Prognosis

The majority of patients respond to therapy with improvement in strength, sensation, and gait. About 75% to 80% of patients will initially respond to intravenous immunoglobulin (IVIG), corticosteroids, or plasma exchange within the first few months of therapy.​[58][59][60][64][133]​ However, response may be incomplete and patients may be left with residual deficit. Distal weakness and paraesthesias commonly do not resolve completely.

Several studies have looked at long-term prognosis and found that most patients continue to do well over 5-10 years, with about 75% of patients having no or only minor symptoms.[4]​​[5][40][70][71][169]​​​​

Approximately one-third of patients will have medication-free remission. About 10% of patients have a poor outcome with either severe disability or death.

Prognostic factors

Several studies have looked at prognostic factors.[4][5][71][169][170]​ Factors associated with a negative prognosis include older age, progressive course, central nervous system involvement, a high number of fibres showing active demyelination, and axonal loss on nerve biopsy. Factors associated with a positive prognosis include younger age, relapsing or subacute presentation, and proximal weakness.

Response to IVIG

A monophasic or relapsing-remitting (not chronic progressive) course and a twofold increase in cerebrospinal fluid protein typically predicts a good response to IVIG.[171] Another study showed that CIDP in patients with diabetes mellitus tends to be more severe, but has a good response to IVIG and fewer relapses than in patients without diabetes mellitus.[172]

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