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The objectives of this article are to present a case of type II cryoglobulinemic vasculitis, explain why mucosa-associated lymphoid tissue (MALT) lymphoma is an unusual cause of type II cryoglobulins and to discuss the aetiology, epidemiology, pathophysiology and treatment of cryoglobulinemic vasculitis. A 67-year-old woman presented with 4 months of weight loss, intermittent epistaxis and a purpuric skin rash. Prior to presentation, she was found to have an elevated rheumatoid factor. Further investigation revealed an acute kidney injury and elevated type II cryoglobulins suspicious for cryoglobulinemic vasculitis, which was confirmed by kidney biopsy. Additional workup for the weight loss included biopsy of newly found splenomegaly. Pathology revealed MALT lymphoma, a rare cause of type II cryoglobulinemic vasculitis. Successful medical therapy required treating the underlying malignancy with rituximab and high-dose steroids. After initial resolution of symptoms with this regimen, the patient’s vasculitis worsened, which was thought to be secondary to undertreatment of the lymphoma. Bendamustine was added to further treat the lymphoma, after which the patient recovered and was able to discharge without recurrence of symptoms at 6 months.

Glucocorticoids (GCs) are the gold standard for treatment of giant cell arteritis (GCA); however, there is a need for studies on GC-sparing agents, given that up to 85% of patients receiving GC only develop adverse events. Previous randomised controlled trials (RCTs) have applied different primary endpoints, limiting the comparison of treatment effects in meta-analyses and creating an undesired heterogeneity of outcomes. The harmonisation of response assessment is therefore an important unmet need in GCA research. In this viewpoint article, we discuss the challenges and opportunities with the development of new, internationally accepted response criteria. A change of disease activity is a fundamental component of response; however, it is debatable whether the ability to taper GC and/or the maintenance of a disease state for a specific time period, as applied in recent RCTs, should be part of response assessment. The role of imaging and novel laboratory biomarkers as possible objective markers of disease activity needs further investigation but might be a possibility when drugs directly or indirectly influence the levels of traditional acute-phase reactants such as erythrocyte sedimentation rate and C reactive protein. Futures response criteria might be constructed as a multidomain set, but the questions about which domains will be included and what their relative weights will be still need to be answered.

A 53-year-old man with hypertension, hyperlipidemia, and a remote, cryptogenic, multifocal, posterior circulation ischemic stroke, came to the emergency department with 1 day of vertigo, ataxic gait, nausea, occipital headache, and painless binocular diplopia. Symptoms were present upon awakening on the day of presentation and progressed throughout the day. The erythrocyte sedimentation rate was 10 mm/h and C-reactive protein level was less than 3 mg/L. Cerebrospinal fluid examination indicated mild lymphocytic pleocytosis with 9 cells/µL, protein value of 45 mg/dL, and glucose level within normal limits. Brain magnetic resonance imaging and magnetic resonance angiography with contrast demonstrated a left caudate head infarction and leptomeningeal and perivascular enhancement involving bilateral temporal lobes, basal ganglia, and frontal lobes. Right middle cerebral artery wall enhancement was also noted. Conventional cerebral angiography showed diffuse dilatation and mural irregularity of the right middle cerebral artery M1 segment, as well as dilatation of the first 2 mm of the left anterior cerebral artery A1 segment. These findings were associated with vessel wall gadolinium enhancement on magnetic resonance imaging, which raised concern for vasculitis. The patient was diagnosed with primary angiitis of the central nervous system. The diagnosis was based on the presence of multiple ischemic infarcts, without cardioembolic source, abnormal brain magnetic resonance imaging and magnetic resonance angiography findings consistent with vasculitis, and the absence of systemic vasculitis, infection, and cancer. After diagnosis, the patient was started on intravenous methylprednisolone, followed by oral prednisone and intravenous cyclophosphamide. Because of some new areas of enhancement on magnetic resonance imaging, the patient was subsequently treated with rituximab. Clinical and radiologic remission was achieved, although the patient had permanent residual gait difficulties. Central nervous system vasculitis is an inflammatory process of the blood vessels in the brain, meninges, and spinal cord. It is called primary angiitis of the central nervous system when the process is limited to the brain and, rarely, the spinal cord. In other circumstances, central nervous system vasculitis can be secondary to a systemic inflammatory syndrome or infectious process.

