Safety and Efficacy of Itacitinib in Adults With Systemic Sclerosis

NCT ID: NCT04789850

Last Updated: 2025-11-20

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Clinical Phase

PHASE2

Total Enrollment

74 participants

Study Classification

INTERVENTIONAL

Study Start Date

2023-02-02

Study Completion Date

2026-02-28

Brief Summary

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The purpose of this study is to determine whether itacitinib is safe and effective in the treatment of systemic sclerosis in adults.

Detailed Description

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Systemic sclerosis (SSc) is a rare systemic autoimmune connective tissue-disease characterized by fibrosis, inflammation, and vasculopathy. SSc is responsible for skin fibrosis that can either be limited or diffuse. The latter phenotype of the disease is commonly associated with visceral involvement and therefore similar to graft versus host disease (GvHD) reaction. It can be life threatening in case of pulmonary or cardiovascular involvement. Nonetheless SSc remains a severe disease responsible for important disability and a poor quality of life.

There is a growing body of evidence that supports the implication of the JAK-STAT tyrosine kinases pathway in the activation of fibroblasts of patients with SSc. A genetic polymorphism of STAT4 was found to be associated with the diffuse form of the disease and inhibition of STAT4 gene is associated with a decrease in TGF-ß and IL-6 cytokines activation, which are two major cytokines implicated in SSc pathogenesis. Recently, Pedroza et al. confirmed the implication of STAT3 in skin fibrosis mechanisms. Indeed, the authors showed an enhanced activation of STAT3 and demonstrated in vivo that the inhibition of STAT3 phosphorylation prevented skin fibrosis in a murine model of SSc. These data were confirmed by a work of Zhang et al. who showed that the inhibition of JAK1 was also needed to prevent skin and lung fibrosis. Altogether these works confirmed the implication of the JAK pathway in fibrosis mechanism.

Itacitinib is a Janus kinase inhibitor that specifically targets JAK1 and decreases STAT3 phosphorylation. Itacitinib was shown to efficiently treat patients with myelofibrosis, rheumatoid arthritis, and chronic plaque psoriasis. Very interestingly, itacitinib efficacy has also been reported in patients with acute GvHD. Altogether these data and studies reinforced the investigator's working hypothesis.

The efficacy and safety of this proposal must be tested.

Conditions

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Systemic Sclerosis

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

QUADRUPLE

Participants Caregivers Investigators Outcome Assessors

Study Groups

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Itacitinib

200mg of oral Itacitinib everyday for 360 days.

Group Type EXPERIMENTAL

Itacitinib

Intervention Type DRUG

200 mg oral for 360 days

Placebo

Oral placebo everyday for 360 days.

Group Type PLACEBO_COMPARATOR

Placebo

Intervention Type DRUG

200 mg oral for 360 days

Interventions

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Itacitinib

200 mg oral for 360 days

Intervention Type DRUG

Placebo

200 mg oral for 360 days

Intervention Type DRUG

Eligibility Criteria

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Inclusion Criteria

* Adult patient (≥18 years old)
* Patient with a diagnosis of diffuse SSc, as defined by the American College of Rheumatology / EULAR 2013 criteria,
* Patient with a diffuse SSc, according to Leroy and Medsger dichotomy
* Patient with a SSc disease duration of less than 36 months (defined as time from first non-Raynaud phenomenon manifestation) or with an active SSc disease, as defined by EUSTAR disease activity score,
* Patient with a modified Rodnan skin score (mRSS) ≥ 10 and ≤ 35 units at screening,
* Negative pregnancy test for woman of childbearing potential, woman of childbearing potential should have reliable contraception for the 12 months' duration of the study,
* Patient able to give written informed consent prior to participation in the study,
* Affiliation to a social security scheme (profit or being entitled)
* If patients receive mycophenolate or methotrexate for SSc, these need to be on stable dose as follows:

* Mycophenolate mofetil/sodium: stable dose for at least 2 months prior to randomisation
* Methotrexate: stable dose and route of administration for at least 2 months prior to randomisation

