Activity in 2022

Research activity:

The Oslo university hospital (OUH) Strategic Research area- Novel personalized management strategies for fibrosing diseases 2022- 2027 with Anna- Maria Hoffmann-Vold as Project leader. Collaboration with Convergence environments= UIO strategic research area with Mona-Elisabeth Rootwelt-Revheim as leader. The overall purpose of the environment is to develop personalized management strategies including early identification, treatment, monitoring, patient dialogue and outcome assessment in patients with fibrosing diseases.
The ReSScue trial: Finalized phase 2 randomized clinical investigator initiated trial (KLINBFORSK)
Senscis-on 1199-225 – From 2018– Dec 2022, industry sponsored RCT
I-FILE - 2021-2023: multicenter registry for home monitoring of ILD, initiated from Erasmus University in Rotterdam
Connective tissue disease associated interstitial lung disease registry and cohort study (group leader)
Molecular profiling of lung tissue in CTD-ILD in collaboration with UCLA and Zurich (USZ)
ILD quantification on serial HRCT in collaboration with UCLA
ERS/EULAR guideline development for CTD-ILD (Anna Hoffmann-Vold convenor and Jens Vikse task force member)
Update of EULAR recommendation for treatment of SSc (Anna Hoffmann-Vold, task force member)
COVID in systemic sclerosis registry from EUSTAR and OUS
Ig coating of gut microbiota in early systemic sclerosis
RA-ILD patient survey, international survey in 11 countries, initiated together with Heidelberg University
Biomarker analysis for ILD progression in systemic sclerosis, multicenter study

Planned projects:

Nor-mILDer home monitoring strategy trial of CTD-ILD, start estimated May 2023

Thesis defense/completed degrees:

Mona-Lovise Talaro Ramsli completed her Master`s degree Nov 2022; The nurses contribution to quality of life in systemic sclerosis and proven pulmonary arterial hypertension – a study based on patients experiences and thoughts in meeting with nurses in their own treatment within the specialist health care in Norway

Ongoing PhD projects:

Hilde Jenssen Bjørkekjær, MD: “Optimizing pulmonary arterial hypertension (PAH) management in systemic sclerosis (SSc)”. Main supervisor: Anna-Maria Hoffmann-Vold
Henriette Didriksen, MD: “Lymphangiogenic biomarkers in Systemic Sclerosis». Main supervisor: Øyvind Molberg
Imon Shoumitra Barua, MD: ”Deciphering interstitial lung disease through advanced imaging of natural history; the nor-precision approach” Main supervisor: Anna-Maria Hoffmann-Vold
Emily Langballe, MD: «Deciphering a contemporary approach to management for interstitial lung disease in rheumatic conditions”. Main supervisor: Anna-Maria Hoffmann-Vold.
Phuong Phuong Diep, MD, pulmonologist, “Evaluation of interstitial lung disease in patients with rheumatoid arthritis and other connective tissue diseases”, co-supervisor:Anna-Maria Hoffmann-Vold

Awards:

Anna Hoffmann-Vold received the Edith-Busch prize
Phuong Phuong Diep “Best lecturer at Norwegian Respiratory Society annual meeting 2022”

Publications (first or last author):

