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Fig. 3 | Pediatric Rheumatology

Fig. 3

From: Consensus protocols for the diagnosis and management of the hereditary autoinflammatory syndromes CAPS, TRAPS and MKD/HIDS: a German PRO-KIND initiative

Fig. 3

a Treat-to-target PRO-KIND Consensus Treatment Plan (CTP) for patients with Cryopyrin-Associated Periodic Syndrome (CAPS)*1 Most patients with CAPS have a continuous disease course, on-demand therapy is only recommended in patients with low disease activity. b Treat-to-target PRO-KIND Consensus Treatment Plan (CTP) for patients with TNF Receptor-Associated Periodic Syndrome (TRAPS). c Treat-to-target PRO-KIND Consensus Treatment Plan (CTP) for patients with Mevalonate Kinase Deficiency Syndrome (MKD). Treat-to-target CTPs for patients with CAPS (a), TRAPS (b) and MKD/HIDS (c) define the treatment targets. After establishing the diagnosis, patients receive either on-demand (only recommended for low disease activity or seasonal disease) or continuous treatment. After 3 months, patients are evaluated for achieving the target. Treatment decisions include continuation, dose adjustments, medication changes, or strategy change. This iterative process aims to ultimately achieve the treatment target of complete remission. Monitoring recommendations and intervals are depicted. AIDAI: AutoInflammatory Diseases Activity Index [26]; ADDI: Autoinflammatory Disease Damage Index for assessment of the extent of organ damage [29]; PGA and PPGA: Physician and patient global assessment using a visual analogue scale. * Definition of remission according to AIDAI [26]

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