How does ivig help cidp
This usually occurs before the end of one year of treatment, or when the doctor tries to renew the prescription. A dose adjustment is actually good advice, especially when the same dose of IVIG is being given month after month and there is still no sign of relapse to suggest the disease is still active.
Looking for all the information possible to alternative therapies to CIDP. Treatments other than IVIg. Notify me when new comments are added. Before submitting your comment, please review the IG Living privacy policy.
Advertise Subscribe to IGL. IG Living Blog share. Posted on 4. Oaklander, A. Treatments for chronic inflammatory demyelinating polyradiculoneuropathy CIDP : an overview of systematic reviews. Cochrane Database Syst Rev. Hughes, R. Corticosteroids for chronic inflammatory demyelinating polyradiculoneuropathy.
Merkies, I. Download references. Gentile, A. Mazzeo, M. Russo, I. Arimatea, G. You can also search for this author in PubMed Google Scholar. Correspondence to L. Reprints and Permissions. Gentile, L. Long-term treatment with subcutaneous immunoglobulin in patients with chronic inflammatory demyelinating polyradiculoneuropathy: a follow-up period up to 7 years. Sci Rep 10, Download citation. Received : 10 February Accepted : 13 April Published : 13 May Anyone you share the following link with will be able to read this content:.
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Download PDF. Subjects Neuroscience Peripheral nervous system. Abstract Chronic Inflammatory Demyelinating Polyneuropathy CIDP is a rare and heterogeneous acquired sensory-motor polyneuropathy with autoimmune pathogenesis.
Introduction Chronic Inflammatory Demyelinating Polyneuropathy CIDP is a rare and heterogeneous acquired sensory-motor polyneuropathy with autoimmune pathogenesis. Full size table. Methods Before starting SCIg treatment at home, the patients were trained in the hospital with nurse assistance. Clinical follow-up included: 1. Discussion CIDP is a rare chronic disease, highly disabling.
References 1. Article Google Scholar 3. Article Google Scholar 4. Article Google Scholar 5. Article Google Scholar 7.
Article Google Scholar 8. Article Google Scholar Author information Author notes These authors contributed equally: L. Gentile and A. Toscano Authors L. Gentile View author publications. View author publications. Therapeutic Ig degradation in extracellular tissue and binding to extracellular matrix may be the cause of lower bioavailability for SCIg potentially resulting in the need for a higher dose than the equivalent IVIg 4.
However, several studies have observed a positive response when adopting an initial dose conversion from IVIg to SCIg 7 , 49 , The PATH study used doses above 0. The importance of maintaining higher trough levels via SCIg vs. One hypothesis is that stable trough levels are important for long-term therapy and better control of systemic AEs, whereas the initial high Ig peak delivered by IVIg may be required to induce improvement rapidly and establish clinical stability 5.
However, the exact mechanism and dose response relationship requires better understanding. Of the relapses on 0. Further data is needed to explore this approach. Many factors should be considered when determining next steps following relapse, such as the severity of the relapse and patient preference.
Physicians should consider reducing a patient's dose after a period of clinical stability. Dose adjustments should be based on a combination of neurological examination, patient-reported symptoms and clinical response. Achieving the lowest effective maintenance dose can be via increasing the interval between infusions or reducing the dose 2.
However, dose-response studies are limited and report mixed results 4 , 19 , During therapy optimization, clinicians should monitor for wear-off effects and be prepared to revert to the prior regimen if necessary 52 — Lunn et al. Ig withdrawal attempts can be made on an annual basis in stable patients The authors suggest that lengthening the interval between Ig doses results in unstable Ig concentrations whereas adjusting the dose can achieve more consistent Ig trough levels while quickly determining the minimal dose required Smaller intervals between IVIg infusions could help avoid wear-off effects and fluctuating symptoms for some patients.
Although, if weekly infusions are required, patients may opt for SCIg as a less invasive and more convenient route of administration.
The weekly SCIg dose can be adjusted gradually to assess Ig dependency. SCIg can be useful for minor responsive dosing adjustments to maximize the dose-response relationship There is a balance between determining Ig dependency, by allowing the patient to deteriorate, and avoiding recurrent relapses which may result in cumulative axonal loss and progressive disability 54 , A number of studies are currently investigating different dosing strategies in CIDP.
The recently published Gripper study investigated the relationship between Ig levels and clinical response The DRIP study is aiming to determine if more frequent, but lower IVIg doses, leads to more stable Ig concentrations with higher trough levels and clinical efficacy These may offer insights into Ig dosing strategies; however, a limitation is the differences in the underlying mechanisms of different disease states.
Fokkink et al. PK parameters remained constant between infusions in the same patient, but varied considerably between patients Ig concentration after 1 week correlated with grip strength A correlation was also observed in a recent case series between Ig concentration and clinical condition in four CIDP patients on high-dose IVIg treatment, where a decrease in Ig concentration led to symptom fluctuations The 8th patient had low Ig trough levels and returned to IVIg In contrast, a review of obesity and Ig concluded that the impact of weight on dosing was not clinically important and should not play a factor in dosing decisions Broyles et al.
In the PATH extension, changes in Ig trough levels were generally maintained with only a slight decline observed in the 0. Ig trough levels fell noticeably in patients who had relapsed on 0. These findings were supported by significant differences in muscle strength in the SCIg group compared with placebo However, no relationship was observed between Ig concentration and muscle strength The authors concluded Ig level was not a reliable prognostic tool, but group-level data may be confounded by high inter-patient variability in PK and differing infusion intervals Fluctuations in muscle and motor performance measures were unchanged over time in the SCIg group compared with significant fluctuations within the IVIg group The extent of how PK differences contribute to treatment response requires further study, but current findings support SCIg as an advantageous approach to maintaining Ig concentration 44 , The transition is important as patients' serum Ig concentrations will change from largely variable peaks and troughs to steady-state values, but maintenance of the trough level appears crucial.
SCIg may require closer monitoring post-transition to achieve the optimal dose due to inter-patient differences in catabolic pathways, gradual release of Ig from subcutaneous tissue, and Ig clearance mechanisms There is also the issue of clinical deterioration and relapse, which can be perceived as a risk by patients whose condition has stabilized on IVIg therapy.
Results from a study in MMN show that, despite different PK profiles elicited by IVIg and SCIg, it is possible to avoid clinical deterioration and preserve disability scores by calculating IVIg-equivalent doses for each patient individually and allowing leeway for dose increases What remains unclear is how best to optimize therapy in individual patients.
PK assessments show that inter-patient variability is high. A better understanding of the influence of PK parameters on clinical response could aid the process of tailoring Ig therapy. Measuring trough Ig levels can allow determination of optimal Ig dosing for an individual patient whether based on IV or SC administration. In addition, patient factors are an important driver of whether IVIg or SCIg is more suitable for maintenance therapy.
Weekly SCIg is a viable alternative for some patients resulting in stable Ig levels while reducing systemic AEs, lowering wear-off risk, and eliminating venous access. Discussions between HCPs and patients to arm them with all the information for either administration route should always take place.
All authors reviewed and approved the final manuscript. The authors declare that this study received funding from CSL Behring for editorial assistance. The funder was not involved in the study design, collection, analysis, interpretation of data, the writing of this article or the decision to submit it for publication. JS has received honoraria for consulting on an advisory board for Alnylam Pharmaceuticals.
The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases: EFNS task force on the use of intravenous immunoglobulin in treatment of neurological diseases. Eur J Neurol. Adverse effects of immunoglobulin therapy.
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