Table 1.

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

Patient	Course/Sex	Age at onset of JDM/RTX, yrs	Symptoms	Ab	Previous Treatment (mo since last treatment)	RTX Regimen	Clinical Outcome/ Relapse	Duration of Followup Since First RTX infusion, yrs
1	1 F	8/8.1	Myositis^, rash^ (d)	—	PRED^* (1.5 mg/kg/day), MP^, PE^, IVIG (0.5)	4 × 375 mg/m²	CCR/No	3
2	2a F	10/12	Myositis^, rash^, calcinosis^, GI disease^ (d)	—	PRED^, MP^, CSA^*, MTX (18)	2 × 500 mg/m²	NE
	2b F	10/13.3	Myositis^, rash^, calcinosis^, GI disease^ (d)	—	PRED^* (0.2 mg/kg/day), MMF^*, CYC (14), CSA (13), MTX (34)	2 × 375 mg/m²	CCR/No	2.5
3	3 F	4/7.1	Myositis^, rash^, calcinosis^* (p)	ANA	PRED^* (1.5 mg/kg/day), MTX^*	4 × 375 mg/m²	CCR/No	3.5
4	4 F	7.6/16	Myositis^, rash^, calcinosis^, GI disease^ (d)	ANA	PRED^^ (0.5 mg/kg/day), PE^*, MP, MMF (37), AZA (7), CSA (1), MTX (6)	2 × 500 mg/m²	NR	2.2
5	5 F	1.5/6.2	Myositis^, rash^, calcinosis^* (d)	—	PRED^*, MTX (20), CSA (40)	2 × 500 mg/m²	NR	2 (death)
6	6 F	8/9.6	Myositis^, rash^ (p)	ANA	PRED^* (0.5 mg/kg/day), MMF^*	NE	NE
7	7 F	9.4/10.3	Myositis^*, rash (d)	Anti-Ku	PRED^* (1 mg/kg/day), MTX^*, PE (1), CSA (3), MP (12)	4 × 375 mg/m²	NR	0.3
8	8 F	7.5/11.5	Myositis, rash, calcinosis^* (d)	ANA	PRED^* (0.5 mg/kg/day), MTX (14), CSA^*	4 × 375 mg/m²	NR	3.5
9	9 M	2.5/9	Myositis, rash, GI disease^* (abnormal lipomatosis), calcinosis^* (d)	ANA	PRED^* (0.2 mg/kg/day), MTX (17), CSA (26), IVIG (14)	4 × 375 mg/m²	NR	4

↵* 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.