khouatra C, Spagnolo P, Strek M, Thivolet-Bejui F, Traclet J, Cottin V. Interstitial pneumonia with autoimmune features: clinical, radiologic, and histological characteristics and outcome in a series of 57 patients. Respir Med. 2017;123:56–62. Epub 2017/02/01. https://doi.org/10.1016/j.rmed.2016.10.017.

    Article  PubMed  Google Scholar 

  45. Yoshimura K, Kono M, Enomoto Y, Nishimoto K, Oyama Y, Yasui H, Hozumi H, Karayama M, Suzuki Y, Furuhashi K, Enomoto N, Fujisawa T, Nakamura Y, Inui N, Sumikawa H, Johkoh T, Colby TV, Sugimura H, Suda T. Distinctive characteristics and prognostic significance of interstitial pneumonia with autoimmune features in patients with chronic fibrosing interstitial pneumonia. Respir Med. 2018;137:167–75. Epub 2018/04/02. https://doi.org/10.1016/j.rmed.2018.02.024.

    Article  PubMed  Google Scholar 

  46. Collins BF, Spiekerman CF, Shaw MA, Ho LA, Hayes J, Spada CA, Stamato CM, Raghu G. Idiopathic interstitial pneumonia associated with autoantibodies: a large case series followed over 1 year. Chest. 2017;152(1):103–12. Epub 2017/03/17. https://doi.org/10.1016/j.chest.2017.03.004.

    Article  PubMed  PubMed Central  Google Scholar 

  47. Kelly BT, Moua T. Overlap of interstitial pneumonia with autoimmune features with undifferentiated connective tissue disease and contribution of UIP to mortality. Respirology. 2018;23(6):600–5. Epub 2018/01/11. https://doi.org/10.1111/resp.13254.

    Article  PubMed  Google Scholar 

  48. Sebastiani M, Cassone G, De Pasquale L, Cerri S, Della Casa G, Vacchi C, Luppi F, Salvarani C, Manfredi A. Interstitial pneumonia with autoimmune features: a single center prospective follow-up study. Autoimmun Rev. 2020;19(2):102451. Epub 2019/12/16. https://doi.org/10.1016/j.autrev.2019.102451.

    Article  PubMed  Google Scholar 

  49. Tsukamoto T, Yamakawa Y. Effects of the cytosol fraction and ATP on the kinetics of cAMP binding to human erythrocyte membranes. Biochem Int. 1988;16(2):253–7. Epub 1988/02/01.

    CAS  PubMed  Google Scholar 

  50. Lim JU, Gil BM, Kang HS, Oh J, Kim YH, Kwon SS. Interstitial pneumonia with autoimmune features show better survival and less exacerbations compared to idiopathic pulmonary fibrosis. BMC Pulm Med. 2019;19(1):120. Epub 2019/07/06. PubMed PMID: 31272428; PMCID: PMC6610995. https://doi.org/10.1186/s12890-019-0868-9.

    Article  PubMed  PubMed Central  Google Scholar 

  51. Sambataro G, Sambataro D, Torrisi SE, Vancheri A, Colaci M, Pavone M, Pignataro F, Del Papa N, Palmucci S, Vancheri C. Clinical, serological and radiological features of a prospective cohort of interstitial pneumonia with autoimmune features (IPAF) patients. Respir Med. 2019;150:154–60. Epub 2019/04/10. https://doi.org/10.1016/j.rmed.2019.03.011.

    Article  PubMed  Google Scholar 

  52. Yamakawa H, Kitamura H, Takemura T, Ikeda S, Sekine A, Baba T, Iwasawa T, Hagiwara E, Sato S, Ogura T. Prognostic factors and disease behaviour of pathologically proven fibrotic non-specific interstitial pneumonia. Respirology. 2018;23(11):1032–40. Epub 2018/04/26. https://doi.org/10.1111/resp.13313.

    Article  PubMed  Google Scholar 

  53. Hernandez-Gonzalez F, Prieto-Gonzalez S, Brito-Zeron P, Cuerpo S, Sanchez M, Ramirez J, Agusti C, Lucena CM, Paradela M, Grafia I, Espinosa G, Sellares J. Impact of a systematic evaluation of connective tissue disease on diagnosis approach in patients with interstitial lung diseases. Medicine (Baltimore). 2020;99(4):e18589. Epub 2020/01/25. PubMed PMID: 31977850; PMCID: PMC7004576. https://doi.org/10.1097/MD.0000000000018589.

    Article  CAS  PubMed  Google Scholar 

  54. McCoy SS, Mukadam Z, Meyer KC, Kanne JP, Meyer CA, Martin MD, Sampene E, Aesif SW, Rice LN, Bartels CM. Mycophenolate therapy in interstitial pneumonia with autoimmune features: a cohort study. Ther Clin Risk Manag. 2018;14:2171–81. Epub 2018/11/23. PubMed PMID: 30464490; PMCID: PMC6219314. https://doi.org/10.2147/TCRM.S173154.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  55. Chung JH, Montner SM, Adegunsoye A, Lee C, Oldham JM, Husain AN, MacMahon H, Noth I, Vij R, Strek ME. CT findings, radiologic-pathologic correlation, and imaging predictors of survival for patients with interstitial pneumonia with autoimmune features. AJR Am J Roentgenol. 2017;208(6):1229–36. Epub 2017/03/30. PubMed PMID: 28350485; PMCID: PMC6536259. https://doi.org/10.2214/AJR.16.17121.

