Also known as Lawrence syndrome, acquired generalized lipodystrophy (AGL) is an ultra-rare condition characterized by the destruction or loss of adipose tissue. Given the small population size, AGL is still poorly understood; only around 100 cases have been described in medical literature. As a result, treatment options are not targeted but symptomatic. A research team hailing from UT Southwestern Medical Center is working to advance our understanding of AGL and potentially lead to new therapeutic interventions. They began this journey, shares News Medical, by discovering the first molecular biomarker for AGL.
Within this study, the results of which were published in Diabetes, the research team sourced clinical data from 46 individuals with AGL over a 30-year period. The data came from the UT Southwestern biorepository, which holds a significant amount of information on acquired generalized lipodystrophy.
Researchers tested 19,500 human proteins to determine what antibodies might be present. They found that the perilipin-1 autoantibody was found in the blood samples of 37% of those with AGL. However, no blood samples from healthy controls showed this autoantibody. Further testing highlighted how patients who had AGL with panniculitis were even more likely to display this autoantibody in their blood samples.
More research is needed—both into perilipin-1 autoantibody and AGL in general. However, this research does suggest that perilipin-1 autoantibody could cause or further disease progression within this realm.
About Acquired Generalized Lipodystrophy (AGL)
Because acquired generalized lipodystrophy can manifest in infancy, childhood, or adulthood, some hypothesize that it is acquired; others believe that an infection or abnormal immune response may cause this. As described above, the perilipin-1 autoantibody has also been implicated. Additional research will provide more insight into AGL and expand our understanding of this disorder. Females are 3x more likely to develop AGL than males. Symptoms of acquired generalized lipodystrophy may include:
- Dark pigmentation in different areas of the body
- An intense appetite
- Newly-onset diabetes, thyroiditis, rheumatoid arthritis, or vitiligo
- Enlarged liver, spleen, and/or genitalia
- Liver cirrhosis
- Low body fat / extreme fat loss
- Prominent muscles and veins
- Excess body hair
- Ovarian cysts
Treatment options include a high-carbohydrate and low-fat diet, regular exercise, and various interventions to reduce symptoms.
