A single center survey study of systemic vasculitis and COVID-19 during the first months of pandemic INTRODUCTION: COVID-19 pandemic created concerns among patients receiving immunosuppressive therapy. Frequency of COVID-19 and impact of lockdown on treatment compliance in patients with vasculitis are largely unknown.PATIENTS AND METHOD: Patients with ANCA-associated and large vessel vasculitis that have been followed-up in our clinic were contacted by phone and a questionnaire containing home isolation status, treatment adherence and history of COVID-19 between March 1st and June 30th, 2020 was applied. RESULTS: The survey was applied to 103 patients (F/M: 59/44, mean age: 53.2±12.5). Thirtythree (32%) patients didn't attend at least one appointment; 98(95.1%) noted that they spent 3 months in home isolation. Five patients (4.8%) received immunosuppressives irregularly and 3(2.9%) developed symptoms due to undertreatment. Four (3.9%) patients admitted to hospital with a suspicion of COVID-19, but none of them had positive PCR or suggestive findings by imaging. COVID-19 diagnosed in a patient with granulomatosis with polyangiitis during hospitalization for disease flare and she died despite treatment. DISCUSSION: Frequency of COVID-19 was low in patients with vasculitis in our single center cohort during the first months of pandemic. Although outpatient appointments were postponed in one-third of our patients, high compliance with treatment and isolation rules ensured patients with vasculitis overcome this period with minimal morbidity and mortality.

Background Hyperinflammation (HI) developing in 2 nd week of COVID‐19 contributes to the worse outcome. Because of relatively milder laboratory findings, available criteria for classification of hemophagocytic lymphohistiocytosis or macrophage activation syndrome could not be helpful. Methods Discovery cohort included symptomatic COVID‐19 patients from Turkey, followed at hospital during the initial wave. Replication cohort consisted of hospitalized patients from a later period; all required oxygen support and received glucocorticoids. Diagnosis of HI was made by an expert panel and the majority received tocilizumab or anakinra. Daily clinical and laboratory data were recorded, and data of treatment start day were compared with the 5 ‐ 6 th day data of other patients. Values maximizing the sensitivity and specificity of each parameter were calculated to determine criteria items. Results 685 patients were analyzed in discovery and 156 in replication cohorts; of whom 150 and 61 received treatment for HI, respectively. Mortality rate was higher in HI patients of discovery cohort (23.3%) compared to the rate of other patients (3.7%), and it was much lower in replication cohort for both groups. The 12‐item criteria were developed to define HI of COVID‐19 (HIC), and score of 35 provided 85.3% sensitivity, 81.7% specificity. The same criteria gave 90.0% sensitivity for HIC in replication cohort, but lower specificity values were observed, due to the inclusion of milder cases of HIC responding only glucocorticoids. Conclusions The new criteria are expected to define patients with HIC better with reasonable sensitivity and specificity and enable us to start treatment as early as possible. This article is protected by copyright. All rights reserved.

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A 20 year old woman presented with right arm pain. Pulses of right upper extremity were weak, acute phase reactants were elevated and MR angiography demonstrated total occlusion of subclavian artery and right axillary artery with collaterals. The diagnosis was Takayasu arteritis and she was treated with prednisolone, azathioprine and acetylsalicylic acid. During follow up, azathioprine was switched to methotrexate. Three years later, patient presented with elevated blood pressure. CT angiography demonstrated reduced calibration of the aorta and almost total occlusion of the lumen of proximal parts of left and right renal arteries. C-reactive protein was elevated. Steroid dose was increased, methotrexate was discontinued and IV tocilizumab and antihypertensive medications were initiated. One month later, she presented to emergency department with elevated blood pressure and blurred vision in the left eye. Fundoscopic examination revealed bilateral grade 3 hypertensive retinopathy and serous detachment of retina in the left eye. Laboratory results revealed normal CRP, elevated creatinine, elevated lactate dehydrogenase, thrombocytopenia, low hemoglobin and low haptoglobin. Peripheral blood smear revealed 2-3 schistocytes in every field. She was admitted to rheumatology department with the diagnosis of thrombotic microangiopathy secondary to malignant hypertension. IV tocilizumab was administered, and methylprednisolone was maintained at a dose of 20 mg/day. Despite treatment with maximum dose of six antihypertensive medications, her blood pressure was not controlled adequately and she became hypervolemic. After undergoing ultrafiltration, balloon dilation was performed in the left renal artery, and a stent was placed there. After stent placement, creatinine and platelet count normalized, hemoglobin increased and hypertension was controlled. In this case, malignant hypertension which was triggered by bilateral renal artery stenosis due to Takayasu arteritis had caused acute kidney injury and advanced stage hypertensive retinopathy. In addition, unlike other Takayasu arteritis cases with malignant hypertension, thrombotic microangiopathy was also detected.

