Article Figures & Data
Figures
- Figure 1.
Combinations of medications for treatment of “typical cases.” Steroid: oral or intravenous corticosteroid; MTX: methotrexate; HCQ: hydroxychloroquine; IVIG: intravenous immunoglobulin; CYCLO: cyclophosphamide.
- Figure 2.
Combinations of medications for treatment of “atypical cases.” See Figure 1 for abbreviations.
- Figure 3.
Dose and route of corticosteroids in mild, moderate, and severe cases. Low-dose oral: < 1.0 mg/kg/day; pulse at diagnosis: pulse methylprednisolone (30 mg/kg/dose/day) for 3–5 consecutive days; ongoing pulses: ongoing intermittent pulses; medium-dose oral: ≥ 1.0 mg/kg/day < 1.5 mg/kg/day; high-dose oral: ≥ 1.5 mg/kg/day.
Tables
Case Features “Typical” Mild Fatigue, typical JDM rash, mild nailfold capillary changes, slow to go up stairs but appears strong and active, MMT 4/5 to 5/5 Moderate Typical JDM rash, nailfold capillaries tortuous and dilated with drop-out, recent swallowing difficulty without choking, no skin ulceration, MMT 3/5 Severe Typical JDM rash, needs to be carried, coughing with liquids, crampy abdominal pain, unable to initiate anti-gravity movement, no skin ulceration, weak nasal voice Ulcerative Severe truncal and proximal muscle weakness, typical JDM rash, trouble swallowing, “aggressive treatment” initiated, 2 weeks later 25–30 cutaneous ulcers develop “Atypical” Late diagnosis Presentation 3 years from onset of symptoms, now has contractures of upper extremities, generalized weakness, typical JDM rash, markedly abnormal nailfold capillaries, mild superficial calcinosis at elbows, elevated CK, AST, and LDH (all 3 × normal) High enzyme Sun-sensitive rash, very mild proximal muscle weakness, mild erythema of eyelids and cheeks, mild abnormalities of nailfold capillaries, persisted mild elevation of CK, AST, ALT, and LDH (all 1.5 × normal) Skin, no muscle involvement Typical JDM rash, abnormal nailfold capillaries, no weakness, AST mildly elevated, CK, aldolase, and LDH normal Muscle, no skin involvement Myalgias, low-grade fever, weight loss, no rash, normal nailfold capillaries, tender proximal muscles, MMT 4/5, 5/5 distal muscles, elevated CK (25 × normal), aldolase, AST, and LDH (5–10 × normal) Lung disease Typical presentation of JDM, 2 years into treatment with corticosteroids and MTX develops dyspnea and nonproductive cough, CT scan of chest shows diffuse interstitial lung disease Refractory rash Typical presentation of JDM, treated with high-dose prednisone and medium-dose subcutaneous MTX, 1 year into treatment (prednisone discontinued) has ongoing JDM rash, strength is normal, CK, aldolase, AST, LDH all normal “Refractory” Typical presentation of JDM, treated with high-dose prednisone and high-dose subcutaneous MTX, at 6 months still on high-dose steroid with persistent rash and moderate weakness, evidence of corticosteroid toxicity, CK, and AST are normal, ALT and LDH just above upper limit of normal Partial response -
MMT: manual muscle testing (in proximal muscles unless otherwise stated); CK: creatine kinase; AST: aspartate aminotransferase; ALT: alanine aminotransferase; LDH: lactate dehydrogenase; CT: computed axial tomography.
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Case Group Response Rate per Case* (%) Representation of Total Surveyed (n = 167), % “Typical” Mild C 29/42 (69) 17 Moderate A, B, C, D, 124/167 (74) 74 Severe B 34/42 (81) 20 Ulcerative A, D 57/83 (69) 34 “Atypical” Late diagnosis B 33/42 (79) 20 High enzyme A 24/41 (59) 14 Skin, no muscle involvement D 35/42 (83) 21 Muscle, no skin involvement B 32/42 (76) 19 Lung disease C 29/42 (69) 17 “Refractory” Partial response A 22/41 (54) 13 Refractory rash C, D 64/84 (76) 38 -
↵* Some subjects completing the “moderate case” did not provide complete data on the individual cases.
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Characteristic n (%) Level of responder Attending physician 95 (77) Trainee 11 (9) Emeritus 1 (0) Did not respond to this question 17 (14) Specialty Pediatric rheumatology 102 (82) Pediatric immunology/rheumatology 5 (4) Adult/pediatric rheumatology 2 (2) Did not respond to this question 15 (12) Year in which began practicing rheumatology* ≤ 1980 8 (7) > 1980 ≤ 1990 22 (19) > 1990 ≤ 2000 36 (32) > 2000 25 (22) Did not respond to this question 22 (19) Estimate of no. of new patients with JDM seen per 5 years 0–5 15 (12) 6–10 35 (28) 11–20 36 (29) 21–50 18 (15) ≥ 51 4 (3) Did not respond 16 (13) -
↵* Trainees not included.
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Case Respondents Indicating They Would Use MTX, n (%) Dosage*, n (%) Route of Administration, n (%) Mild 22/29 (76) Low 3 (14) Oral 5 (23) Med 9 (41) SC 15 (68) High 10 (45) IV 2 (9) Moderate 104/124 (84) Low 11 (11) Oral 17 (16) Med 51 (49) SC 66 (63) High 42 (40) IV 6 (6) > 1 option 15 (14) Severe 27/34 (79) Low 1 (4) Oral 4 (15) Med 12 (44) SC 17 (63) High 14 (52) IV 4 (15) IM 1 (4) > 1 option 1 (4) Ulcerative 38/57 (67) Low 4 (11) Oral 2 (5) Medium 10 (26) SC 21 (55) High 19 (50) IV 6 (16) Did not respond 5 (13) > 1 option 4 (11) Did not respond 5 (13) -
↵* Low: ≤ 10 mg/m2/week; Med: > 10 mg < 20 mg/m2/week; High: ≥ 20 mg/m2/week. SC: subcutaneous; IV: intravenous; IM: intramuscular.
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