Tennøe, A. H., Murbræch, K., Didriksen, H., Ueland, T., Palchevskiy, V., Weigt, S. S., Fretheim, H., Midtvedt, Ø., Garen, T., Brunborg, C., Aukrust, P., Molberg, Ø., Belperio, J. A., & Hoffmann-Vold, A. M. (2022). Serum markers of cardiac complications in a systemic sclerosis cohort. Scientific reports, 12(1), 4661. https://doi.org/10.1038/s41598-022-08815-8
Didriksen, H., Molberg, Ø., Mehta, A., Jordan, S., Palchevskiy, V., Fretheim, H., Gude, E., Ueland, T., Brunborg, C., Garen, T., Midtvedt, Ø., Andreassen, A. K., Lund-Johansen, F., Distler, O., Belperio, J., & Hoffmann-Vold, A. M. (2022). Target organ expression and biomarker characterization of chemokine CCL21 in systemic sclerosis associated pulmonary arterial hypertension. Frontiers in immunology, 13, 991743. https://doi.org/10.3389/fimmu.2022.991743
Hoffmann-Vold AM, Brunborg C, Airò P, Ananyeva LP, Czirják L, Guiducci S, Hachulla E, Li M, Mihai C, Riemekasten G, Sfikakis P, Valentini G, Kowal-Bielecka O, Allanore Y, Distler O. Cohort Enrichment Strategies for Progressive Interstitial Lung Disease in Systemic Sclerosis From European Scleroderma Trials and Research. Chest. 2022 Oct 14:S0012-3692(22)03925-3. doi: 10.1016/j.chest.2022.09.044
Hoffmann-Vold AM, Volkmann ER, Allanore Y, Assassi S, de Vries-Bouwstra JK, Smith V, Tschoepe I, Loaiza L, Kanakapura M, Distler O. Safety and tolerability of nintedanib in patients with interstitial lung diseases in subgroups by sex: a post-hoc analysis of pooled data from four randomised controlled trials. The Lancet Rheumatol 2022, Oct e679-e687
Hoffmann-Vold AM, Distler O, Bruni C, Denton CP, de Vries-Bouwstra J, Matucci Cerinic M, Vonk MC, Gabrielli A. Systemic sclerosis in the time of COVID-19. Lancet Rheumatol. 2022 Aug;4(8):e566-e575.
Hoffmann-Vold AM, Distler O, Crestani B, Antoniou KM. Recent advances in the management of systemic sclerosis-associated interstitial lung disease. Curr Opin Pulm Med. 2022 Sep 1;28(5):441-447.
Kreuter M, Hoffmann-Vold AM, Matucci-Cerinic M, Saketkoo LA, Highland KB, Wilson H, Alves M, Erhardt E, Schoof N, Maher TM. Impact of lung function and baseline clinical characteristics on patient-reported outcome measures in systemic sclerosis-associated interstitial lung disease. Rheumatology (Oxford). 2022 May 31:keac325. doi: 10.1093/rheumatology/keac325

Co-author publications:

Wang Y, Xie X, Zheng S, Du G, Chen S, Zhang W, Zhuang J, Lin J, Hu S, Zheng K, Mikish A, Xu Z, Zhang G, Gargani L, Bruni C, Hoffmann-Vold AM, Matucci-Cerinic M, Furst DE. Serum B-cell activating factor and lung ultrasound B-lines in connective tissue disease related interstitial lung disease. Front Med (Lausanne). 2022 Dec 15;9:1066111
Campochiaro C, Hoffmann-Vold AM, Avouac J, Henes J, de Vries-Bouwstra J, Smith V, Siegert E, Airò P, Oksel F, Pellerito R, Vanthuyne M, Pozzi MR, Inanc M, Sibilia J, Gabrielli A, Distler O, Allanore Y; EUSTAR collaborators. Sex influence on outcomes of patients with systemic sclerosis-associated interstitial lung disease: an EUSTAR database analysis. Rheumatology (Oxford). 2022 Nov 22:keac660.
Kuster S, Jordan S, Elhai M, Held U, Steigmiller K, Bruni C, Cacciapaglia F, Vettori S, Siegert E, Rednic S, Codullo V, Airo P, Braun-Moscovici Y, Hunzelmann N, Joao Salvador M, Riccieri V, Gheorghiu AM, Alegre Sancho JJ, Romanowska-Prochnicka K, Castellví I, Kötter I, Truchetet ME, López-Longo FJ, Novikov PI, Giollo A, Shirai Y, Belloli L, Zanatta E, Hachulla E, Smith V, Denton C, Ionescu RM, Schmeiser T, Distler JHW, Gabrielli A, Hoffmann-Vold AM, Kuwana M, Allanore Y, Distler O; EUSTAR collaborators. Effectiveness and safety of tocilizumab in patients with systemic sclerosis: a propensity score matched controlled observational study of the EUSTAR cohort. RMD Open. 2022 Nov;8(2):e002477.
Henry B, Benoit S, Vikse J, Favaloro E, Benoit J, Lippi G. Anti-Endothelial Cell Antibodies are not frequently elevated in hospitalized patients with COVID-19. Acta Biomed. 2022;93(2):e2022043.
Mutua V, Henry BM, Csefalvay C von, et al. Tocilizumab in addition to standard of care in the management of COVID-19: a meta-analysis of RCTs. Acta Biomed. 2022;93(1):e2022014.
Henry BM, Benscoter AL, de Oliveira MHS, Vikse J, Perry T, Cooper DS. Outcomes of extracorporeal life support for respiratory failure in children with primary immunodeficiencies. Perfusion. 2023;38(1):37-43.
Lescoat A, Huscher D, Schoof N, Airò P, de Vries-Bouwstra J, Riemekasten G, Hachulla E, Doria A, Rosato E, Hunzelmann N, Montecucco C, Gabrielli A, Hoffmann-Vold AM, Distler O, Ben Shimol J, Cutolo M, Allanore Y; EUSTAR collaborators. Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region. Rheumatology (Oxford). 2022 Oct 12:keac576.
Roofeh D, Brown KK, Kazerooni EA, Tashkin D, Assassi S, Martinez F, Wells AU, Raghu G, Denton CP, Chung L, Hoffmann-Vold AM, Distler O, Johannson KA, Allanore Y, Matteson EL, Kawano-Dourado L, Pauling JD, Seibold JR, Volkmann ER, Walsh SLF, Oddis CV, White ES, Barratt SL, Bernstein EJ, Domsic RT, Dellaripa PF, Conway R, Rosas I, Bhatt N, Hsu V, Ingegnoli F, Kahaleh B, Garcha P, Gupta N, Khanna S, Korsten P, Lin C, Mathai SC, Strand V, Doyle TJ, Steen V, Zoz DF, Ovalles-Bonilla J, Rodriguez-Pinto I, Shenoy PD, Lewandoski A, Belloli E, Lescoat A, Nagaraja V, Ye W, Huang S, Maher T, Khanna D. Systemic Sclerosis Associated Interstitial Lung Disease: A Conceptual Framework for Subclinical, Clinical, and Progressive Disease. Rheumatology (Oxford). 2022 Sep 29:keac557.
Iudici M, Mongin D, Siegert E, Carreira PE, Distler J, Henes J, Zanatta E, Hachulla E, De Luca G, Müller CS, Santiago T, Tandaipan JL, Bianchi BV, De Santis M, Hoffmann-Vold AM, Gabrielli A, Distler O, Courvoisier DS; EUSTAR collaborators. Glucocorticoids prescribing practices in systemic sclerosis: an analysis of the EUSTAR database. Rheumatology (Oxford). 2022 Sep 13:keac533.
Garaiman A, Steigmiller K, Gebhard C, Mihai C, Dobrota R, Bruni C, Matucci-Cerinic M, Henes J, de Vries-Bouwstra J, Smith V, Doria A, Allanore Y, Dagna L, Anić B, Montecucco C, Kowal-Bielecka O, Martin M, Tanaka Y, Hoffmann-Vold AM, Held U, Distler O, Becker MO; EUSTAR Collaborators. Use of platelet inhibitors for digital ulcers related to systemic sclerosis: EUSTAR study on derivation and validation of the DU-VASC model. Rheumatology (Oxford). 2022 Jul 29:keac405. doi: 10.1093/rheumatology/keac405.
Bruni C, Chung L, Hoffmann-Vold AM, Assassi S, Gabrielli A, Khanna D, Bernstein EJ, Distler O; EUSTAR and SCTC collaborators. High-resolution computed tomography of the chest for the screening, re-screening and follow-up of systemic sclerosis-associated interstitial lung disease: a EUSTAR-SCTC survey. Clin Exp Rheumatol. 2022 Jun 30. doi: 10.55563/clinexprheumatol/7ry6zz
Hughes M, Huang S, Alegre-Sancho JJ, Carreira PE, Engelhart M, Hachulla E, Henes J, Kerzberg E, Pozzi MR, Riemekasten G, Smith V, Szücs G, Vanthuyne M, Zanatta E, Distler O, Gabrielli AG, Hoffmann-Vold AM, Steen VD, Khanna D; EUSTAR Collaborators. Late Skin Fibrosis in Systemic Sclerosis: A Study from the EUSTAR Cohort. Rheumatology (Oxford). 2022 Jun 22:keac363.
Matteson EL, Kelly C, Distler JHW, Hoffmann-Vold AM, Seibold JR, Mittoo S, Dellaripa PF, Aringer M, Pope J, Distler O, James A, Schlenker-Herceg R, Stowasser S, Quaresma M, Flaherty KR; INBUILD Trial Investigators. Nintedanib in Patients with autoimmune disease-related progressive fibrosing interstitial lung diseases: Subgroup Analysis of the INBUILD Trial. Arthritis Rheumatol. 2022 Jun;74(6):1039-1047
Zanatta E, Huscher D, Ortolan A, Avouac J, Airò P, Balbir-Gurman A, Siegert E, Cerinic MM, Cozzi F, Riemekasten G, Hoffmann-Vold AM, Distler O, Gabrielli A, Heitmann S, Hunzelmann N, Montecucco C, Morovic-Vergles J, Ribi C, Doria A, Allanore Y; EUSTAR collaborators. Phenotype of limited cutaneous systemic sclerosis patients with positive anti-topoisomerase I antibodies: data from EUSTAR cohort. Rheumatology (Oxford). 2022 Mar 28:keac188. doi: 10.1093/rheumatology/keac188
Bruni C, Heidenreich S, Duenas A, Hoffmann-Vold AM, Gabrielli A, Allanore Y, Chatelus E, Distler JHW, Hachulla E, Hsu VM, Hunzelmann N, Khanna D, Truchetet ME, Walker UA, Alves M, Schoof N, Saketkoo LA, Distler O. Patient preferences for the treatment of systemic sclerosis-associated interstitial lung disease: a discrete choice experiment. Rheumatology (Oxford). 2022 Mar 3:keac126. doi: 10.1093/rheumatology/keac126
Volkmann ER, Kreuter M, Hoffmann-Vold AM, Wijsenbeek M, Smith V, Khanna D, Denton CP, Wuyts WA, Miede C, Alves M, Sambevski S, Allanore Y; SENSCIS trial investigators. Dyspnoea and cough in patients with systemic sclerosis-associated interstitial lung disease in the SENSCIS trial. Rheumatology (Oxford). 2022 Feb 12:keac091. doi: 10.1093/rheumatology/keac091.
Hilberg O, Hoffmann-Vold AM, Smith V, Bouros D, Kilpeläinen M, Guiot J, Morais A, Clemente S, Daniil Z, Papakosta D, Fretheim H, Neves S, Alfaro TM, Antoniou KM, Valveny N, Asijee G, Soulard S, Wuyts W. Epidemiology of interstitial lung diseases and their progressive-fibrosing behaviour in six European countries. ERJ Open Res. 2022 Jan 24;8(1):00597-2021. doi: 10.1183/23120541.00597-2021. eCollection 2022 Jan
Nina M van Leeuwen, Maaike Boonstra, Håvard Fretheim, Cathrine Brunborg, Øyvind Midtvedt, Torhild Garen, Øyvind Molberg, Tom W J Huizinga, Jeska K de Vries-Bouwstra, Anna-Maria Hoffman-Vold. Gastrointestinal symptom severity and progression in systemic sclerosis. Rheumatology (Oxford) 2022 Oct; 61(10): 4024–4034. Published online 2022 Mar 3. doi: 10.1093/rheumatology/keac118
Janine Schniering, Malgorzata Maciukiewicz, Hubert S. Gabrys, Matthias Brunner, Christian Blüthgen, Chantal Meier, Sophie Braga-Lagache, Anne-Christine Uldry, Manfred Heller, Matthias Guckenberger, Håvard Fretheim, Christos T. Nakas, Anna-Maria Hoffmann-Vold, Oliver Distler, Thomas Frauenfelder, Stephanie Tanadini-Lang, Britta Maurer. Eur Respir J. 2022 May; 59(5): 2004503. Published online 2022 May 19. doi: 10.1183/13993003.04503-2020. PMCID: Computed tomography-based radiomics decodes prognostic and molecular differences in interstitial lung disease related to systemic sclerosis.

