A patient with total lipodystrophy, juvenile rheumatoid arthritis, 5’ nucleotidase deficiency and abnormal immune function

The acquired form of total lipodystrophy (LD) is iracterized by the disappearance of subcutaneous fat ue, hyperlipidemia, non-ketotic insulin resistant perglycemia, hepatomegaly and, in some cases, pigntary disturbances; this condition has also been orted in association with autoimmune diseases, such scleroderma and sicca syndrome. The present study cribes a 15-year-old boy who suffered from polyicular juvenile rheumatoid arthritis (JRA), which appeared at the age of ten years to be followed by tures of total lipodystrophy, liver cirrhosis, lipidemia, ulin responsive non-ketotic hyperglycemia, hypermentation and moderate hypertension. He also showed rked immunological abnormalities which included the sence of autoantibodies, elevated immunoglobulins, a inction of total T cells and increased B cells. The per/suppressor cell ratio was high and suppressor cell iction was absent. Lymphocyte 5 ’ nucleotidase activity s decreased. The possible relationship between the kiency of 5 ’ nucleotidase activity and the decrease of ' pressor cells will be discussed. copyright.