Clinical Features, Metabolic and Autoimmune Derangements in Acquired Partial Lipodystrophy (Barraquer–Simons Syndrome)
Authors' conclusion
Our data reveal high risk of metabolic comorbidities and drusen in patients with APL, with earlier onset of these complications in those with C3 hypocomplementemia.
Study context
- Population
- Patients with acquired partial lipodystrophy (APL; Barraquer–Simons syndrome); 77 female, 9 male; median age 40 years (range 8–78)
- Participants
- 86 patients
- Outcome
- Clinical features, metabolic and autoimmune derangements
Methodological detailsmulticentre, 2 US tertiary referral centers
- Multicentre
- 2 US tertiary referral centers
Main finding
As reported in the abstract
- Median age of onset of lipodystrophy
- 7 years (range, 2-51 years)
- Autoimmune diseases
- About 15%
- DM or glucose intolerance
- 38%
- Hypertriglyceridemia
- 43%
- Fatty liver or MASH
- 61%
- Low serum C3
- 71%
- Membranoproliferative glomerulonephritis
- 8%
- Drusen, among those with fundus examination (n = 21)
- 62%
Observational data: associations, not effects.
Further findings
Age of onset of complications, C3 hypocomplementemia vs normal C3
- DM or glucose intolerance, median age, C3 hypocomplementemia vs normal C3
- 36 vs 56.5 years, P = .007
- Hypertriglyceridemia, C3 hypocomplementemia vs normal C3
- 30 vs 48 years, P = .03
- Drusen, C3 hypocomplementemia vs normal C3
- 33 vs 60 years, P = .14
- Earlier onset reported with C3 hypocomplementemia
Original publication
Clinical Features, Metabolic and Autoimmune Derangements in Acquired Partial Lipodystrophy (Barraquer–Simons Syndrome). J Clin Endocrinol Metab. Published 28 May 2025. doi:10.
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