Chronische inflammatorische demyelinisierende Polyradikuloneuropathie (CIDP)

Die chronische inflammatorische demyelinisierende Polyradikuloneuropathie (CIDP) ist die häufigste chronische Autoimmunerkrankung des peripheren Nervensystems. Sie ist häufig schwierig zu diagnostizieren. Die CIDP ist mittlerweile mittels verschiedener Immuntherapeutika gut behandelbar. In diesem Beitrag wird die Diagnose und Therapie der CIDP anhand eines typischen Fallbeispiels erläutert.

Kernaussagen

• Die chronische inflammatorische demyelinisierende Polyneuropathie (CIDP) ist die häufigste chronische Immunneuropathie.

• Wichtige Differenzialdiagnosen der CIDP umfassen andere entzündliche Polyneuropathien wie das Guillain-Barré-Syndrom, Anti-MAG-IgM-Neuropathie, das CANOMAD-Syndrom, oder andere metabolische Neuropathien wie zum Beispiel die diabetische Polyneuropathie oder die ATTRv

• Die Elektroneurografie ist die wichtigste Zusatzdiagnostik für die Diagnose einer CIDP, andere wertvolle Untersuchungsmethoden sind

  • Nervenultraschall,

  • Liquoruntersuchung,

  • Nervenbiopsie.

• Autoantikörper spielen in der Diagnostik der CIDP nach wie vor eine untergeordnete Rolle. Bei etwa jedem 10. Patienten mit der Verdachtsdiagnose einer CIDP findet man Antikörper gegen Proteine, die im Bereich des Ranvier’schen Schnürrings exprimiert werden.

• Therapie der 1. Wahl sind Steroide, Immunglobuline und Plasmapherese. Darüber hinaus können auch andere Immunsuppressiva und monoklonale Antikörper (Off-Label Use) eingesetzt werden.

Schlüsselwörter

Autoimmunerkrankung - peripheres Nervensystem - Immuntherapie - polyneuropathische Symptome

