Anti-HMGCR myopathy: Diversity of clinical presentations in a national cohort in New Zealand
Authors' conclusion
Recurrent falls, a long prodrome and dominant lower limb proximal weakness were common in this anti-HMGCR myopathy cohort.
Study context
- Population
- Adults positive for serum anti-HMGCR autoantibodies (line blot or enzyme-immunoassay followed by immunoprecipitation), New Zealand
- Participants
- 75 participants
- Outcome
- Clinical presentation of anti-HMGCR myopathy
Methodological detailsnational cohort
- National cohort
Main finding
As reported in the abstract
- Weakness as presenting symptom
- 72 (96 %)
- Predominantly or solely lower limb weakness (of 65 with reliable description of proximal weakness)
- 22/65 (33.8 %)
- Subacute onset (symptoms >4 weeks -≤6 months)
- 45 of 75 (60 %)
- Indolent chronic onset (symptoms >6 months)
- 22/75 (29.3 %)
- Falls
- 18 of 75 (24 %)
- "General decline"
- 2/75 (2.7 %)
- No weakness described (2 myalgia, 1 Jessner lymphocytic skin rash)
- 3
- Median creatine kinase at presentation, U/L (range 1050-25,500)
- 7337
Observational data: associations, not effects.
Further findings
Muscle biopsy performed
- Muscle biopsy
- 38 (50.7 %)
Associated malignancy
- Associated malignancy was infrequent
Recovery without immunosuppression
- Recovered without immunosuppression
- 4
Survival · 5 and 10 years
%
- 5-year survival
- 92.7 (95 % CI 80.6-97.4 %)
- 10-year survival
- 82.5 (95 % CI 61.2-92.8 %)
Original publication
Anti-HMGCR myopathy: Diversity of clinical presentations in a national cohort in New Zealand. Semin Arthritis Rheum. Published 14 July 2024. doi:10.
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