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Table 3 Severity factors for successful steroid-free maintenance therapy in patients with anti-HMGCR myopathy stratified by the use of steroid-sparing immunosuppressants in monotherapy or in combination, with or without IVIG (N = 41)

From: Statin-induced anti-HMGCR myopathy: successful therapeutic strategies for corticosteroid-free remission in 55 patients

 Successful maintenance with SSI monotherapy, n = 23Unsuccessful or unevaluable maintenance with SSI monotherapy
Overall, n = 18Remission with SSI monotherapy and IVIG, n = 5Remission with SSI combination therapy (± IVIG), n = 5Unsuccessful maintenance with SSI therapies, n = 6*Maintenance with SSI therapy not evaluable, n = 2
Weakness at treatment onset, n (%)21 (91)16 (89)5 (100)5 (100)5 (83)1 (50)
Age at treatment onset, median (range) years70.5 (50.1–83.6)67.5 (44.0–83.1)69.4 (56.7–78.4)66.2 (44.0–78.8)63.0 (46.5–74.8)67.5 (73.0–83.1)
CK at treatment onset, median (range) UI/L5380 (696–23,000)8234 (1556–14,098)4750 (2770–14,098)8300 (1556–11,755)6737 (2267–13,339)6327 (2832–9821)
Severity score, mean (SD)2.2 ± 1.12.7 ± 1.22.8 ± 0.83.4 ± 0.92.5 ± 1.41.5 ± 2.1
Severity score ≥ 3, n (%)8 (35)11 (61)3 (60)4 (80)3 (50)1 (50)
Delay from first increased serum CK (< 500 UI/L) to treatment, median (range) months1.7 (0–24.9)12.7 (0–95.0)14 (0–95.0)13.4 (0.4–26.0)6.6 (0–42.2)0 and 79.0
IVIG at last follow-up011 (61)5 (100)2 (40)3 (50)1 (50)
Corticosteroid dosage at last follow-up
 No corticosteroids22 (96)11 (61)4 (80)4 (80)3 (50)0
 Prednisone ≤ 5 mg per day1 (4)4 (22)1 (20)1 (20)1 (17)1 (50)
 Prednisone > 5 mg per day03 (17)002 (33)1 (50)
Drug-free remission, n (%)4 (17)00000
Normal strength at last follow-up, n (%)20 (87)8 (44)2 (40)3 (60)2 (33)1 (50)
  1. SSI steroid-sparing immunosuppressant
  2. *Unsuccessful maintenance with SSI therapy included failure to SSI monotherapy (n = 3), SSI monotherapy + IVIG (n = 2) and SSI combination therapy + IVIG (n = 1)