Systemic sclerosis & Raynaud's
Proximal weakness and creatine kinase elevation in systemic sclerosis: Clinical correlates, prognosis and functional implications
Authors' conclusion
Clinical features of myopathy are highly prevalent in SSc, affecting almost half of our study cohort. Detection of PW and elevated CK alone, even without imaging or histopathological identification of SSc-myopathy, identified important clinical associations and are associated with poorer function and overall prognosis.
Study context
- Population
- Australian Scleroderma Cohort Study participants with systemic sclerosis (SSc) and ≥1 creatine kinase (CK) and proximal power assessment
- Participants
- 1,786 participants
- Exposure
- Concurrent PW&CK elevation, PW alone or CK elevation alone vs neither
- Outcome
- Frequency of clinical evidence of muscle disease
Methodological detailstime-varying-covariate Cox regression, multinomial logistic and linear regression
- Time-varying-covariate Cox regression
- Multinomial logistic and linear regression
Main finding
As reported in the abstract
Observational data: associations, not effects.
Further findings
| Outcome | HR (95 % CI) | P value | |
|---|---|---|---|
| Mortality (multivariable)CK elevation alonevs Neither | 0.7 (0.4–1.1) | P = 0.09 | |
| 0.512Hazard ratio (log scale) |
| Outcome | Fold increased mortality (95 % CI) | P value |
|---|---|---|
| Mortality (multivariable)PW&CK elevation vs Neither | 3.6 (1.9–6.6) | P < 0.01 |
| Mortality (multivariable)PW alone vs Neither | 2.1 (1.4–3.0) | P < 0.01 |
Clinical phenotype of PW&CK elevation
- More frequent dcSSc
- P < 0.01
- More frequent tendon friction rubs
- P < 0.01
- More frequent synovitis
- P < 0.01
- More frequent digital ulceration
- P = 0.03
Comorbidity in PW, with and without CK elevation (most common)
- Multimorbidity
- P < 0.01
- Ischaemic heart disease
- P < 0.01
- PAH
- P < 0.01
Physical function in PW regardless of CK elevation
- Reduced six-minute-walk-distance
- P < 0.01
- Higher HAQ-DI scores
- P < 0.01
- Increased patient-reported dyspnoea
- P = 0.04
CK elevation alone
- Most frequent in men with anti-Scl70 positivity, without other significant clinical differences
Original publication
Proximal weakness and creatine kinase elevation in systemic sclerosis: Clinical correlates, prognosis and functional implications. Semin Arthritis Rheum. Published 24 January 2024. doi:10.
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