A Pilot Study of KPL-914 in Recurrent Pericarditis
Condition: Recurrent Pericarditis
MeSH terms: Pericarditis; Recurrence
Intervention: KPL-914
Intervention description: KPL-914 (rilonacept) will be provided in its commercially available formulation as a lyophilized powder to be reconstituted for SC administration.
Adult participants (≥ 18 years of age) KPL-914 will be administered as an initial loading dose of 320 mg SC, delivered as 2 subcutaneous injections of 160 mg SC each on Day 0, then 160 mg SC dosed once weekly for 5 subsequent weeks.
Pediatric participants (6 to <18 years of age) KPL-914 will be administered with an initial loading dose of 4.4 mg/kg, up to a maximum of 320 mg, delivered as 2 subcutaneous injections of 2.2 mg/kg each with a maximum single-injection volume of 2 mL.
Participants considered to be 'treatment responders' will be offered participation in an optional 18-week extension period (EP) in which KPL-914 can be continued for a total duration of KPL-914 treatment of up to 24 weeks.
Intervention MeSH terms: Rilonacept
Phase: Phase 2
Study type: Interventional
Primary purpose: Treatment
Allocation: Non-Randomized
Intervention model: Parallel Assignment
Masking: None (Open Label)
Enrollment: 26.0
Sex: All
Minimum age: 6 Years
Maximum age: 75 Years
Healthy volunteers: No

Summary:

      The purpose of this study is to assess the preliminary efficacy and safety of KPL-914
      treatment in participants with recurrent pericarditis.
    

Eligibility criteria:

        Inclusion Criteria for All Participants:

          -  Has given consent (or assent, if applicable) and signed an Informed Consent Form (ICF)
             (or informed assent form, if applicable).

          -  Male or female, of any ethnic origin.

          -  6 to 75 years of age, inclusive.

          -  If used, has received non-steroidal anti-inflammatory drugs (NSAIDs), and/or
             colchicine and/or corticosteroids (in any combination) at stable dose levels for at
             least 7 days prior to study drug dosing (although stable doses for a shorter period
             will be acceptable if in the opinion of the Investigator, in consultation with the
             Sponsor, a shorter period of stability is not anticipated to alter the baseline CRP
             values) and is anticipated to continue these concomitant medications at these dose
             levels for the duration of the active Treatment Period.

          -  If female of child-bearing potential, must be nonpregnant and nonlactating and must
             agree to use an effective method of contraception, e.g., hormonal contraception or
             double-barrier birth control.

          -  Is able to adequately maintain a medication diary.

          -  Agrees to refrain from making any new, major life-style changes that may affect
             pericarditis symptoms (e.g., starting a new diet or changing exercise pattern) from
             the time of signature of the ICF (or informed assent form, if applicable) to the
             End-of-Trial Visit.

        Parts 1, 2 and 4:

        Subjects eligible for Parts 1, 2 and 4 have to present during a symptomatic episode of
        recurrent idiopathic pericarditis (RIP; Parts 1 and 2) and post pericardiotomy syndrome
        (PPS; Part 4) and a history of at least one pericarditis recurrence. They can be enrolled
        into Part 1 or 4 if the CRP value at screening is >1 mg/dL, and into Part 2 if a CRP ≤1
        mg/dL (attributed to concomitant medications e.g., corticosteroids), and there is an
        evidence of pericardial inflammation on cardiac MRI confirmed by the imaging core lab.

        Enrollment into Part 3 and 5:

        Subjects eligible for Part 3 of this study have to present with corticosteroid-dependent
        RIP or PPS and history of at least 2 pericarditis recurrences.

        Exclusion Criteria for All Participants:

          -  Has a diagnosis of pericarditis that was secondary to specific excluded etiologies,
             including tuberculous, neoplastic, or purulent etiologies, post-myocardial infarction
             (early or late), thoracic trauma, myocarditis, or systemic diseases including
             autoinflammatory diseases, autoimmune diseases (e.g., rheumatoid arthritis, systemic
             lupus erythematosus, etc.).

          -  Has a history of immunodepression, including a positive human immunodeficiency virus
             test result.

          -  Has received treatment within the 6-month period before dosing with any systemic
             immunosuppressants (other than, for example, corticosteroids or mycophenolate) which,
             in the opinion of the Investigator (in consultation with the Sponsor), may interfere
             with the study endpoints.

          -  Currently receiving other interleukin (IL)-1 or IL-6 blockers, Janus-activating kinase
             (JAK) or tumor necrosis factor (TNF) inhibitors.