TableĀ 1

Overarching principles for CAPS, TRAPS and MKD

Overarching principlesLSAgree (%)
Management of patients with AID should ideally be guided by a multidisciplinary team in a tertiary centre with expertise in AID, with access to genetic counselling4D100
The care of patients with AID should include shared patient-centred and family-centred decision-making with the multidisciplinary team4D100
Aims of the treatment of AID include:
  • Early and rapid control of disease activity

  • Prevention of disease and treatment-related damage

  • Enabling participation in daily activities

  • Improvement of health-related quality of life

In patients with AID, psychosocial support is recommended as appropriate4D100
  • L, level of evidence; 4, expert opinion; S, strength of recommendation; D, based on level 4 evidence.9 Agree, percentage of experts who agreed on the recommendation during the final voting round of the consensus meeting.

  • AID, autoinflammatory diseases; CAPS, cryopyrin-associated periodic syndromes; MKD, mevalonate kinase deficiency; TRAPS, tumour necrosis factor receptor-associated periodic syndrome.