Spontaneous Improvement of Aortitis Associated with Severe COVID-19 Infection-A Case Report.

Aortitis is a rare complication of the coronavirus disease 2019 (COVID-19) and is often treated empirically with steroids. We present a case of spontaneous resolution of aortitis without treatment. A 65-year-old man was admitted to our intensive care unit for severe COVID-19 pneumonia and underwent rehabilitation in the general ward. On day 12, he developed fever, and on day 13, he developed right cervical pain and increased inflammatory markers. On day 16, a cervical echocardiogram showed vasculitis in the right common carotid artery, and on day 17, computed tomography (CT) of the neck showed thickening of the arterial wall of the right common to the internal carotid arteries. A retrospective assessment of the CT scan on day 12 showed wall thickening from the thoracic aorta to the abdominal aorta, and a diagnosis of aortitis was made. Autoantibody analysis, culture, and magnetic resonance imaging (MRI) of the head and neck showed no abnormalities. During the investigation of the cause of aortitis, the fever and inflammatory reaction spontaneously resolved and the right cervical pain gradually improved. Therefore, the patient was diagnosed with transient COVID-19-related aortitis. To our knowledge, this is the first report describing the spontaneous resolution of COVID-19-related aortitis.

Challenges in the Diagnosis and Investigation of Giant Cell Arteritis during Covid- 19 Pandemic Restrictions: Does the Southend Pretest Probability Score Help to Guide Clinical Diagnosis?

Background/Aims During 2020-2021 many usual hospital services were affected as focus turned towards managing COVID-19. Elective outpatient surgery ceased and rheumatology staff were redeployed to covid wards. This reduced the availability of temporal artery biopsy (TAB) and temporal artery ultrasound (TAUS) to aid in diagnosing giant cell arteritis (GCA). The rheumatology team making diagnoses of GCA or not-GCA were doing so often based entirely on clinical and laboratory findings. We aimed to determine referral patterns and investigations for suspected GCA during the covid pandemic, compare diagnoses at 6 months after initial assessment and retrospectively apply the Southend Pretest Probability Score (PTPS) and correlate with the diagnosis of GCA or not-GCA. Methods We reviewed all electronic referrals for suspected GCA from July 2020 - June 2021. Clinical details and investigations reviewed. PTPS applied giving a result of low, intermediate or high probability of GCA. Results 84 referrals for suspected GCA over 12 months. 20 diagnosed GCA/ large vessel vasculitis (LVV), 64 not-GCA. Peak referral months Nov 2020 and April 2021 with 13 and 16 referrals. Lowest in October 2020 with 1 referral. 57 female, 27 male. Mean age 70.1 years. 19% male referrals diagnosed GCA, 26% female diagnosed GCA. All LVV and PMR diagnoses were female. 27 TAUS, 6 TAB, 7 PET, 13 CT, 3 MRI performed. 30 patients had no additional investigations. Of 20 GCA;14 had supporting investigations, 6 were clinical diagnoses. All GCA diagnoses were consistent at 6 months. One not-GCA case was subsequently diagnosed with LVV on CTPET. All other not-GCA diagnoses were consistent at 6 months. The PTPS was retrospectively applied based on available clinical information in all except 2 cases, and compared to GCA/not-GCA diagnosis and investigations undertaken. Conclusion Referral numbers for suspected GCA were higher than previous years however the number of actual GCA diagnoses was similar. With limitations on diagnostic investigations due to covid, diagnoses of GCA with and without additional tests were accurate at 6 months, and correlated with a high probability score. The PTPS is a therefore valuable clinical tool in the assessment of GCA. (Table Presented).

