Table 1 Patients' demographics, clinical and laboratory characteristics and response to anakinra treatment.
| Demographics/symptoms/signs/laboratory findings | Patient 1 | Patient 2 | Patient 3 | Patient 4 |
|---|---|---|---|---|
| Sex | F | M | M | M |
| Age (years) | 36 | 18 | 54 | 17 |
| Fever >39°C | Yes | Yes | Yes | Yes |
| Arthritis/arthralgias | Arthralgias | Arthralgias | Symmetric polyarthritis | Arthralgias |
| Sore throat | Yes | Yes | No | Yes |
| Rash | Maculopapular | No | No | No |
| Lymphadenopathy/splenomegaly/hepatomegaly | Lymphadenopathy/hepatomegaly | Lymphadenopathy | Lymphadenopathy | Splenomegaly |
| Serositis | Pleural effusion | Polyserositis | Pleural effusion | No |
| Serum ferritin (ng/ml) | >5000 | Not applicable | 3743 | 2456 |
| Neutrophils/mm3 (%) | 13200 (87) | 35 000 (83) | 17890 (87.5) | 31200 (93) |
| ESR (mm/h) | 77 | 85 | 81 | 115 |
| CRP (mg/dl) (normal <0.8) | 9.8 | 22 | 22 | 29 |
| Abnormal liver function tests | AST: 226 U/l | AST: 113 U/l | AST: 82 U/l | AST: 68 U/l |
| ALT: 365 U/l | ALT: 350 U/l | ALT: 87 U/l | ALT: 118 U/l | |
| γ‐GT:71 | ||||
| Diagnostic examination for infections, malignancy and other rheumatic diseases* | Negative | Negative | Negative | Negative |
| Resolution of symptoms on initiation of anakinra | Within a few hours | Within a few hours | Within a few hours | Within a few hours |
| Response of serum markers of inflammation on initiation of anakinra | Within 3 weeks | Within 4 weeks | Within 3 weeks | Within 2 weeks |
ALT, alanine aminotransferase; AST, aspartate aminotransferase; CRP, C reactive protein; ESR, erythrocyte sedimentation rate; γ‐GT, γ‐glutamyltransferase.
*Including: x rays, CT scans, PPD tests, microbial cultures (blood, urine, cerebrospinal and pleural fluid), serum cancer markers, tests for anti‐nuclear antibodies, extractable nuclear antigens, NA, anti‐DNA, anti‐neutrophil cytoplasmic antibodies, rheumatoid factor, viral serology (HIV, hepatitis A virus, hepatitis B virus, hepatitis C virus, cytomegalovirus, Epstein–Barr virus, herpes simplex virus), specific antibodies (for toxoplasma, leptospira, leishmania, rickettsias and borrelias), antistreptolysin titre, Widal and Wright tests. For patient 1 thick blood smear examination for plasmodia and liver, skin, muscle and bone marrow biopsies were also performed.