Abstract
MCTD is more severe in children than in adults. Since children are more susceptible to long term corticosteroid side effects, we treated a 12 y/o white female with biweekly plasmapheresis, and induced remission of her MCTD. She presented with Raynaud's phenomenon of 5 months duration plus diffuse swelling of distal extremities. Three months later tender 1 cm. red nodules appeared in the anterior aspects of her legs. This was followed by anorexia, weight loss, and increasing weakness. Her fingertips were cyanotic, but did not have ulcers. There was moderate weakness of the proximal muscles without synovitis or limitation of motion. She had leukopenia, hypergammaglobulinemia, and elevated sedrate. SGOT was 155, and LDH 588 units. ANA was (+) at 1/6400 with a speckled pattern. ENA was (+) at 1/10,240, but only to RPN fraction. C1Q of 2.4% was normal, but Raji cell assay was stronly (+). Antids DNA studies were negative. EMG revealed inflammatory findings became normal. On plasmapheresis q3w she has remained asymptomatic with normal lab titers. Her favorable responses suggest a wider use for this novel treatment in MCTD and other immune complex diseases of children.
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Hertzman, A., Rodriguez, G., Sharp, D. et al. 925 PLASMAPHERESIS IN ADOLESCENT MIXED CONNECTIVE TISSUE DISEASE(MCTD). Pediatr Res 15 (Suppl 4), 596 (1981). https://doi.org/10.1203/00006450-198104001-00950
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DOI: https://doi.org/10.1203/00006450-198104001-00950