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L.W. is a 48-year-old right handed female with a scleroderma overlap syndrome (scleromyositis) manifested by systemic sclerosis (skin and gastrointestinal involvement) with a predominantly proximal necrotizing myositis. It is likely that this is a rare polymyositis/scleroderma overlap or anti-synthetase syndrome. She is persistently antibody negative for U1RNP, PmScl, Ro, La, Jo-1 and Ku antibodies. Antibodies to smooth muscle and skeletal muscle are also negative. Her symptoms were treatment resistant to immunosuppression until B cell depletion with rituximab. We discuss her course and prognosis. The patient presented in 2008 with progressive gastrointestinal symptoms with malabsorption, eventually requiring total parenteral nutrition. Total parenteral nutrition resulted in improved general condition with weight gain. She was being treated with combination of methotrexate and prednisolone when she developed insidiously progressive proximal weakness, with parallel progression of skin thickening. Peak creatine kinase (CK) was recorded at 1800 u/L. There was no improvement in muscle strength with steroid reduction. Electromyogram in 2010 suggested a proximal predominant necrotizing myositis. We postulate she has a scleroderma overlap syndrome. She was treated with rituximab in January 2012. L.W. was monitored clinically with CK levels. B cell depletion was confirmed with CD20 count of