Chronic inflammatory demyelinating polyradiculoneuropathy: long-term course and treatment of 60 patients.
The objective of this study was to assess the long-term course and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We evaluated, according to a predefined protocol, a series of 60 CIDP patients who received a long-term course of steroids and immunosuppressants. Eighteen of them also had monoclonal gammopathy of undetermined significance (MGUS). Mean follow-up was 4.4 years and was similar for CIDP and CIDP-MGUS patients. At the end of the follow-up, improvement was ascertained in 60% of patients (69% CIDP, 39% CIDP-MGUS). Complete remission was achieved in 13%. Out of 26 patients receiving steroids as a monotherapy, 19 improved (73%). The following variables were predictive of a better outcome: female gender, younger age at onset, relapsing-remitting course, and absence of axonal damage at neurophysiologic study. In the multivariate analysis, younger age at onset and demyelination without axonal damage still retained an independent positive value.
National Neurological Institute C. Besta, Milano, Italy.
A
A
Sghirlanzoni
A
A
Solari
C
C
Ciano
C
C
Mariotti
E
E
Fallica
D
D
Pareyson
D000293:Adolescent; D000328:Adult; D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D001379:Azathioprine / Q000627:therapeutic use*; D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004594:Electrophysiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D011241:Prednisone / Q000627:therapeutic use*; D011379:Prognosis; D012074:Remission Induction
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D001379:Azathioprine; D011241:Prednisone