SUN-642 Mortality Outcomes in Patients with Lipodystrophy: Key Insights from the LD-Lync Study

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Abstract Disclosure: M. Celik Guler: None. O. Besci: None. M.C. Foss de Freitas: None. M. Yosef: None. S. Khalatbari: None. M. Ashmus: None. D. Guler: None. D. Gilio: None. D. Kaba: None. C. Uwandu: None. A. Dill Gomes: None. R. Tuska: None. M. Brush: None. M. Hwang: None. T. Neal: None. B. Tracey: None. M. Okawa: None. B. Brite: None. C. Richison: None. I. Yildirim Simsir: None. B. Akinci: Chiesi Farmaceutici, Alnylam, Regeneron Pharmaceuticals, Marea Therapeutics, AstraZeneca, Novo Nordisk, Boehringer Ingelheim, Sanofi, Bilim Ilac, ARIS, Servier. R.J. Brown: Pfizer, Inc., Regeneron Pharmaceuticals, Marea Therapeutics, Chiesi Farmaceutici. E.A. Oral: Chiesi Farmaceutici, Ionis Pharmaceuticals Inc., Regeneron Pharmaceuticals, Rhythm Pharmaceuticals, Marea Therapeutics, Novo Nordisk, Rejuvenate Bio, Fractyl, Morphic Medical. Background: Lipodystrophy syndromes (LD) represent rare disorders characterized by adipose tissue deficiency, accompanied by severe metabolic complications such as uncontrolled diabetes, hypertriglyceridemia, pancreatitis, and hepatic steatosis, all of which contribute to morbidity and mortality. Despite advancements in understanding LD, data on mortality and its contributing factors remain limited by retrospective data; this study seeks to address this gap by evaluating mortality rates and underlying determinants across LD subgroups. Methods: Data were analyzed from an ongoing prospective, multicenter study (LD-Lync; NCT03087253, at the end of January 2025). Demographics and medical histories were collected at baseline and annual visits. This analysis examines mortality rates during the follow-up period, providing insights into patient LD subtypes, comorbidities, and causes of death. Findings were summarized using descriptive statistics and presented as median with interquartile range (IQR). Results: Among 348 patients with LD, nine (2.6%) died during the study follow-up period. The median age at LD diagnosis for these patients was 45 years (IQR: 13.5-57), with a median time to mortality of 15 years (IQR: 7-28) after diagnosis. The deceased comprised four patients with familial partial lipodystrophy type 1 (FPLD1), three with FPLD type 2 (FPLD2), and two with acquired generalized lipodystrophy (AGL). The median age at death was 51 years (IQR: 39.5-72). In the FPLD1 subgroup, a 64-year-old patient died from aspiration pneumonia with underlying coronary artery disease, a 36-year-old patient died due to multiorgan failure following an acute exacerbation of chronic pancreatitis and a 70-year-old patient who suffered from concomitant end-stage liver disease and hepatic encephalopathy died from aspiration pneumonia. Additionally, a 51-year-old patient with FPLD1 passed away, though the cause of death was undocumented. Among patients with FPLD2, a 37-year-old died from metastatic endometrial cancer, a 74-year-old from myocardial infarction, and a 78-year-old from decompensated cirrhosis. A 42-year-old with AGL developed, endocarditis induced hemophagocytic lymphohistiocytosis and acute respiratory failure secondary to gastrointestinal bleeding. A 43-year-old patient with AGL who also harbored titin mutation, who had a variety of complications, including autoimmune hepatitis, type 1 diabetes, polymyositis, and rheumatoid arthritis, died from cardiogenic shock due to chronic nonischemic cardiomyopathy. Conclusions: This study underscores the substantial mortality burden associated with LD. Findings emphasize the critical need for early identification and comprehensive management of metabolic abnormalities and associated conditions to enhance survival outcomes and reduce the overall disease burden in this population. Presentation: Sunday, July 13, 2025

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