A maioria dos pacientes responde à terapia com uma melhora da força, sensibilidade e marcha. Cerca de 75% a 80% dos pacientes responderão inicialmente à imunoglobulina intravenosa (IGIV), corticosteroides ou plasmaférese nos primeiros meses de terapia.[58]Bus SRM, Broers MC, Lucke IM, et al. Clinical outcome of CIDP one year after start of treatment: a prospective cohort study. J Neurol. 2022 Feb;269(2):945-55.
https://link.springer.com/article/10.1007/s00415-021-10677-5
http://www.ncbi.nlm.nih.gov/pubmed/34173873?tool=bestpractice.com
[59]Merkies ISJ, van Schaik IN, Léger JM, et al; PRIMA Trial Investigators and the PATH Study Group. Efficacy and safety of IVIG in CIDP: combined data of the PRIMA and PATH studies. J Peripher Nerv Syst. 2019 Mar;24(1):48-55.
https://onlinelibrary.wiley.com/doi/10.1111/jns.12302
http://www.ncbi.nlm.nih.gov/pubmed/30672091?tool=bestpractice.com
[60]van Lieverloo GGA, Peric S, Doneddu PE, et al. Corticosteroids in chronic inflammatory demyelinating polyneuropathy: a retrospective, multicentre study, comparing efficacy and safety of daily prednisolone, pulsed dexamethasone, and pulsed intravenous methylprednisolone. J Neurol. 2018 Sep;265(9):2052-9.
https://link.springer.com/article/10.1007/s00415-018-8948-y
http://www.ncbi.nlm.nih.gov/pubmed/29968199?tool=bestpractice.com
[64]Godil J, Barrett MJ, Ensrud E, et al. Refractory CIDP: clinical characteristics, antibodies and response to alternative treatment. J Neurol Sci. 2020 Nov 15;418:117098.
http://www.ncbi.nlm.nih.gov/pubmed/32841917?tool=bestpractice.com
[133]Mehndiratta, MM, Hughes, RA, Pritchard, J. Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev. 2015 Aug 25;(8):CD003906.
https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD003906.pub4/full
http://www.ncbi.nlm.nih.gov/pubmed/26305459?tool=bestpractice.com
No entanto, a resposta pode ser incompleta, e os pacientes podem ficar com deficits residuais. Fraqueza distal e parestesias normalmente não se resolvem por completo.
Vários estudos analisaram o prognóstico em longo prazo e revelaram que a maioria dos pacientes continua bem ao longo de 5 a 10 anos, com cerca de 75% dos pacientes sem sintomas ou apenas sintomas menores.[4]McCombe PA, Pollard JD, McLeod JG. Chronic inflammatory demyelinating polyradiculoneuropathy: clinical and electrophysiological study of 92 cases. Brain. 1987 Dec;110 (Pt 6):1617-30.
http://www.ncbi.nlm.nih.gov/pubmed/3427403?tool=bestpractice.com
[5]Hattori N, Misu K, Koike H, et al. Age of onset influences clinical features of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci. 2001 Feb 15;184(1):57-63.
http://www.ncbi.nlm.nih.gov/pubmed/11231033?tool=bestpractice.com
[40]Barohn RJ, Kissel JT, Warmolts JR, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: clinical characteristics, course, and recommendations for diagnostic criteria. Arch Neurol. 1989 Aug;46(8):878-84.
http://www.ncbi.nlm.nih.gov/pubmed/2757528?tool=bestpractice.com
[70]Gorson KC, Allam G, Ropper AH. Chronic inflammatory demyelinating polyneuropathy: clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy. Neurology. 1997 Feb;48(2):321-8.
http://www.ncbi.nlm.nih.gov/pubmed/9040714?tool=bestpractice.com
[71]Bouchard C, Lacroix C, Plante V, et al. Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy. Neurology. 1999 Feb;52(3):498-503.
http://www.ncbi.nlm.nih.gov/pubmed/10025777?tool=bestpractice.com
[169]Simmons Z, Albers JW, Bromberg MB, et al. Long-term follow-up of patients with chronic inflammatory demyelinating polyradiculoneuropathy, without and with monoclonal gammopathy. Brain. 1995 Apr;118 (Pt 2):359-68.
http://www.ncbi.nlm.nih.gov/pubmed/7735878?tool=bestpractice.com
Aproximadamente um terço dos pacientes terá remissão sem medicação. Cerca de 10% dos pacientes têm um desfecho desfavorável com incapacidade grave ou morte.
Fatores de prognóstico
Vários estudos analisaram os fatores de prognóstico.[4]McCombe PA, Pollard JD, McLeod JG. Chronic inflammatory demyelinating polyradiculoneuropathy: clinical and electrophysiological study of 92 cases. Brain. 1987 Dec;110 (Pt 6):1617-30.
http://www.ncbi.nlm.nih.gov/pubmed/3427403?tool=bestpractice.com
[5]Hattori N, Misu K, Koike H, et al. Age of onset influences clinical features of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci. 2001 Feb 15;184(1):57-63.
http://www.ncbi.nlm.nih.gov/pubmed/11231033?tool=bestpractice.com
[71]Bouchard C, Lacroix C, Plante V, et al. Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy. Neurology. 1999 Feb;52(3):498-503.
http://www.ncbi.nlm.nih.gov/pubmed/10025777?tool=bestpractice.com
[169]Simmons Z, Albers JW, Bromberg MB, et al. Long-term follow-up of patients with chronic inflammatory demyelinating polyradiculoneuropathy, without and with monoclonal gammopathy. Brain. 1995 Apr;118 (Pt 2):359-68.
http://www.ncbi.nlm.nih.gov/pubmed/7735878?tool=bestpractice.com
[170]Sghirlanzoni A, Solari A, Ciano C, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: long-term course and treatment of 60 patients. Neurol Sci. 2000 Feb;21(1):31-7.
http://www.ncbi.nlm.nih.gov/pubmed/10938200?tool=bestpractice.com
Fatores associados a um prognóstico negativo incluem idade avançada, evolução progressiva, envolvimento do sistema nervoso central, alto número de fibras mostrando desmielinização ativa e perda axonal na biópsia do nervo. Fatores associados a um prognóstico positivo incluem idade mais jovem, apresentação recidivada ou subaguda e fraqueza proximal.
Resposta à IGIV
Uma evolução monofásica ou recidivante/remitente (não progressiva crônica) e um aumento de duas vezes no nível de proteínas no líquido cefalorraquidiano (LCR) normalmente indicam uma boa resposta à IGIV.[171]Tackenberg B, Lünemann JD, Steinbrecher A, et al. Classifications and treatment responses in chronic immune-mediated demyelinating polyneuropathy. Neurology. 2007 May 8;68(19):1622-9.
http://www.ncbi.nlm.nih.gov/pubmed/17485651?tool=bestpractice.com
Outro estudo mostrou que a PDIC em pacientes com diabetes mellitus tende a ser mais grave, mas tem boa resposta a IGIV e menos recidivas do que em pacientes sem diabetes mellitus.[172]Pedersen K, Pandolfo M, Mavroudakis N. Chronic inflammatory demyelinating polyneuropathy in a diabetic patient: deterioration after intravenous immunoglobulins treatment and favorable response to steroid treatment. Acta Neurol Belg. 2007 Mar;107(1):14-7.
http://www.ncbi.nlm.nih.gov/pubmed/17569228?tool=bestpractice.com