EPIDEMIOLOGY
Kabadayi1 , associated of small and tracts can cases, it the clinical mainly in n fact, it was thought to be underestimated, as it is often asymptomatic. In this study, we aimed to investigate systematically the frequency of splenic infarct and related factors in our AAV patients. We also evaluated the role of splenic involvement in the differentiation of AAV subgroups. Methods: Patients with a diagnosis of AAV in whom abdomen/thorax computed tomography (CT) was performed were included in the study. An experienced radiologist examined CT images for the presence of splenic involvement. The clinical and demographic data were retro-spectively collected.Results: In total 70 (30 [43%] female and mean age 56.1 15.7 years) AAV patients (38 [54%] had GPA; 20 [29%] microscopic polyangiitis; 11 [16%] renal-limited disease and 1 [1%] eosinophilic granulomatosis with polyangiitis) were included in the analysis. Splenic pathologies including splenomegaly, hypodense lesion/s, lobulation, and infarction were seen in 21 (30%) patients with AAV. Splenic infarct was observed in seven (10%) patients and all had GPA with renal involvement and PR3ANCA positive. Three of them had total splenic infarct or auto-splenectomy. None of these patients had a history of endocarditis, shock or malignancy before CT. Splenic infarction was found to be negatively correlated with age at diagnosis (p ¼ 0.017; rho¼-0.285), and positively associated with ENT (ear-nose-throat) (p ¼ 0.002; rho¼0,370) and eye involvements (p ¼ 0.013; rho¼0,324).
Conclusion: Our results show that splenic pathologies might not be rare in AAV however, infarction can help to separate GPA from other AAVs. In young GPA patients, in particular, those with ENT and eye involvement, physicians should remember splenic infarction. Since almost half of our cases had severe infarction or autosplenectomy, clinicians might consider immunization in GPA patients for vaccine-preventable infections.
Disclosures: None
125. NEW MAJOR ORGAN INVOLVEMENT IS LOWER IN
YOUNG MALE BEHC¸ ET’S PATIENTS COMPARED TO
RETROSPECTIVE SERIES: FIVE-YEAR RESULTS OF A PROSPECTIVE COHORT Fatma Alibaz-Oner1 , Belgin Aldag1 , Emrah Karatay1 , Ihsan Nuri Akpinar1 , Gonca Mumcu1 , Tulin Ergun1
and Haner Direskeneli1
1
Marmara University, School of Medicine Istanbul, Turkey
Background: Major organ involvement such as vascular or ocular disease, especially in young males, is one of the main causes of mortality and morbidity in Behc¸et’s Disease (BD). However, the prognosis and predictors of major organ involvement is insufficiently studied. We aimed to follow young, male BD patients with only mucocutaneous symptoms which have the highest risk for new major organ involvement prospectively.
Methods: Thirty-six male patients with BD consecutively consulted in the outpatient clinics of Marmara University, Istanbul, 35 males with ankylosing spondylitis and 36 healthy males were included in the study. Bilateral upper and lower extremity venous doppler ultrasono-graphy (US) and brachial and carotid arterial US (for assessing endothelial dysfunction) were performed in baseline visit for all study groups and in the first year follow-up visit for BD patients. Patients with BD were assessed prospectively with 3-6 months intervals and in any urgent visits.
Results: At baseline, the mean disease duration was 3.3 years in patients with BD. The rate of venous insufficiency was higher in male BD patients without vascular events compared to healthy controls (BD vs HC: 30.5% vs 0%) and similar to patients with AS (BD vs AS. 30.5% vs 32%). Markers of endothelial dysfunction (FMD and NID) were similar between BD patients and healthy controls, however CIMT (Carotid intima media thickness) was significantly higher in BD (0.54 mm vs 0.47 mm, p ¼ 0.033). The mean follow-up duration was 59.6 months. Major organ involvement developed in 5 (13.8%, 3 vascular and 2 ocular involvement) patients during follow-up. Immunosuppressive (IS) therapy was required in 27% (n ¼ 10) of patients, due to major organ involvement in 5 (13.8%), refractory mucocutaneous symptoms in four (11%) and chronic arthritis in one (2.7%) patient. In the first year follow-up visit, endothelial functions and CIMT were observed to be significantly improved compared to baseline (Baseline vs Follow-up: 6.84 vs 10.94.5, p ¼ 0.003 for FMD, 0.550.13 vs 0.470.1 for CIMT, p ¼ 0.004). Patients requiring IS treatment in the follow-up had significantly lower FMD at baseline compared to the rest of the group (4.4 vs 8.5, p ¼ 0.005).
Conclusion: Our study demonstrated a lower incidence of major vascular events in male BD patients during prospective follow-up compared to historic controls in the literature. The decreased rate of baseline FMD in patients with later IS requirement suggest that FMD might be a predictor for major organ involvement in BD.
Disclosure: None
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ß The Author(s) 2019. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com
ABSTRACT125 TABLE1: Clinical characteristics of patients with immunosuppressive need during follow-up.
Reason for IS use Age at Diagnosis
Disease duration when IS started
IS agent
Patient 1 Pulmonary aneurysm 35 1 year Azathioprine
Patient 2 Refractory OU 25 5 years Cyclosporine
Patient 3 Deep venous thrombosis 38 10 years Azathioprine
Patient 4 Uveitis 20 5 years Azathioprine
Patient 5 Refractory OU 28 7 years Azathioprine
Patient 6 Refractory OU 23 6 years Cyclosporine
Patient 7 Refractory EN 35 1 year Azathioprine
Patient 8 Deep venous thrombosis 23 1 year Azathioprine
Patient 9 Arthritis 28 7 years Methotrexate
Patient 10 Uveitis 18 4. Azathioprine
F/U: Follow-up, IS: Immunosuppressive, OU: Oral ulcer, EN: ertyhema nodosum
