Primary Systemic Vasculitis Research Articles

Cardiac involvement and cardiovascular risk factors in pediatric primary systemic vasculitides.

Pediatric primary systemic vasculitides are a complex group of diseases. Vasculitis subgroups are mainly determined according to the size of the predominantly affected vessels. In patients with primary systemic vasculitis, the location of vascular involvement, the size of the vessels, the extent of vascular damage, and the underlying pathology determine the disease phenotype and severity. Cardiac involvement is rare in some pediatric vasculitis, such as IgA vasculitis and polyarteritis nodosa, while it is more common in some others like Kawasaki disease and Takayasu arteritis. On the other hand, chronic inflammation in the setting of systemic vasculitis forms a major cardiovascular risk factor. Accelerated atherosclerosis and the tendency to thrombosis are the main issues determining the cardiovascular risks in pediatric systemic vasculitis. Early diagnosis and treatment are essential in these patients to minimize morbidity and mortality. In this review, we aimed to raise physicians' awareness of cardiac involvement and cardiovascular risks in pediatric patients with primary systemic vasculitis.

A Spotlight on Drug-Induced Vasculitis.

Drug-induced vasculitis (DIV) is a rare form of vasculitis related to the use of various drugs. DIV primarily affects small to medium size vessels, but it can potentially involve vessels of any size. Differentiating between primary systemic vasculitis and DIV can be challenging; however, it is crucial, so that the offending agent can be discontinued promptly. The clinical phenotype of DIV is protean and depends on the size of the affected vessels. It ranges from arthralgias, to an isolated cutaneous rash, to severe single or multi-organ involvement. While withdrawal of the offending drug is the most important step in management, a significant number of patients require immunosuppressive therapy for varying periods of time. DIV can affect any vascular bed size, leading to protean vasculitic syndromes. Increased awareness among general practitioners, specialty, andsubspecialty physicians is crucial for early recognition, and withdrawal of drug for better outcomes.

Alemtuzumab for refractory primary systemic vasculitis\u2014a randomised controlled dose ranging clinical trial of efficacy and safety (ALEVIATE)

BackgroundPrimary systemic vasculitis (PSV) is a heterogeneous group of autoimmune conditions. There is an unmet need for alternative therapies that lead to sustained remission in patients with refractory disease. Alemtuzumab, an anti-CD52 antibody, depletes lymphocytes for prolonged periods and, in retrospective studies, has induced sustained, treatment-free remissions in patients with refractory/relapsing vasculitis but has raised safety concerns of infection and secondary autoimmunity. This phase IIb clinical trial aimed to assess the efficacy and safety of alemtuzumab, at two different doses, in inducing remission in refractory vasculitis patients.MethodsThe ALEVIATE trial was a randomised, prospective, open-label, dose ranging clinical trial. Patients with refractory ANCA-associated vasculitis (AAV) or Behçet’s disease (BD) were randomised to receive either 60 mg or 30 mg alemtuzumab. Treatments were administered at baseline and 6 months or earlier where clinically appropriate. A maximum of three treatments were allowed within the 12-month study period.ResultsTwenty-three patients received at least one dose of alemtuzumab. Twelve had AAV, and 11 a diagnosis of BD. The median age was 40 years (range 28–44), with a prior disease duration of 61 months (42–103). Sixteen (70%) achieved either complete (6/23, 26%) or partial (10/23, 44%) response at 6 months. Eight (35%) maintained remission to the end of the trial without relapse. Ten severe adverse events were observed in 7 (30%) patients; 4 were related to alemtuzumab. There were no differences in clinical endpoints between the 60 and 30 mg alemtuzumab treatment groups.ConclusionIn a selected group of refractory vasculitis patients, alemtuzumab led to remission in two thirds of patients at 6 months. Remission was maintained to 12 months in a third of the patients, and the safety profile was acceptable.Trial registrationClinicalTrials.gov identifier: NCT01405807, EudraCT Number: 2009-017087-17. Registered on April 07, 2011.

Primary vasculitides in childhood and adulthood

Primary systemic vasculitides can be observed at any age. Some vasculitides occur preferentially in childhood, such as Kawasaki syndrome or immunoglobulinA (IgA) vasculitis, whereas others, such as giant cell arteritis, occur beyond the age of 50years. Vasculitides occurring in childhood or adolescence and adulthood may have different phenotypes, different disease courses and outcomes depending on the age of manifestation. For example, those with Takayasu arteritis beginning in adolescence have different vascular involvement, ahigher degree of systemic inflammation and amore aggressive course of disease than those with adult-onset disease. In contrast, IgA vasculitis is more severe in adults than in children. The causes for the age predilections and different age-dependent disease manifestations have not yet been clarified. The therapeutic principles are similar for vasculitides occurring in children or adolescents and adults. The first international evidence-based treatment recommendations are now available for juvenile vasculitides, although the evidence for certain forms of treatment is still very limited. The treatment of adult vasculitides can be guided by numerous national and international guidelines and recommendations. