INDIVIDUAL TEST 620
Autoimmune Nodopathy/CIDP – Screen IgG
Indication
Suspicion of Inflammatory Neuropathy/Autoimmune Nodopathy that is refractory to standard treatment.
Sample material
Serum
- Minim. volume: 0,5 mL
Transport
Within Sweden
- room temperature
International
- cold
Method
Cell-Based Assay (CBA), Indirect immunofluorescence (IIF)
Reference interval
- <1:10 negative
Result
Results are reported as negative or positive. A positive result is followed by a titer.
Interpretation
Autoimmune nodopathies have until recently been regarded as subgroups of CIDP but are distinct in both disease course and treatment. They are now regarded as separate disorders.
The panel includes the analysis of the following auto-antibodies that may be helpful in the investigation of patients with CIDP-like symptoms:
- Neurofascin-155 and Neurofascin-186 antibodies (IgG). Neurofascin-155 (NF155) is a paranodal protein, while Neurofascin-186 (NF186) is a protein present in the nodes of Ranvier. IgG antibodies against NF155 and NF186 have been reported in autoimmune nodopathy. The simultaneous presence of antibodies against NF155 and NF186 is seen in pan-neurofascin nodopathy.
- Contactin-1 antibodies (IgG). Contactin-1 (CNTN1) is a paranodal protein. IgG antibodies against CNTN1 have been reported in autoimmune nodopathy.
- CASPR1 antibodies (IgG). Contactin-associated protein 1 (CASPR1) is a paranodal protein. IgG antibodies against CASPR1 have been reported in autoimmune nodopathy.
References
- Van den Bergh PYK et al. J Peripher Nerv Syst. 2021. 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. PMID: 34085743
- Dong M et al. Clin Neurol Neurosurg. 2022. Characterization of the patients with antibodies against nodal-paranodal junction proteins in chronic inflammatory demyelinating polyneuropathy. PMID: 36401951
- Querol LA et al. Neurotherapeutics. 2022. The Role of the Complement System in Chronic Inflammatory Demyelinating Polyneuropathy: Implications for Complement-Targeted Therapies. PMID: 35378684
- Cortese A et al. Neurol Neuroimmunol Neuroinflamm. 2019. Antibodies to neurofascin, contactin-1, and contactin-associated protein 1 in CIDP: Clinical relevance of IgG isotype. PMID: 31753915
- Delmont E et al. Brain. 2017. Autoantibodies to nodal isoforms of neurofascin in chronic inflammatory demyelinating polyneuropathy. PMID: 28575198
- Appeltshauser L et al. Brain. 2023. Anti-pan-neurofascin antibodies induce subclass-related complement activation and nodo-paranodal damage. PMID: 36346134
Last updated: 2024-09-10
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ENSKILD ANALYS 620
Autoimmun nodopati/CIDP – screen IgG
Indikation
Misstanke om inflammatorisk neuropati/autoimmun nodopati
Provmaterial
Serum
- Minim. volym: 0,5 mL
Transport
Inom Sverige
- rumstemperatur
Internationellt
- kylt
Metod
Cell-based assay (CBA), Indirekt immunofluorescens (IIF)
Referensintervall
- <1:10 negativt
Resultat
Resultat anges som negativt eller positivt. Positivt resultat titreras.
Tolkning
Autoimmun nodopatier har tidigare ansetts vara undergrupper till CIDP men både förlopp och behandling särskiljer sig och autoimmuna nodopatier definieras numera som distinkta diagnoser.
Panelen inkluderar analys av följande autoantikroppar som kan vara till hjälp vid utredning av patienter med CIDP-liknande symtom:
- Neurofascin-155 och Neurofascin 186 antikroppar (IgG). Neurofascin-155 (NF155) är ett paranodalt protein medan Neurofascin-186 (NF186) är ett protein i Ranviers nod. IgG antikroppar mot NF155 och NF186 har beskrivits vid autoimmun nodopati. Samtidig förekomst av antikroppar mot NF155 och NF186 ses vid pan-neurofascin nodopati.
- Contactin-1 antikroppar (IgG). Contactin-1 (CNTN1) är ett paranodalt protein. IgG antikroppar mot CNTN1 har beskrivits vid autoimmun nodopati.
- CASPR1 antikroppar (IgG). Contactin-associated-protein 1 (CASPR1) är ett paranodalt protein. IgG antikroppar mot CASPR1 har beskrivits vid autoimmun nodopati.
Referenser
- Van den Bergh PYK et al. J Peripher Nerv Syst. 2021. 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. PMID: 34085743
- Dong M et al. Clin Neurol Neurosurg. 2022. Characterization of the patients with antibodies against nodal-paranodal junction proteins in chronic inflammatory demyelinating polyneuropathy. PMID: 36401951
- Querol LA et al. Neurotherapeutics. 2022. The Role of the Complement System in Chronic Inflammatory Demyelinating Polyneuropathy: Implications for Complement-Targeted Therapies. PMID: 35378684
- Cortese A et al. Neurol Neuroimmunol Neuroinflamm. 2019. Antibodies to neurofascin, contactin-1, and contactin-associated protein 1 in CIDP: Clinical relevance of IgG isotype. PMID: 31753915
- Delmont E et al. Brain. 2017. Autoantibodies to nodal isoforms of neurofascin in chronic inflammatory demyelinating polyneuropathy. PMID: 28575198
- Appeltshauser L et al. Brain. 2023. Anti-pan-neurofascin antibodies induce subclass-related complement activation and nodo-paranodal damage. PMID: 36346134
Senast uppdaterat: 2024-09-10