NCT03610217

Brief Summary

Systemic Sclerosis (SSc) is an autoimmune connective tissue disease characterized by autoantibodies, fibrosis and microvascular injury and endothelial cell activation that results in vascular damage. Vascular injury induces both innate and acquired immune responses resulting in fibroblast activation and organ fibrosis. SSc may target multiple organs, including: skin, lungs, heart, vascularization, kidneys, the gastrointestinal tract and musculoskeletal structures. Mortality among scleroderma patients is significant, with a 3.5 standardized mortality ratio (SMR) in studies of prevalent cases. This mortality may be increased in the early years of the disease, reaching a SMR of 4 in a multinational inception cohort. In general, treatment strategies target involved organs as early as possible to avoid damage. Many treatment options are available for each manifestation, but evidence with respect to the order of treatment is scarce. Financial costs, the lack of proper outcome measures, difficulty to recruit patients as a rare disease, all prevent the development of new big clinical trials, oppositely to other common diseases such as stroke or cancer. The heterogeneous features of SSc may make trials challenging. The current guidelines available are the British guidelines (2017) , and the updated European League Against Rheumatism (EULAR) guidelines, published in 2017. Management guidelines have some gaps regarding second-line treatment, combinations and there are no proposed algorithms. With the pragmatic trials, the investigators intend to fill the gap between the complicated randomized clinical trials and the observational studies. Using the treatments that have already been proved useful in SSc, in an open-label randomized way and based on some refined expert-made algorithms, will allow the investigators to establish the order in how to use them. Patients will be offered to participate with the collection of their clinical data and, if they give their consent, they will be randomized according to the algorithms. There will be an optional part of the study consisting in the collection of blood samples and skin samples for future research.

Trial Health

43
At Risk

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Trial has exceeded expected completion date
Enrollment
400

participants targeted

Target at P75+ for not_applicable

Timeline
Completed

Started Oct 2018

Typical duration for not_applicable

Geographic Reach
1 country

1 active site

Status
unknown

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

First Submitted

Initial submission to the registry

July 25, 2018

Completed
7 days until next milestone

First Posted

Study publicly available on registry

August 1, 2018

Completed
2 months until next milestone

Study Start

First participant enrolled

October 1, 2018

Completed
3 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

October 1, 2021

Completed
Same day until next milestone

Study Completion

Last participant's last visit for all outcomes

October 1, 2021

Completed
Last Updated

August 6, 2018

Status Verified

August 1, 2018

Enrollment Period

3 years

First QC Date

July 25, 2018

Last Update Submit

August 2, 2018

Conditions

Outcome Measures

Primary Outcomes (11)

  • Forced vital capacity %

    Variation of the forced vital capacity %

    1 year

  • Bleeding

    Documentation of bleeding

    1 year

  • Raynaud's phenomenon visual analog scale

    Raynaud's phenomenon visual analog scale variation ranging from 0 to 100 mm (0 no Raynaud's phenomenon, 100 very intense Raynaud's phenomenon)

    3 months

  • Time to the healing of a digital ulcer

    Time to the healing of a digital ulcer

    1 year

  • Time to the development of a new digital ulcer

    Time to the development of a new digital ulcer

    1 year

  • Disease activity score 28

    Disease activity score 28 accounting for tender and swollen joints over 28 possible joints. Values \<2.6 remission, values \<3.2 low disease activity, values \>5.1 high disease activity

    3 months

  • GERD-HRQL

    Variation of the Gastro-esophageal reflux disease-health related quality of life questionnaire, ranging from 0 (no symptoms) to 75 (worst symptoms)

    3 months

  • Diarrhea visual analog scale

    Diarrhea visual analog scale variation ranging from 0 to 100 mm (0 no diarrhea, 100 very intense diarrhea)

    3 months

  • Constipation visual analog scale

    constipation visual analog scale variation ranging from 0 to 100 mm (0 no constipation, 100 very intense constipation)

    3 months

  • Modified Rodnan skin score

    Modified Rodnan skin score variation. Ranging from a total of 0 (no induration) to 51 (maximum induration)

    1 year

  • Pain visual analog scale

    Pain visual analog scale variation, ranging from 0 to 100 mm (0 no pain, 100 very intense pain)

    3 months

Study Arms (10)

Interstitial lung disease induction

EXPERIMENTAL
Other: Interstitial lung disease induction algorithm

Pulmonary arterial hypertension

EXPERIMENTAL
Other: Pulmonary arterial hypertension algorithm

Raynaud's phenomenon

EXPERIMENTAL
Other: Raynaud's phenomenon algorithm

Digital ulcers

EXPERIMENTAL
Other: Digital ulcer algorithm

Inflammatory arthritis

EXPERIMENTAL
Other: Inflammatory arthritis algorithm

Gastroesophageal reflux

EXPERIMENTAL
Other: Gastroesophageal reflux algorithm

Bacterial overgrowth

EXPERIMENTAL
Other: Bacterial overgrowth algorithm

Constipation

EXPERIMENTAL
Other: Constipation algorithm

Skin involvement

EXPERIMENTAL
Other: Skin involvement algorithm

Pain

EXPERIMENTAL
Other: Pain algorithm

Interventions

Patients failing first line mycophenolic acid (MFA) will be randomized to MFA plus rituximab or intravenous cyclophosphamide. If they fail the second line they will be crossed over to the other option.

