Research question and objectives
The objective of this non-interventional study is to monitor and further explore long-term outcome of CAPS patients treated with Ilaris® in terms of disease course as well as quality of life and health economic aspects - under conditions of standard clinical practice in Germany. The usage of Ilaris® and the population receiving Ilaris® for CAPS under conditions of standard clinical practice shall be described.
Endpoints:
 This prospective, non-interventional, multi-center study will evaluate the following endpoints:
 Describe the usage and patterns of dosing of Ilaris® in routine clinical practice.
 Description of the population receiving Ilaris® for CAPS under conditions of standard clinical practice regarding details on CAPS diagnosis and laboratory parameters.
 Physician global assessment (PGA) of autoinflammatory disease activity [5-point scale (absent, minimal, mild, moderate, or severe)] comprising urticarial skin rash, arthralgia,
Novartis Vertraulich
Beobachtungsplan Nicht-interventionelle Studie ACZ885 / Ilaris / CACZ885DDE06
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myalgia, headache, migraine, conjunctivitis, fatigue or malaise, abdominal pain, amongst other symptoms that may appear.
 The long-term impact of Ilaris® on disease progression / improvement consisting of but not limited to AA-Amyloidosis as evidenced by renal function, neurologic and ophthalmologic symptoms and sensorineural deafness, skin disease, serologic markers of inflammation (CRP, ESR, serum amyloid A-protein (SAA), IL-6) in CAPS patients treated with Ilaris.
 Explore growth and development patterns (normal / retarded) of children aged ≥2 to ≤17 years of age exposed to Ilaris®.
 Patient-reported outcome as captured by disease-specific questionnaire (used as in clinical routine; VAS / AIDAI) capturing amongst others appearance of fever/chills, skin rash, joint/muscle, pain, eye discomfort/redness, fatigue, headache.
 Ressource utilization
 Routine safety monitoring. Adverse events will be reported according to pharmacovigilance requirements.
Patients who previously had been enrolled into a Novartis clinical study with Canakinumab (Ilaris) will be asked if they volunteer for compiled data analysis from previous data collection.