Clinical trial NCT05428826: Early Discontinuation of Steroid Treatment in Negative FDG-PET/CT Patients With Idiopathic Retroperitoneal Fibrosis
Official title: Early Discontinuation of Steroid Treatment in Negative FDG-PET/CT Patients With Idiopathic Retroperitoneal Fibrosis. A Prospective Multicentric Study
Study type: INTERVENTIONAL
Overall status: RECRUITING
Phase: PHASE4
Conditions: Idiopathic Retroperitoneal Fibrosis
Keywords: Steroid treatment
Lead sponsor: Assistance Publique - Hôpitaux de Paris
Sponsor class: OTHER
First posted: 2022-06-23
Enrollment: 41
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Allocation: NA
Intervention model: SINGLE_GROUP
Intervention model description: Phase 4 Prednisone Dose : 1mg/kg/day at inclusion Route of administration : oral Duration of treatment: 9 to 21 months.
Primary purpose: TREATMENT

Brief summary: Adult patients with a diagnosis of idiopathic retroperitoneal fibrosis Prospective multicentric cohort study Intervention : administration of prednisone during 9 to 21 months at 1mg/kg/day at inclusion.

Detailed description: At baseline visit: Eligible patients will be screen during a standard visit care (consultation or hospitalization). A clinical examination, an abdominal CT scan, blood and urine biological tests will be performed.

At inclusion visit: After verification of inclusion and non inclusion criteria, if the patient meets the eligibility criteria, the investigator, will provide the patient with information and details regarding the trial. The consent is obtained and signed after a reflection period of 30 minutes.

The following procedure will be scheduled within 7 days:

* 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET/CT) (pregnancy test if mandatory)
* Specimens for the biocollection Patients with positive FDG PET/CT (hypermetabolism grade II or III) at M0 will receive oral steroids (prednisone) at 1mg/kg/day during 1 month and then the dose will be tapered to obtain \<10mg/day at 6 months and \<7,5mg/day at 9 months.

Patients with a negative FDG-PET/CT (hypermetabolism grade 0 or I) at M0 will be excluded of the study.

Follow-up visits : M6, M9,M12,M15,M21, relapse At M6, M12, and M15: During these visits clinical examination (blood pressure measurement, body temperature, heart rate, weight and clinical signs or symptoms related to IRF) will be performed. An abdominal CT scan may be performed as part of the care depending on the clinician's judgment. Glucocorticoid compliance and tapering, concomitant medications and adverse events (including serious cardiovascular adverse events) will be assessed and recorded. A nurse will collect blood and urine.

At M9, M21 or relapse : During these visits clinical examination, an abdominal CT scan, a FDG-PET/CT blood and urine biological tests will be performed.

At M9: The patients who failed to reach remission at M9 are considered as treatment failure and will be treated on best medical judgment by the investigator and excluded to the study. The patients who had a dose of prednisone ≥7,5mg / day at M9 will also be excluded to the study.

Patients who achieved remission at M9 and have a retroperitoneal fibrosis visual score grade 0 or I under a dose of prednisone \<7,5mg / day will discontinue steroids treatment.

Patients who achieved remission at M9 and have a retroperitoneal fibrosis visual score grade II or III under a dose of prednisone \<7,5mg / day will continue steroids treatment at the actual dose (medical judgment).

Eligibility criteria: Inclusion Criteria:

* Patient over 18 years old
* New onset or untreated relapsing of active idiopathic retroperitoneal fibrosis (IRF) defined by the association of:
* Related-disease symptoms (Appendix 17.2) or elevated CRP level (\>20 mg/l) AND
* Retroperitoneal peri-aortic mass that surrounds the abdominal vessels on CT-scan

Exclusion Criteria:

* Secondary retroperitoneal fibrosis including drug-related retroperitoneal fibrosis, active infections (such as tuberculosis) or malignancies, systemic vasculitis (such as Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis), Erdheim-Chester disease (Appendix 17.3),patients with IgG4 disease may be enrolled
* Contraindication to perform FDG-PET/CT,
* Contraindication to perform CT scan with injection of contrast agent,
* Contraindication to treatment by prednisone
* Active infection
* Acute or chronic liver disease that is deemed sufficiently severe to impair their ability to participate in the trial,
* Active or history of malignancy in last 5 years. Individuals with squamous cell or basal cell skin carcinomas and individuals with cervical carcinoma in situ may be enrolled if they have received curative surgical treatment,
* Serum creatinine level greater than 400 µmol/L that cannot be attributed to underlying IRF,
* Live vaccination received from 4 weeks before inclusion,
* Inhaled glucocorticoids (except for patients with documented asthma),
* Any previous treatment with rituximab, methotrexate, alemtuzumab, cyclophosphamide, azathioprine, mycophenolate mofetil, infliximab, adalimumab, etanercept within the past 3 months,
* Pregnancy or breastfeeding,
* Non-affiliation to a social security regime,
* Subject deprived of freedom, subject under a legal protective measure
* Refusal to participate

