Table 2 Severity factors in patients with anti-HMGCR myopathy and successful steroid-based induction therapy, stratified by concomitant use of IVIG therapy and early vs late remission (N = 41)
Dual steroids/SSI cohort (n = 19) | Triple steroids/IVIG/SSI cohort (n = 22) | |||
|---|---|---|---|---|
Early remission (≤ 3 months), n = 10 | Late remission (> 3 months), n = 9 | Early remission (≤ 3 months), n = 12 | Late remission (> 3 months), n = 10 | |
Weakness at treatment onset, n (%) | 8 (80) | 8 (89) | 11 (92) | 10 (100) |
Age at treatment onset, median (range) years | 70.6 (59.9–83.6) | 69.4 (50.1–81.3) | 73.6 (46.5–83.1) | 60.4 (44.0–74.3) |
CK level at treatment onset, median (range) UI/L | 2673 (696–12,000) | 6405 (3573–10,465) | 7317 (1556–13,339) | 10,789 (2267–23,000) |
Severity score, mean (SD) | 1.4 (0.8) | 2.5 (0.9) | 2.5 (1.2) | 3.3 (0.9) |
Severity score ≥ 3, n (%) | 1 (10) | 4 (44) | 6 (50) | 8 (80) |
Delay from first increased serum CK (> 500 UI/L) to treatment, median (range) months | 1.4 (0–79.2) | 13.4 (0–24.9) | 0.8 (0–42.2) | 11.5 (0–95) |
Delay from treatment to serum CK < 500 UI/L, median (range) months | 1.7 (0.4–3) | 11.5 (4–50.7) | 2.0 (0.6–3) | 15.0 (3.2–53) |
Needed induction strategies to obtain remission, n (%) | ||||
1 | 10 (100) | 8 (89) | 12 (100) | 6 (60) |
2 | 0 | 1 (11) | 0 | 1 (10) |
3 | 0 | 0 | 0 | 2 (20) |
≥ 4 | 0 | 0 | 0 | 1 (10) |
Successful maintenance with SSI monotherapy, n (%) | 8 (80) | 8 (89) | 5 (42) | 2 (20) |
Corticosteroid dosage at last follow-up | ||||
No corticosteroids | 8 (80) | 8 (89) | 9 (75) | 8 (80) |
Prednisone ≤ 5 mg per day | 2 (20) | 1 (11) | 1 (8) | 1 (10) |
Prednisone > 5 mg per day | 0 | 0 | 2 (17) | 1 (10) |
Drug-free remission, n (%) | 3 (30) | 1 (11) | 0 | 0 |
Normal strength at last follow-up, n (%) | 9 (90) | 8 (89) | 6 (50) | 5 (50) |