Publication History

Article published online:
08 December 2023

• Literatur

• 1 Lehmann HC, Burke D, Kuwabara S. Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment. J Neurol Neurosurg Psychiatry 2019; 90: 981-987 DOI: 10.1136/jnnp-2019-320314. (PMID: 30992333)
• 2 Sommer C, Koch S, Lammens M. et al. Macrophage clustering as a diagnostic marker in sural nerve biopsies of patients with CIDP. Neurology 2005; 65: 1924-1929 DOI: 10.1212/01.wnl.0000188879.19900.
• 3 Vural A, Doppler K, Meinl E. Autoantibodies against the node of ranvier in seropositive chronic inflammatory demyelinating polyneuropathy: diagnostic, pathogenic, and therapeutic relevance. Front Immunol 2018; 9: 1029 DOI: 10.3389/fimmu.2018.01029. (PMID: 29867996)
• 4 Bäumer D, Grant DJ, Knight R. et al. CIDP presenting as recurrent severe back pain without weakness or sensory loss. Pract Neurol 2016; 16: 488-492 DOI: 10.1136/practneurol-2016-001397. (PMID: 27313227)
• 5 Di Guglielmo G, Di Muzio A, Torrieri F. et al. Low back pain due to hypertrophic roots as presenting symptom of CIDP. Ital J Neurol Sci 1997; 18: 297-299
• 6 Mahdi-Rogers M, Hughes RC. Epidemiology of chronic inflammatory neuropathies in southeast England. Eur J Neurol 2014; 21: 28-33 DOI: 10.1111/ene.12190. (PMID: 23679015)
• 7 Lefter S, Hardiman O, Ryan AM. A population-based epidemiologic study of adult neuromuscular disease in the Republic of Ireland. Neurology 2017; 88: 304-313 DOI: 10.1212/WNL.0000000000003504. (PMID: 27927941)
• 8 Mengel D, Fraune L, Sommer N. et al. Costs of illness in chronic inflammatory demyelinating polyneuropathy in Germany. Muscle Nerve 2018; 58: 681-687 DOI: 10.1002/mus.26315. (PMID: 30073683)
• 9 Guptill JT, Bromberg MB, Zhu L. et al. Patient demographics and health plan paid costs in chronic inflammatory demyelinating polyneuropathy. Muscle Nerve 2014; 50: 47-51 DOI: 10.1002/mus.24109. (PMID: 24639235)
• 10 Kaplan A, Brannagan TH. Evaluation of patients with refractory chronic inflammatory demyelinating polyneuropathy. Muscle Nerve 2017; 55: 476-482 DOI: 10.1002/mus.25271. (PMID: 27463215)
• 11 Allen JA, Lewis RA. CIDP diagnostic pitfalls and perception of treatment benefit. Neurology 2015; 85: 498-504 DOI: 10.1212/WNL.0000000000001833. (PMID: 26180143)
• 12 Allen JA, Ney J, Lewis RA. Electrodiagnostic errors contribute to chronic inflammatory demyelinating polyneuropathy misdiagnosis. Muscle Nerve 2018; 57: 542-549 DOI: 10.1002/mus.25997. (PMID: 29053880)
• 13 Van den Bergh PYK, Doorn PA van, Hadden RDM. et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint Task Force-Second revision. J Peripher Nerv Syst 2021; 26: 242-268 DOI: 10.1111/jns.12455. (PMID: 34085743)
• 14 Üçeyler N, Necula G, Wagemann E. et al. Endoneurial edema in sural nerve may indicate recent onset inflammatory neuropathy. Muscle Nerve 2016; 53: 705-710 DOI: 10.1002/mus.24930. (PMID: 26439587)
• 15 Grimm A, Axer H, Heiling B. et al. Nerve ultrasound normal values – Readjustment of the ultrasound pattern sum score UPSS. Clin Neurophysiol 2018; 129: 1403-1409 DOI: 10.1016/j.clinph.2018.03.036. (PMID: 29729596)
• 16 Querol L, Nogales-Gadea G, Rojas-Garcia R. et al. Neurofascin IgG4 antibodies in CIDP associate with disabling tremor and poor response to IVIg. Neurology 2014; 82: 879-886
• 17 Doppler K, Appeltshauser L, Wilhelmi K. et al. Destruction of paranodal architecture in inflammatory neuropathy with anti-contactin-1 autoantibodies. J Neurol Neurosurg Psychiatry 2015; 86: 720-728
• 18 Schaik IN van, Eftimov F, Doorn PA van. 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 DOI: 10.1016/S1474-4422(10)70021-1. (PMID: 20133204)
• 19 Lehmann HC, Hartung H-P. Plasma exchange and intravenous immunoglobulins: mechanism of action in immune-mediated neuropathies. J Neuroimmunol 2011; 231: 61-69 DOI: 10.1016/j.jneuroim.2010.09.015. (PMID: 21056913)
• 20 Cornblath DR, Doorn PA van, Hartung H-P. et al. Randomized trial of three IVIg doses for treating chronic inflammatory demyelinating polyneuropathy. Brain J Neurol 2022; 145: 887-896 DOI: 10.1093/brain/awab422. (PMID: 35038723)
• 21 Adrichem ME, Lucke IM, Vrancken AFJE. et al. Withdrawal of intravenous immunoglobulin in chronic inflammatory demyelinating polyradiculoneuropathy. Brain J Neurol 2022; 145: 1641-1652
• 22 Yoon MS, Chan A, Gold R. et al. Standard and escalating treatment of chronic inflammatory demyelinating polyradiculoneuropathy. Ther Adv Neurol Disord 2011; 4: 193-200 DOI: 10.1177/1756285611405564.
• 23 Isose S, Mori M, Misawa S. et al. Long-term regular plasmapheresis as a maintenance treatment for chronic inflammatory demyelinating polyneuropathy. J Peripher Nerv Syst 2010; 15: 147-149 DOI: 10.1111/j.1529-8027.2010.00263.x.
• 24 Lehmann HC, Hartung H-P, Hetzel GR. et al. Plasma exchange in neuroimmunological disorders: part 2. Treatment of neuromuscular disorders. Arch Neurol 2006; 63: 1066-1071 DOI: 10.1001/archneur.63.8.1066. (PMID: 16908731)
• 25 Lieker I, Slowinski T, Harms L. et al. A prospective study comparing tryptophan immunoadsorption with therapeutic plasma exchange for the treatment of chronic inflammatory demyelinating polyneuropathy. J Clin Apheresis 2017; 32: 486-493
• 26 Davies AJ, Fehmi J, Senel M. et al. Immunoadsorption and plasma exchange in seropositive and seronegative immune-mediated neuropathies. J Clin Med 2020; 9: 2025 DOI: 10.3390/jcm9072025. (PMID: 32605107)
• 27 Liu L, Chen J, Zhang Y. et al. Case report: Immunoadsorption therapy for anti-caspr1 antibody-associated nodopathy. Front Immunol 2022; 13: 986018 DOI: 10.3389/fimmu.2022.986018. (PMID: 36211443)
• 28 Good JL, Chehrenama M, Mayer RF. et al. Pulse cyclophosphamide therapy in chronic inflammatory demyelinating polyneuropathy. Neurology 1998; 51: 1735-1738
• 29 Querol L, Rojas-García R, Diaz-Manera J. et al. Rituximab in treatment-resistant CIDP with antibodies against paranodal proteins. Neurol Neuroimmunol Neuroinflammation 2015; 2: e149
• 30 Härtig F, Ross M, Dammeier NM. et al. Nerve ultrasound predicts treatment response in chronic inflammatory demyelinating polyradiculoneuropathy – a prospective follow-up. Neurotherapeutics 2018; 15: 439-451 DOI: 10.1007/s13311-018-0609-4. (PMID: 29435815)
• 31 Balke M, Wunderlich G, Brunn A. et al. Chronische inflammatorische demyelinisierende Polyneuropathie. Fortschr Neurol Psychiatr 2016; 84: 756-769