Trip Answers

Question answered:12/09/07 Warning! this question is over two years old.

Unfortunately, we cannot answer this question. We searched the TRIP and Medline databases as well as the National Institute of Neurological Disorders and Stroke (NINDS) website but found no information concerning prognosis or life expectancy for patients with CIPD who fail to respond to steroid or immunoglobin therapy.

 

NINDS state in an information sheet on CIPD:

 

“The course of CIDP varies widely among individuals. Some may have a bout of CIDP followed by spontaneous recovery, while others may have many bouts with partial recovery in between relapses. The disease is a treatable cause of acquired neuropathy and initiation of early treatment to prevent loss of nerve axons is recommended. However, some individuals are left with some residual numbness or weakness.” [1]

 

The Guillian Barre Syndrome and CIPD Support Group note in their information leaflet:

 

“Like GBS, CIDP can improve without treatment. However, recovery may be very slow and the illness can either get progressively better or worse, or can follow a relapsing/remitting course. Most patients are given treatment in the forms of plasmapheresis, immunoglobulin or corticosteroids. Other drugs may be used in difficult cases.” [2]

 

The following two Medline abstracts may be of general interest to you (although they do not answer your question):

 

“Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated disease of the peripheral nervous system with an estimated prevalence of 1-2/100,000. The clinical presentation is heterogeneous, but the most common form causes symmetrical progressive or relapsing weakness affecting proximal and distal muscles. CIDP is among the most treatable peripheral nerve disorders and corticosteroids, plasmapheresis and intravenous immunoglobulin have been shown to be effective in short-term prospective, randomized controlled trials. Data however indicate that approximately one-third of patients do not respond to these treatment modalities, nor do they provide equivalent evidence for a durable clinical response. There is a lack of good quality controlled trials of any other immunosuppressive agent, but cyclophosphamide and cyclosporin may be of value in patients with poor response to first-line modalities. Alternatively, the use of combination therapy may increase the efficacy in unresponsive patients. This review highlights the current status of CIDP treatment trials and discusses the significance of any therapeutic option in terms of efficacy, tolerability and cost-effects.” [3]

 

A second article, one by Kuntzer also discusses treatment options for CIPD:

 

“Limits of treatment in chronic inflammatory demyelinating poly(radiculo)neuropathies (CIDP) patients are better known thanks to recent Cochrane reviews. (1) Randomized controlled trials have only focused on short-term effects, but most patients need long-term therapy, (2) There are three proven effective treatments available (prednisone; intravenous immunoglobulin or IVIg and plasma exchange or PE) which are useful in more than 60 p. 100 of patients, (3) New open studies indicated possible efficacy for mycophenolate, rituximab, etanercept, ciclosporine and interferons, and (4) Whether CIDP variants need specific treatment is still unknown. Many CIDP patients need treatment for years. The fear of side effects during long-term steroid treatment, the high costs of IVIg, the necessity for specialized equipment and the invasive nature of PE, are important factors determining the choice for one of these treatments.

 

In most up-to-date treatment options, patients are initially treated with IVIg at a dosage of 2 g/kg administered for 25 days, clinical improvement can be judged within 10 days. The percentage of patients responding seems to be approximately 70 percent, with a very high chance (approximately 85 percent) that repeated administration of IVIg will be necessary, explaining why most neurologists add an immunosuppressive drug at this stage, but there is no consensus concerning the best drug to be used. Combinations of drugs are most likely to be useful in the next future, using IVIg, prednisone, and a immunosuppressor agent, such as mycophenolate, rituximab, etanercept, or ciclosporine. General measures to rehabilitate patients and to manage symptoms like fatigue and other residual findings are important.” [4]

 

Given the heterogeneity of CIPD and lack of information on prognosis in non-responders to steroid and immunoglobin therapies, the NLH Q & A Service can only suggest discussing this case with a neurologist and/or contacting the Guillain-Barre Syndrome Support group for further assistance:

 

“Can I talk to someone about GBS or CIDP now?
Yes. Call the GBS/CIDP Helpline on 0800 374 803.”

 

References
1. NIND.  Chronic inflammatory demyelinating polyneuropathy. July 2007. (http://www.ninds.nih.gov/disorders/cidp/cidp.htm)
2. Lünemann JD, Prass K and Zschenderlein R.  [Immunotherapy of chronic inflammatory demyelinating polyneuropathy]. Fortschr Neurol Psychiatr. 2004 Dec;72(12):672-8. (http://www.hubmed.org/display.cgi?uids=15580532)
3. Kuntzer T [Treatment options for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)]. Rev Neurol (Paris). 2006 Apr;162(4):539-43. (http://www.hubmed.org/display.cgi?uids=16585918)
4. Guillain-Barre Syndrome Support Group. (http://www.gbs.org.uk/professionals.shtml)

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