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Complement abnormalities in acquired lipodystrophy revisited.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	O'Rahilly, Stephen Chaudhry, Afzal N. Raymond-Barker, Philippa Alexander, Graeme J. M. Cochran, Elaine K. Cinti, Saverio Semple, Robert K. Scobie, Ian Savage, David B. Clatworthy, Menna R. Murano, Incoronata Smith, Kenneth G. C. Lyons, Paul A. Gorden, Phillip Morgan, B. Paul Mufti, Ghulam J. Thiru, Sathia Murgatroyd, Peter R. Adams, Claire
Description	Country affiliation: United kingdom Author Affiliation: Savage DB ( Metabolic Research Laboratories, Institute of Metabolic Science, University of Cambridge, Addenbrooke's Hospital, Hills Road, Cambridge CB2 0QQ, United Kingdom. dbs23@medschl.cam.ac.uk)
Abstract	CONTEXT: Lipodystrophy is a heterogeneous condition characterized by an inherited or acquired deficiency in the number of adipocytes required for the storage of energy as triglycerides. Acquired lipodystrophy is frequently associated with other autoimmune disorders. One well-studied form is characterized by the selective loss of upper body fat in association with activation of the alternative complement pathway by C3 nephritic factor, low complement factor C3, and mesangiocapillary glomerulonephritis. OBJECTIVE: We now describe an immunologically distinct form of acquired generalized lipodystrophy, with evidence of activation of the classical complement pathway (low C4) and autoimmune hepatitis. Patients and Research Design: Three unrelated patients with acquired lipodystrophy and low complement C4 levels are described. In vitro analysis of the complement pathway was undertaken to determine the reason for the low C4 complement levels. Biopsies were obtained from liver, bone marrow, and adipose tissue for histological analysis. RESULTS: All three patients manifested near-total lipodystrophy, chronic hepatitis with autoimmune features, and low C4 complement levels. Additional autoimmune diseases, including severe hemolytic anemia, autoimmune thyroid disease, and polyneuropathy, were variably present. Detailed studies of complement pathways suggested constitutive classical pathway activation. CONCLUSIONS: Although the previously described syndrome, which typically results in a cephalad pattern of partial lipodystrophy, results from activation of the alternative complement pathway, this form, in which lipodystrophy is generalized, is associated with activation of the classical pathway. Future therapeutic approaches to these disorders may benefit from being tailored to their distinct immunopathogenesis.
ISSN	0021972X
e-ISSN	19457197
Journal	The Journal of Clinical Endocrinology & Metabolism
Issue Number	1
Volume Number	94
Language	English
Publisher	Oxford University Press
Publisher Date	2009-01-01
Publisher Place	United States
Access Restriction	Open
Subject Keyword	Complement Activation Complement C4 Deficiency Complement Pathway, Classical Lipodystrophy Immunology Child, Preschool Lupus Erythematosus, Systemic Research Support, Non-u.s. Gov't Discipline Endocrinology Discipline Metabolism
Content Type	Text
Resource Type	Article Case study
Subject	Biochemistry (medical) Endocrinology, Diabetes and Metabolism Clinical Biochemistry Biochemistry Endocrinology

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