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Familial Mediterranean FeverPhase 2NCT00582907

Rilonacept for Treatment of Familial Mediterranean Fever (FMF)

A Phase 2 study testing Rilonacept in people with Familial Mediterranean Fever, run by The Cleveland Clinic at 5 sites in United States.

Completed

Finished. The study has ended and is not enrolling. Results may be posted on the official record.

Official record
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Trial summary

Familial Mediterranean fever (FMF) is a genetic disease resulting in recurrent attacks of fever, abdominal pain, chest pain, arthritis and rash. There are 5-15% of patients who continue to have FMF attacks despite treatment with colchicine or who cannot tolerate colchicine. Read more about this study

Who is it for?
Ages 4 years and older
Conditions the study is for
Familial Mediterranean Fever
What is being tested?
Rilonacept
Type of treatment
Injection or infusion
Could I receive a placebo?
Yes, some participants may receive a placebo
How many people
14 participants
Where
5 sitesUnited States
Study dates
Aug 2008to Sep 2011
Phase
Phase 2
Are there results?
Yes, posted in the registrySummary in the doctors' view

Key requirements

Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.

  • Ages 4 years and older
  • All sexes
  • No healthy volunteers
  • Child, Adult, Older adult

Who may be able to join

  • Subject has a definitive diagnosis of FMF as by the Tel-Hashomer clinical criteria (long version of criteria) with at least one mutation on one of the MEFV gene alleles. However, subjects with an isolated heterozygous mutation of exon 2 of the MEFV gene (including E148Q) will not be eligible.
  • Subject must have an estimated mean of at least one acute FMF attack per month before and during the month of screening.
  • Subject is at least four years of age (with no upper limit of age).
  • Subjects must have received an adequate trial of colchicine defined as treatment of at least 1.5 mg/d for at least 3 months if ≥6 years old or 1.2 mg/d if less than 6 years, or an inability to tolerate colchicine due to adverse effects in a dose that controls acute attacks in the frequency of less than one attack per month.
  • If subject is being treated with anakinra at the time of consent, washout must be done (about 3 days). Subject must experience 2 attacks before randomization visit can occur.
  • If subject has been treated previously with anti-TNF drugs, appropriate washout must be done. Etanercept must be discontinued for 4 weeks prior to randomization; Adalimumab and Infliximab must be discontinued for 8 weeks prior to randomization.
Show all 11Show fewer
  • If subject is a female of childbearing potential, she must agree to use adequate contraception (adequate contraception can include abstinence) for the duration of the trial and 3 months after and must have a negative serum or urine pregnancy test prior to administration of study medication.
  • If subject is a male and has reached puberty, he must agree to use adequate contraception or abstinence during the study and for 3 months after discontinuation from study.
  • Subject's parent or legal guardian has provided written informed consent prior to screening for this study or if subject is older than 18 years has provided informed consent him/herself.
  • Subject, if applicable, has assented to participate prior to screening for this study.
  • Subject and, if applicable, parent/legal guardian, agree to comply with study requirements and are able to come to the clinic for all required study visits.

Who cannot join

  • The subject has existing biopsy proven amyloidosis or proteinuria >0.5 gram per day.
  • The subject has another active inflammatory rheumatic disease.
  • The subject has an active malignancy of any type, or history of a malignancy.
  • The subject has active GI disease (e.g., inflammatory bowel disease), a chronic or acute renal or hepatic disorder, or a significant coagulation defect.
  • The subject has an AST (SGOT), ALT (SGPT) or BUN >2 x ULN or creatinine >1.5 mg/dL or any other laboratory abnormality considered by the examining physician to be clinically significant within 28 days before the Baseline visit.
  • Current use of an anti-tumor necrosis factor drug.
Show all 19Show fewer
  • The subject has, in the investigator's opinion, a chronic condition (e.g., diabetes, epilepsy) that is either not stable or well-controlled and may interfere with the conduct of the study.
  • The subject has received any investigational medication within 30 days before the first dose of study medication or is scheduled to receive an investigational drug, other than study medications described in this protocol, during the course of the study.
  • The subject has chronic or active infection or any major episode of infection requiring hospitalization or treatment with i.v. antibiotics within 30 days or oral antibiotics within 14 days prior to the screening evaluation.
  • The subject has known positive human immunodeficiency virus (HIV) status.
  • The subject has known past or current hepatitis.
  • The subject has received a live virus vaccine within 1 month prior to the baseline visit.
  • The subject has a positive PPD test.
  • The subject is sexually active and not practicing effective birth control.
  • The subject is pregnant or breast feeding a child.
  • Any concurrent medical condition which would, in the investigator's opinion, compromise the subject's ability to tolerate the study drug or would make the subject unable to cooperate with the protocol.
  • History of/or current psychiatric illness that would interfere with ability to comply with protocol requirements or give informed consent.
  • Subject has a history of alcohol or drug abuse within the past 6 months that would interfere with ability to comply with protocol requirements.
  • Inability to comply with the study requirements for any reason.

Trial locations and contacts

5 sites in 1 country.

5 sites in 1 country

United States5 sites
  • NIH

    Bethesda, Maryland, 20892

  • Cleveland Clinic

    Cleveland, Ohio, 44195

  • Children's Hospital Los Angeles/Cedars-Sinai Medical Center

    Los Angeles, California, 90027

  • Children's Hospital of Central California

    Madera, California, 93638

  • NYU Hospital for Joint Diseases

    New York, New York, 10003

Other trials in United States

About this study

Official title: Phase 2 Study of IL-1 Trap (Rilonacept) for Treatment of Familial Mediterranean Fever (FMF)

Familial Mediterranean fever (FMF) is a genetic disease resulting in recurrent attacks of fever, abdominal pain, chest pain, arthritis and rash. There are 5-15% of patients who continue to have FMF attacks despite treatment with colchicine or who cannot tolerate colchicine. Currently there are no alternatives to colchicine. Pyrin, the protein that has a defect in FMF has an important role in the regulation of a molecule called interleukin (IL)-1 beta production and activity. This molecule is very important in the process of inflammation in FMF.

Therefore we propose to use IL-1 Trap (Rilonacept), a medication that binds and neutralizes IL-1.

Read more

We will enroll in this study 17 subjects from the age of 4 years, including adults with active FMF despite colchicine therapy. Subjects will receive in random order two 3-month courses of Rilonacept at 2.2 mg/kg (maximum 160 mg) by weekly subcutaneous injection and two 3-month courses of placebo injection. If patients have at least two FMF attacks during a treatment course they will be able to get if they choose the other treatment until the end of that treatment course. Our hypothesis is that Rilonacept will decrease the number of acute FMF attacks and will be safe to use. This study may confirm the importance of IL-1 in the cause of FMF.

Funding source - FDA Office of Orphan Products Development

Read the detailed description

Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory genetic disorder resulting in recurrent attacks of fever, serositis, arthritis and rash. Late complications of untreated FMF include the development of renal amyloidosis. FMF is a rare orphan disease in the United States. Treatment with colchicine is effective in reducing the frequency of episodes in most patients and the development of amyloidosis in nearly all patients. However, there are still 5-15% of patients who continue to have acute FMF attacks despite colchicine therapy or are intolerant of colchicine, usually from gastrointestinal adverse effects. Currently there are no effective alternatives to colchicine. Pyrin, the mutated protein in FMF has an important role in the regulation of IL-1 beta production and activity. Mutations in pyrin result in increased IL-1 beta levels in mice and humans. IL-1 beta is an important pro-inflammatory cytokine. Thus, we hypothesize that inhibition of IL-1 will decrease acute attacks in patients with FMF. We propose to use IL-1 Trap (Rilonacept), a fusion protein consisting of human IL-1 cytokine receptor extracellular domains and the FC portion of human IgG1 that binds and neutralizes IL-1.

