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.
Sghirlanzoni
A
A
National Neurological Institute C. Besta, Milano, Italy.
Solari
A
A
Ciano
C
C
Mariotti
C
C
Fallica
E
E
Pareyson
D
D
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