Intractable CIDP treated with cyclosporin - case report

Japanese neurologists reporting their success with cyclosporin for intractable chronic inflammatory demyelinating polyneuropathy (CIDP) suggest a treatment protocol that includes the immunosuppressive agent for maintenance therapy in intractable cases.The report describes a patient with pure motor CIDP who showed no improvement with prednisolone and cyclophosphamide therapy and required repeated plasma exchange and IVIg. Cyclosporin therapy resulted in fewer relapses requiring IVIg. Further therapy resulted in him being relapse-free for 27 months, after which the patient's cyclosporin dose was tapered and stopped. However, hand weakness reappeared a few weeks later, and cyclosporin was restarted along with five days' IVIg treatment. The authors report that there have been no further relapses since restarting cyclosporin, the patient's condition has been stable for 20 months, and he has normal strength in all limbs. Apart from hypertrichosis, there have been no side effects.The authors briefly report their findings in a further 14 patients treated with cyclosporin, including three patients who showed improvement. On the basis of their clinical experience and evidence from controlled trials, they suggest a treatment protocol for CIDP that includes cyclosporin as a consideration in intractable cases."In the treatment of CIDP, a distinction needs to be made between initial and maintenance therapy," the authors point out. They recommend IVIg for initial therapy in eligible patients and plasma exchange in patients not suitable for IVIg. A trial of oral steroids should be offered as maintenance therapy, although additional IVIg courses may be required until maximal effect is achieved. The steroid dose should be slowly reduced over a 12-month period, and it's during this time that the authors suggest cyclosporin for relapsing patients. The authors caution against the use of cyclosporin in patients with severe renal impairment or hypertension, and say that serum levels should be kept between 100 and 150 ng/mL and creatinine concentrations should be closely monitored.Reference...