Background: The impact and consequences of the COVID-19 pandemic on people with rheumatic disease are unclear. We developed the COVID-19 Global Rheumatology Alliance Patient Experience Survey to assess the effects of the COVID-19 pandemic on people with rheumatic disease worldwide. Methods: Survey questions were developed by key stakeholder groups and disseminated worldwide through social media, websites, and patient support organisations. Questions included demographics, rheumatic disease diagnosis, COVID-19 diagnosis, adoption of protective behaviours to mitigate COVID-19 exposure, medication access and changes, health-care access and communication with rheumatologists, and changes in employment or schooling. Adults age 18 years and older with inflammatory or autoimmune rheumatic diseases were eligible for inclusion. We included participants with and without a COVID-19 diagnosis. We excluded participants reporting only non-inflammatory rheumatic diseases such as fibromyalgia or osteoarthritis. Findings: 12 117 responses to the survey were received between April 3 and May 8, 2020, and of these, 10 407 respondents had included appropriate age data. We included complete responses from 9300 adults with rheumatic disease (mean age 46·1 years; 8375 [90·1%] women, 893 [9·6%] men, and 32 [0·3%] participants who identified as non-binary). 6273 (67·5%) of respondents identified as White, 1565 (16·8%) as Latin American, 198 (2·1%) as Black, 190 (2·0%) as Asian, and 42 (0·5%) as Native American or Aboriginal or First Nation. The most common rheumatic disease diagnoses included rheumatoid arthritis (3636 [39·1%] of 9300), systemic lupus erythematosus (2882 [31·0%]), and Sjogren's syndrome (1290 [13·9%]). Most respondents (6921 [82·0%] of 8441) continued their antirheumatic medications as prescribed. Almost all (9266 [99·7%] of 9297) respondents adopted protective behaviours to limit SARS-CoV-2 exposure. A change in employment status occurred in 2524 (27·1%) of 9300) of respondents, with a 13·6% decrease in the number in full-time employment (from 4066 to 3514). Interpretation: People with rheumatic disease maintained therapy and followed public health advice to mitigate the risks of COVID-19. Substantial employment status changes occurred, with potential implications for health-care access, medication affordability, mental health, and rheumatic disease activity. Funding: American College of Rheumatology.

Objectives Multicentric reticulohistiocytosis (MRH), a rare histiocytic disease that can mimic other rheumatic conditions, may be associated with cancer and other autoimmune disorders. To better understand the disorder and its other associations, we aimed to evaluate clinical correlates and outcomes of all patients with MRH seen at Mayo Clinic, Rochester between 1980 and 2017. Methods A retrospective medical record review was conducted to identify all patients with MRH between 1 January 1980 and 30 April 2017. Results We identified 24 patients with biopsy-proven MRH (58% female, 75% Caucasian, median age at diagnosis 52 years, median follow-up of 2.3 years). All patients had cutaneous and articular involvement; 23 (96%) patients had papulonodular skin lesions (87% periungual and dorsal hand) and seven (30%) mucosal nodules; and 22 (92%) patients had arthralgias, 21 (88%) joint effusions and 13 (54%) synovitis. Most frequently used therapies included corticosteroids, cyclophosphamide, methotrexate and bisphosphonates. Biologics were used in four patients. Nine patients had symptomatic resolution at 1 year and 12 partial improvement. Radiological findings included erosive changes in three (60%) patients and arthritis mutilans in two patients (40%). Twenty-nine per cent of patients had a concomitant autoimmune disease and 25% malignancy including melanoma, endometrial, peritoneal and lung carcinoma. The 5-year survival rate was 85% (95% CI: 74, 100%). Conclusion To our knowledge, this is the largest single-centre series of patients with MRH highlighting the rarity of the condition and an unmet need for treatment options that can allow sustained disease remission. It also highlights the need for a high vigilance for malignancy and autoimmune diseases.

