Table 1.
Characteristics of the patient.
| Age (years) | 4 | 8 | 12 | 15 | 19 | 20 | 21 | 22 |
| Clinical manifestations | ||||||||
| Sterile pyogenic arthritis | + | – | – | – | – | – | – | – |
| Pyoderma gangrenosum | – | + | + | – | – | – | – | – |
| Cystic acne | – | – | + | – | – | + | – | – |
| Hepato splenomegaly |
+ | + | + | + | + | + | + | + |
| Growth failure | – | – | – | + | + | + | + | + |
| Laboratory tests | ||||||||
| Hemoglobin (g/dL) | 11 | 10 | 11 | 11 | 11 | 11 | 11 | 11 |
| While blood cells (number/mmc) | 3,000 | 3,760 | 3,000 | 3,260 | 7,270 | 10,630 | 4,900 | 4,000 |
| C-reactive protein (mg/dL) | ND | ND | 5.32 | 1.64 | 1.3 | 11.8 | 0.86 | 0.6 |
| Serum amyloid A (mg/L) | ND | ND | 73 | 14.8 | 42.6 | 213 | 3.7 | 4 |
| Creatininemia (mg/dL) | ND | ND | 0.54 | 0.81 | 0.79 | 1.55 | 1.57 | 1.7 |
| Proteinuria (g/24 h) | Absent | Absent | Absent | Absent | 4.49 | 4.28 | 3.18 | 3.2 |
| Treatments | ||||||||
| Intra-articular steroid injections | + | – | – | – | – | – | – | – |
| Topical steroids and antibiotics | – | + | + | – | – | – | – | – |
| Oral prednisone | – | – | – | – | – | + | + | – |
| Canakinumab | – | – | – | + | + | – | + | + |
| Tacrolimus | – | – | – | – | – | + | – | – |
| Allopurinol | – | – | – | – | – | + | + | + |
| ACE inhibitor | – | – | – | – | – | – | + | + |
ACE, angiotensin-converting enzyme; ND, not determined.