PMID- 30027593 OWN - NLM STAT- MEDLINE DCOM- 20191022 LR - 20191022 IS - 1529-8027 (Electronic) IS - 1085-9489 (Linking) VI - 23 IP - 3 DP - 2018 Sep TI - Conventional and unconventional therapies in typical and atypical chronic inflammatory demyelinating polyneuropathy with different clinical course of progression. PG - 183-189 LID - 10.1111/jns.12282 [doi] AB - Intravenous immunoglobulin (IVIG), corticosteroids and therapeutic plasma exchange (TPE) are evidence-based conventional treatments for chronic inflammatory demyelinating polyneuropathy (CIDP). In many centres, unconventional treatments are frequently used as alternatives. We evaluated the outcome of conventional and unconventional therapies in 31 CIDP patients. Overall response rate with conventional first-line immunotherapies was 77% (20/26), comparable between IVIG and corticosteroids (80% vs 70%). Use of TPE was limited. Treatment response among typical and atypical CIDP were comparable (76 vs 80%). Non-responders were patients with progressive form of typical CIDP and DADS. Majority (21/26, 81%) of patients with persistent neurological deficits received maintenance therapy. Two subgroups of patients frequently treated with maintenance immunosuppressants were those with improving or stable disease following first-line treatment (12, 57%) and those with progressive form of CIDP (2, 10%). Primary indications for immunosuppressant use were corticosteroids-sparing and additional immunosuppression effects. Nine (64%) patients with improving or stable disease given azathioprine were taken off corticosteroids after a median duration of 14 months (range 12-108). Two (14%) eventually achieved cure or clinical remission without treatment. Maintenance IVIg was given to 6 (29%) relapsing CIDP patients; none of achieved cure or remission after similar median duration of treatment. Less potent immunosuppressant drugs (azathioprine, mycophenolate mofetil, and methotrexate) were frequently used, with moderate adverse effect profiles. In resource limited setting, unconventional treatments were commonly used among CIDP patients with different clinical course of progression. In most cases, careful risk-benefit re-assessment is required to justify its further use. CI - (c) 2018 Peripheral Nerve Society. FAU - Hung, Stefanie K Y AU - Hung SKY AD - Department of Neurology, Kuala Lumpur Hospital, Kuala Lumpur, Malaysia. FAU - Hiew, Fu L AU - Hiew FL AD - Department of Neurology, Kuala Lumpur Hospital, Kuala Lumpur, Malaysia. FAU - Viswanathan, Shanthi AU - Viswanathan S AD - Department of Neurology, Kuala Lumpur Hospital, Kuala Lumpur, Malaysia. FAU - Puvanarajah, Santhi AU - Puvanarajah S AD - Department of Neurology, Kuala Lumpur Hospital, Kuala Lumpur, Malaysia. LA - eng PT - Journal Article DEP - 20180814 PL - United States TA - J Peripher Nerv Syst JT - Journal of the peripheral nervous system : JPNS JID - 9704532 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Immunoglobulins, Intravenous) RN - 0 (Immunosuppressive Agents) SB - IM MH - Adrenal Cortex Hormones/*therapeutic use MH - Adult MH - Aged MH - Female MH - Humans MH - Immunoglobulins, Intravenous/*therapeutic use MH - Immunosuppressive Agents/*therapeutic use MH - Immunotherapy/*methods MH - Male MH - Middle Aged MH - Plasma Exchange/methods MH - Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/*therapy MH - Retrospective Studies MH - Treatment Outcome OTO - NOTNLM OT - chronic inflammatory demyelinating polyneuropathy OT - conventional treatment OT - corticosteroids OT - intravenous immunoglobulin OT - plasma exchange OT - unconventional treatment EDAT- 2018/07/22 06:00 MHDA- 2019/10/23 06:00 CRDT- 2018/07/21 06:00 PHST- 2018/05/08 00:00 [received] PHST- 2018/07/14 00:00 [revised] PHST- 2018/07/16 00:00 [accepted] PHST- 2018/07/22 06:00 [pubmed] PHST- 2019/10/23 06:00 [medline] PHST- 2018/07/21 06:00 [entrez] AID - 10.1111/jns.12282 [doi] PST - ppublish SO - J Peripher Nerv Syst. 2018 Sep;23(3):183-189. doi: 10.1111/jns.12282. Epub 2018 Aug 14.