Clinical Characteristics of Patients With Acquired Partial Lipodystrophy: A Multicenter Retrospective Study
Authors' conclusion
BSS has a childhood onset and is often associated with autoimmune diseases. Skinfold thickness measurements and fat assessment by dual energy X-ray absorptiometry are useful tools to identify the disease. C3 hypocomplementemia and the presence of autoantibodies may be used as additional diagnostic supportive criteria but the prevalence of C3 hypocomplementemia may be lower than previously reported.
Study context
- Population
- Patients with Barraquer-Simons syndrome (BSS), an acquired partial lipodystrophy, diagnosed by accepted international-guideline criteria
- Participants
- 26 patients
- Outcome
- Clinical, immunological and metabolic features of BSS
Main finding
As reported in the abstract
- Female
- 73%
- Fat loss during childhood
- 77%
- Median age at fat loss (years)
- 8
- Diabetes
- 11.5%
- Dyslipidemia
- 34.6%
- Hepatic steatosis
- 26.9%
- C3 hypocomplementemia in children
- 75%
- C3 hypocomplementemia in adults
- 50%
- Positive for ≥1 autoantibody
- 76%
- Patients with a single LMNB2 variant
- 1
Observational data: associations, not effects.
Original publication
Clinical Characteristics of Patients With Acquired Partial Lipodystrophy: A Multicenter Retrospective Study. J Clin Endocrinol Metab. Published 7 December 2023. doi:10.
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