Background:Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disorder caused by the MEFV gene variants. Although association between FMF and spondyloarthritis (SpA) has previously been reported, clinical and laboratory features of patients with FMF and SpA have not been defined in detail.Objectives:We aimed to evaluate clinical and laboratory characteristics, disease outcome and biologic responses of patients with FMF+SpA compared to patients with only SpA who were followed-up in our tertiary referral center.Methods:Database of FMF Clinic was screened for FMF patients with coexistent SpA and 113 patients were identified fulfilling Tel Hashomer and ASAS criteria for FMF and SpA, respectively. A group of patients with SpA without FMF matched for age, gender and disease duration were selected as the control group.Results:Thirteen patients were excluded because of missing data, and 100 patients (F/M: 52/48) were included into the analysis. Mean follow-up time was 93.6 ± 77 (range[r]: 3-324) months and mean patient age was 43.3 ± 12 (r: 20-87) years. Mean age of onset for FMF was 12.5 ± 8 (r: 1-36) and for SpA was 25 ± 11 (r: 7-72) years. SpA findings was classified as axial in 35.4%, axial and peripheral in 47.9% and only peripheral in 16.7% in FMF+SpA group. Half (49%) of the patients had hip involvement (70% bilaterally), and 21.5% of them needed total hip joint replacement (TJR), which were significantly more frequent compared to control group. Two exon 10 MEFV variants were found in 69.4%, and most (69.8%) had homozygous M694V. Hip involvement was more frequent in patients with two exon 10 variants (p=0.036; OR=4.4) compared to those with one variant; and TJR was more frequent in those with homozygous M694V compared to other exon 10 variants (p=0.001; OR=10). Radiographic sacroiliitis was less frequent in patients with homozygous M694V (p=0.019; OR=5.48). HLA-B27 positivity was not associated with hip or axial involvement in patients with FMF+SpA.Biologics were used in 60 patients (anti-TNF in 43, secukinumab in 1, and tocilizumab in 2). Anti-IL-1 drugs were used in 23 patients for refractory FMF. In 9 patients, anti-TNF and anti-IL-1 drugs were tried for refractory joint involvement: 5 switched to anti-TNFs from anti-IL-1, 4 patients switched to anti-IL-1 from anti-TNFs. Biologic DMARD requirement was more frequent in patients with two exon 10 variants (p=0.006; OR=7.4), especially in those with homozygous M694V (p=0.006; OR=7.6). Although anti-IL-1 usage did not differ among MEFV variants, anti-TNF was used more frequently in patients with homozygous M694V (p=0.007; OR=7.2). FMF+SpA patients had higher serum CRP and developed amyloidosis more frequently than those patients with SpA.Table 1.Comparison of clinical and laboratory findings between the patients with FMF+SpA and SpA controlFMF + SpA (n=100)SpA (n=217)P valueAge (years)*43.3 ± 1243.4 ± 110.6Sex (n, %) Male48104 (47.9)0.99 Female52113 (52.1)Duration of SpA (monnths)*181.6 ± 108180.2 ± 1120.8Age onset of SpA (years)*25.1±1128.4±80.008Peripheral arthritis (n, %)35/80 (43.8)79/212 (37.3)0.3HLA-B27 positivity (n, %)6/21 (28.6)105/139 (75.5)<0.001 (OR=18.9)CRP (mg/dL)*26.7 ± 25**18.96±290.001ESR (mm/hour)*39.7 ± 2739.4 ± 280.8Hip involvement (n, %)47/96 (49)23/118 (19.5)<0.001 (OR=20.9)TJR (n, %)20/93 (21.5)8/205 (3.9)<0.001 (OR=23.3)Fulfilling mNY criteria (n, %)52/81 (64.2)164/199 (82.4)0.001 (OR=10.8)Biologic DMARD (n, %)6068/214 (31.8)<0.001 (OR=22.5)Anti-TNF (n, %)4668/214 (31.8)0.015 (OR=5.96)Amyloidosis (n, %)165/205 (2.4)<0.001 (OR=19.3)* mean±standard deviation, **during the attack-free periodConclusion:In this group of FMF+SpA patients, hip involvement and need for TJR were more frequent and associated with penetrant MEFV variants rather than HLA-B27 positivity. These patients had higher inflammatory response and risk of developing amyloidosis, and they needed biologics more frequently compared to SpA group. More severe disease course in FMF+SpA patients requires further attention and analysis in larger cohorts.Disclosure of Interests:None declared