Jan. 19, 2011 -- Updated guidelines from the American Academy of Neurology recommend plasma exchange (plasmapheresis) for the treatment of certain types of multiple sclerosis (MS) and other inflammatory neurological conditions, including Guillain-Barré syndrome and CIDP (chronic inflammatory demyelinating polyneuropathy).
Plasma exchange involves replacing the plasma (the liquid part of a person’s blood) with plasma from a donor. Plasma exchange removes factors in the blood’s plasma thought to play a role in these disorders.
The time-consuming therapy involves separating the plasma from other blood components such as blood cells, replacing the plasma, and returning the other components. Risks may include infection, allergic reaction, and blood-clotting issues.
The new guidelines, which appear in the Jan. 18 issue of Neurology, were last updated in 1996. While many of the core recommendations remain the same, the new guidelines state that there is insufficient evidence to support or refute the use of plasma exchange in the treatment of myasthenia gravis, a chronic condition that causes muscles to weaken easily, and pediatric autoimmune neuropsychiatric disorders that result from streptococcal infection.
“This is really an established therapy and we know more about which diseases it is useful in, and which diseases it isn’t useful or needs to be looked at again,” says guidelines author Alex Rae-Grant, MD, a neurologist at The Neurological Institute at Cleveland Clinic in Ohio.
For example, “we are not saying you can’t use it in myasthenia gravis, but it’s ripe for reassessment and we need to think about whether we could do a trial in more rigorous fashion,” he says. Plasma exchange is currently used in many medical centers to treat myasthenia gravis and myasthenic crisis, which occurs when myasthenia gravis affects the breathing muscles and the disease hits a crisis point.
Plasma Exchange in MS
“In acute, sudden severe MS relapses that don’t respond to steroids, we would recommend plasmapheresis, but it is not helpful for chronic secondary progressive MS,” Grant says.
There are several different types of MS. The most common is the relapsing-remitting form of MS. This type of MS is characterized by exacerbations or relapses that are followed by a complete or partial remission. By contrast, secondary progressive MS is marked by a progressive worsening of symptoms with or without relapses. The new guidelines suggest plasmapheresis can be useful in the short-term treatment of CIDP, but longer trials may help carve out a role for this therapy for a longer duration, he says. CIDP is a neurological disorder characterized by progressive weakness and impaired sensory function in the limbs. It occurs as a result to damage to the myelin sheath that protects the peripheral nerves.
Guillain-Barré syndrome occurs when your body's immune system attacks peripheral nerves. Plasma exchange may be beneficial in severe forms of this disease and is a consideration in milder forms, the new guidelines state.
Grant says the new guidelines could aid in reimbursement practices for the areas that plasma exchange is now recommended.
Slow and Costly
“Plasmapheresis is effective in very specific circumstances when you have acute disorders, but it is not a treatment for chronic diseases,” says Nancy L. Sicotte, MD, a neurologist and the multiple sclerosis program director at the Cedars-Sinai Medical Center in Los Angeles.
There may be a bigger role for plasmapheresis for a subset of people with MS, she says. “Some MS patients seem to respond very well and there are others that don’t and there is some evidence that the ones who may be responding may have particular forms of MS such as neuromyelitis optica.”
As it stands, “we do this when steroids don’t work, but not because another marker says this is likely to help,” Sicotte says.
Plasma exchange is time-consuming to administer and costly, and it can take several days to know if it was effective, so it would be helpful to know if someone was likely to benefit before initiating the therapy, she says.