Interstitial lung disease induction

Patients diagnosed with pulmonary arterial hypertension secondary to systemic sclerosis will be randomized to receive anticoagulation (warfarin, rivaroxaban or apixaban)

Pulmonary arterial hypertension

Patients with mild Raynaud's phenomenon not responding to the first line treatment (nifedipine), will be randomized to receive losartan or nifedipine plus atorvastatin or nifedipine plus losartan. If they fail the second line they will be crossed over to the other options randomly. Patients with severe Raynaud's phenomenon not responding to the first line treatment (nifedipine) or failing the previous mild Raynaud's algorithm, will be randomized to receive nifedipine plus sildenafil or nifedipine plus intravenous iloprost. If they fail the second line they will be crossed over to the other option.

Raynaud's phenomenon

Patients with active digital ulcers not healing after 3 months or developing new ones with nifedipine will be randomized to nifedipine plus sildenafil or nifedipine plus intravenous iloprost. If they fail the second line they will be crossed over to the other option. Patients who develop new digital ulcers under treatment with nifedipine will be randomized to nifedipine plus atorvastatin plus standard of care or nifedipine plus standard of care. If they fail the second line they will be crossed over to the other option.

Digital ulcers

Patients with inflammatory arthritis failing methotrexate and/or prednisone and/or hydroxychloroquine and/or sulfasalazine will be randomized to receive intravenous rituximab or subcutaneous tocilizumab. If they fail the second line they will be crossed over to the other option.

Inflammatory arthritis

Patients with gastroesophageal reflux failing standard doses of proton pump inhibitors (PPI) will be randomized to receive double doses of PPI or standard dose of PPI plus ranitidine or double doses of PPI plus domperidone or double doses of PPI plus prucalopride/erythromycin. If they fail the second line they will be crossed over to the other options randomly.

Gastroesophageal reflux

Patients with bacterial overgrowth will be randomized to receive erythromycin or metronidazole or amoxicillin. If they fail the second line they will be crossed over to the other options randomly.

Bacterial overgrowth

Patients with constipation will be randomized to receive bisacodyl or magnesium sulphate or polyethylene glycol or senna.If they fail the second line they will be crossed over to the other options randomly.

Constipation

Patients with skin involvement and modified Rodnan skin score \<32 will be randomized to receive methotrexate or mycophenolic acid or methotrexate plus mycophenolic acid. If they fail the second line they will be crossed over to the other option. Patients with skin involvement and modified Rodnan skin score \>32 will be randomized to receive methotrexate plus mycophenolic acid or intravenous cyclophosphamide. If they fail the second line they will be crossed over to the other option.

Skin involvement

Patients with pain failing first line treatment with acetaminophen or celecoxib or ibuprofen will be randomized to receive pregabalin or duloxetine. Patients with skin involvement and modified Rodnan skin score \<32 will be randomized to receive methotrexate or mycophenolic acid or methotrexate plus mycophenolic acid. If they fail the second line they will be crossed over to the other option. In case they fail the second line they will be treated with medical marijuana.

Pain

Eligibility Criteria

Age18 Years+
Sexall
Healthy VolunteersNo
Age GroupsAdult (18-64), Older Adult (65+)

You may qualify if:

  • Any patient with an age \>18 years meeting the 2013 SSc classification criteria managed at the Rheumatology division, St. Joseph's Healthcare London.
  • Patients who refuse to be randomized for treatments but wish to provide their data for the registry will also be included, after signing the informed consent form.

You may not qualify if:

  • Refusal to participate or to sign an informed consent form.

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (1)

Saint Joseph's Health Care London

London, Ontario, n6a 4v2, Canada

Location

Related Publications (1)

  • Fernandez-Codina A, Walker KM, Pope JE; Scleroderma Algorithm Group. Treatment Algorithms for Systemic Sclerosis According to Experts. Arthritis Rheumatol. 2018 Nov;70(11):1820-1828. doi: 10.1002/art.40560. Epub 2018 Sep 17.

Related Links

MeSH Terms

Conditions

Scleroderma, Systemic

Condition Hierarchy (Ancestors)

Connective Tissue DiseasesSkin and Connective Tissue DiseasesSkin Diseases

Central Study Contacts

Janet E Pope, MD, MPH, FRCPSC

CONTACT

Andreu Fernandez-Codina, MD, MSc

CONTACT

Study Design

Study Type
interventional
Phase
not applicable
Allocation
RANDOMIZED
Masking
NONE
Purpose
TREATMENT
Intervention Model
SEQUENTIAL
Sponsor Type
OTHER
Responsible Party
PRINCIPAL INVESTIGATOR
PI Title
Principal investigator

Study Record Dates

First Submitted

July 25, 2018

First Posted

August 1, 2018

Study Start

October 1, 2018

Primary Completion

October 1, 2021

Study Completion

October 1, 2021

Last Updated

August 6, 2018

Record last verified: 2018-08

Data Sharing

IPD Sharing
Will not share

Locations