Table 1 (abstract P1024). See text for description
From: 10th Congress of International Society of Systemic Auto-Inflammatory Diseases (ISSAID)
Pts | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 910 |
|---|---|---|---|---|---|---|---|---|---|
Systemic symptoms | Fever |  | Fever |  |  | Fever | Fever | Fever | FeverFever, Hemophagocytic lymphohistiocytosis |
CNS Cerebral calcification | Â | Epilepsy | Epilepsy, mental retardation | Â | / | Â | Â | Hemiplegia | |
Gastrointestinal | Epatosplenomegaly, aspecific IBD | Â | Â | IBD simil-RCU | Abdominal pain, diarrhoea | Â | Abdominal pain | / | Hypertransaminasemia |
Skin/osteoarticular | Arthro-myalgia, , panniculitis | Arthro-myalgia, arthritis, urticarial rash | Arthro-myalgia, vasculitis | Eczema | Arthro-myalgia | erythema polymorphe, HSP | Arthro-myalgia | / | 9783 28 16 |
C3 (90-180 mg/dl) | 144 | 127 | 81 | 133 | 53 | 137 | 96 | 203 | |
 C4 (10-40 mg/dl) | 34 | 24 | 6 | 23 | 20 | 54 | 17 | 38 | |
Immunodeficit autoimmunity | Â | ANA 1:2.560 Anti ds-DNA 1:640 | ANA 1:10.240 Anti dsDNA 1:1280 | Immunodeficit ANA neg | Neg | Â | Neg | Neg | Immuno Deficit / |
WBC/ mmc3 | 5,2 | 4,4 | 9,8 | 3,9 | 4,6 | 8,1 | 6,4 | 4,4 | 3,51,7 |
Hb g/dl | 10,4 | 12 | 12,9 | 10,2 | 13 | 11.1 | 14,1 | 12,1 | 16,5Â 9,7 |
PLT/mmc3 | 199 | 381 | 194 | 86 | 110 | 277 | 214 | 358 | 150Â 56 |
IS | 14,2 | 171,5 | 54,73 | 60,09 | 27,8 | 17,2 | 53,9 | 40,6 | 32,618,19 |
Genetic analysis | Neg | PSMB9:p.Arg60Cys OTULIN:p.Gln115His | CARD8:p.Leu426Phe | IFIH1: p.Arg374His | Â | Â | Neg | Â | ACP5:DNASE2: p.Arg269p.Ala45Gly Tr |