We will enroll 17 subjects from the age of 4 years, including adults, from multiple centers in the United States with active FMF (at least 1 attack per month) despite receiving at least 1.2-1.5 mg/d of colchicine (dose dependent on age) or are intolerant of colchicine. Subjects will be diagnosed by clinical criteria with at least one heterozygote mutation of the MEFV (pyrin) gene. After screening subjects will be monitored for a month to observe for acute FMF attacks or if they did not develop an attack in that month until they develop two attacks. We will then use a single-subject alternating treatments design with subjects receiving in random order two 3-month courses of Rilonacept at 2.2 mg/kg (max 160 mg) by weekly SC injection and two 3-month courses of comparable volume placebo. Subjects will continue the usual colchicine dose they were on when they started the study. Subjects with 2 acute FMF attacks during a treatment course will be able to crossover to the other treatment arm until the end of that treatment course. There will be 10 study visits: 1. Screening. 2. Treatment baseline after one month or after subjects have developed FMF attacks as described above. After 1 month of each treatment course and at the end of each treatment course (overall 8 visits). At each visit subjects will return completed diary forms, used and unused drug, queried on adverse effects, undergo a physical examination and laboratory tests obtained for inflammation, safety and in some visits for translational studies. Subjects will also fill out quality of life questionnaires and give an overall estimation of the disease activity. Results will be analyzed by traditional frequency statistics (using an intent to treat analysis) and by Bayesian hierarchical modeling. Our primary aim is to assess the efficacy of Rilonacept in decreasing the number of acute FMF attacks while monitoring drug safety.

The significance of the study includes short and long-term benefits. Fewer FMF attacks will result in less functional impairment and a higher quality of life in colchicine resistant or intolerant patients. Once weekly injections have the potential to improve treatment compliance. Fewer acute attacks of arthritis may prevent the development of chronic joint damage. In the long-term, better FMF control may prevent amyloidosis. This study may confirm the importance of IL-1 in the pathogenesis of FMF and provide support for an FDA filing for use of Rilonacept in FMF. The study design may serve as a template for trials of new biologic drugs for rare diseases.

Being tested
Rilonacept, Placebo
Keywords
FMF, IL1 Trap (Rilonacept), Periodic fever syndromes, Autoinflammatory syndromes, Familial Mediterranean Fever, colchicine
Medical subject headings
Familial Mediterranean Fever

What participants receive

What participants receive. In a randomised study each person is assigned to one group by chance.

  • RilonaceptDrug

    Also called Arcalyst, IL-1 Trap

    2.2 mg/kg/wk by subcutaneous injection, for 3 months

  • PlaceboDrug

    placebo by subcutaneous injection weekly for 3 months

Study groups in NCT00582907
Study groupRoleWhat they receive
1ExperimentalTreatment Arm A: Rilonacept (IL-1 Trap) at a dose of 2.2 mg/kg/wk (max 160 mg)given by subcutaneous injection for 3 months plus colchicine at a stable dose for those subjects already taking colchicine, or without colchicine for those intolerant or non-compliant with colchicine. Since the colchicine dose is stable throughout the study for each subject, at the prestudy dose, colchicine was not considered an intervention
Receives: Drug: Rilonacept
2Placebo comparatorTreatment Arm B: Placebo given by subcutaneous injection weekly with or without colchicine for 3 months. Since the colchicine dose is stable throughout the study for each subject, at the prestudy dose, colchicine was not considered an intervention.
Receives: Drug: Placebo

What is being measured

The primary outcome is the main question the study is designed to answer.

  1. To Assess the Efficacy of Rilonacept in Decreasing the Number of Acute FMF Attacks.

    Difference in number of attacks per treatment month between rilonacept and placebo

    Measured: attacks were assessed at the end of each 3 month treatment course (overall up to 6 month of rilonacept and 6 months of placebo, each)

  2. To Determine if There is a Medically Important Difference Between the Safety Profiles of Rilonacept vs. Placebo.

    Differences in adverse events (AEs) between rilonacept and placebo per patient-month of treatment. We separately analyzed injection site reactions and infectious adverse events. Other adverse events were too small in number to analyze. The upper table (and first statistical analysis) regards injection site reactions and lower table (and second statistical analysis) regards infections.

