PLEX TREATMENT TRIAL
A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease. (37) Weinshenker BG, O’Brien PC, Petterson TM, Noseworthy JH, Lucchinetti CF, Dodick DW, Pineda AA, Stevens LN, Rodriguez M. Treatment of acute transverse myelitis and its early complications. Idiopathic transverse myelitis: corticosteroids, plasma exchange, or cyclophosphamide. (35) Greenberg BM, Thomas KP, Krishnan C, Kaplin AI, Calabresi PA, Kerr DA. (34) Beh SC, Greenberg BM, Frohman T, Frohman EM. In: UpToDate, Dash JF (Ed), UpToDate, Waltham, MA, 2021. Consultation with a neuroimmunologist should strongly be considered when recurrence occurs, and immunosuppressive treatments may be recommended. Recurrence of idiopathic TM is rare and warrants a comprehensive evaluation for known causes of recurrent myelitis. While most studies support the use of corticosteroids and/or PLEX in acute demyelinating syndromes, IVIG can be considered in certain circumstances. Like corticosteroids and PLEX, there are no data confirming the value of IVIG in the setting of acute events. Reactions after an infusion can be more serious and include migraine headaches, aseptic meningitis, renal impairment and blood clots.
PLEX TREATMENT SKIN
Potential adverse reactions are uncommon, but usually occur during or immediately after an infusion and include headache, nausea, muscle pain, fever, chills, chest discomfort, skin and anaphylactic reactions. As the name suggests, IVIG is given intravenously. Immunoglobulin comes from pooled blood that is donated from thousands of healthy people. 35 Cyclophosphamide should be administered under the supervision of an experienced oncology team, and caregivers should monitor the patient carefully for hemorrhagic cystitis and cytopenias.Īnother option for treating acute inflammation is intravenous immunoglobulin (IVIG). For those who were classified at a disability level of ASIA A at their nadir, they showed a significant benefit when given combination therapy with steroids, PLEX and IV cyclophosphamide. From the Johns Hopkins TM Center experience, it has been reported that PLEX provided an added benefit to steroids in patients who were not at a disability level of ASIA A and who did not have a history of autoimmune disease. 19 Cyclophosphamide is known to have immunosuppressive properties. If there is continued progression despite intravenous steroid therapy and PLEX, pulse dose intravenous cyclophosphamide (800–1200 mg/m 2) is considered. PLEX has been shown to be effective in adults with TM and other inflammatory disorders of the CNS. 40 PLEX is believed to work in autoimmune CNS diseases through the removal of specific or nonspecific soluble factors likely to mediate, be responsible for, or contribute to inflammatory-mediated organ damage. 19 It is often given as five treatments, each with exchanges of 1.1 to 1.5 plasma volumes, every other day for 10 days. PLEX is often initiated in individuals with motor impairment 36 or who show little clinical improvement after intravenous steroid treatment, 37-39 but may also be initiated at first presentation for those with significant deficits. 19 The decision to offer continued steroids or to add a new treatment is often based on the clinical course and MRI appearance at the end of 5 days of steroids. The standard of care includes intravenous methylprednisolone (30 mg/kg up to 1000 mg daily) or dexamethasone (120 to 200 mg daily for adults) for 3 to 5 days unless there are compelling reasons to avoid this therapy.
Though there is no randomized double-blind placebo-controlled study that supports this approach, evidence from related disorders and clinical experience support this treatment. Corticosteroids have multiple mechanisms of action including anti-inflammatory activity, immunosuppressive properties, and antiproliferative actions. Intravenous steroid treatment is the first line of therapy often used in acute TM. The acute therapies most frequently used to treat an inflammatory attack include: high dose intravenous steroids (methylprednisolone), Plasmapheresis (Plasma Exchange or PLEX), Immunoglobulin Therapy (IVIG), and cyclophosphamide. Treatment in the acute or early stages involves quieting down the immune system as quickly as possible, before damage is done. It is extremely important to begin treatments as soon as possible after a rare neuroimmune diagnosis.
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