Volkmann ER, Andréasson K, Smith V. Systemic sclerosis. Lancet. 2023;401(10373):304–18.
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. Ann Rheum Dis. 2013;72(11):1747–55.
Google Scholar
Volkmann ER, Fischer A. Update on morbidity and mortality in systemic sclerosis–related interstitial lung disease. J Scleroderma Relat Disorders. 2021;6(1):11–20.
Google Scholar
Hoffmann-Vold AM, Allanore Y, Alves M, Brunborg C, Airó P, Ananieva LP, et al. Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database. Ann Rheum Dis. 2021;80(2):219–27.
Google Scholar
Vonk MC, Smith V, Sfikakis PP, Cutolo M, del Galdo F, Seibold JR. Pharmacological treatments for SSc-ILD: systematic review and critical appraisal of the evidence. Autoimmun Rev. 2021;20(12):102978.
Google Scholar
Allanore Y, Vonk MC, Distler O, Azuma A, Mayes MD, James A, et al. Continued nintedanib in patients with systemic sclerosis-associated interstitial lung disease: 3-year data from SENSCIS-ON. RMD Open. 2025;11(1):e005086.
Google Scholar
Bonhomme O, André B, Gester F, de Seny D, Moermans C, Struman I, et al. Biomarkers in systemic sclerosis-associated interstitial lung disease: review of the literature. Rheumatology (Oxford). 2019;58(9):1534–46.
Google Scholar
Khanna D, Tashkin DP, Denton CP, Renzoni EA, Desai SR, Varga J, Etiology. Risk factors, and biomarkers in systemic sclerosis with interstitial lung disease. Am J Respir Crit Care Med. 2020;201(6):650–60.
Google Scholar
Lavis P, Garabet A, Cardozo AK, Bondue B. The fibroblast activation protein alpha as a biomarker of pulmonary fibrosis. Front Med. 2024;11.
Sollini M, Kirienko M, Gelardi F, Fiz F, Gozzi N, Chiti A. State-of-the-art of FAPI-PET imaging: a systematic review and meta-analysis. Eur J Nucl Med Mol Imaging. 2021;48(13):4396–414.
Google Scholar
Park JE, Lenter MC, Zimmermann RN, Garin-Chesa P, Old LJ, Rettig WJ. Fibroblast activation protein, a dual specificity Serine protease expressed in reactive human tumor stromal fibroblasts. J Biol Chem. 1999;274(51):36505–12.
Google Scholar
Lay AJ, Zhang HE, McCaughan GW, Gorrell MD. Fibroblast activation protein in liver fibrosis. Front Bioscience (Landmark edition). 2019;24:1–17.
Google Scholar
Fitzgerald AA, Weiner LM. The role of fibroblast activation protein in health and malignancy. Cancer Metastasis Rev. 2020;39(3):783–803.
Google Scholar
Acharya PS, Zukas A, Chandan V, Katzenstein AL, Puré E. Fibroblast activation protein: a Serine protease expressed at the remodeling interface in idiopathic pulmonary fibrosis. Hum Pathol. 2006;37(3):352–60.
Google Scholar
Fan MH, Zhu Q, Li HH, Ra HJ, Majumdar S, Gulick DL, et al. Fibroblast activation protein (FAP) accelerates collagen degradation and clearance from lungs in mice. J Biol Chem. 2016;291(15):8070–89.
Google Scholar
Egger C, Cannet C, Gérard C, Suply T, Ksiazek I, Jarman E, et al. Effects of the fibroblast activation protein inhibitor, PT100, in a murine model of pulmonary fibrosis. Eur J Pharmacol. 2017;809:64–72.
Google Scholar
Kimura T, Monslow J, Klampatsa A, Leibowitz M, Sun J, Liousia M, et al. Loss of cells expressing fibroblast activation protein has variable effects in models of TGF-β and chronic bleomycin-induced fibrosis. Am J Physiol Lung Cell Mol Physiol. 2019;317(2):L271–82.
Google Scholar
Bergmann C, Distler JHW, Treutlein C, Tascilar K, Müller A-T, Atzinger A, et al. 68Ga-FAPI-04 PET-CT for molecular assessment of fibroblast activation and risk evaluation in systemic sclerosis-associated interstitial lung disease: a single-centre, pilot study. Lancet Rheumatol. 2021;3(3):e185–94.
Google Scholar
Corsi F, Sorrentino L, Albasini S, Colombo F, Cigognini M, Massari A et al. Circulating fibroblast activation protein as potential biomarker in patients with inflammatory bowel disease. Front Med. 2021;8.