Exclusion Criteria

* Previous treatment with itacitinib or a Janus kinase (JAK) inhibitor,
* Contra-indications to itacitinib or Janus kinase inhibitor,
* Failure to sign the informed consent or unable to consent
* Patient participating in another investigational therapeutic study,
* Acute or chronic active infections, including HBV, HCV, HIV,
* Patient with other uncontrolled diseases, including drug or alcohol abuse, severe psychiatric diseases, that could interfere with participation in the trial according to the protocol,
* Patient suspected not to be observant to the proposed treatments,
* Patient who have white blood cell count ≤ 4,000/mm3,
* Patient who have platelet count ≤ 100,000/mm3,
* Patients who have ALT or AST level greater that 3 times the upper limit of normal,
* Patient who have triglyceride level greater than 5g/L
* Pregnant or breastfeeding woman,
* Protected adults (including individual under guardianship by court order),
* Patient receiving or having received cyclophosphamide or rituximab within the last three months (possible inclusion beyond 3 months),
* Patient receiving or having received a biotherapy (anti-TNF, abatacept or tocilizumab) in the last 3 months (possible inclusion beyond 3 months)
* Patient with Systemic Lupus, or Sjögren's syndrome with systemic manifestations justifying immunosuppressive therapy
* Atherosclerotic cardiovascular disease as defined by a history of myocardial infarction, ischaemic stroke, or peripheral artery thrombosis
* Anti-phospholipid syndrome
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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URC-CIC Paris Descartes Necker Cochin

