Table 1.

Demographic characteristics at onset of treatment and outcome og 9 patients with JDM treated with rituximab (RTX).

PatientCourse/SexAge at onset of JDM/RTX, yrsSymptomsAbPrevious Treatment (mo since last treatment)RTX RegimenClinical Outcome/ RelapseDuration of Followup Since First RTX infusion, yrs
11 F8/8.1Myositis*, rash* (d)PRED* (1.5 mg/kg/day), MP*, PE*, IVIG (0.5)4 × 375 mg/m2CCR/No3
22a F10/12Myositis*, rash*, calcinosis*, GI disease* (d)PRED*, MP*, CSA*, MTX (18)2 × 500 mg/m2NE
2b F10/13.3Myositis*, rash*, calcinosis*, GI disease* (d)PRED* (0.2 mg/kg/day), MMF*, CYC (14), CSA (13), MTX (34)2 × 375 mg/m2CCR/No2.5
33 F4/7.1Myositis*, rash*, calcinosis* (p)ANAPRED* (1.5 mg/kg/day), MTX*4 × 375 mg/m2CCR/No3.5
44 F7.6/16Myositis*, rash*, calcinosis*, GI disease* (d)ANAPRED** (0.5 mg/kg/day), PE*, MP, MMF (37), AZA (7), CSA (1), MTX (6)2 × 500 mg/m2NR2.2
55 F1.5/6.2Myositis*, rash*, calcinosis* (d)PRED*, MTX (20), CSA (40)2 × 500 mg/m2NR2 (death)
66 F8/9.6Myositis*, rash* (p)ANAPRED* (0.5 mg/kg/day), MMF*NENE
77 F9.4/10.3Myositis*, rash (d)Anti-KuPRED* (1 mg/kg/day), MTX*, PE (1), CSA (3), MP (12)4 × 375 mg/m2NR0.3
88 F7.5/11.5Myositis, rash, calcinosis* (d)ANAPRED* (0.5 mg/kg/day), MTX (14), CSA*4 × 375 mg/m2NR3.5
99 M2.5/9Myositis, rash, GI disease* (abnormal lipomatosis), calcinosis* (d)ANAPRED* (0.2 mg/kg/day), MTX (17), CSA (26), IVIG (14)4 × 375 mg/m2NR4
  • * Present at the time of starting rituximab; treatment was under way at time of starting rituximab, at a stable dosage for at least 3 months. PRED: prednisone; MP: methylprednisolone; PE: plasma exchange; IVIG: intravenous immunoglobulins; CSA: cyclosporine; MTX: methotrexate; MMF: mycophenolate mofetil; AZA: azathioprine; ANA: antinuclear antibodies; CCR: complete clinical response; NR: no response; NE: not evaluable; GI: gastrointestinal; (p): probable JDM according to Bohan and Peter criteria1; (d): definite JDM according to Bohan and Peter criteria; Ab: autoantibodies N: normal values.