Abstracts:

Submitted to EULAR 2023: Bjørkekjær HJ, Bruni C, Carreira P, Airò P, Aznar CPS, Truchetet ME, Giollo A, Balbir-Gurman A, Martin M, Denton CP, Gabrielli A, Fretheim H, Barua I, Bitter H, Midtvedt Ø, Garen T, Broch K, Andreassen A, Tanaka Y, Riemekasten G, Müller-Ladner U, Matucci-Cerinic M, Castellví I, Siegert E, Hachulla E, Distler O, Hoffmann-Vold AM. Comparison of four risk stratification models for prediction of mortality in SSc-PAH in the EUSTAR cohort
Oral presentation at the Nordic PH forum 2022: Bjørkekjær HJ, Bruni C, Carreira P, Airò P, Aznar CPS, Truchetet ME, Giollo A, Balbir-Gurman A, Martin M, Denton CP, Gabrielli A, Fretheim H, Barua I, Bitter H, Midtvedt Ø, Broch K, Andreassen A, Tanaka Y, Riemekasten G, Müller-Ladner U, Matucci-Cerinic M, Castellví I, Siegert E, Hachulla E, Distler O, Hoffmann-Vold AM. Risk Stratification of Patients with Systemic Sclerosis-associated Pulmonary Arterial Hypertension in EUSTAR Using the New Definition of Pulmonary Hypertension
Presented as a poster and orally in the systemic sclerosis community hub at ACR 2022: Bjørkekjær HJ, Bruni C, Carreira P, Airò P, Aznar CPS, Truchetet ME, Giollo A, Balbir-Gurman A, Martin M, Denton CP, Gabrielli A, Fretheim H, Barua I, Bitter H, Midtvedt Ø, Broch K, Andreassen A, Tanaka Y, Riemekasten G, Müller-Ladner U, Matucci-Cerinic M, Castellví I, Siegert E, Hachulla E, Distler O, Hoffmann-Vold AM. Risk Stratification of Patients with SSc-PAH Using the Current and New Proposed Criteria in EUSTAR
Poster tour at EULAR 2022: Bjørkekjær HJ, Bruni C, Carreira P, Airò P, Aznar CPS, Truchetet ME, Giollo A, Balbir-Gurman A, Martin M, Denton CP, Gabrielli A, Fretheim H, Barua I, Bitter H, Midtvedt Ø, Broch K, Andreassen A, Tanaka Y, Riemekasten G, Müller-Ladner U, Matucci-Cerinic M, Castellví I, Siegert E, Hachulla E, Distler O, Hoffmann-Vold AM. Risk stratification approaches perform differently in SSc-associated PAH in EUSTAR
Oral presentation at the Systemic Sclerosis World Congress 2022: Bjørkekjær HJ, Fretheim H, Barua I, Inselseth HM, Bitter H, Midtvedt Ø, Garen T, Andreassen A, Molberg Ø, Broch K, Hoffmann-Vold AM. Risk stratification and treatment regimens in systemic sclerosis-associated pulmonary arterial hypertension
Poster presentation at ACR 2022: Didriksen H, Molberg Ø, Mehta A, Jordan S, Fretheim H, Gude E, Ueland T, Brunborg C, Palchevskiy V, Garen T, Midtvedt Ã, Andreassen A, Lund-Johansen F, Distler O, Belperio J, Hoffmann-Vold A. The Role of CCL21 in Serum Samples from Systemic Sclerosis Patients [abstract]. Arthritis Rheumatol. 2022; 74 (suppl 9). https://acrabstracts.org/abstract/the-role-of-ccl21-in-serum-samples-from-systemic-sclerosis-patients/. Accessed January 31, 2023.
Abstract submission to EULAR 2023 based on Master thesis;
Ramsli ML, Midtvedt Ø, Garen T, Bjørkeskjær HJ, Karterud D, Hoffmann-Vold AM (2022).
High awareness among nurses of the symptom burden patients with SS-PAH is important to provide the best possible patient care.
EULAR 2022: Vikse J, Midtvedt Ø, Molberg Ø et al. Phenotypes and disease characteristics of IgG4-related disease in Norway