Background Takayasu arteritis is a rare large-vessel vasculitis that predominantly affects females of Asian descent. This retrospective analysis was performed to increase understanding of the epidemiology of the disease in the United States. Methods We performed a retrospective cohort study in 2 tertiary centers. Patients were selected according to the American College of Rheumatology classification criteria for Takayasu arteritis. Data collected included demographic characteristics and details of physical examinations, treatments, and surgical interventions. Data were managed with REDCap (Research Electronic Data Capture) tools. Results The study included 57 patients. The female:male ratio was 4.2:1, the median age at diagnosis was 29 years, 61.4% of the patients were Caucasians, and 86% of the patients had stenosis on imaging. Hata V was the most common angiographic classification (37.5% of patients). Vascular interventions were required in 43.9% of patients. The most frequent complications were hypertension (56.1%), renal artery stenosis (28.1%), and aortic insufficiency (19.3%). Conclusions Takayasu arteritis continues to be a rare large-vessel vasculitis. In the United States, it tends to affect predominantly Caucasian females, with cervicobrachial involvement. This cohort reflects the morbidity, multiple interventions, and complications experienced by patients with Takayasu arteritis.

Objective Antirheumatic disease therapies have been used to treat coronavirus disease 2019 (COVID‐19) and its complications. We conducted a systematic review and meta‐analysis to describe the current evidence. Methods A search of published and preprint databases in all languages was performed. Included studies described one or more relevant clinical outcomes in five or more people who were infected with SARS‐CoV‐2 and were treated with antirheumatic disease therapy between 01/01/2019 and 05/29/2020. Pairs of reviewers screened articles and extracted data and assessed risk of bias. A meta‐analysis of effect sizes using the random‐effects models was performed when possible. Results The search identified 3,935 articles, of which 45 were included (4 randomized controlled trials, 29 cohort studies, and 12 case series). All studies evaluated hospitalized patients and 29 out of 45 had been published in a peer‐reviewed journal. In a meta‐analysis of three cohort studies with a low risk of bias, hydroxychloroquine use was not significantly associated with mortality (pooled hazard ratio (HR) 1.41, 95% confidence interval (CI) 0.83‐2.42). In a meta‐analysis of two cohort studies with some concerns/high risk of bias, anakinra use was associated with lower mortality (pooled HR 0.2, 95% CI 0.1‐0.4). Evidence was inconclusive with regard to other antirheumatic disease therapies and the majority of other studies had a high risk of bias. Conclusion In this systematic review and meta‐analysis, hydroxychloroquine use was not associated with benefit or harm with regard to COVID‐19 mortality. The evidence supporting the effect of other antirheumatic disease therapies in COVID‐19 is currently inconclusive.

Purpose of the study To reduce the number of unnecessary laboratory tests ordered through a measurement of effects of education and cost awareness on laboratory ordering behaviour by internal medicine residents for common tests, including complete blood cell count (CBC) and renal profile (RP), and to evaluate effects of cost awareness on hospitalisation, 30-day readmission rate and mortality rate. Study design 567 patients admitted during February, March and April 2014 were reviewed as the control group. Total CBC, CBC with differential and RP tests were counted, along with readmission and mortality rates. Interventions were education and visual cost reminders. The same tests were reassessed for 629 patients treated during 12 months after intervention in 2015. Results Data showed a significant increase in CBCs ordered after the intervention (mean number per hospitalisation changed from 1.7 to 2.3 (p Conclusions Education in the form of cost reminders did not significantly reduce the overall ordering of the most common daily laboratory testing in our academic teaching service. We believe further research is needed to fully evaluate the effectiveness of other education forms on the redundant ordering of tests in the hospital setting.

Background: Longitudinally extensive transverse myelitis (LETM) accompanying systemic lupus erythematosus (SLE) is often due to coexisting aquaporin-4-IgG seropositive neuromyelitis optica spectrum disorder but has not been associated with myelin oligodendrocyte glycoprotein-IgG (MOG-IgG). Objective and methods: Case report at an academic medical center. Results: A 32-year-old woman developed severe transverse myelitis (paraplegia) shortly after SLE onset in the post-partum period. Magnetic resonance imaging (MRI) revealed an LETM, cerebrospinal fluid showed marked inflammation, and testing for infections was negative. Serum live-cell-based assay for MOG-IgG was positive but aquaporin-4-IgG was negative. Conclusion: In patients with SLE and LETM, MOG-IgG testing should be considered, in addition to AQP4-IgG.

Background: Takayasu’s Arteritis (TAK) is a rare granulomatous inflammation of the large vessels. Its incidence has been reported to be 2-3 cases per million people in the United States. To our knowledge an inpatient nationwide study of TAK in the United States has not been conducted to date. The aim was to evaluate the demographic characteristics, reason for admission, procedures, mortality and resource utilization of patients with TAK using a national database. Objectives: The primary goal was to estimate the national inpatient prevalence of admissions involving patients with TAK. Secondary outcomes were top reasons for admission, mortality, morbidity, resource utilization, length of hospital stay (LOS), and total hospitalization charges and costs, adjusted for inflation using the Consumer-Price Index. Methods: All patients with a principal ICD9 diagnosis code for TAK were included using the NIS 2013 and 2014, the largest publically available inpatient database in the US. Controls were 1:1 matched using a propensity score including age, gender, and Charlson Comorbidity Index (CCI). Multivariate logistic regression models were used to adjust for race, sex, patient zip code, income, CCI, hospital region, location, size and teaching status. Results: Of a total of 2,840 hospital admissions for patients with TAK, 2,680 were propensity-matched to admissions without TAK and included in the study. Mean age was 49 years, 81% were female. The inpatient prevalence of TAK was 4.6/100,000 admissions. After adjusting for confounders, patients with TAK had propensity-matched mortality odds of 1.44 (p=0.43) when compared to patients without TAK. The top reasons for admission were large vessel vasculitis, sepsis secondary to pneumonia and urinary tract infection, carotid artery stenosis/occlusion, and arthralgias. For secondary outcomes, patients with TAK displayed increased odds of stroke, aortic aneurysm, peripheral vascular disease and aortic valvulopathy when compared to patients without TAK. When CCI was evaluated it was noted that all the patients had an index lower than 4 and the great majority lower than 2 which could be related to the young age of this population. Despite the low CCI, these patients had 7.35 days of in-hospital stay which represented 2.1 days more when compared to non-TAK patients. For resource utilization, patients with TAK displayed higher odds of peripheral vascular intervention and arteriography when compared to patients without TAK. Patients with TAK displayed significantly higher adjusted total hospital costs, charges and LOS compared to patients without TAK. Conclusion: The inpatient prevalence was higher than suspected and could be related to the severity of the disease and complications associated with TAK. TAK was associated with an increased risk of stroke, aortic aneurysm, peripheral vascular disease and vascular interventions, but no increase mortality in this group of patients. The costs associated with hospitalization were substantial. References: [1] Watanabe Y, Miyata T, Tanemoto K. Current Clinical Features of New Patients With Takayasu Arteritis Observed From Cross-Country Research in Japan: Age and Sex Specificity. Circulation. 2015;132(18):1701-9. [2] Schmidt J, Kermani TA, Bacani AK, Crowson CS, Cooper LT, Matteson EL, et al. Diagnostic features, treatment, and outcomes of Takayasu arteritis in a US cohort of 126 patients. Mayo Clinic proceedings.2013;88(8):822-30. Disclosure of Interests: None declared

Background Giant cell arteritis (GCA) is a large vessel vasculitis that primarily affects the aorta and its branches. Extracranial branches of the carotid artery are frequently affected; however, intracranial involvement in GCA is rare [1]. Objectives To determine the clinical features and outcome of patients with intracranial giant cell arteritis (IC-GCA). Methods A retrospective medical record review was performed to identify all patients with IC-GCA from 1/1996 through 5/2018. Demographic, clinical, laboratory, radiographic, and treatment data at baseline and subsequent follow-up visits were collected. Data was summarized using descriptive statistics. Results The study cohort included 9 patients with IC-GCA (78% male, mean [±SD] age 72.1 [±7.9] years). 8 of 9 patients had positive temporal artery biopsies. One patient with negative biopsy had evidence of large-vessel GCA on thoracic CT angiography. The mean time from onset of GCA to intracranial involvement was 17 months (±38). All patients had neurologic symptoms, 33% (n:3) had a stroke and 22% (n:2) had a transient ischemic attack. IC-GCA was diagnosed by cranial imaging in 8 patients and by autopsy in one. Cranial imaging modalities used included magnetic resonance angiography (n=8), CT angiography (n=2) and cerebral angiography (n=2). Intracranial vasculitis most commonly affected the internal carotid artery 78% (N=7) followed by the, vertebral artery 56% (n=5), posterior cerebral artery 56% (n=5), middle cerebral artery 44% (n=4), anterior cerebral artery 33% (n=3) and posterior inferior cerebral artery 11% (n=1). Stenosis was present in 89%, occlusion in 33%, dilatation in 11%, and wall thickening in 11%. All patients received glucocorticoids. For treatment of intracranial disease, additional agents included: cyclophosphamide (67%), Tocilizumab (22%). Despite treatment, outcomes for patients with IC-GCA were poor. Five of nine patients died during a mean length of follow-up of 2.4 months. Conclusion Although rare, IC-GCA is associated with significant morbidity and mortality. It occurs predominantly in men, presenting with ischemic cerebrovascular events. Current treatment strategies appear to be of limited efficacy for IC-GCA. Reference [1] Salvarani C, Giannini C, Miller DV, Hunder G. Giant cell arteritis: Involvement of intracranial arteries. Arthritis Rheum. 2006;55(6):985-9. Disclosure of Interests None declared

To the Editor: Takayasu arteritis (TA) is a rare inflammatory condition of the large blood vessels that affects the aorta and its branches. Young females of Asian descent are typically the most affected by this disease; however, in the United States, most patients with TA are White1,2,3. Data regarding the epidemiology of TA is limited, with most studies of the incidence and prevalence of the disease coming from Japan, where TA is more prevalent. The described incidence rate of TA ranges from 0.3 to 3.3 per million per year and the prevalence ranges from 4.7 to 360 cases per million4. Only 2 studies have evaluated the epidemiology of TA in the US; one of them demonstrated an incidence rate of 2.6 cases per million per year in Olmsted County, Minnesota5, and the other study described a prevalence of 0.9 cases per million in New York, which is the …

Infiltrative cardiomyopathies include a variety of disorders that lead to myocardial thickening resulting in a constellation of clinical manifestations and eventually heart failure that could be the first clue to reach the diagnosis. Among the more described infiltrative diseases of the heart is amyloid cardiomyopathy. The disease usually presents with subtle, nonspecific symptoms. Herein, we illustrate a case of recurrent syncope as the initial presenting symptom for systemic amyloid with polyneuropathy and cardiomyopathy as a cause of syncope. The article illustrates the role of advanced cardiac imaging in the diagnosis of the disease with a focused literature review. We also highlight the role of early, shared decision-making between patient, family, and medical team in the management of cardiac amyloidosis.

Pericarditis is the most common pericardial disease worldwide, and can be recurrent in one third of the patients with viral and idiopathic pericarditis [1]. The diagnosis of pericarditis should be based on clinical criteria, history, clinical findings, electrocardiographic (ECG) changes and evidence of a pericardial effusion [2]. Elevation of the inflammatory markers and evidence of pericardial inflammation by an imaging technique such as contrast enhancement on the pericardium-CT scan or pericardial edema and pericardial late gadolinium enhancement on cardiac magnetic resonance imaging (MRI) are additional supportive criteria in the diagnosis [1].

In recent years there has been growing interest in the use of tocilizumab for the treatment of large vessel vasculitis, particularly giant cell arteritis.1 Nakaoka et al recently suggested that tocilizumab may be of therapeutic benefit in patients with relapsing Takayasu arteritis (TAK).2 In this journal, a case of aortic ulceration in a patient with TAK while on tocilizumab was described.3 We report two additional patients who had disease progression despite tocilizumab therapy. ### Case 1 A 44-year-old woman with a 20-year history of seropositive rheumatoid arthritis in clinical remission on adalimumab presented with new-onset right upper extremity claudication. A diagnosis of TAK was made after angiography demonstrated arterial thickening of the aorta and arch branches with multifocal narrowing of the subclavian and common carotid arteries. …