Table 1.
Cases | Case 1 | Case 2 | Case 3 |
Age of onset (years) | 60 | 68 | 62 |
Gender | Female | Female | Male |
Statin | Atorvastatin and rosuvastatin | Atorvastatin | Atorvastatin |
Duration from symptom onset to presentation | 2 months | 1 month | 12 months |
Duration from commencement of statin to symptom onset | Atorvastatin: few months Rosuvastatin: 2 months |
Atorvastatin: 3 years | Atorvastatin: 3 years |
Pattern of weakness | Proximal upper and lower limbs | Proximal upper and lower limbs | Proximal, especially hip girdle |
Initial peak CK | 11 003 | 3900 | 5000 |
Anti-HMGCR antibody at presentation | 54.4 RU (normal range <11.0 RU) | 49.0 RU (normal range <11.0 RU) | 0.84 (normal <0.24 OD units) |
Muscle biopsy | Focal segmental necrotising myopathy with minimal inflammation | Moderate necrotising myopathy with minimal inflammation | Necrotising myopathy |
Immunotherapies trialled | Prednisolone Methotrexate Cyclosporine IVIG |
Prednisolone Methotrexate Cyclosporine IVIG |
Prednisolone Methotrexate Cyclosporine IVIG Plasma exchange |
Current immunotherapy | Methotrexate Rituximab |
Methotrexate IVIG Rituximab |
Methotrexate IVIG Rituximab |
Current status | Stable CK (<100 U/L) Normal anti-HMGCR antibody level Mild weakness |
Stable CK (200~300 U/L) Reduced anti-HMGCR antibody level Mild weakness |
Stable CK (100~300 U/L) Anti-HMGCR antibody level still elevated Mild weakness |
anti-HMGCR, antibodies to 3-hydroxy-3-methylglutaryl coenzyme A reductase; CK, creatine kinase; IVIG, intravenous immunoglobulin; OD, Optical Density; RU, Reference Unit.