{
  "abstract": "Objectives Peripheral neurological manifestations of SLE are often under-characterised. Autoimmune nodopathy (AN), a nodal/paranodal antibody–mediated neuropathy within the chronic inflammatory demyelinating polyneuropathy (CIDP)-spectrum is characterised by IgG to neurofascins (NF155/NF186), contactin-1 or Caspr1 and often responds poorly to IVIg but well to B-cell depletion. To our knowledge, AN has not previously been reported in SLE. Herein we describe two SLE cases with anti-neurofascin–positive AN and highlight robust responses to B-cell–depleting therapy.Methods Case 1: An 18-year-old woman with juvenile-onset SLE presented with subacute proximal weakness, areflexia and nephrotic-range proteinuria. Nerve conduction studies supported an inflammatory polyradiculoneuropathy with an MRI brain/spine demonstrating cranial and cervical nerve-root enhancement. Despite high-dose glucocorticoids and IVIg, she deteriorated to respiratory failure requiring mechanical ventilation. Serum was positive for IgG1 NF155 and NF140/186. IVIg and plasma exchange was given without efficacy so treatment with obinutuzumab plus cyclophosphamide was initiated, with gradual neurological recovery to baseline by 3 months.Case 2: A pregnant 25-year-old of 14 weeks’ gestation presented with rapidly progressive, ascending weakness, nephrotic-range proteinuria and Class III lupus nephritis on renal biopsy. NCS indicated acute inflammatory demyelinating polyneuropathy. She developed acute myocarditis early in admission with a troponin rise of 36,766 ngL (normal range: <6 ngL). Serology confirmed IgG1/2/3 NF140/186 and NF155 antibodies. Management with prednisolone, hydroxychloroquine, azathioprine and tacrolimus (pregnancy-compatible) plus rituximab was initiated, resulting in progressive neurological recovery. She returned to baseline strength without deficits within two months and delivered a small for gestational age infant.Relevant investigation findings are highlighted in the table below.Results Both patients demonstrated autoimmune nodopathy serology in the setting of active SLE with nephrotic-range proteinuria. Conventional CIDP therapies (IVIg, plasma exchange) where insufficient B cell depletion was associated with marked neurological recovery in both cases.Abstract PO:04:096 Table 1Baseline neurologic examination and laboratory tests of the patient at the initial presentationConclusions Autoimmune nodopathy can occur in SLE and may drive acute demyelinating neuropathies within peripheral NPSLE. Recognition is critical because IVIg may be ineffective, whereas B-cell depletion is often highly efficacious, including in pregnancy-tailored regimens. We advocate routine nodal/paranodal antibody testing (NF155/NF186, CNTN1, Caspr1) in atypical or severe neuropathies and early consideration of B-cell depletion when AN is confirmed or strongly suspected.",
  "authors": [
    {
      "affiliations": [
        "King’s College Hospital, London, UK"
      ],
      "name": "Hadi Rabee"
    },
    {
      "affiliations": [
        "King’s College Hospital, London, UK"
      ],
      "name": "Aly Alzayyat"
    },
    {
      "affiliations": [
        "King’s College Hospital, London, UK"
      ],
      "name": "Shanindra De Alwis"
    },
    {
      "affiliations": [
        "King’s College Hospital, London, UK"
      ],
      "name": "Ismail Awad"
    },
    {
      "affiliations": [
        "King’s College London, London, UK"
      ],
      "name": "Kate Bramham"
    },
    {
      "affiliations": [
        "King’s College Hospital, London, UK"
      ],
      "name": "Susanne Watkins"
    },
    {
      "affiliations": [
        "King’s College Hospital, London, UK"
      ],
      "name": "Rob Hadden"
    },
    {
      "affiliations": [
        "King’s College Hospital, London, UK"
      ],
      "name": "Lizemarie Wium"
    },
    {
      "affiliations": [
        "King’s College Hospital, London, UK"
      ],
      "name": "Sancho Villar"
    },
    {
      "affiliations": [
        "King’s College Hospital, London, UK"
      ],
      "name": "Chris Wincup"
    }
  ],
  "title": "PO:04:096 Refractory demyelinating neuropathy in SLE unmasked as autoimmune nodopathy: evidence for B cell–targeted therapy",
  "uid": "af67a3d6-d3a7-571b-9f57-ab2baaab726e"
}