Primary outcomes:
measure: To compare the cumulative IRF relapse rate 12 months after discontinuation of steroids.
timeFrame: 12 months after discontinuation of steroids
Secondary outcomes:
measure: To analyze the characteristics of hypermetabolism of IRF in FDG-PET/CT and their evolution at inclusion
timeFrame: at inclusion
measure: To analyze the characteristics of hypermetabolism of IRF in FDG-PET/CT and their evolution at inclusion
timeFrame: at inclusion
measure: To analyze the characteristics of hypermetabolism of IRF in FDG-PET/CT and their evolution at inclusion
timeFrame: at inclusion
measure: To analyze the characteristics of hypermetabolism of IRF in FDG-PET/CT and their evolution at remission (M9)
timeFrame: 9 months after the inclusion
measure: To analyze the characteristics of hypermetabolism of IRF in FDG-PET/CT and their evolution at remission (M9)
timeFrame: 9 months after the inclusion
measure: To analyze the characteristics of hypermetabolism of IRF in FDG-PET/CT and their evolution at remission (M9)
timeFrame: 9 months after the inclusion
measure: To analyze the characteristics of hypermetabolism of IRF in FDG-PET/CT and their evolution at M21
timeFrame: 21 months after the inclusion
measure: To analyze the characteristics of hypermetabolism of IRF in FDG-PET/CT and their evolution at M21
timeFrame: 21 months after the inclusion
measure: To analyze the characteristics of hypermetabolism of IRF in FDG-PET/CT and their evolution at M21
timeFrame: 21 months after the inclusion
measure: To analyze the characteristics of hypermetabolism of IRF in FDG-PET/CT and their evolution at relapse
timeFrame: between inclusion and 21 months after the inclusion
measure: To analyze the characteristics of hypermetabolism of IRF in FDG-PET/CT and their evolution at relapse
timeFrame: between inclusion and 21 months after the inclusion
measure: To analyze the characteristics of hypermetabolism of IRF in FDG-PET/CT and their evolution at relapse
timeFrame: between inclusion and 21 months after the inclusion
measure: To assess the performance of hypermetabolism of IRF in FDG-PET/CT for diagnosis of disease activity,
timeFrame: 21 months after the inclusion
measure: To assess the performance of hypermetabolism of IRF in FDG-PET/CT for diagnosis of disease activity,
timeFrame: 21 months after the inclusion
measure: To compare at M21 the corticosteroids therapy - related adverse events between patients who continue or discontinue the treatment at M9.
timeFrame: 21 months after the inclusion
Other outcomes:
Locations:
city: Agen
country: France
facility: Médecine Interne
city: Bordeaux
country: France
facility: Médecine interne et maladies infectieuses - GH Sud Haut Lévêque
city: Boulogne-Billancourt
country: France
facility: Médecine interne - Ambroise Paré
city: Brest
country: France
facility: Médecine interne
city: Créteil
country: France
facility: Médecine interne - Henri-Mondor
city: Dijon
country: France
facility: Médecine interne et immunologie clinique - Dijon
city: Lille
country: France
facility: Médecine interne - Lille
city: Marseille
country: France
facility: Médecine Interne - La Timone
city: Paris
country: France
facility: Médecine interne - Saint Antoine
city: Paris
country: France
facility: Médecine Interne, Vascularites et Myosites - La Pitié Salpêtrière
city: Paris
country: France
facility: Médecine interne - Cochin
city: Paris
country: France
facility: Médecine vasculaire - HEGP
city: Paris
country: France
facility: Médecine Interne - Bichat
city: Paris
country: France
facility: Néphrologie - Bichat
city: Saint-Denis
country: France
facility: Médecine interne - Delafontaine
Study officials:
affiliation: Assistance Publique - Hôpitaux de Paris (AP-HP)
name: Aline DECHANET
role: STUDY_CHAIR
References: