Kapoor, M;
Reilly, MM;
Manji, H;
Lunn, MP;
Aisling, SC;
(2020)
Dramatic clinical response to ultra-high dose IVIg in otherwise treatment resistant inflammatory neuropathies.
International Journal of Neuroscience
10.1080/00207454.2020.1815733.
(In press).
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Reilly_Dramatic clinical response to ultra-high dose IVIg in otherwise treatment resistant inflammatory neuropathies.High Dose IJN 02082020.pdf - Accepted Version Download (408kB) | Preview |
Abstract
Background Intravenous immunoglobulin (IVIg) has short and long-term efficacy in both chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy with conduction block (MMNCB). There is potential for under and over-treatment if trial regimens are strictly adhered to in clinical practice where titrating dose to clinical response is recommended. Methods We report the response to high-dose IVIg (>2 g/kg/6 weeks) in a subgroup of patients with definite CIDP or MMNCB who were unresponsive to ‘usual’ dosing. IVIg frequency and dosing was determined for each individual by subjective and objective outcome measures for impairment, grip strength, and activity and participation. Results Six patients (three with chronic inflammatory demyelinating polyneuropathy (CIDP), three with MMN) were included. Two patients (one CIDP and one MMNCB) returned to full-time work on fractionated IVIg doses of 5 g/kg/month and 9 g/kg/month. Patient three (CIDP) failed numerous other immunosuppressants but responded to short-term fractionated 4 g/kg/month of IVIg. Patient four has severe, refractory, childhood-onset CIDP, remains stable but dependent currently on 6.9 g/kg/month of IVIg. Patients five and six, both with MMNCB, required short term 4.5–5 g/kg/month to recover significant bilateral hand strength. No IVIg-related adverse events occurred in any individual. Conclusions These six cases demonstrate the safety and effectiveness of a treatment approach that includes individualised but evidence-based clinical assessment and, when necessary, high-doses of IVIg to restore patients’ strength and ability to participate in activities of daily activities. Careful patient selection is important.
| Type: | Article |
|---|---|
| Title: | Dramatic clinical response to ultra-high dose IVIg in otherwise treatment resistant inflammatory neuropathies |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1080/00207454.2020.1815733 |
| Publisher version: | https://doi.org/10.1080/00207454.2020.1815733 |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
| Keywords: | Science & Technology, Life Sciences & Biomedicine, Neurosciences, Neurosciences & Neurology, inflammatory neuropathy, intravenous immunoglobulin, therapeutic dosing, outcome measure, SUBCUTANEOUS IMMUNOGLOBULIN THERAPY, MULTIFOCAL MOTOR NEUROPATHY, DEMYELINATING POLYRADICULONEUROPATHY, INTRAVENOUS IMMUNOGLOBULIN, IGG LEVELS, GLOBULIN, SERUM |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Department of Neuromuscular Diseases |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10113925 |
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