Transcript
CIDP heterogeneity
Claudia Sommer, MD
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- CIDP is a chronic immune-mediated disease. It's characterised by various degrees of demyelination and axonal damage and it manifests with proximal and distal muscle weakness, hypo or areflexia, some sensory disturbances. And although it is a rare disease, I think every neurologist sees cases of CIDP. There are a variety of presentations. The easiest to diagnose is probably the typical CIDP with the distal and proximal paresis and the motor, multifocal, sensory, distal, and focal CIDPs, all have a number of differential diagnosis. So it may be more challenging to make a diagnosis here. And as you can see here in the middle, we have the variants of CIDP, together with the typical CIDP, which is the most frequent one. And among the typical ones, there are a few with an acute onset. And if you have a patient that looks like CIDP with an acute onset, it might be an autoimmune nodopathy. So check out the nodal, paranodal antibodies. And this is now no longer part of CIDP in the classification, it's a disorder of its own right. And if you have a very acute onset, it might also be GBS. And on the left side, we look at the haematological comorbidities. So if you find an IgG or IgA in your patient with CIDP, this would still count as CIDP. So actually, it should be inside the circle. But if you find an IgM with anti MAG, again, it's a separate entity because it responds differently to treatment. Patients do have a significant burden with CIDP, and I will just let you look at these sentences that are from patient's histories. And it can be the pain, it can be the lack of muscle strength, it can be the misdiagnosis. So there are many things that are burdensome to CIDP patients, and we are all looking for the cure. We say CIDP is a demyelinating disorder.
Of course, it is. It's in the name, it's in all our definitions. But recent studies suggest that axonal damage occurs early in the disease and it is also predictive of long-term disability and may indicate a more aggressive disease cause. And even total disability is largely determined by the extent of axonal damage. So this implies that the destruction of the axonal integrity is an intrinsic part of CIDP pathogenesis. And although it may vary, it would be very good if we could prevent this axonal damage.
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