Recommendations for the treatment of CAPS, TRAPS and MKD
| L | S | Agree (%) | |
|---|---|---|---|
| Treatment CAPS | |||
| IL-1 inhibition is indicated for the whole spectrum of CAPS, at any age | 1B–2A* | A–B | 94.4 |
| To prevent organ damage, long-term IL-1 inhibition should be started as early as possible in patients with active disease | 2B | B | 100 |
| There is no evidence for the efficacy of DMARDs or biological therapy other than IL-1 blockade in CAPS | 4 | D | 94.4 |
| For symptomatic adjunctive therapy, short courses of NSAIDs and corticosteroids may be used,# but they should not be used for primary maintenance therapy## | #3 ##4 | #C ##D | 100 |
| In patients with CAPS, adjunctive therapy (eg, physiotherapy, orthotic devices, hearing aids) is recommended as appropriate | 4 | D | 100 |
| Treatment TRAPS | |||
| NSAIDs may provide symptom relief during inflammatory attacks | 3 | D | 100 |
| Short-term glucocorticoids, with or without NSAIDs, are effective for terminating inflammatory attacks | 3 | C | 100 |
| The beneficial effect of corticosteroids can decline over time so that increasing doses are required to achieve an equivalent response | 3 | C | 100 |
| IL-1 blockade is beneficial in the majority of patients with TRAPS. | 2B | B | 100 |
| Etanercept can be effective in some patients, but the effect might decline over time | 2B | C | 93.8 |
| With frequent attacks and/or subclinical inflammation between attacks, maintenance therapy with IL-1 blockade or etanercept is recommended and may limit corticosteroid exposure | 2B–3* | C | 100 |
| If one IL-1 blocking agent at adequate dose is ineffective or intolerable, a switch to etanercept or another IL-1 blocking agent should be considered Likewise, if etanercept is ineffective or intolerable, a switch to an IL-1 blocking agent should be considered | 4 | D | 100 |
| Although a beneficial effect is reported in a few cases, the use of anti-TNF monoclonal antibodies is not advised, due to the possible detrimental effect | 3 | C | 100 |
| Treatment MKD | |||
| NSAIDs may provide symptom relief during inflammatory attacks | 3 | C | 100 |
| Short-term glucocorticoids, with or without NSAIDs, may be effective for alleviating inflammatory attacks | 3 | C | 100 |
| Colchicine or statins are not efficacious; therefore we do not recommend their use | 3 | C | 100 |
| Short-term IL-1 blockade may be effective for terminating inflammatory attacks and should be considered to limit or prevent steroid side effects | 2B | C | 100 |
| With frequent attacks and/or subclinical inflammation between attacks, maintenance therapy with IL-1 blockade or etanercept is recommended, and may limit corticosteroid exposure | 2B–3* | C | 93.3 |
| If one IL-1 blocking agent at adequate dose is ineffective or intolerable, a switch to another IL-1 blocking agent or another biological agent (including TNF-α blockade or IL-6 blockade) should be considered. Likewise, if TNF-α blockade is ineffective or intolerable, a switch to another biological agent (including an IL-1 or IL-6 blocking agent) should be considered | 4 | D | 100 |
| In selected cases with severe refractory disease with poor quality of life, referral to a specialist centre for consideration of allogeneic haematopoietic stem cell transplantation is recommended. | 3 | D | 93.3 |
L, level of evidence; 1B, randomised controlled study; 2A, controlled study without randomisation; 2B, quasi-experimental study; 3, descriptive study; 4, expert opinion; S, strength of recommendation; A, based on level 1 evidence; B, based on level 2 or extrapolated from level 1; C, based on level 3 or extrapolated from level 1 or 2; D, based on level 4 or extrapolated from level 3 or 4 evidence.9 Agree, percentage of experts who agreed on the recommendation during the final voting round of the consensus meeting.
*See table 4 for detailed information on evidence and approval of IL-1 blocking and TNF-blocking agents.
CAPS, cryopyrin-associated periodic syndromes; DMARDs, disease-modifying antirheumatic drugs; IL, interleukin; MKD, mevalonate kinase deficiency; NSAIDs, non-steroidal anti-inflammatory drugs; TNF, tumour necrosis factor; TRAPS, tumour necrosis factor receptor-associated periodic syndrome.