    Article  PubMed  PubMed Central  Google Scholar 

  56. Wiertz IA, van Moorsel CHM, Vorselaars ADM, Quanjel MJR, Grutters JC. Cyclophosphamide in steroid refractory unclassifiable idiopathic interstitial pneumonia and interstitial pneumonia with autoimmune features (IPAF). Eur Respir J. 2018;51(4):1702519. Epub 2018/03/03. https://doi.org/10.1183/13993003.02519-2017.

    Article  CAS  PubMed  Google Scholar 

  57. Richeldi L, du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, Cottin V, Flaherty KR, Hansell DM, Inoue Y, Kim DS, Kolb M, Nicholson AG, Noble PW, Selman M, Taniguchi H, Brun M, Le Maulf F, Girard M, Stowasser S, Schlenker-Herceg R, Disse B, Collard HR, Investigators IT. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071–82. Epub 2014/05/20. https://doi.org/10.1056/NEJMoa1402584.

    Article  CAS  PubMed  Google Scholar 

  58. Crestani B, Huggins JT, Kaye M, Costabel U, Glaspole I, Ogura T, Song JW, Stansen W, Quaresma M, Stowasser S, Kreuter M. Long-term safety and tolerability of nintedanib in patients with idiopathic pulmonary fibrosis: results from the open-label extension study, INPULSIS-ON. Lancet Respir Med. 2019;7(1):60–8. Epub 2018/09/19. https://doi.org/10.1016/S2213-2600(18)30339-4.

    Article  CAS  PubMed  Google Scholar 

  59. Richeldi L, Cottin V, du Bois RM, Selman M, Kimura T, Bailes Z, Schlenker-Herceg R, Stowasser S, Brown KK. Nintedanib in patients with idiopathic pulmonary fibrosis: combined evidence from the TOMORROW and INPULSIS((R)) trials. Respir Med. 2016;113:74–9. Epub 2016/02/27. https://doi.org/10.1016/j.rmed.2016.02.001.

    Article  PubMed  Google Scholar 

  60. Costabel U, Inoue Y, Richeldi L, Collard HR, Tschoepe I, Stowasser S, Azuma A. Efficacy of Nintedanib in idiopathic pulmonary fibrosis across prespecified subgroups in INPULSIS. Am J Respir Crit Care Med. 2016;193(2):178–85. Epub 2015/09/24. https://doi.org/10.1164/rccm.201503-0562OC.

    Article  CAS  PubMed  Google Scholar 

  61. Distler O, Highland KB, Gahlemann M, Azuma A, Fischer A, Mayes MD, Raghu G, Sauter W, Girard M, Alves M, Clerisme-Beaty E, Stowasser S, Tetzlaff K, Kuwana M, Maher TM, Investigators ST. Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med. 2019;380(26):2518–28. Epub 2019/05/22. https://doi.org/10.1056/NEJMoa1903076.

    Article  CAS  PubMed  Google Scholar 

  62. Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y, Richeldi L, Kolb M, Tetzlaff K, Stowasser S, Coeck C, Clerisme-Beaty E, Rosenstock B, Quaresma M, Haeufel T, Goeldner RG, Schlenker-Herceg R, Brown KK, Investigators IT. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019;381(18):1718–27. Epub 2019/10/01. https://doi.org/10.1056/NEJMoa1908681.

    Article  CAS  PubMed  Google Scholar 

  63. Flaherty KR, Brown KK, Wells AU, Clerisme-Beaty E, Collard HR, Cottin V, Devaraj A, Inoue Y, Le Maulf F, Richeldi L, Schmidt H, Walsh S, Mezzanotte W, Schlenker-Herceg R. Design of the PF-ILD trial: a double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease. BMJ Open Respir Res. 2017;4(1):e000212. Epub 2017/10/12. PubMed PMID: 29018526; PMCID: 5604725. https://doi.org/10.1136/bmjresp-2017-000212.

    Article  PubMed  PubMed Central  Google Scholar 

  64. Noble PW, Albera C, Bradford WZ, Costabel U, Glassberg MK, Kardatzke D, King TE Jr, Lancaster L, Sahn SA, Szwarcberg J, Valeyre D, du Bois RM, Group CS. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377(9779):1760–9. Epub 2011/05/17. https://doi.org/10.1016/S0140-6736(11)60405-4.

    Article  CAS  PubMed  Google Scholar 

  65. King TE Jr, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, Gorina E, Hopkins PM, Kardatzke D, Lancaster L, Lederer DJ, Nathan SD, Pereira CA, Sahn SA, Sussman R, Swigris JJ, Noble PW, Group AS. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083–92. Epub 2014/05/20. https://doi.org/10.1056/NEJMoa1402582.

