The Diagnosis and Management of Lipodystrophy Syndromes: A Multi-Society Practice Guideline
Year: 2016
Type: article
Abstract: OBJECTIVE: Lipodystrophy syndromes are extremely rare disorders of deficient body fat associated with potentially serious metabolic complications, including diabetes, hypertriglyceridemia, and steatohepatitis. Due to their rarity, most clinicians are not familiar with their diagnosis and management. This practice guideline summarizes the diagnosis and management of lipodystrophy syndromes not associated with HIV or injectable drugs. PARTICIPANTS: Seventeen participants were nominated by worldwide endocrine societies or selected by the committee as content experts. Funding was via an unrestricted educational grant from Astra Zeneca to the Pediatric Endocrine Society. Meetings were not open to the general public. EVIDENCE: A literature review was conducted by the committee. Recommendations of the committee were graded using the system of the American Heart Association. Expert opinion was used when published data were unavailable or scarce. CONSENSUS PROCESS: The guideline was drafted by committee members and reviewed, revised, and approved by the entire committee during group meetings. Contributing societies reviewed the document and provided approval. CONCLUSIONS: Lipodystrophy syndromes are heterogeneous and are diagnosed by clinical phenotype, supplemented by genetic testing in certain forms. Patients with most lipodystrophy syndromes should be screened for diabetes, dyslipidemia, and liver, kidney, and heart disease annually. Diet is essential for the management of metabolic complications of lipodystrophy. Metreleptin therapy is effective for metabolic complications in hypoleptinemic patients with generalized lipodystrophy and selected patients with partial lipodystrophy. Other treatments not specific for lipodystrophy may be helpful as well (eg, metformin for diabetes, and statins or fibrates for hyperlipidemia). Oral estrogens are contraindicated.
DOI: 10.1210/jc.2016-2466
Institutions: National Institutes of Health, Universidade de Santiago de Compostela, University of Cambridge, National Institute of Diabetes and Digestive and Kidney Diseases, University of Hong Kong, Medical Research Council, National Institute for Health and Care Research, Wellcome/MRC Institute of Metabolic Science, MRC Epidemiology Unit, NIHR Cambridge Biomedical Research Centre, Wellcome Trust, The University of Texas Southwestern Medical Center, The University of Sydney, Royal North Shore Hospital, Osaka City General Hospital, University of Nairobi, University of Michigan, Michigan Medicine, Universität Ulm, Lomonosov Moscow State University, Endocrinology Research Center, Harvard University, Massachusetts General Hospital, Inserm, Université Pierre-et-Marie-Curie, Cambridge University Hospitals NHS Foundation Trust, Assistance Publique – Hôpitaux de Paris, Hôpital Saint-Antoine, Centre de Recherche Saint-Antoine, Fondation pour l’innovation en Cadiométabolisme et Nutrition
Language: English
Subfield: Cardiology and Cardiovascular Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $3,750
OpenAlex ID: W2528176296