    Measured: 12 months of entire study length

Secondary outcomes (12)
  1. To Determine the Difference in the Length of Attacks During Treatment With Rilonacept vs. Placebo.

    This outcome was the difference in days in the length of attacks between rilonacept and placebo.

    Measured: 12 months

  2. Percentage of Treatment Courses Without FMF Attacks in Rilonacept Courses as Compared to Placebo Courses.

    The percentage of rilonacept and placebo treatment courses without FMF attacks.

    Measured: Each treatment course of up to 3 months

  3. To Determine the Proportion of Courses in Which Subjects Attained at Least a 50% Decrease in Acute FMF Attacks During Rilonacept Courses as Compared to Placebo Courses.

    Differences between rilonacept and placebo in the percentage of courses that attained at least a 50% decrease in FMF attacks when compared to attacks in the screening period.

    Measured: Up to 3 months for each treatment course

  4. To Determine Differences in the Time to the Development of Attacks Between the Treatment Arms (Rilonacept vs. Placebo).

    In a survival analysis we measured the difference (in days) until the development of the first and second attack within a treatment course of up to 3 months and examined differences in this parameter between rilonacept and placebo. Data regarding the development of the second attack are reported below. In regards to the first attack there were no significant differences between rilonacept and placebo (20 days (7.5,>90)for rilonacept; 15 (8,32) for placebo, P=0.066).

    Measured: 3 months

  5. To Determine the Differences in the Erythrocyte Sedimentation Rate Between the Treatment Arms (Rilonacept vs. Placebo).

    Erythrocyte sedimentation rate - ESR (mm/h)

    Measured: 3 months (each treatment course, overall 12 months)

  6. To Determine the Differences in C-Reactive Protein Between the Treatment Arms (Rilonacept vs. Placebo)

    Differences between the treatment courses in the C-Reactive Protein levels mg/L

    Measured: 3 months (each treatment course, overall 12 months)

  7. To Determine the Differences in the Platelet Count Between the Treatment Arms (Rilonacept vs. Placebo)

    The difference between the treatment arms in the platelet count X 10 to the power of 9

    Measured: 3 months (each treatment course, overall 12 months)

  8. To Determine the Differences in the Fibrinogen Levels Between the Treatment Arms (Rilonacept vs. Placebo)

    The differences between treatment arms in the fibrinogen level (micromol/L)

    Measured: 3 months (each treatment course, overall 12 months)

  9. To Determine the Differences in Serum Amyloid A Levels Between the Treatment Arms (Rilonacept vs. Placebo)

    The difference between the treatment arms in serum amyloid A levels (mg/L)

    Measured: 3 months (each treatment course, overall 12 months)

  10. To Determine the Differences in the Quality of Life Between the Treatment Arms (Rilonacept vs. Placebo).

    Differences in the health-related quality of life (HRQOL) during treatment with rilonacept vs. placebo. HRQOl was measured by the Childhood Health Questionnaire which was adopted also for adults. There are 2 summary scores: 1. Physical summary score. 2. Psychosocial summary score. The data reported below in the upper table is the physical summary composite score and in the lower table the psychosocial summary composite score. Scores were from 0-100 (higher is better) with a score of 50 representing the mean of the normal population.

    Measured: 12 months

  11. To Determine the Differences in the FMF Severity Score of the Subjects Between the Treatment Arms (Rilonacept vs. Placebo).

    Differences in the Armenian Evaluation Score between rilonacept and placebo courses. The Armenian Evaluation Score is a composite score of disease severity based on the frequency, duration and character of attacks (degree of fever and severity of serositis). It was adapted to calculate a score for a 3-month treatment course. The lowest (best) score is 0 and higher values are worse. In theory there is no upper limit to the scale. The total score is reported (there are no subscales).

    Measured: overall 12 months

  12. To Determine the Differences in the Proportion of Time Subjects Received Rilonacept vs Placebo

    The proportion of time within the trial that participants received rilonacept as opposed to placebo. The reason for this outcome is that participants who had at least 2 attacks within an individual treatment course were able to "escape" in a blinded manner to the other treatment arm until the end of that treatment course and then resume the original randomization sequence. Thus participants may have been treated for a longer time with one treatment arm or the other.