The Incidence of Large Vessel Vasculitis in Norfolk

Background/Aims There are no data on the collective incidence of the large vessel vasculitides. The incidence of GCA and Takayasu arteritis in the UK has been based on clinical coding in routine administrative datasets. There are no data on the incidence of these diseases based on clinically verfied diagnoses. We studied the incidence of the large vessel vasculitides in a stable population with a predominant Northern European ancestry. Methods Individuals attending a secondary care hospital with a clinically verified diagnosis of primary systemic vasculitis made between 2011-2020, who lived within the NR postcode districts of Norfolk county were included if they met classification criteria for GCA or Takayasu arteritis, or had definite tissue or imaging evidence of large vessel vasculitis. Population above the age of 18 as in the 2011 census, available from the office of national statistics, was accepted as the denominator. If classification criteria for both GCA and TAK were met, physician judgement was accepted as final diagnosis. Results 272 adults were diagnosed with large vessel vasculitis out of a population of 454,316. The annual incidence of large vessel vasculitis in Norfolk is 59.9/million in the adult population. The annual incidence of GCA is 9.9/100,000 over the age of 50 using the ACR 1990 criteria and 10.6/100,000 using the ACR/EULAR 2022 criteria. The rise in the incidence from 2017 onwards coincides with the establishment of a fast-track pathway (Table 1). The dip in the incidence in 2020 coincides with suspension of services during the SARS-CoV-2 pandemic. The annual incidence peaks at 168.5/100,000 in the 9th decade and is commoner in females (12.3/100,000 vs 7.3/100,000). The annual incidence of Takayasu arteritis is 3.3/million in the adult population using the ACR 1990 criteria and 1.1/million using the ACR/EULAR 2022 criteria. Conclusion This is the first study that reports the incidence of all objectively diagnosed large vessel vasculitis in a stable population in Norfolk county. The incidence of GCA rose with the establishment of a fasttrack pathway and its peak may have been affected by the SARS-CoV- 2 pandemic. GCA is commoner in females and peaks in the 8th and 9th decades. (Table Presented).

Takayasu arteritis associated with autoimmune/inflammatory syndrome induced by adjuvants: a case-based review.

Takayasu's arteritis (TA) is a chronic granulomatous vasculitis that predominantly affects the aorta and its major branches. Despite advancements in the understanding of the pathogenic pathways of vascular inflammation, the etiology and predisposing factors of TA remain to be fully understood. In susceptible individuals, exposure to adjuvants may trigger, unlock or unmask an autoimmune disorder, presenting as non-specific constitutional symptoms or a fully developed autoimmune syndrome such as vasculitis. Here, we hypothesize that TA could be triggered by siliconosis, a subtype of the autoimmune/inflammatory syndrome induced by adjuvants (ASIA). ASIA, also known as Shoenfeld syndrome, encompasses a wide range of autoimmune and immune-mediated diseases resulting from dysregulation of the immune response after exposure to agents with adjuvant activity. This case report describes the development of large artery vasculitis, TA, in an individual one year following the placement of silicone breast implants. The patient initially presented with non-specific symptoms, and multiple imaging methods were employed, including ultrasound diagnostics, CT angiography, and 18-fluorodeoxyglucose positron emission tomography/CT. These techniques revealed vasculitic alterations in the carotid arteries and thoracic aorta. Initial treatment with glucocorticosteroids proved ineffective, prompting the addition of steroid-sparing immunosuppressive agents. Due to the distinct clinical symptoms, disease progression, implant-associated fibrosis, and resistance to therapy, the potential involvement of implants in the development of large-vessel vasculitis was considered, and a potential association with ASIA was postulated. Although there is limited evidence to support a direct link between adjuvants and the pathogenesis of TA, similarities in cellular immunity between the two conditions exist. The diagnosis of this complex and potentially debilitating condition requires a comprehensive clinical examination, laboratory evaluation, and instrumental assessment. This will aid in identifying potential contributing factors and ensuring successful treatment.

Vasculitis post COVID-19 vaccinations in an infection naive population: A temporal association?

Background/Purpose: To date, isolated cases of vasculitis linked to COVID-19 vaccinations have been reported. Western Australia (WA), a state of 2.8 million habitants, had largely been shielded initially from COVID-19 infections with only 3.7 cases/1,000 people identified prior to March 2022. In addition, there were only 3.4 cases/1,000 people detected between July 2021 and March 2022(1). Vaccination rates during this period were high with an adult double vaccination rate recorded at over 95%(2). The combination of these has led to a unique study population. We describe a case-series of patients who developed a medium or large vessel vasculitis post-COVID- 19 vaccination. Method(s): Patients who developed a medium or large vessel vasculitis with symptom onset within four weeks of a COVID-19 vaccine were identified from public and private Rheumatology departments in WA between July 2021 and March 2022. Result(s): The clinical features and diagnosis of 10 identified patients are presented in Table 1. Ages ranged between 41-83 and a various subtypes of vasculitis were observed. Nine patients had symptom onset within two weeks post vaccine. All patients received high dose corticosteroids and seven received steroid-sparing therapies with initial good outcomes. Conclusion(s): The link between vaccinations and induction of autoimmunity remains disputed. It must be emphasised that COVID-19 vaccinations have been critical in this pandemic. The reporting of such cases serves to expand the literature base and generate discussion. Biases (recall and observer) and coincidence could account for these observations. However, the time interval between symptom onset and characteristic features of certain cases in an infection naive population should raise further questions. (Table Presented).

Large-Vessel Giant Cell Arteritis following COVID-19-What Can HLA Typing Reveal?

Giant cell arteritis (GCA) is an immune-mediated vasculitis that affects large arteries. It has been hypothesized that viruses may trigger inflammation within the vessel walls. Genetic studies on human leukocyte antigens (HLAs) have previously reported HLA-DRB1*04 as a susceptible allele for GCA and HLA-DRB1*15 as a protective allele for GCA. Here, we discuss the clinical presentation, laboratory findings, HLA class I and class II analysis results, and management of patients with extracranial large-vessel (LV) GCA, detected at least six weeks after recovery from COVID-19. This case series encompassed three patients with LV-GCA (two males and a female with an age range of 63-69 years) whose leading clinical presentation included the presence of constitutional symptoms and significantly elevated inflammatory markers. The diagnosis of LV-GCA was confirmed by CT angiography and FDG-PET/CT, revealing inflammation in the large vessels. All were treated with corticosteroids, while two received adjunctive therapy. By analyzing HLA profiles, we found no presence of the susceptible HLA-DRB1*04 allele, while the HLA-DRB1*15 allele was detected in two patients. In conclusion, LV-GCA may be triggered by COVID-19. We highlight the importance of the early identification of LV-GCA following SARS-CoV-2 infection, which may be delayed due to the overlapping clinical features of GCA and COVID-19. The prompt initiation of therapy is necessary in order to avoid severe vascular complications. Future studies will better define the role of specific HLA alleles in patients who developed GCA following COVID-19.

EXCELLENT PROGNOSIS of RHEUMATIC MANIFESTATIONS FOLLOWING COVID-19 VACCINATION: 7 MONTHS FOLLOW-UP DATA

Background: It is well established that severe forms of SARS-CoV2 infection can induce a massive cytokine storm, which may disrupt the immune system stability and conceivably stimulate the development of reactive manifestations through a molecular mimicry process. Likewise, anti-COVID-19 vaccines, which have so far proved an excellent tolerability and safety profile, are able boost the immune response via different biologic technologies and adjuvant combinations possibly facilitating, in predisposed subjects, the onset of infammatory or even autoimmune manifestations. Objectives: We report a case series of suspected rheumatic adverse events following immunization (AEFI) associated with anti-COVID-19 vaccine. We focused our attention on the prognosis of these patients by analysing their available follow-up data. Methods: We included patients evaluated at frst-aid rheumatologic consultancy and at rheumatologic outpatient and inpatient clinic at Padua University Hospital between May and September 2021 presenting with new-onset rheumatic manifestation or a fare of an underlying rheumatic disease within 30 days after receiving an anti-COVID-19 vaccine dose. Inclusion and exclusion criteria were in accordance with the World Health Organization guidelines for AEFI surveillance. All patients were re-evaluated in January 2022: telemedicine or face-to-face visit. Response to therapy was classifed as complete, good or absent according to the clinician's judgment based on clinical examination, patient's reporting and analysis of laboratory data. Results: We identifed 30 cases of suspected rheumatic AEFI reported in Table 1. Comprehensively the most common manifestations were infammatory arthritis (40.0%), rheumatic polymyalgia (26.7%) and adult-onset Still disease (13.3%). Among patients with an underlying rheumatic disease we recorded an AOSD fare, a rheumatoid arthritis fare with involvement of hands proximal inter-phalangeal joints, one case of wrist arthritis in a patient with psoriatic arthritis, one of aortitis in a patient with large vessels vasculitis, one case of polyarthritis in undifferentiated connective tissue disease and a nephritis fare in a patient with systemic lupus erythematosus. Treatment for the suspected AEFI was based on systemic glucocorticoids (GC) alone (63.3%), systemic GC plus IL-1R antagonists (13.3%), non-steroidal autoinfammatory drugs (13.3%), intra-articular GC (6.6%), colchicine (3.3%) and non-steroidal anti-infammatory drugs (13.3%). At last follow-up contact (7.8±1.5 months) 26 patients (89.6%) were classified as complete responders. Eleven of them (42.3%) withdrew therapy without experiencing recurrence of disease manifestation. One patient with lupus nephritis had a proteinuric flare after the first BNT162b dose;he showed an initial good response to increased glucocorticoid therapy but had a new 24h proteinuria increase at second follow-up visit three months later requiring implementation of immunosuppressive therapy. Another patient with AOSD was in remission at last FU visit in December 2021 but required hospitalization in January 2022 for disease relapse due to a suspected gastrointestinal infection. Finally, one patient hospitalized for a seronegative polyarthritis after the first BNT162b dose achieved complete remission at last available contact (one month after hospital discharge) but was then lost in follow-up. Conclusion: After a mean follow-up of 7.8±1.5 months nearly all of patients showed a complete/good response to standard therapy and about half of them withdrew the treatment without losing the remission status.

SEROLOGICAL RESPONSE FOLLOWING THREE DOSES of SARS-COV-2 MRNA VACCINE in PATIENTS with GIANT CELL ARTERITIS: EXPERIENCE of A SINGLE-CENTRE COHORT

Background: The spread of COVID-19 pandemic raised the need to perform an additional vaccine dose to overcome the diffusion of the infection and possible life-threatening disease complications. Certain population subsets seem to be at increased risk of developing such complications, such as elderly and/or immu-nocompromised patients. Objectives: To assess the persistence of immunity following SARS-CoV-2 mRNA vaccine and the magnitude of the humoral response after the booster dose in a cohort of patients affected by giant cell arteritis (GCA). Methods: Patients with GCA regularly followed at the Rheumatology Department of the University of Pavia, Italy, who received a booster dose of SARS-CoV-2 mRNA vaccine (BNT162b2 Pfizer/BioNtech or mRNA-1273 Moderna) between October 1st and December 31st, 2021 were included. Humoral response was assessed by measuring SARS-CoV-2 Trimeric S (TSAbs) and Neutralizing (NAbs) antibodies, with a cut-off of 33.8 Binding Antibody Units (BAU)/mL and 1:10 dilution, respectively. Blood samples from each patient were drawn at least 4 months after the second and three weeks after the third vaccine dose. Results: Forty-two patients who received the booster dose of SARS-CoV-2 mRNA vaccine were enrolled. Thirty (71.4%) were females, mean age 73.2±4.7 years, disease duration 58±38 months, 19 (45.2%) had large-vessel vasculitis. Thirty-two (76.2%) were on glucocorticoids (GCs) at a mean dose of 4.9±7.8 mg/day prednisone equivalent, with 7 (16.7%) receiving ≥7.5 mg/day. Eighteen (42.9%) were on methotrexate (MTX) (mean dose 14.2±3.5 mg/week) and 8 (19.0%) were treated with subcutaneous tocilizumab (TCZ) 162 mg/week. SARS-CoV-2 serology was tested prior to the third vaccine dose at an average of 5.4±0.4 months from the former vaccination scheduled: 37 (88.1%) retained TSAbs and 30 (71.4%) NAbs. The median TSAb titre was 134 BAU/mL (IQR 97-292). Four out of 5 patients (80.0%) without TSAbs and 7 out of 12 (58.3%) without NAbs were on both GCs and MTX. Moreover, those on GCs plus MTX had lower pre-third dose TSAb titres as compared to other treatment subgroups (Figure 1A). GC doses ≥7.5 mg/day prednisone equivalents seemed to blunt NAb levels along time: 28.6% patients on GCs ≥7.5 mg/day prednisone equivalents had negative NAbs before the third dose vs. 80.0% of those taking <7.5 mg/day (p=0.007) as well as lower NAb titres (Figure 1B). Data regarding antibody response after the booster dose were available for 35 patients (83.3%). Blood collection occurred at a median of 25 days (IQR 24-32) after the third vaccine dose. All patients developed TSAbs, even those who did not respond to the previous shots. The median TSAb titre rose to 2080 BAU/mL (IQR 2080-2080) (p<0.001), while the median NAb titre increased from 1:10 to 1:320 (p<0.001). One patient (2.9%) treated with prednisone 8.75 mg/day plus MTX 12.5 mg/week did not develop NAbs. NAb levels were lower in patients taking MTX as compared to those who did not (Figure 1C,D), whereas treatment with TCZ or GCs, along with the GC dose, did not affect the magnitude of the antibody response. There were no serious adverse events from the vaccine. However, 3 patients (8.6%) experienced a disease relapse 24±5 days after the booster dose. Conclusion: In our cohort, most patients who seroconverted after the second dose of vaccine retained the humoral immunity, with excellent serocon-version rates following the booster dose. However, GCs, especially at doses ≥7.5 mg/day prednisone equivalents, may contribute to the waning of NAb titres. On the other hand, immunosuppressants like MTX, especially when combined with GCs, might impair the magnitude of the humoral response to the booster dose.

Isolated arteritis misdiagnosed as bilateral orbital tumors in a patient with acute ischemic stroke.

Vasculitis is a heterogeneous group of disorders characterized by multifocal segmental inflammation of the small and medium vessels of the central nervous system. The predominant symptoms of cerebral vasculitis are stroke, headache, and encephalopathy. Additional symptoms include seizures, cranial nerve palsies, and myelopathy. Imaging techniques play a crucial role in identifying the diagnosis of vasculitis and demonstrating brain involvement. An 89-year-old woman with permanent atrial fibrillation developed an embolic stroke. In treatment, intravenous thrombolysis and thrombectomy with complete antegrade reperfusion of the left middle cerebral artery was used, without the clinical effectiveness. Brain MRI revealed bilateral oval lesions in medial parts of the orbits, which were initially misinterpreted as orbital tumors. Final diagnosis confirmed thickened arterial walls as orbital changes due to inflammatory arteritis. Ten days later, follow-up MRI was performed and showed complete regression of the orbital masses. Primary central nervous system vasculitis, manifesting as acute ischemic stroke, may be reversible with early systemic thrombolytic treatment.

Overlapping Symptoms of COVID-19 and Giant Cell Arteritis: The Need for a Higher Degree of Suspicion for Diagnostic Differentiation.

Giant cell arteritis (GCA) is a large cell vasculitis that can present with a plethora of symptoms affecting several different systems. Before the COVID-19 pandemic, diagnosis of GCA was straightforward since the list of differential diagnoses for this disease was relatively short. However, the development of a SARS-CoV-2 viral infection challenges this standard. COVID-19 is a viral illness that also can present with similar vascular symptoms as GCS and creates a substantial inflammatory reaction, similar to most vasculitis. We present a case of a patient who had developed GCA after recovering from a COVID-19 viral illness. This is a rare presentation of GCA in the setting of COVID-19, and recognition of the nuanced differences between the two diseases may significantly change a patient's prognosis if not detected early.

A CASE OF REFRACTORY PAEDIATRIC COGAN'S SYNDROME TREATED WITH TOCILIZUMAB

Background/Aims Cogan's syndrome is a rare vasculitis, characterised by progressive sensorineural bilateral hearing loss, vestibular symptoms and nonsyphilitic interstitial keratitis. We present a paediatric case which was refractory to initial treatment and subsequently successfully treated with Tocilizumab. Methods Case report. Results A previously well 14-year-old boy presented with sudden onset hearing loss, tinnitus and vertigo. There was no history of new medication, trauma or tick bite. There was no family history of autoimmune disease or genetic hearing losses. On examination his cranial nerves except for vestibulocochlear were grossly intact. He had a steady gait with no cerebellar signs. Otoscopy was unremarkable. Audiology showed a moderate to severe sensorineural hearing loss bilaterally, worse on the right. He had normal type A tympanograms. An autoimmune screen was carried out which showed normal FBC, inflammatory markers, ACE and Chitotriosidase. ANCA, Rheumatoid factor and lyme serology were negative. MRI of the internal auditory meati was normal. He subsequently developed visual disturbance and was diagnosed with bilateral interstitial keratitis. A unifying diagnosis of Cogan's syndrome was made. An MRI scan of his head, neck and upper thorax looking for evidence of large vessel vasculitis was normal. Genetics looking for evidence of a monogenic autoinflammatory disorder and primary immunodeficiency were negative. He was initially treated with a weaning course of oral prednisolone with improvement in symptoms. Unfortunately, 4 months later had clinical and audiological deterioration in hearing. He therefore received pulsed IV methylprednisolone and commenced subcutaneous methotrexate and adalimumab. 3 months later, there was both clinical and audiological improvement and he started to wean prednisolone. 7 months later, he presented with a 24-hour history of reduced hearing on the right, confirmed on audiogram. He received a further pulse of IV methylprednisolone and a short course of high-dose oral prednisolone, followed by a slowly weaning course. 3 months later, again he felt his hearing had deteriorated and this was confirmed on audiogram, with a 40-decibel loss in his previously good ear. He received two doses of IV methylprednisolone and background steroids were increased to 20mg daily. Due to frequent relapses, adalimumab was changed to IV tocilizumab at 10mg/kg 2-weekly, alongside methotrexate. IV tocilizumab was changed to the subcutaneous route during the COVID-19 pandemic and was tolerated well. His hearing subsequently improved and tocilizumab interval was extended to 3-weekly in Feb 2021. At last review he was stable and successfully transitioned to adult services. Conclusion Evidence regarding treatment options in paediatric patients is lacking due to the rarity of the condition and consequent difficulty in arranging high-quality trials. This is the first case report of use of tocilizumab for Cogan's syndrome in children, highlighting it as a well-tolerated and successful treatment modality.

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). Thirty-three (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. 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.

Large vessel vasculitis related to COVID-19 infection

Case report-IntroductionBy June 2020, 175,000 cases of COVID-19 had been identified in London alone. The most common symptoms include fever, headache, loss of smell, cough, myalgia, and sore throat. The major complication is acute respiratory distress syndrome (ARDS) but systemic complications such as cardiomyopathy, acute kidney injury, encephalopathy and coagulopathy are being identified. A delayed multi-system inflammatory syndrome in children has also been recognised and further complications are likely to be identified as our experience increases. Here, we report the case of a patient with large vessel vasculitis who initially presented with symptoms highly likely to be due to COVID-19 infection.Case report-Case descriptionA 36-year-old black African nurse presented in May 2020, with acute onset 7 days prior of high-grade fevers, rigors, nights sweats, generalised myalgia, sore throat, headache with photophobia, anosmia, dysgeusia and a widespread rash. She was a smoker with no other relevant medical, travel nor sexual history, and no drug use. A COVID-19 swab on day 2 had been negative and she had taken a course of Doxycycline.Examination revealed firm palpable subcutaneous nodules on lower limbs, upper back and forehead and cervical lymphadenopathy. She was photophobic with no meningism. The rest of her physical examination was normal. BP was 116/97 mmHg, heart rate 109 bpm and satO2 100%. Investigations demonstrated C-reactive protein 330mg/L, erythrocyte sedimentation rate 140, Ferritin 479, lymphopaenia 0.7x109, eGFR 54 with no haematoproteinuria, D-dimer 3.05 mg/L with INR 1.1, aPTT 1.3, fibrinogen 8.8 g/L. Hb, WCC, liver function, CK, serum ACE and triglycerides were normal. Infectious screen revealed negative blood cultures, HIV, Hepatitis B and C, EBV, CMV and Treponema pallidum serology. CT brain and CSF analysis were normal including bacterial culture and viral PCR. ANA, ENA, dsDNA, ANCA and aPL antibodies were negative with normal complement levels. Throat swab grew group A streptococcus and she was treated with broad spectrum antibiotics for 7 days maintaining fevers up to 39oC. Skin biopsy was non-specific with negative direct immunofluorescence but showed microvascular thrombi in the papillary dermis. COVID-19 PCR tests (three naso-pharyngeal swabs and one stool PCR) and IgG test (day 38) were negative. CT showed no pneumonitis but non-specific retroperitoneal stranding with medium/large vessel vasculitis involving both proximal renal arteries and a 6 cm segment of mid abdominal aorta on PET-CT. We started oral prednisolone 40mg with immediate resolution of her fevers, myalgia, and inflammatory markers, remaining well a month later.Case report-DiscussionTakayasu's arteritis is the most common autoimmune large vessel vasculitis (LVV) affecting young females and involves inflammation of the arterial wall ultimately resulting in stenosis and obstruction of the vessel. However, it is rare in patients with African heritage and usually presents with a prolonged prodromal phase. Given the atypical presentation and symptoms consistent with COVID-19 infection we feel that this patients' LVV may have been a complication of COVID-19 infection. The relationship between infections and vasculitis is complex. TB and syphilis cause aortitis and a relationship between infection and vasculitis has been proven in HBV associated PAN and HCV associated cryoglobulinemia.Experimental data supports a possible association between CMV and herpes virus and Takayasu arteritis. It could, therefore, be hypothesised that COVID-19 infection can trigger LVV. Our patient had a throat swab positive for Streptococcus pyogenes which is an uncommon cause of infective endocarditis and mycotic aneurism, but this patient had no evidence of either endocarditis or aneurism formation and so it was felt the throat swab finding was incidental. Our patient had repeated negative COVID-19 nasopharyngeal swabs and a negative antibody test at day 38.Although this argues against a diagnosis of COVID-19 related illness, the relative lack of inform tion we currently have regarding sensitivities of the tests, at what point COVID-19 PCR becomes negative in the illness and when/if patients develop antibodies, means these negative tests in the presence of typical symptoms cannot exclude the diagnosis. We believe this case is extremely important to highlight a possible novel inflammatory complication of COVID-19 infection. We decided to treat this patient in line with guidance for the management of LVV, including the introduction of methotrexate, but it will be interesting to observe her long-term outcome.Case report-Key learning points Increasing numbers of COVID-19 related systemic inflammatory conditions are likely to be recognised over the coming months. We present the case of patient with large vessel vasculitis who initially presented atypically with symptoms consistent with COVID-19 infectionTo identify these complications, COVID-19 symptoms questioning should be part of any routine medical historyMore information is required regarding the sensitivity of COVID-19 PCR and antibody tests to aid the diagnosis of these conditionsThe long-term management of inflammatory conditions associated with COVID-19 infection is not clear and a discussion is warranted as to whether DMARDs should be initiated.

Aortitis in COVID-19.

Corona Virus Disease of 2019 (COVID-19) pandemic has affected more than 67.9 million individuals world-wide and led to more than 15.5 million Deaths. In the initial studies from China, 88.7 % of the patient were noted to have fever, 67 % of the patient had cough and 56.4 % had ground glass changes on the chest imaging. With time, the presentation of patients has been found to be highly variable and unpredictable. COVID-19 is reported to present with various complications, ranging from gastrointestinal (GI) manifestations, such as loss of sensation of taste, abdominal pain, diarrhea, vomiting, pancreatitis and hepatobiliary disease, to neurological manifestations of encephalitis and stroke, and cardiovascular manifestations like myocarditis, heart failure and arrythmia. We report a rare case of COVID-19 presenting with abdominal pain from aortitis.

Fast-Track Ultrasound Clinic for the Diagnosis of Giant Cell Arteritis Changes the Prognosis of the Disease but Not the Risk of Future Relapse.

Background: Color Duplex sonography (CDS) of temporal arteries and large vessels (LV) is a recently validated diagnostic methodology for Giant Cell Arteritis (GCA). CDS combined with a fast-track approach (FTA) has improved the early diagnosis of the disease. Objectives: To assess FTA effects on the prevention of permanent visual loss (PVL), relapse and late complications of GCA compared to conventional practice. To assess the impact of COVID-19 pandemic on outcomes of GCA patients assessed with FTA. Methods: GCA patients diagnosed up to June 2020 at the Rheumatology Department, University of Pavia, were included. FTA was implemented since October 2016. FTA consists in the referral within 1 working day of a suspected GCA case to an expert rheumatologist who performs clinical evaluation and CDS. Results: One hundred sixty patients were recruited [female 120 (75%), mean age 72.4 ± 8.2 years]. Sixty-three (39.4%) evaluated with FTA, 97 (60.6%) with conventional approach. FTA patients were older (75.1 ± 7.6 vs. 70.6 ± 8.2 years old; p < 0.001). Median follow-up duration was shorter in the FTA group compared to the conventional one (0.9 vs. 5.0 years; p < 0.001). There was no difference between the two cohorts regarding major vessel district involvement (LV-GCA 17.5% vs. 22.7%; p = 0.4). PVL occurred in 8 (12.7%) FTA patients and 26 (26.8%) conventional ones (p = 0.03). The relative risk of blindness in the conventional group was 2.11 (95% C.I. 1.02-4.36; P = 0.04) as compared to FTA. Median symptom latency of patients experiencing PVL was higher in the conventional group (23 days IQR 12-96 vs. 7 days IQR 4-10, p = 0.02). During COVID-19 there was a significant increase in the occurrence of PVL (40%) including bilateral blindness despite a regularly operating FTA clinic. Cumulative incidence of relapses and time to first relapse did not change after FTA introduction (P = 0.2). No difference in late complications (stenosis/aneurysms) was detected. Conclusions: FTA including CDS evaluation contributed to a substantial reduction of PVL in GCA by shortening the time to diagnosis and treatment initiation. Relapse rate did not change upon FTA introduction, highlighting the need for better disease activity monitoring and treatment strategies optimization based on risk stratification that would predict the occurrence of relapse during glucocorticoid de-escalation.

Treatment of Giant Cell Arteritis and Takayasu Arteritis-Current and Future.

PURPOSE OF REVIEW: Guidelines for the management of large vessel vasculitides have been recently updated by several scientific societies. We have evaluated the current recommendations for treatment of giant cell arteritis (GCA) and Takayasu arteritis (TA) and addressed potential future therapeutic strategies. RECENT FINDINGS: While glucocorticoids (GCs) remain the gold standard for induction of remission, many patients relapse and acquire high cumulative GC exposure. Thus, GC-sparing therapies such as methotrexate are recommended for selected patients with GCA and all patients with TA. Recent high-quality evidence shows that tocilizumab is an effective GC-sparing agent in GCA. Non-biologic and biologic immunomodulators also appear to have GC-sparing properties in TA. Tocilizumab is now considered to be part of the standard treatment for GCA, particularly with relapsing disease, but questions on its use such as length of treatment and monitoring of disease activity remain open. High-quality evidence to guide treatment of TA is still lacking.