Journal of clinical apheresis
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Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system that is probably due to an autoimmune mechanism with an acute presentation and a monophasic course. The management of patients with ADEM is based on supportive therapy, corticosteroids, and intravenous immunoglobulin, and in selected cases, with therapeutic plasma exchange (TPE). The aim of our study is to evaluate the efficacy of TPE, as adjuvant therapy in pediatric patients with ADEM. ⋯ The treatment can be complemented with intravenous immunoglobulin and plasmapheresis. Available data suggests that plasma exchange is beneficial in children with ADEM who fail these treatments. The good tolerance of the procedure without adverse reactions and the progressive neurological improvement detected in the reviewed cases suggest that the use of TPE in pediatric patients is a good therapeutic option when performed in an experienced center.
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Case Reports
Therapeutic plasma exchange in antisynthetase syndrome with severe interstitial lung disease.
Antisynthetase syndrome (ASS) is a rare condition characterized by interstitial lung disease (ILD), inflammatory myositis, fever, Raynaud phenomenon, mechanic's hand, and inflammatory polyarthritis in the setting of antibodies to amino acyl-transfer RNA synthetases, with anti-Jo-1 antibody being the most common. Prognosis is very poor especially when there is associated ILD. To date, there is no standardized treatment for ILD associated ASS. ⋯ She underwent five sessions of TPE. At the end of the 5th session, the anti-Jo-1 antibody levels dropped to 3.6 AI (antibody index) and her creatinine kinase (CK) level from 875 to 399 U L(-1) (Units per liter) with overall improvement in her respiratory status. This case suggests TPE may be a promising treatment option in patients with ILD associated ASS refractory to steroids and other immunosuppressive therapy, particularly those with severe disease.