309. Real life experience of evaluation and treatment of large Vessel Vasculitis: A Single Centre Experience.
Creators: name: Azeem Ahmed1
name: Khin Yein1
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Background: </strong>Giant Cell Arteritis (GCA) is a large vessel vasculitis with overlapping phenotypes including classic cranial arteritis and large-vessel GCA (LV-GCA). Imaging studies of patients with GCA have demonstrated large-vessel involvement is present in up to 83% of patients<sup>1</sup>. LV-GCA increases the likelihood of aneurys and this is associated with significant mortality. Treatment includes corticosteroids and immunosuppressants such as Methotrexate or Tocilizumab. <strong>Methods: </strong>All patients were diagnosed by CT-angiogram or CT-PET from 2011 to 2020. Clinical, biochemical and radiographic data prior to and 6 months following therapy were analysed. <strong>Results: </strong>Of 24 patients, 12/24 patients were female. Median age was 64.5 years (IQR 60.8-72). Median time from sympto to diagnosis was 2 months (IQR 1-6.3). Of 24 patients analysed, 18 were diagnosed by CT-PET, 5 by CT angiogram and 1 on thoracic aorta biopsy. The clinical features included: refractory Polymyalgia Rheumatica (45.8%), weight loss (33.3%), chest pain (25%), abdominal pain (20.8%), BP difference &gt;10mmHg (20.8%), fever (16.6%), limb claudication (12.5%), cranial GCA (8.33%), y cough (8.3%) and joint swelling (4.16%). The vessels involved in decreasing order were: Aorta (91.6%), Subclavians (54.16%), both carotids (33.33%), iliacs (29.16%), both axillaries (20.83%), both femorals (16.66%), mesenteric (12.5%), both vertebrals (8.33%) and popliteal (4.16%). 22/24 patients were given prednisolone. Prednisolone was not given in 2 patients for relative contraindications, multifocal motor neuropathy and possible cancer. 23/24 were given additional immunosuppresants including Methotrexate (n=10), Cyclophosphamide (n=7), Mycophenolate (n=3), Leflunomide (n=2) and Tocilizumab (n=1). 6/24 patients required a second agent for progressive or refractory disease. Two patients were given Tocilizumab, One patient Cyclophosphamide and three patients were given Rituximab. At 6 months, mean CRP fell from 108.6 to 4.97mg/L (p&lt;0.001), ESR from 70.1 to 15mm/hr (p&lt;0.001), Prednisolone dose from 43.9 to 15.7mg (p&lt;0.001) (Wilcoxon).18/24 had follow up imaging to date. 16/18 (88.8%) improved or resolved and were treated with Methotrexate (n=6), Cyclophosphamide (n=5), Tocilizumab (n=1), Prednisolone only (n=1), Mycophenolate (n=1), Cyclophosphamide followed by Rituximab (n=1), Mycophenolate/Rituximab combination (n=1). 1/18 imaging was stable (Mycophenolate/Rituximab combination) and 1/18 progressed despite Cyclophosphamide. Two patients died during follow up. One patient developed chronic myelomonocytic leukaemia. The second patient was initially diagnosed with LV-GCA on biopsy after developing aortic dissection whilst being treated for longstanding seronegative rheumatoid arthritis with tocilizumab. Despite Cyclophosphamide therapy she progressed and died from ruptured aortic aneurysm. <strong>Conclusions: </strong>We highlight common presentations for large vessel GCA include refractory Polymyalgia Rheumatica and weight loss. We found 91.6% had involvement of the aorta. 88.8% responded at 6 month with initial Prednisolone plus additional immunosuppressant. All GCA patients need close follow up for evolving aortic involvement especially if they are taking Tocilizumab. Tocilizumab therapy can normalise inflammatory markers and may mask progressive underlying vasculitis revealed on histology<sup>2</sup>. <strong>Disclosures:</strong> The authors have declared no conflicts of interest. <strong>References</strong> Blockmans D, de CL, Vanderschueren et al. Repetitive <sup>18</sup>F-fluorodeoxyglucose positron emission tomography in giant cell arteritis: a prospective study of 35 patients. Arthritis Rheum 2006;55:131-7 Unizony S, Arias-Urdaneta E, Miloslavsky E et al. Tocilizumab for the treatment of Large-Vessel Vasculitis (Giant Cell Artertitis, Takayasu Arteritis) and Polymyalgia Rheumatica. Arthritis Care &amp; Research Nov 2012;64(11):1720-29