    Measured: 12 months

Timeline

From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.

  1. First posted28 Dec 2007 (estimated)
  2. Study startAug 2008
  3. Primary completionSep 2011
  4. Study completionSep 2011

Resources

More details

For doctors and researchers: how the study is designed, who runs it, and its registry IDs.

Study design
Study type
Interventional
Phase
Phase 2
Primary purpose
Treatment
Allocation
Randomised
Intervention model
Crossover
Masking
Triple blind
Who is masked
Participant, Care provider, Investigator
Enrolment
14 participants (actual)
IDs, dates and publications
ClinicalTrials.gov ID
NCT00582907
Sponsor's protocol ID
1R01FD003435-01
Other IDs
FDA 1RO1FD003435-01
Status last verified
Feb 2013
First submitted
19 Dec 2007
Last updated
11 Feb 2013
Data monitoring committee
Yes

Publications

  • Hashkes PJ, Spalding SJ, Giannini EH, Huang B, Johnson A, Park G, Barron KS, Weisman MH, Pashinian N, Reiff AO, Samuels J, Wright DA, Kastner DL, Lovell DJ. Rilonacept for colchicine-resistant or -intolerant familial Mediterranean fever: a randomized trial. Ann Intern Med. 2012 Oct 16;157(8):533-41. doi: 10.7326/0003-4819-157-8-201210160-00003.

    PubMed 23070486
  • Hashkes PJ, Spalding SJ, Hajj-Ali R, Giannini EH, Johnson A, Barron KS, Weisman MH, Pashinian N, Reiff AO, Samuels J, Wright D, Lovell DJ, Huang B. The effect of rilonacept versus placebo on health-related quality of life in patients with poorly controlled familial Mediterranean fever. Biomed Res Int. 2014;2014:854842. doi: 10.1155/2014/854842. Epub 2014 May 15.

    PubMed 25147819
  • Huang B, Giannini EH, Lovell DJ, Ding L, Liu Y, Hashkes PJ. Enhancing crossover trial design for rare diseases: limiting ineffective exposure and increasing study power by enabling patient choice to escape early. Contemp Clin Trials. 2014 Jul;38(2):204-12. doi: 10.1016/j.cct.2014.05.001. Epub 2014 May 12.

    PubMed 24833067

Questions about this trial

What is this clinical trial studying?

A Phase 2 study testing Rilonacept in people with Familial Mediterranean Fever, run by The Cleveland Clinic at 5 sites in United States.

The full registered description is in the About this study section and the official record.

Who may be eligible to take part?

The sponsor lists 11 inclusion and 19 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.

Is this clinical trial currently recruiting?

The record says: finished, as of the sponsor's last update on 11 Feb 2013. Status can change between updates, so confirm with the study contact before making plans.

Where is the study taking place?

The record lists 5 sites in United States. Whether a site is taking part right now should be confirmed with that site. See every site.

What are the possible benefits and risks?

They depend on the study and the treatment, and no study can promise a benefit. Some participants in this study may receive a placebo. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.

What does this phase mean?

Phase 2 studies give the treatment to a larger group to see whether it has the intended effect and to keep checking safety.

Not every study follows the same path, and some studies (observational studies, device studies) have no phase.

Are results available for this study?

Yes. Results are posted in the registry (first posted 31 Oct 2012). A summary is in the For doctors view, and the full results are on ClinicalTrials.gov.

How can a doctor refer a patient or contact the research team?

The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.

What does the primary outcome measure?

To Assess the Efficacy of Rilonacept in Decreasing the Number of Acute FMF Attacks. (time frame: attacks were assessed at the end of each 3 month treatment course (overall up to 6 month of rilonacept and 6 months of placebo, each)). The study lists 2 primary outcomes in all. It is what the study was designed to measure, not a finding.

Where can I read the original study information?

On the ClinicalTrials.gov record for NCT00582907, which also lists 3 linked publications.

Does a listing mean the treatment is approved or proven to work?

No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.

How often is this information updated?

AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 11 Feb 2013.

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