Interstitial Pneumonia with Autoimmune Features: 8 Years after Nomenclature and Classification—Where are We Not and Where Are We Headed?

Aletaha D, Neogi T, Silman AJ, Funovits J, Felson DT, Bingham CO 3rd, et al. 2010 rheumatoid arthritis classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Ann Rheum Dis. 2010;69(9):1580–8.

Article  PubMed  Google Scholar 

van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013 Classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum. 2013;65(11):2737–47.

Article  PubMed  PubMed Central  Google Scholar 

Lundberg IE, Tjarnlund A, Bottai M, Werth VP, Pilkington C, Visser M, et al. 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Ann Rheum Dis. 2017;76(12):1955–64.

Article  PubMed  Google Scholar 

Kinder BW, Collard HR, Koth L, Daikh DI, Wolters PJ, Elicker B, et al. 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 

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.

Article  PubMed  PubMed Central  Google Scholar 

Vij R, Noth I, Strek ME. Autoimmune-featured interstitial lung disease: a distinct entity. Chest. 2011;140(5):1292–9.

Article  PubMed  PubMed Central  Google Scholar 

•• Fischer A, Antoniou KM, Brown KK, Cadranel J, Corte TJ, du Bois RM, et al. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J. 2015;46(4):976–87. Research statement discussing the rationale and definition of IPAF.

Aletaha D, Neogi T, Silman AJ, Funovits J, Felson DT, Bingham CO 3rd, et al. 2010 Rheumatoid arthritis classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Arthritis Rheum. 2010;62(9):2569–81.

Article  PubMed  Google Scholar 

Kumar Y, Bhatia A, Minz RW. Antinuclear antibodies and their detection methods in diagnosis of connective tissue diseases: a journey revisited. Diagn Pathol. 2009;4:1.

Article  CAS  PubMed  PubMed Central  Google Scholar 

• Hazarika K, Sahoo RR, Mohindra N, Wakhlu A, Manoj M, Bafna P, et al. Clinical, radiologic and serologic profile of patients with interstitial pneumonia with autoimmune features: a cross-sectional study. Rheumatol Int. 2022;42(8):1431–41. More recent study describing the characteristics and presentation of patients with IPAF.

Oldham JM, Adegunsoye A, Valenzi E, Lee C, Witt L, Chen L, et al. Characterisation of patients with interstitial pneumonia with autoimmune features. Eur Respir J. 2016;47(6):1767–75.

Article  CAS  PubMed  PubMed Central  Google Scholar 

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.

Article  PubMed  Google Scholar 

Ahmad K, Barba T, Gamondes D, Ginoux M, Khouatra C, Spagnolo P, et al. Interstitial pneumonia with autoimmune features: clinical, radiologic, and histological characteristics and outcome in a series of 57 patients. Respir Med. 2017;123:56–62.

Article  PubMed  Google Scholar 

Ito Y, Arita M, Kumagai S, Takei R, Noyama M, Tokioka F, et al. Serological and morphological prognostic factors in patients with interstitial pneumonia with autoimmune features. BMC Pulm Med. 2017;17(1):111.

Article  PubMed  PubMed Central  Google Scholar 

Dai J, Wang L, Yan X, Li H, Zhou K, He J, et al. 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.

Article  PubMed  Google Scholar 

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.

Article  PubMed  Google Scholar 

Kim HC, Lee JH, Chae EJ, Song JS, Song JW. Long-term clinical course and outcome of interstitial pneumonia with autoimmune features. Respirology. 2020;25(6):636–43.

Article  PubMed  Google Scholar 

Sebastiani M, Cassone G, De Pasquale L, Cerri S, Della Casa G, Vacchi C, et al. Interstitial pneumonia with autoimmune features: a single center prospective follow-up study. Autoimmun Rev. 2020;19(2):102451.

Article  PubMed  Google Scholar 

Jee AS, Parker MJS, Bleasel JF, Troy LK, Lau EM, Jo HE, et al. Baseline characteristics and survival of an australian interstitial pneumonia with autoimmune features cohort. Respiration. 2021;100(9):853–64.

Article  CAS  PubMed  Google Scholar 

Collins BF, Spiekerman CF, Shaw MA, Ho LA, Hayes J, Spada CA, et al. Idiopathic interstitial pneumonia associated with autoantibodies: a large case series followed over 1 year. Chest. 2017;152(1):103–12.

Article  PubMed  PubMed Central  Google Scholar 

Khanna D, Tashkin DP, Denton CP, Lubell MW, Vazquez-Mateo C, Wax S. Ongoing clinical trials and treatment options for patients with systemic sclerosis-associated interstitial lung disease. Rheumatology (Oxford). 2019;58(4):567–79.

Article  CAS  PubMed  Google Scholar 

Kawano-Dourado L, Lee JS. Management of connective tissue disease-associated interstitial lung disease. Clin Chest Med. 2021;42(2):295–310.

Article  PubMed  Google Scholar 

Matson SM, Demoruelle MK. Connective tissue disease associated interstitial lung disease. Immunol Allergy Clin North Am. 2023;43(2):229–44.

Article  PubMed  Google Scholar 

Dsouza KG, Alexander AS, Watts JR Jr, Kulkarni T. Management of interstitial lung disease in patients with autoimmune disease-related interstitial lung disease. Multidiscip Respir Med. 2023;18(1). https://doi.org/10.4081/mrm.2023.890.

Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006;354(25):2655–66.

Article  CAS  PubMed  Google Scholar 

Tashkin DP, Roth MD, Clements PJ, Furst DE, Khanna D, Kleerup EC, et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med. 2016;4(9):708–19.

Article  CAS  PubMed  PubMed Central  Google Scholar 

•• Maher TM, Tudor VA, Saunders P, Gibbons MA, Fletcher SV, Denton CP, et al. Rituximab versus intravenous cyclophosphamide in patients with connective tissue disease-associated interstitial lung disease in the UK (RECITAL): a double-blind, double-dummy, randomised, controlled, phase 2b trial. Lancet Respir Med. 2023;11(1):45–54. First RCT reporting the efficacy of rituximab in patients with progressive CTD-ILD.

Khanna D, Lin CJF, Furst DE, Goldin J, Kim G, Kuwana M, et al. Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Respir Med. 2020;8(10):963–74.

Article  CAS  PubMed  Google Scholar 

• Joerns EK, Adams TN, Newton CA, Bermas B, Karp D, Batra K, et al. Variables associated with response to therapy in patients with interstitial pneumonia with autoimmune features. J Clin Rheumatol. 2022;28(2):84–8. This study is one of the few small studies reporting treatment patterns for IPAF.

McCoy SS, Mukadam Z, Meyer KC, Kanne JP, Meyer CA, Martin MD, et al. Mycophenolate therapy in interstitial pneumonia with autoimmune features: a cohort study. Ther Clin Risk Manag. 2018;14:2171–81.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Nieto MA, Sanchez-Pernaute O, Vadillo C, Rodriguez-Nieto MJ, Romero-Bueno F, Lopez-Muniz B, et al. Functional respiratory impairment and related factors in patients with interstitial pneumonia with autoimmune features (IPAF): multicenter study from NEREA registry. Respir Res. 2023;24(1):19.

Article  CAS  PubMed  PubMed Central  Google Scholar 

D'Silva KM, Ventura IB, Bolster MB, Castelino FV, Sharma A, Little BP, et al. Rituximab for interstitial pneumonia with autoimmune features at two medical centres. Rheumatol Adv Pract. 2021;5(Suppl 2):ii1–ii9.

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).

King TE Jr, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083–92.

Article  PubMed  Google Scholar 

Richeldi L, Cottin V, Flaherty KR, Kolb M, Inoue Y, Raghu G, et al. Design of the INPULSIS trials: two phase 3 trials of nintedanib in patients with idiopathic pulmonary fibrosis. Respir Med. 2014;108(7):1023–30.

Article  PubMed  Google Scholar 

Distler O, Highland KB, Gahlemann M, Azuma A, Fischer A, Mayes MD, et al. Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med. 2019;380(26):2518–28.

Article  CAS  PubMed  Google Scholar 

Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y, et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019;381(18):1718–27.

Article  CAS  PubMed  Google Scholar 

Behr J, Prasse A, Kreuter M, Johow J, Rabe KF, Bonella F, et al. Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial. Lancet Respir Med. 2021;9(5):476–86.

Article  CAS  PubMed  Google Scholar 

Maher TM, Corte TJ, Fischer A, Kreuter M, Lederer DJ, Molina-Molina M, et al. 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.

Article  CAS  PubMed  Google Scholar 

Raghu G, Remy-Jardin M, Richeldi L, Thomson CC, Inoue Y, Johkoh T, et al. Idiopathic Pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2022;205(9):e18–47.

Article  PubMed  PubMed Central  Google Scholar 

Yamano Y, Kataoka K, Takei R, Sasano H, Yokoyama T, Matsuda T, et al. Interstitial pneumonia with autoimmune features and histologic usual interstitial pneumonia treated with anti-fibrotic versus immunosuppressive therapy. Respir Investig. 2023;61(3):297–305.

Article  CAS 

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