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Table 1

From: Long-term efficacy of IL-1 blockers in PAPA patients

Pt Sex Mutation Main manifestations Manifestations in the 24 months before treatment N± of flares during follow-up Treatment (dose, duration)
1 F E256G Pyogenic arthritis
Pyoderma gangrenosum
Cystic acne/foruncolosis
2 flares of pyogenic arthritis
5 pyoderma gangrenosum
Cystic acne
0 Anakinra
100 mg/day
(36 months)
2 F E250Q Pyogenic arthritis
Sterile osteomyelitis
Palpebral edema
3 flares of pyogenic arthritis
1 sterile osteomyelitis
1 palpebral edema
0 Anakinra
2 mg/kg/day
(21 months)
3 M E250Q Pyogenic arthritis 7 flares of pyogenic arthritis (polyarticular) 3 (mild) articular flares Anakinra
1.5 mg/kg/day
(38 months) and low dose steroid
4 M WT Cutaneous abscesses
Pyoderma gangrenosum
Pyogenic muscular abscess
1 persistent muscular abscess 0 Anakinra
100 mg/day
(26 months)
5 M E250K Pyogenic arthritis
Pyoderma gangrenosum
Severe anemia
Splenomegaly
Growth delay
1 cutaneous abscess
3 pyoderma gangrenosum
Anemia
1 pyoderma gangrenosum (resolved after Canakinumab) Anakinra
2 mg/kg/day
(31 months)
-
Canakinumab
(4 months)
6 F E250Q Dactylitis/tendinitis
Pyogenic arthritis
Acne and furunculosis
6 articular flares 0 Anakinra
100 mg/day
(4 months)