OTHER

Sponsor Role collaborator

Assistance Publique - Hôpitaux de Paris

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Luc Mouthon, MD-PhD

Role: STUDY_CHAIR

Assistance Publique - Hôpitaux de Paris

Benjamin Chaigne, MD

Role: PRINCIPAL_INVESTIGATOR

Assistance Publique - Hôpitaux de Paris

Locations

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CH Amiens

Amiens, , France

Site Status RECRUITING

CHU Angers

Angers, , France

Site Status RECRUITING

CHU Annecy

Annecy, , France

Site Status RECRUITING

CHU Besançon

Besançon, , France

Site Status RECRUITING

Avicenne Hospital

Bobigny, , France

Site Status RECRUITING

CHU Bordeaux

Bordeaux, , France

Site Status RECRUITING

CHU Bordeaux

Bordeaux, , France

Site Status RECRUITING

Ambroise Paré hospital

Boulogne-Billancourt, , France

Site Status RECRUITING

Hôpital de la Cavale Blanche

Brest, , France

Site Status RECRUITING

CHU Caen

Caen, , France

Site Status RECRUITING

CHU Gabriel Montpied

Clermont-Ferrand, , France

Site Status RECRUITING

Henry Mondor hospital

Créteil, , France

Site Status RECRUITING

CH Dax-Côte d'ARgent

Dax, , France

Site Status RECRUITING

CHU Dijon

Dijon, , France

Site Status RECRUITING

CHU Grenoble

Grenoble, , France

Site Status RECRUITING

CHU Grenoble

Grenoble, , France

Site Status RECRUITING

CH Le Mans

Le Mans, , France

Site Status RECRUITING

CHU Lille

Lille, , France

Site Status RECRUITING

CHU Limoges

Limoges, , France

Site Status RECRUITING

CHU Lyon sud

Lyon, , France

Site Status RECRUITING

Hôpital Nord

Marseille, , France

Site Status RECRUITING

La Timone Hospital

Marseille, , France

Site Status RECRUITING

La Timone Hospital

Marseille, , France

Site Status RECRUITING

Robert Schuman Hospital

Metz, , France

Site Status RECRUITING

CHU Montpellier - rhumatology

Montpellier, , France

Site Status RECRUITING

CHU Montpellier - St Eloi Hospital

Montpellier, , France

Site Status RECRUITING

CHU Nancy

Nancy, , France

Site Status RECRUITING

CHU Nantes

Nantes, , France

Site Status RECRUITING

Hopital L'Archet 1

Nice, , France

Site Status RECRUITING

Hospital Pasteur - CHU Nice

Nice, , France

Site Status RECRUITING

Saint Antoine Hospital

Paris, , France

Site Status NOT_YET_RECRUITING

La Pitié-Salpêtrière

Paris, , France

Site Status RECRUITING

La Pitié-Salpêtrière

Paris, , France

Site Status RECRUITING

Cochin Hospital

Paris, , France

Site Status RECRUITING

Hospital Croix St Simon

Paris, , France

Site Status NOT_YET_RECRUITING

CHU Poitiers

Poitiers, , France

Site Status RECRUITING

CH de Cornouaille

Quimper, , France

Site Status RECRUITING

Robert Debré Hospital

Reims, , France

Site Status RECRUITING

Hôpital Sud

Rennes, , France

Site Status RECRUITING

CHU Rouen

Rouen, , France

Site Status NOT_YET_RECRUITING

CHU Saint Etienne

Saint-Etienne, , France

Site Status RECRUITING

Nouvel Hospital Civil

Strasbourg, , France

Site Status RECRUITING

Rangueil Hospital

Toulouse, , France

Site Status RECRUITING

CHU Tours

Tours, , France

Site Status RECRUITING

CH Valenciennes

Valenciennes, , France

Site Status RECRUITING

Hôpitaux de Barbois

Vandœuvre-lès-Nancy, , France

Site Status RECRUITING

Countries

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France

Central Contacts

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Benjamin Chaigne, MD

Role: CONTACT

+33 1 58 41 41 17

Adèle BELLINO

Role: CONTACT

+33 1 58 41 11 95

Facility Contacts

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Benjamin Chaigne, MD

Role: primary

+33 1 58 41 41 17

References

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Almeida C, Almeida I, Vasconcelos C. Quality of life in systemic sclerosis. Autoimmun Rev. 2015 Dec;14(12):1087-96. doi: 10.1016/j.autrev.2015.07.012. Epub 2015 Jul 23.

Reference Type BACKGROUND
PMID: 26212726 (View on PubMed)

Mouthon L. SSc in 2011: From mechanisms to medicines. Nat Rev Rheumatol. 2012 Jan 10;8(2):72-4. doi: 10.1038/nrrheum.2011.203.

Reference Type BACKGROUND
PMID: 22231235 (View on PubMed)

Distler JH, Feghali-Bostwick C, Soare A, Asano Y, Distler O, Abraham DJ. Review: Frontiers of Antifibrotic Therapy in Systemic Sclerosis. Arthritis Rheumatol. 2017 Feb;69(2):257-267. doi: 10.1002/art.39865. No abstract available.

Reference Type BACKGROUND
PMID: 27636741 (View on PubMed)

Wang Y, Fan PS, Kahaleh B. Association between enhanced type I collagen expression and epigenetic repression of the FLI1 gene in scleroderma fibroblasts. Arthritis Rheum. 2006 Jul;54(7):2271-9. doi: 10.1002/art.21948.

Reference Type BACKGROUND
PMID: 16802366 (View on PubMed)

Landi C, Bargagli E, Bianchi L, Gagliardi A, Carleo A, Bennett D, Perari MG, Armini A, Prasse A, Rottoli P, Bini L. Towards a functional proteomics approach to the comprehension of idiopathic pulmonary fibrosis, sarcoidosis, systemic sclerosis and pulmonary Langerhans cell histiocytosis. J Proteomics. 2013 May 27;83:60-75. doi: 10.1016/j.jprot.2013.03.006. Epub 2013 Mar 23.

Reference Type BACKGROUND
PMID: 23528693 (View on PubMed)

Kubo M, Ihn H, Yamane K, Tamaki K. Up-regulated expression of transforming growth factor beta receptors in dermal fibroblasts in skin sections from patients with localized scleroderma. Arthritis Rheum. 2001 Mar;44(3):731-4. doi: 10.1002/1529-0131(200103)44:33.0.CO;2-U. No abstract available.

Reference Type BACKGROUND
PMID: 11263790 (View on PubMed)

Zhang Y, Liang R, Chen CW, Mallano T, Dees C, Distler A, Reich A, Bergmann C, Ramming A, Gelse K, Mielenz D, Distler O, Schett G, Distler JHW. JAK1-dependent transphosphorylation of JAK2 limits the antifibrotic effects of selective JAK2 inhibitors on long-term treatment. Ann Rheum Dis. 2017 Aug;76(8):1467-1475. doi: 10.1136/annrheumdis-2016-210911. Epub 2017 May 6.

