Table.
Characteristics of the Patients.
| Patient 1 | Patient 2 | |
|---|---|---|
| Age at onset (years) | 17 | 23 |
| Age at diagnosis of CRMO | 48 | 26 |
| Periodic fever | - | - |
| Family history | - | - |
| Polyarthritis | +, Bilateral shoulders, right elbow and right wrist | +, MP and PIP joints, bilateral elbows and left knee |
| Joint destruction in X-rays | Poor | Poor |
| Higher intensity of muscles on MRI | + | + |
| Osteomyelitis | + | + |
| Sternoclavicular joint abnormality | - | - |
| Skin lesion | - | Subcutaneous nodule, (biopsy specimen showed necrotizing vasculitis of subfascial artery) |
| RF/ACPA/ANA/ANCA | -/-/-/- | -/-/-/- |
| MMP-3 | WNR | WNR |
| WBC count | 8,500/μL 10,700/μL (PSL 13 mg/day) | 6,730/μL 8,430/μL (PSL 13 mg/day) |
| CRP ≥1.0 mg/dL | + | + |
| PSL | NE (max dose; 10 mg) | NE (max dose; 20 mg) |
| Colchicine | NE (max dose; 0.5 mg) | NE (max dose; 1 mg) |
| MTX | NE (max dose; 8 mg/week) | NE (max dose; 12 mg/week) |
| NSAIDs | NE | NE |
| Bisphosphonate | NE (Minodronate) | - |
| TCZ | Effective | Effective |
| Clinical score system for CRMO1) | 55 (≥39 out of a maximum of 63 is considered CRMO) | 55(≥39 out of a maximum of 63 is considered CRMO) |
CRMO: chronic recurrent multiple osteomyelitis, MP joints: metacarpophalangeal joints, PIP joint, proximal interphalangeal joint, MRI: magnetic resonance imaging, CK: creatinine kinase, RF: rheumatoid factor, ACPA: anti-cyclic citrullinated peptide antibody, ANA: anti-nuclear antibody, ANCA: myeloperoxidase and proteinase 3 antineutrophil cytoplasmic antibody, MMP-3: matrix metalloproteinase-3, WBC: white blood cell, CRP: C-reactive protein, WNR: within the normal range, PSL: prednisolone, NE: not effective, MTX: methotrexate, NSAIDs: non-steroidal anti-inflammatory drugs, TCZ: tocilizmab