Tillmanns J, Widera C, Habbaba Y, Galuppo P, Kempf T, Wollert KC, et al. Circulating concentrations of fibroblast activation protein α in apparently healthy individuals and patients with acute coronary syndrome as assessed by sandwich ELISA. Int J Cardiol. 2013;168(4):3926–31.
Google Scholar
Uitte de Willige S, Keane FM, Bowen DG, Malfliet J, Zhang HE, Maneck B, et al. Circulating fibroblast activation protein activity and antigen levels correlate strongly when measured in liver disease and coronary heart disease. PLoS ONE. 2017;12(6):e0178987.
Google Scholar
Sinnathurai P, Lau W, Vieira de Ribeiro AJ, Bachovchin WW, Englert H, Howe G, et al. Circulating fibroblast activation protein and dipeptidyl peptidase 4 in rheumatoid arthritis and systemic sclerosis. Int J Rheum Dis. 2018;21(11):1915–23.
Google Scholar
Kosałka-Węgiel J, Lichołai S, Pacholczak-Madej R, Dziedzina S, Milewski M, Kuszmiersz P, et al. Serum IL-17 and TNFα as prognostic biomarkers in systemic sclerosis patients: a prospective study. Rheumatol Int. 2024;44(1):119–28.
Google Scholar
Ruiter G, Lanser IJ, de Man FS, van der Laarse WJ, Wharton J, Wilkins MR, et al. Iron deficiency in systemic sclerosis patients with and without pulmonary hypertension. Rheumatology. 2013;53(2):285–92.
Google Scholar
Bellando-Randone S, Del Galdo F, Lepri G, Minier T, Huscher D, Furst DE, et al. Progression of patients with raynaud’s phenomenon to systemic sclerosis: a five-year analysis of the European scleroderma trial and research group multicentre, longitudinal registry study for very early diagnosis of systemic sclerosis (VEDOSS). Lancet Rheumatol. 2021;3(12):e834–43.
Google Scholar
Sinha A, Lutter R, Xu B, Dekker T, Dierdorp B, Sterk PJ, et al. Loss of adaptive capacity in asthmatic patients revealed by biomarker fluctuation dynamics after rhinovirus challenge. eLife. 2019;8:e47969.
Google Scholar
Hoffmann-Vold AM, Petelytska L, Fretheim H, Barua I, Becker MO, Bjørkekjær HJ, et al. POS1291 Prediction of mortality in SSC-ILD depends on definition of ILD progression. Ann Rheum Dis. 2023;82(Suppl 1):992–3.
Google Scholar
Matteson EL, Kelly C, Distler JHW, Hoffmann-Vold A-M, Seibold JR, Mittoo S et al. Nintedanib in Patients With Autoimmune Disease–Related Progressive Fibrosing Interstitial Lung Diseases: Subgroup Analysis of the INBUILD Trial. Arthritis & Rheumatology.n/a(n/a).
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.
Google Scholar
Dorst DN, van Caam APM, Vitters EL, Walgreen B, Helsen MMA, Klein C et al. Fibroblast activation protein targeted photodynamic therapy selectively kills activated skin fibroblasts from systemic sclerosis patients and prevents tissue contraction. Int J Mol Sci. 2021;22(23).
Nirk EL, Reggiori F, Mauthe M. Hydroxychloroquine in rheumatic autoimmune disorders and beyond. EMBO Mol Med. 2020;12(8):e12476.
Google Scholar
Prior TS, Hoyer N, Davidsen JR, Shaker SB, Hundahl MP, Lomholt S, et al. Fibroblast activation protein and disease severity, progression, and survival in idiopathic pulmonary fibrosis. Scand J Immunol. 2024;100(3):e13392.
Google Scholar
Röhrich M, Leitz D, Flechsig P, Kahn N, Mall M, Michael K, et al. Fibroblast activation Protein – specific PET/CT imaging in idiopathic pulmonary fibrosis with lung cancer. J Nucl Med. 2019;60(supplement 1):298.
Yang P, Luo Q, Wang X, Fang Q, Fu Z, Li J, et al. Comprehensive analysis of fibroblast activation protein expression in interstitial lung diseases. Am J Respir Crit Care Med. 2023;207(2):160–72.
Google Scholar
Tillmanns J, Weiglein JM, Neuser J, Fraccarollo D, Galuppo P, König T, et al. Circulating soluble fibroblast activation protein (FAP) levels are independent of cardiac and extra-cardiac FAP expression determined by targeted molecular imaging in patients with myocardial FAP activation. Int J Cardiol. 2024;406:132044.
Google Scholar
Petelytska L, Bonomi F, Cannistrà C, Fiorentini E, Peretti S, Torracchi S et al. Heterogeneity of determining disease severity, clinical course and outcomes in systemic sclerosis-associated interstitial lung disease: a systematic literature review. RMD Open. 2023;9(4).