Reference Type BACKGROUND
PMID: 28478401 (View on PubMed)

Migita K, Izumi Y, Torigoshi T, Satomura K, Izumi M, Nishino Y, Jiuchi Y, Nakamura M, Kozuru H, Nonaka F, Eguchi K, Kawakami A, Motokawa S. Inhibition of Janus kinase/signal transducer and activator of transcription (JAK/STAT) signalling pathway in rheumatoid synovial fibroblasts using small molecule compounds. Clin Exp Immunol. 2013 Dec;174(3):356-63. doi: 10.1111/cei.12190.

Reference Type BACKGROUND
PMID: 23968543 (View on PubMed)

Xu Y, Wang W, Tian Y, Liu J, Yang R. Polymorphisms in STAT4 and IRF5 increase the risk of systemic sclerosis: a meta-analysis. Int J Dermatol. 2016 Apr;55(4):408-16. doi: 10.1111/ijd.12839. Epub 2015 Dec 29.

Reference Type BACKGROUND
PMID: 26712637 (View on PubMed)

Avouac J, Furnrohr BG, Tomcik M, Palumbo K, Zerr P, Horn A, Dees C, Akhmetshina A, Beyer C, Distler O, Schett G, Allanore Y, Distler JH. Inactivation of the transcription factor STAT-4 prevents inflammation-driven fibrosis in animal models of systemic sclerosis. Arthritis Rheum. 2011 Mar;63(3):800-9. doi: 10.1002/art.30171.

Reference Type BACKGROUND
PMID: 21360510 (View on PubMed)

Kremer JM, Bloom BJ, Breedveld FC, Coombs JH, Fletcher MP, Gruben D, Krishnaswami S, Burgos-Vargas R, Wilkinson B, Zerbini CA, Zwillich SH. The safety and efficacy of a JAK inhibitor in patients with active rheumatoid arthritis: Results of a double-blind, placebo-controlled phase IIa trial of three dosage levels of CP-690,550 versus placebo. Arthritis Rheum. 2009 Jul;60(7):1895-905. doi: 10.1002/art.24567.

Reference Type BACKGROUND
PMID: 19565475 (View on PubMed)

Fridman JS, Scherle PA, Collins R, Burn T, Neilan CL, Hertel D, Contel N, Haley P, Thomas B, Shi J, Collier P, Rodgers JD, Shepard S, Metcalf B, Hollis G, Newton RC, Yeleswaram S, Friedman SM, Vaddi K. Preclinical evaluation of local JAK1 and JAK2 inhibition in cutaneous inflammation. J Invest Dermatol. 2011 Sep;131(9):1838-44. doi: 10.1038/jid.2011.140. Epub 2011 Jun 16.

Reference Type BACKGROUND
PMID: 21677670 (View on PubMed)

Deverapalli SC, Rosmarin D. The use of JAK inhibitors in the treatment of progressive systemic sclerosis. J Eur Acad Dermatol Venereol. 2018 Aug;32(8):e328. doi: 10.1111/jdv.14876. Epub 2018 Mar 6. No abstract available.

Reference Type BACKGROUND
PMID: 29444362 (View on PubMed)

Gordon JK, Martyanov V, Franks JM, Bernstein EJ, Szymonifka J, Magro C, Wildman HF, Wood TA, Whitfield ML, Spiera RF. Belimumab for the Treatment of Early Diffuse Systemic Sclerosis: Results of a Randomized, Double-Blind, Placebo-Controlled, Pilot Trial. Arthritis Rheumatol. 2018 Feb;70(2):308-316. doi: 10.1002/art.40358. Epub 2017 Dec 29.

Reference Type BACKGROUND
PMID: 29073351 (View on PubMed)

Other Identifiers

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2019-003430-16

Identifier Type: EUDRACT_NUMBER

Identifier Source: secondary_id

APHP180613

Identifier Type: -

Identifier Source: org_study_id

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