ACR 2022: Vikse J, Midtvedt Ø, Molberg Ø et al. Rituximab in IgG4-RD: an open-label non-randomized observational study
Poster presentation at ACR 2022; Maylen Nordgård Carstens, Håvard Fretheim, Imon Barua, Torhild Garen, Henriette Didriksen, Øyvind Midtvedt, Øyvind Molberg, Anna-Maria Hoffmann-Vold. Discordance of patient, physician and nurse assessment of disease burden in systemic sclerosis.
Submitted to EULAR 2023; Maylen Nordgård Carstens, Torhild Garen, Henriette Didriksen, Håvard Fretheim, Imon Barua, Inger-Lise Knutsen, Tone Karaaslan, Tove Hatletveit, Maiju Pesonen, Øyvind Midtvedt, Øyvind Molberg, Anna-Maria Hoffmann-Vold. Sensitivity to change of the ScleroID compared to the UCLA GIT score in a cohort of SSc patients with lower GI symptoms.
Submitted to EULAR 2023: Emily Langballe, Phuong Phuong Diep, Helena Andersson, Cosimo Bruni, Håvard Fretheim, Torhild Garen, Ragnar Gunnarsson, Øyvind Midtvedt, Øyvind Molberg, Øyvind Palm, Silje Reiseter, Trond M Aaløkken, Oliver Distler, Anna-Maria Hoffmann-Vold. ILD progression on standard of care varies in patients with connective tissue disease
EULAR 2022: Barua IS, Vyachelav Palchevskiy, Håvard Fretheim, Torhild Garen, Henriette Didriksen,Trond Mogens Aaløkken, Stephen Weigt, Øyvind Molberg, John Belperio, Anna-Maria Hoffmann-Vold. High levels of both CCL2 and CCL17 were associated with more severe SSc-ILD
ACR 2022: EULAR 2022: Barua IS, Vyachelav Palchevskiy, Håvard Fretheim, Torhild Garen, Henriette Didriksen,Trond Mogens Aaløkken, Stephen Weigt, Øyvind Molberg, John Belperio, Anna-Maria Hoffmann-Vold. High levels of both CCL2 and CCL17 were associated with more severe SSc-ILD.
Submitted to EULAR 2023: Barua IS, Håvard Fretheim, Maylen Nordgård Carstens Torhild Garen, Henriette Didriksen, Øyvind Midtvedt, Øyvind Molberg, Anna-Maria Hoffmann-Vold. SmartPill Technology provides safe and effective assessment of gastrointestinal function in patients with systemic sclerosis.
EULAR and ACR 2022: Håvard Fretheim, Imon Barua, Øyvind Midtvedt, Torhild Garen, Phuong Phuong Diep, Michael Durheim, Cathrine Brunborg, Anna-Maria Hoffmann-Vold. Patients with Systemic Sclerosis have impaired Forced vital capacity, even in the absence of interstitial lung disease.

Grants:

Research Funding for an investigator-initiated study entitled: “Risk stratification, treatment regimens and outcomes in systemic sclerosis associated pulmonary arterial hypertension”.
National funding for the IIS Nor-mILDer homemonitoring strategy trial (Åpen Prosjekt støtte, HSØ)
Simon Fougner Hartmans Familiefond for home monitoring development

Popular science:

Podcast production; "Revmatologi" with 13 episodes in 2022, Jens Vikse.
Members of our group has been presenting webinars for patients in the Norwegian Rheumatology Association
Hilde Jenssen Bjørkekjær was Interviewed by Medpage Today about the abstract presented at the ACR Convergence 2022 “Risk Stratification of Patients with SSc-PAH Using the Current and New Proposed Criteria in EUSTAR”
Overview article about IgG4-RD to the Gastromedicines member magazine, “NGF-nytt” and “Revmatologen”. Jens Vikse