Table 2.

Published treatment options for juvenile dermatomyositis.

Medication	Dosing	Route	Mechanism of Action	JDM Publications
Anti-Inflammatory
IVIG	2 g/kg every 4 wks	IV	Uncertain, inhibits macrophages	Touimy et al., 2013 [64]
Infliximab	3 mg/kg at 0, 2, 6 wks; then every 8 wks	IV	Inhibits TNF-α	Riley et al., 2008 [66]
Abatacept	10 mg/kg at 0, 2, 4 wks; then every 4 wks	IV, SQ	Binds CD80/CD86 inhibiting T cell costimulatory signal	Arabshahi et al., 2012 [67]
Depomedrone, intra-lesional	80 mg once	Local injection	Uncertain, facilitates dissolving and resorption of calcified materials	Al-Mayouf et al., 2010 [68]
Thalidomide	50 mg/d (1.3 mg/kg/d) x 4 wks; then increased to 75 mg/d	PO	Inhibits TNF-α and IL-6, anti-angiogenesis	Miyamae et al., 2010 [69]
Rituximab	750 – 1000 mg/m2/wk x 2 wks	IV	Binds CD20 on B cells
Calcium and phosphate modulators
Diltiazem	4–6 mg/kg/d	PO	Calcium channel blocker	Oliveri et al., 1996 [73]
Pamidronate	1 mg/kg/d x 3 d; repeated every 3 mo	IV	Bisphosphonate: reduces bone resorption via reduced osteoclast activity, inhibits macrophages, deregulates expression of genes involved in phosphate homeostasis and mineralization	Slimani et al, 2010 [81]
	2 mg/kg/yr	IV		Marco Puche et al., 2010 [82]
Alendronate	0.4 mg/kg/d	PO	Bisphosphonate	Ambler et al., 2005 [83]
Sodium Thiosulfate	10–25% cream, daily	Topical	Uncertain, dissolves calcium deposits, chelates free calcium	Pagnini et al., 2014 [93]
	10 g, 3/wk x 2 wks; then 15 g, 2/wk x 3 mo	IV		Arabshahi et al., 2012 [67]
Aluminum Hydroxide	1.8–2.4 g/d	PO	Decreases intestinal absorption of phosphate	Aihara et al., 1994 [94] Nakagawa et al., 1993 [95] Wang et al., 1988 [96]
Probenecid	500 mg/d titrated up to 1000–1250 mg/d	PO	Uncertain: increases renal excretion of phosphate, decreases extracelullar ATP levels with resultant decrease in PPi	Nakamura et al., 2006 [97] Harel et al., 2001 [98]
Colchicine	1–1.8 mg/d	PO	Uncertain, inhibits microtubule polymerization, inhibits IL-1β and inflammasome activation	Taborn et al., 1978 [99]
Surgical Excision	N/A	Surgical	Direct removal of calcium deposits	Al-Mayouf et al., 2010 [68] Vitale et al., 2009 [102] Wu et al., 2008 [2008]

Note: IVIG, intravenous immunoglobulin; g, gram; kg, kilogram; mg, milligram; d, day; wk, week; mo, month; yr, year; IV, intravenous; SQ, subcutaneous; PO, by mouth; TNF-a, Tumor Necrosis Factor alpha; IL, interleukin.