• Changes in Auto-Inflammatory Diseases Activity Index score at the 1st and 3rd month over baseline [ Time Frame: The previous 1 month before treatment and the 1st and 3rd month after treatment ]
  Patients or their parents completed a 1-month (31 days) prospective diary with 12 yes/no items( Fever ≥38°C, Overall symptoms, Abdominal pain, Nausea/vomiting, Diarrhoea, Headaches, Chest pain, Painful nodes, Arthralgia or myalgia, Swelling of the joints, Eye manifestations, Skin rash) at the previous 1 month before treatment, and the 1st and 3rd month after treatment . Each item of this diary was dichotomised as no (0)=absence of symptom or yes (1)=presence of symptom. The calculation of the Auto-Inflammatory Diseases Activity Index score is straightforward, consisting of the sum of all 12 items (0-372 in a month of 31 days). Higher values represent higher disease activity.
• Changes in inflammatory markers, including C-reactin protein, erythrocyte sedimentation rate, serum amyloid protein, interleukin-1β and interleukin-18， at 1, 3 and 6 months over baseline [ Time Frame: Baseline and at 1, 3 and 6 months after treatment ]
  C-reactin protein, erythrocyte sedimentation rate, serum amyloid protein, interleukin-1β and interleukin-18 are measured at baseline，1, 3 and 6 months after treatment.
• Changes in physician global assessment of disease activity on a 0-10 visual analog scale (VAS) at 1, 3 and 6 months over baseline [ Time Frame: Baseline and 1, 3 and 6 months after treatment ]
  Visual analogue scale (VAS) for overall disease activity were completed by the physician at each visit (Baseline and 1, 3 and 6 months after treatment).
• Changes in parent/patient global assessment of well-being on a 0-10 visual analogue score (VAS) at 1, 3 and 6 months over baseline [ Time Frame: Baseline and 1, 3 and 6 months after treatment ]
  Visual analogue scale (VAS) for overall disease activity were completed by the parent/patient at each visit (Baseline and 1, 3 and 6 months after treatment).
• Changes in CSF white blood cell count for CINCA patients [ Time Frame: Baseline and 6 months after treatment. ]
  For CINCA patients, Lumbar punctures (LPs) were performed at baseline and 6 months after treatment.
• Changes in MRI of the brain and inner ear for CINCA patients [ Time Frame: Baseline and 6 months after treatment. ]
  For CINCA patients, MRIs with gadoliniumenhanced fluid-attenuated inversion recovery (FLAIR) sequences of the brain and inner ear were performed and scored at baseline and 6 months after treatment.
• Changes in audiology data for CINCA patients [ Time Frame: Baseline and 6 months after treatment. ]
  For CINCA patients, Hearing assessment included audiological evaluations. Outcomes in each ear were categorised as 'stable' or 'worsened', according to a modification of the American Speech and Hearing Association (ASHA) criteria, comparing the results at 6 months after treatment over baseline.
• Number of participants with adverse effect [ Time Frame: Up to 6 months ]
  Treatment-related adverse effect, including abnormal liver function, hematuria and decreased white blood cells

Inclusion Criteria:

• All patients must meet the following diagnostic criteria of CAPS and have pathogenic mutation(s) in NLRP3 gene.

  1. Raised inflammatory markers (CRP/SAA) (mandatory criteria)

  2. ≥2 of 6 CAPS typical signs/symptoms:

     1. Urticaria-like rash;
     2. Cold/stress triggered episodes;
     3. Sensorineural hearing loss;
     4. Musculoskeletal symptoms (arthralgia/arthritis/myalgia);
     5. Chronic aseptic meningitis;
     6. Skeletal abnormalities (epiphyseal overgrowth/frontal bossing).

Exclusion Criteria:

• Patients will not be included if meets any of the following criteria:

  1. Being treated with IL-1 inhibitor, other biological agents and immunosuppressants
  2. Pregnant and lactating women
  3. Serious organ function failure, expected life time less than 6 months