FRI0191 CRANIAL-LIMITED AND LARGE-VESSEL GIANT CELL ARTERITIS: PRESENTING FEATURES AND OUTCOME
Authors: A. Tomelleri, C. Campochiaro, S. Sartorelli, N. Farina, E. Baldissera, L. Dagna
Journal: Annals of the Rheumatic Diseases (2020)

Background: Giant cell arteritis (GCA) comprises two main phenotypes: cranial (C) and large-vessel (LV) disease 1 . A full baseline steroid-free vascular imaging evaluation is required to properly diagnose LV involvement 2 Objectives: To compare presenting and prognostic features of LV-GCA and C-GCA patients after an adequate vascular imaging evaluation at baseline Methods: Data from GCA patients followed-up at our Institution were retrospectively collected. Only patients who underwent large-vessel imaging (PET, CTA, MRA) at disease onset or within 1 week after steroid introduction were included. Patients with evidence of LV involvement were classified as LV-GCA. Differences between LV-GCA and C-GCA patients regarding presenting features, treatment, prognosis were evaluated. Non-parametric tests were used Results: In our cohort, we identified 161/280 patients who underwent LV-imaging study at baseline. Of these, 100 (62.1%) had signs of LV inflammation. Table 1 compares demographic features, diagnostic delay, pre-existing comorbidities and complementary treatment between the 2 groups. Table 2 compares disease features at diagnosis. Mean follow-up was similar between LV- and C-GCA patients (31.8±31.8 vs 27.8±29.1 months; 70% vs 73.8% followed-up ≥12 months). Corrected cumulative prednisone dose (CCPD, grams/months) was equivalent (LV, 0.67±0.57; C, 0.87±1.37; p=0.871). A DMARD was added in 73% of LV- and in 55.7% of C-GCA patients (p=0.027), but, notably, it was introduced at baseline in 52% of LV- vs 23.5% of C-GCA patients (p=0.006). CCPD was equivalent even considering only patients who did not receive DMARDs (LV, 0.92±0.81; C, 0.94±1.18; p=0.522). Frequency of relapses was not significantly different (LV, 51%; C, 57.3%, p=0.515), even when considering only DMARD-receiving patients (LV, 36.1%; C, 38.2%, p=0.833). Aortic aneurysms incidence at 5 years was similar (LV, 17.3%; C, 15.7%; p=0.826). Rate of metabolic and infective complications was similar, in terms of arterial hypertension (LV, 3%; C, 0%, p=0.286), diabetes (2% vs 0%, p=0.524), osteoporotic fractures (7% vs 5%, p=0.742), severe infections (3% vs 3.3%, p=1) Table 1. Demographic features, diagnostic delay, pre-existing comorbidities, and complementary treatment at baseline in LV and C-GCA patients LV imaging + n=100 (% ) LV imaging - n=61 (% ) p-value Age (years) 73.2 ± 8.9 76 ± 8.8 0.018 Sex (female) 65 (65) 40 (65) 1 Diagnostic delay (months) 3.5 ± 4.6 2.3 ± 4.9 0.001 Pre-existing comorbidities - CAD 3 (3) 7 (11.5) 0.043 - Diabetes 4 (4) 6 (9.8) 0.181 - Dyslipidemia 17 (17) 17 (27.9) 0.114 - Hypertension 42 (42) 34 (55.7) 0.105 - Stroke 3 (3) 3 (5) 0.674 - Cancer 20 (20) 6 (9.8) 0.122 Ongoing complementary treatment - Antiplatelet 18 (18) 15 (25) 0.322 - Anticoagulant 1 (1) 6 (9.8) 0.012 - Statin 14 (14) 14 (23) 0.198 Table 2. Diseases features at onset in LV and C-GCA patients LV imaging + n=100 (% ) LV imaging - n=61 (% ) p-value Temporal biopsy positive 17/31 (55) 9(43) 0.573 Symptoms - Headache 65 (65) 52 (85) 0.006 - Jaw claudication 22 (22) 20 (32.8) 0.142 - Scalp tenderness 31 (31) 26 (42.6) 0.174 - Ocular symptoms 14 (14) 20 (32.8) 0.006 - Ischemic optic neuropathy 7 (7) 17 (27.9) &lt;0.001 - Stroke 3 (3) 0 (0) 0.290 - Polymyalgia rheumatica 42 (42) 31 (50.8) 0.328 - Fever 44 (44) 12 (19.7) 0.002 - Fatigue 72 (72) 21 (34.4) &lt;0.001 - Weight loss 37 (37) 7 (11.5) &lt;0.001 - Cough 10 (10) 1 (1.6) 0.053 Laboratory findings, mean - C-reactive protein, mg/L 80.8 ± 60.8 65.7 ± 58.2 0.057 - Erythrocyte sedimentation rate 76.8 ± 30 71.5 ± 27 0.360 - Hemoglobin, g/dL 11.4 ± 1.5 12 ± 1.6 0.007 - Platelet count 389.4 ± 116.6 366.8 ± 125.2 0.758 Conclusion: LV-GCA patients are younger and suffer of a greater diagnostic delay. Although a greater systemic inflammation seems to be a feature of LV-GCA patients, the vascular prognosis is similar to C-GCA patients, who, conversely, have a greater incidence of ocular complications References: [1]Dejaco C, et al. Nat Rev Rheumatol (2017) [2]Kermani T, et al. Rheumatology (2019) Disclosure of Interests: Alessandro Tomelleri: None declared, Corrado Campochiaro Speakers bureau: Novartis, Pfizer, Roche, GSK, SOBI, Silvia Sartorelli: None declared, Nicola Farina: None declared, Elena Baldissera Speakers bureau: Novartis, Pfizer, Roche, Alpha Sigma, Sanofi, Lorenzo Dagna Grant/research support from: Abbvie, BMS, Celgene, Janssen, MSD, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, SG, SOBI, Consultant of: Abbvie, Amgen, Biogen, BMS, Celltrion, Novartis, Pfizer, Roche, SG, and SOBI