    Article  CAS  PubMed  Google Scholar 

  66. Nathan SD, Albera C, Bradford WZ, Costabel U, Glaspole I, Glassberg MK, Kardatzke DR, Daigl M, Kirchgaessler KU, Lancaster LH, Lederer DJ, Pereira CA, Swigris JJ, Valeyre D, Noble PW. Effect of pirfenidone on mortality: pooled analyses and meta-analyses of clinical trials in idiopathic pulmonary fibrosis. Lancet Respir Med. 2017;5(1):33–41. Epub 2016/11/24. https://doi.org/10.1016/S2213-2600(16)30326-5.

    Article  CAS  PubMed  Google Scholar 

  67. Noble PW, Albera C, Bradford WZ, Costabel U, du Bois RM, Fagan EA, Fishman RS, Glaspole I, Glassberg MK, Lancaster L, Lederer DJ, Leff JA, Nathan SD, Pereira CA, Swigris JJ, Valeyre D, King TE Jr. Pirfenidone for idiopathic pulmonary fibrosis: analysis of pooled data from three multinational phase 3 trials. Eur Respir J. 2016;47(1):243–53. Epub 2015/12/10. PubMed PMID: 26647432; PMCID: PMC4697914. https://doi.org/10.1183/13993003.00026-2015.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  68. Maher TM, Corte TJ, Fischer A, Kreuter M, Lederer DJ, Molina-Molina M, Axmann J, Kirchgaessler KU, Samara K, Gilberg F, Cottin V. Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial. Lancet Respir Med. 2020;8(2):147–57. Epub 2019/10/04. https://doi.org/10.1016/S2213-2600(19)30341-8.

    Article  CAS  PubMed  Google Scholar 

  69. Cantin AM, Hubbard RC, Crystal RG. Glutathione deficiency in the epithelial lining fluid of the lower respiratory tract in idiopathic pulmonary fibrosis. Am Rev Respir Dis. 1989;139(2):370–2.

    Article  CAS  PubMed  Google Scholar 

  70. Behr J, Maier K, Degenkolb B, Krombach F, Vogelmeier C. Antioxidative and clinical effects of high-dose N-acetylcysteine in fibrosing alveolitis. Adjunctive therapy to maintenance immunosuppression. Am J Respir Crit Care Med. 1997;156(6):1897–901.

    Article  CAS  PubMed  Google Scholar 

  71. Behr J, Neuser P, Prasse A, Kreuter M, Rabe K, Schade-Brittinger C, Wagner J, Gunther A. Exploring efficacy and safety of oral pirfenidone for progressive, non-IPF lung fibrosis (RELIEF) - a randomized, double-blind, placebo-controlled, parallel group, multi-center, phase II trial. BMC Pulm Med. 2017;17(1):122. Epub 2017/09/08. PubMed PMID: 28877715; PMCID: 5588600. https://doi.org/10.1186/s12890-017-0462-y.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  72. Maher TM, Corte TJ, Fischer A, Kreuter M, Lederer DJ, Molina-Molina M, Axmann J, Kirchgaessler KU, Cottin V. Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: design of a double-blind, randomised, placebo-controlled phase II trial. BMJ Open Respir Res. 2018;5(1):e000289. Epub 2018/09/21. PubMed PMID: 30233802; PMCID: PMC6135451. https://doi.org/10.1136/bmjresp-2018-000289.

    Article  PubMed  PubMed Central  Google Scholar 

  73. A Study of pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease. https://ClinicalTrials.gov/show/NCT03099187.

Download references

Author information

Authors and Affiliations

  1. Division of Pulmonary Sciences and Critical Care Medicine, National Jewish Health, Denver, CO, USA

    Amen Sergew

  2. Bristol Myers Squibb, Denver, CO, USA

    Aryeh Fischer

  3. National Jewish Health, Denver, CO, USA

    Kevin Brown

Authors
  1. Amen Sergew
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Aryeh Fischer
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Kevin Brown
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Amen Sergew .

Editor information

Editors and Affiliations

  1. Department of Respiratory Medicine, National Coordinating Reference Centre for Rare Pulmonary Diseases (OrphaLung), Louis Pradel Hospital, University of Lyon, Lyon, France

    Vincent Cottin

  2. Fondazione Policlinico A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Roma, Italy

    Luca Richeldi

  3. National Jewish Health, Denver, CO, USA

    Kevin Brown

  4. Division of Pulmonary, Critical Care, and Sleep Medicine Department of Internal Medicine, University of Cincinnati, Cincinnati, OH, USA

    Francis X. McCormack

Rights and permissions

Reprints and permissions

Copyright information

© 2023 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Sergew, A., Fischer, A., Brown, K. (2023). Interstitial Pneumonia with Autoimmune Features. In: Cottin, V., Richeldi, L., Brown, K., McCormack, F.X. (eds) Orphan Lung Diseases. Springer, Cham. https://doi.org/10.1007/978-3-031-12950-6_15

Download citation

  • DOI: https://doi.org/10.1007/978-3-031-12950-6_15

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-031-12949-0

  • Online ISBN: 978-3-031-12950-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation