Evidence-based guideline update: Plasmapheresis in neurologic disorders
Abraham CJStork, MD, Polyneuropathy Expertise Ctr, Dept of Neurology, Rudolf Magnus Inst of Neuroscience, Utrecht, Nethastork@umcutrecht.nl
Nicolette C Notermans, Alexander FJE Vrancken, Leonard H van den Berg and W-Ludo van der Pol
Submitted July 05, 2011
Cortese et al. conclude that there is level A evidence for effectiveness of plasmapheresis for treatment of acute inflammatory demyelinating neuropathy (AIDP), Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuropathy (CIDP), and level B evidence for neuropathy associated with IgA or IgG monoclonal gammopathy of undetermined significance (MGUS). [1]
The conclusion that plasmapheresis might be an effective treatment strategy for the latter group of patients is based on one Class I study. [10] In this study, patients treated with plasmapheresis showed improvement on neuropathy disability and weakness scores compared to sham-treated patients, although these differences were not always statistically significant. Further analysis showed that improvement was greater in plasmapheresis treated patients with IgA or IgG than IgM MGUS. [1]
Lack of benefit of plasmapheresis in the IgM group was later confirmed by others. [11] Dyck et al. considered the differences in efficacy depending on the isotype of the MGUS and polyneuropathy associated with IgM MGUS started to emerge as a nosological entity. [10] Polyneuropathy associated with IgA or IgG MGUS encompasses a variety of clinical phenotypes including: POEMS; demyelinating neuropathies that cannot be distinguished from CIDP; and axonal neuropathies. Although Dyck et al. do not provide detailed results from nerve conduction studies [10], it is likely that those patients who responded to plasmapheresis would now be diagnosed with CIDP.
We identified 76 patients with polyneuropathy associated with IgA/IgG MGUS in our prospective cohort of 216 patients with polyneuropathy and MGUS. [12] Nerve conduction studies from 17 (24%) patients had demyelinating features and 9 fulfilled the AAN criteria for CIDP. These patients cannot be distinguished from patients with CIDP without MGUS, and have been included in recent CIDP trials. [13]
These data do not support Cortese et al.’s conclusion that there is Level B evidence that plasmapheresis is effective for patients with polyneuropathy associated with IgA or IgG MGUS.
References
10. Dyck PJ, Low PA, Windebank AJ, et al. Plasma exchange in polyneuropathy associated with monoclonal gammopathy of undetermined significance. New Engl J Med 1991; 325: 1482-1486.
11. Oksenhendler E, Chevret S, Leger JM, Louboutin JP, Bussel A, Brouet JC. Plasma exchange and chlorambucil in polyneuropathy associated with monoclonal IgM gammopathy. J Neurol Neurosurg Psychiatry 1995;59:243-247.
12. Niermeijer JM, Eurelings M, Franssen H, Wokke JHJ, Notermans NC. Prognosis of polyneuropathy due to IgM monoclonal gammopathy. Neurology 2010;74:406-412.
13. Van Schaik IN, Eftimov F, Van Doorn PA, et al. Pulsed high-dose dexamethasone versus standard prednisolone treatment for chronic inflammatory demyelinating polyradiculoneuropathy (PREDICT study): a double-blind, randomised, controlled trial. Lancet Neurol 2010;9:245-253.
Disclosure: Dr. van den Berg serves on the scientific advisory board of ARISLA (the Italian ALS Association); serves as a consultant for and has received funding for travel from Baxter International Inc.; and receives research support from the Prinses Beatrix Fonds, Netherlands ALS Foundation, VSB Fonds, and Adessium Foundation.
Dr. Van der Pol received travel grants from Baxter and grants from the Prinses Beatrix Fonds, a non-commercial organisation that funds neuromuscular research. Drs. Stork, Notermans and Vrancken report no disclosures.
Cortese et al. conclude that there is level A evidence for effectiveness of plasmapheresis for treatment of acute inflammatory demyelinating neuropathy (AIDP), Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuropathy (CIDP), and level B evidence for neuropathy associated with IgA or IgG monoclonal gammopathy of undetermined significance (MGUS). [1]
The conclusion that plasmapheresis might be an effective treatment strategy for the latter group of patients is based on one Class I study. [10] In this study, patients treated with plasmapheresis showed improvement on neuropathy disability and weakness scores compared to sham-treated patients, although these differences were not always statistically significant. Further analysis showed that improvement was greater in plasmapheresis treated patients with IgA or IgG than IgM MGUS. [1]
Lack of benefit of plasmapheresis in the IgM group was later confirmed by others. [11] Dyck et al. considered the differences in efficacy depending on the isotype of the MGUS and polyneuropathy associated with IgM MGUS started to emerge as a nosological entity. [10] Polyneuropathy associated with IgA or IgG MGUS encompasses a variety of clinical phenotypes including: POEMS; demyelinating neuropathies that cannot be distinguished from CIDP; and axonal neuropathies. Although Dyck et al. do not provide detailed results from nerve conduction studies [10], it is likely that those patients who responded to plasmapheresis would now be diagnosed with CIDP.
We identified 76 patients with polyneuropathy associated with IgA/IgG MGUS in our prospective cohort of 216 patients with polyneuropathy and MGUS. [12] Nerve conduction studies from 17 (24%) patients had demyelinating features and 9 fulfilled the AAN criteria for CIDP. These patients cannot be distinguished from patients with CIDP without MGUS, and have been included in recent CIDP trials. [13]
These data do not support Cortese et al.’s conclusion that there is Level B evidence that plasmapheresis is effective for patients with polyneuropathy associated with IgA or IgG MGUS.
References
10. Dyck PJ, Low PA, Windebank AJ, et al. Plasma exchange in polyneuropathy associated with monoclonal gammopathy of undetermined significance. New Engl J Med 1991; 325: 1482-1486.
11. Oksenhendler E, Chevret S, Leger JM, Louboutin JP, Bussel A, Brouet JC. Plasma exchange and chlorambucil in polyneuropathy associated with monoclonal IgM gammopathy. J Neurol Neurosurg Psychiatry 1995;59:243-247.
12. Niermeijer JM, Eurelings M, Franssen H, Wokke JHJ, Notermans NC. Prognosis of polyneuropathy due to IgM monoclonal gammopathy. Neurology 2010;74:406-412.
13. Van Schaik IN, Eftimov F, Van Doorn PA, et al. Pulsed high-dose dexamethasone versus standard prednisolone treatment for chronic inflammatory demyelinating polyradiculoneuropathy (PREDICT study): a double-blind, randomised, controlled trial. Lancet Neurol 2010;9:245-253.
Disclosure: Dr. van den Berg serves on the scientific advisory board of ARISLA (the Italian ALS Association); serves as a consultant for and has received funding for travel from Baxter International Inc.; and receives research support from the Prinses Beatrix Fonds, Netherlands ALS Foundation, VSB Fonds, and Adessium Foundation. Dr. Van der Pol received travel grants from Baxter and grants from the Prinses Beatrix Fonds, a non-commercial organisation that funds neuromuscular research. Drs. Stork, Notermans and Vrancken report no disclosures.