051 Overlapping syndrome of giant cell arteritis and ANCA-associated vasculitis complicated by severe axonal neuropathy: a case report
| العنوان: | 051 Overlapping syndrome of giant cell arteritis and ANCA-associated vasculitis complicated by severe axonal neuropathy: a case report |
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| المؤلفون: | Abhishek Malhotra, Catriona McLean, Hedley Griffiths, Fariha Islam, Laura Perju-Dumbrava, Rohitha Makonahalli |
| المصدر: | Journal of Neurology, Neurosurgery & Psychiatry. 90:A17.1-A17 |
| بيانات النشر: | BMJ, 2019. |
| سنة النشر: | 2019 |
| مصطلحات موضوعية: | medicine.medical_specialty, business.industry, Sural nerve, medicine.disease, Gastroenterology, Psychiatry and Mental health, chemistry.chemical_compound, Giant cell arteritis, Tocilizumab, chemistry, Giant cell, Large vessel vasculitis, Internal medicine, Prednisolone, medicine, Surgery, Rituximab, Neurology (clinical), business, Vasculitis, medicine.drug |
| الوصف: | CaseA 61-year- old woman presented with what was thought to be a refractory, temporal artery biopsy-proven giant cell arteritis (GCA). After 4 months of therapy she was unable to get the prednisolone dose below 40 mg/day, so weekly subcutaneous Tocilizumab was introduced but with minimal response after 8 weeks. She was then admitted to hospital with a severe rapidly progressive length dependent sensorimotor peripheral neuropathy. Nerve conduction studies showed predominantly an axonal neuropathy with multiple pseudo-conduction blocks.Sural nerve and gastrocnemius biopsies revealed a necrotising vasculitis with numerous giant cells and active axonal degeneration. She proved to be ANCA-PR3 positive, without other systemic manifestations. ANCA-associated vasculitic neuropathy was diagnosed and she was treated with two 1g Rituximab infusions, 2 weeks apart.On follow-up after a month, she had regained some strength but still required a wheelchair for mobility. Further Rituximab infusion after 6 months is planned; prednisolone had been successfully weaned to 10 mg/day.ConclusionPeripheral nerve involvement, which is relatively common in ANCA-associated vasculitis, has also been reported in 14% of GCA cases. But ANCA-PR3 positivity is rare in biopsy-proven GGA, with only a handful of well-documented cases.Heightened suspicion of an alternative diagnosis in the face of an unusual clinical course (lack of steroid response and appearance of small vessel vasculitic symptoms for what is accepted to be a large vessel vasculitis) is critical and our experience highlights the important fact that a diagnosis of one of these disorders does not preclude the subsequent diagnosis of the other. |
| تدمد: | 1468-330X 0022-3050 |
| DOI: | 10.1136/jnnp-2019-anzan.45 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_________::d6dad796418282cbce406bc7c7bc1ac1 https://doi.org/10.1136/jnnp-2019-anzan.45 |
| Rights: | OPEN |
| رقم الانضمام: | edsair.doi...........d6dad796418282cbce406bc7c7bc1ac1 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 1468330X 00223050 |
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| DOI: | 10.1136/jnnp-2019-anzan.45 |