1st International Workshop on Adverse Drug Reactions and Lipodystrophy in HIV 26–28 June 1999 - San Diego, CA, USA

GENETIC AND ACQUIRED LIPODYSTROPHY SYNDROMES

Antiviral Therapy 1999; 4(Suppl. 2):25 (abstract no. 8)

A Garg
University of Texas Southwestern Medical Center at Dallas, Dallas, Texas, USA

The lipodystrophies are disorders characterized by loss of adipose tissue. Fat loss can be limited with well-demarcated subcutaneous (sc) indentations as in localized lipodystrophies or extensive as in generalized lipodystrophies. Metabolic complications, such as insulin resistance, diabetes mellitus, hypertriglyceridaemia and fatty liver, are related to the extent of fat loss. The lipodystrophies can be classified into two broad categories: (1) familial and (2) acquired. The two main types of familial lipodystrophies are an autosomal recessive, congenital generalized lipodystrophy (CGL) and an autosomal dominant, familial partial lipodystrophy, Dunnigan type (FPLD). Patients with CGL have near complete lack of metabolically active adipose tissue since birth but mechanical adipose tissue is well preserved. Patients with FPLD lose sc fat from the extremities at puberty and tend to accumulate excess fat in the face and neck. The FPLD gene was recently localized to chromosome 1q21-22 but the genetic basis of CGL and FPLD remains to be elucidated. Patients with acquired generalized lipodystrophy have generalized loss of sc fat but those with acquired partial lipodystrophy have fat loss limited to face, trunk and upper extremities. Autoimmunity underlies both types of acquired lipodystrophies, which occur more commonly in females usually starting during childhood. Recently, patients infected with HIV, receiving therapy including HIV-1 protease inhibitors, have been reported to develop a lipodystrophy characterized by loss of sc adipose tissue from the extremities and face but excess fat deposition in the neck and trunk. The underlying mechanisms, however, of lipodystrophy in HIV-infected patients remain unknown. Current management of patients with lipodystrophy includes cosmetic surgery, diet and drug therapy for control of diabetes and dyslipidaemia.

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