Table 2.

Medications received by 320 patients with juvenile dermatomyositis.

	Number and Percent of Patients			Time to Start Medication*
	Ever Used	Before 1997	After 1997	All patients	Before 1997	After 1997
	(N=320)	(N=220)	(N=100)	(N=320)	(N=220)	(N=100)
	N (%)	N (%)	N (%)	Months [IQR]	Months [IQR]	Months [IQR]
Oral Prednisone	318 (99.4)	218 (99.1)	100 (100.0)	0 [0–3]	0 [0–3]	0 [0–3]
IV Methylprednisolone1	182 (56.9)†	112 (50.9)	70 (70.0)‡	0 [0–251]†	0 [0–312.8]	3 [0–183.8]
Methotrexate2	225 (70.3)†§	130 (59.1)	95 (95.0)**	2.0 [0.1–13.0]†§	6.0 [0.8–22.0]	0.2 [0–2.0]**
IV Immunoglobulin3	119 (37.2)†§‖	55 (25.0)	64 (64.0)**	7.1 [0.6–20.0]†§	10.0 [0.5–25.0]	5.1 [0.7–19.0]
Other DMARDs4	66 (20.6)†§‖¶	25 (11.4)	41 (41.0)**	14.5 [3.4–31.2]†‖§	23.0 [5.5–51.4]	13.3 [3.2–24.9]
Cytotoxic/Biologics therapies5	40 (12.5)†§‖¶	12 (5.5)	28 (28.0)**	9.0 [3.1–31.5]†‖§	5.5 [2.7–13.1]	9.0 [3.1–42.6]
Antimalarial drugs6	153 (47.8)†‖⨍#	83 (37.7)	70 (70.0)**	3.3 [0.1–20.7]†‖⨍	7.0 [0.1–31.7]	2.3 [0–12.2]
Maximum combination of medications received, Median [IQR]	2.0 [2.0–3.0]	2.0 [1.0–3.0]	4.0 [2.0–4.8]**
1 major medication	76 (23.8)	71 (32.3)	5 (5.0)**	0 [0–0]	0 [0–0]	0 [0–0]
2 major medications7	107 (33.4)	83 (37.7)	24 (24.0)	2.9 [0.1–13.9]††	3.0 [0.1–22.0]	1.0 [0–2.9]
3 major medications8	71 (22.2)	51 (23.2)	20 (20.0)	8.0 [1.0–23.0]††	11.5 [2.0–37.2]	5.7 [1.1–21.2]
4 or more major medications	64 (20.0)	13 (5.9)	51 (51.0)**	10.1 [2.0–26.6]††	12.0 [6.0–41.0]	6.4 [1.9–25.4]

Abbreviations: IQR, Interquartile range; IV, intravenous; DMARDs, disease-modifying anti rheumatic drugs; Ever used, total patients who received each medication at least once; Before 1997, patients diagnosed before 1997; After 1997, patients diagnosed after 1997

^*Time to start medication from start of initial treatment for Oral prednisone, IV methylprednisolone and 1 major agent are shown in days instead of months.

^‡P <0.05,

^**P <0.01, vs Before 1997 patients,

^†P <0.01 vs Oral Prednisone,

^§P <0.01 vs IV Methylprednisolone,

^‖P <0.01 vs Methotrexate,

^¶P <0.01 vs IV Immunoglobulin,

^⨍P <0.01 vs Other DMARDs,

^#P <0.01 vs Cytotoxic/Biologics Agents,

^††P <0.01 vs 1 major agent

¹The median dose of IV methylprednisolone (IVMP) initial pulse therapy was 30.0 [IQ range 22.1–30.0] mg/kg/dose, and the median dose for other intervals was 22.0 [14.7–30.0] mg/kg/dose. IVMP was given as an initial pulse, primarily for 3 days, in 31.3% who received IVMP, and as weekly infusions in 24.8%, every 2 week infusions in 11.4%, and monthly infusions in 19.4%.

²Among the patients who received methotrexate (MTX), 99.2% received it weekly. The median initial dose of MTX was 0.50 [ IQ range 0.40–0.76] mg/m²/week. The administration route of MTX was: PO in 47.5%, SQ in 35.3%, IV in 12.6 and IM in 4.6% of patients receiving MTX; 40.4% of patients changed the route of MTX administration later.

³The median dose of IV immunoglobulin (IVIG) was 2 [IQR 1–2] g/kg/dose. The Interval of IVIG was monthly in 71.7% who received IVIG, every 2 weeks in 10.6% and every 3 weeks in 10.3%.

⁴Other DMARDs included cyclosporine in 59.1% of patients who received other DMARDs, mycophenolate mofetil 30.3%, azathioprine 28.8%, tacrolimus 12.1%, and leflunomide 3.0%. The percentages are >100 because 15 patients received more than 1 other DMARD.

⁵Cytoxic/Biologic therapies included intravenous cyclophosphamide in 50.0% of patients who received cytotoxic/biologic agents, and of these, 75.8% received it monthly with median dose of 750 mg/m², rituximab 32.5%, etanercept 27.5%, infliximab 22.5%, oral cyclophosphamide 15.0% with median dose of 1mg/kg/day; 14 patients received more than 1 cytotoxic/biologic therapy. For the 13 patients who received rituximab, the median number of IV rituximab infusions was 4.0 [IQ range 2.5–4.0]; doses ranged from 500 mg/m² to 1000 mg per infusion, and 6 patients received 4 weekly IV infusions and 6 received 2 weekly IV infusions(13).

⁶Antimalarial drugs included hydroxychloroquine in 100% of patients who received antimalarial drugs, chloroquine 1.3%, and quinacrine 1.3%; 4 patients received more than 1 antimalarial drug.

⁷The most frequent 2 medication combinations were prednisone and methotrexate in 84.6%, and prednisone and IV immunoglobulin in 12.3% among 228 patients who received 2 medications in combination.

⁸The most frequent 3 medication combinations were: prednisone, methotrexate and IV methylprednisolone in 35.4% of patients, prednisone, methotrexate and IV immunoglobulin in 35.4%, prednisone, IV methylprednisolone and IV immunoglobulin in 10.0%, and prednisone, methotrexate and cyclosporine 9.2% among 130 patients who received 3 medications in combination.