Patient no.Previously failed ISConcomitant ISBaseline prednisone doseKey clinical feature
All2.6 (1.1) IS failed+all failed predMean (SD): 2.4 (0.8)
Pred: 11; MTX: 5; AZA: 3; TAC: 1; MMF: 5; HCQZ: 2
Mean (SD) dose: 19.5 (15.3)Severe muscle weakness: 8 (one dropped out at week 6)
Moderate-severe DM rash: 5
1Pred (30 months, 60 mg taper), MTX (30 months, 15 mg), MMF (15 months, 2 g)Pred, MTX (15 mg), MMF (2 g)7.5 mgModerate-severe rashes and mild muscle weakness
2Pred (>12 months, 60 mg taper), TAC (39 months, 4 mg), AZA (unknown, 200 mg), MMF (unknown, 2 g), MTX (4 months, 15 mg)Pred, HCQZ (400 mg)20 mgModerate-severe rashes, no muscle weakness
3Pred (17 months, 60 mg taper), MTX (16 months, 20 mg), AZA (14 months, 100 mg)Pred, AZA (100 mg), MTX (15 mg)42.5 mgSevere muscle weakness
4Pred (14 months, 80 mg taper), MTX (14 months, 25 mg), MMF (2 months, 2 g), AZA (6 months, 100 mg)Pred, AZA (100 mg), MMF (2 g)10 mgSevere muscle weakness
5Pred (11 months, 60 mg taper), MTX (5 months, 20 mg), MMF (3 months, 3 g), AZA (3 months, 100 mg)Pred, AZA (100 mg), MMF (3 g)50 mgModerate-severe rashes and severe muscle weakness
6Pred (15 months, 60 mg taper), TAC (3 months, 3 mg), AZA (3 months, 100 mg), MMF (5 months, 3 g), MTX (9 months, 25 mg)Pred, TAC (3 mg)7.5 mgSevere rashes and mild muscle weakness
7Pred (18 months, 60 mg taper), IVIG (6 months), MMF (7 months, 3 g), MTX (18 months, 22.5 mg)Pred, MTX (22.5 mg), MMF (3 g), HCQZ (400)10 mgModerate-severe rashes and severe muscle weakness
8Pred (24 months, 40 mg taper), MTX (8 months, 17.5 mg)Pred, MTX (17.5 mg)2.5 mgSevere muscle weakness; dysphagia
9Pred (24 months, 60 mg taper), IVIG (8 months, 2 g/kg), rituximab (12 months ago, 2 g), MTX (18 months, unknown)Pred20 mgSevere muscle weakness; dysphagia
10Pred (6 months, 60 mg taper), MTX (5 months, 25 mg)Pred, MTX15 mgSevere muscle weakness; mild arthritis
11Pred (8 months, 50 mg taper), IVIG (8 months, 1 g/kg), MMF (5 months, 3 g)Pred, MMF (3 g)30 mgSevere muscle weakness
  • All medication doses are daily doses except MTX which is weekly.

  • *Muscle enzymes are represented as times the upper limit of normal of the most abnormal enzyme (ie, CK, aldolase, LDH, AST, ALT) at baseline.

  • AA, African–American; ALT, alanine transaminase; AST, aspartate transaminase; AZA, azathioprine; DK, creatine kinase; DM, dermatomyositis; HCQZ, hydroxychloroquine; IS, immunosuppression; IVIG, intravenous immunoglobulin; LDH, lactate dehydrogenase; MMF, mycophenolate; MMT, Manual Muscle Testing; MTX, methotrexate; NM, necrotising myopathy; PM, polymyositis; pred, prednisone; TAC, tacrolimus; VAS, Visual Analogue Scale.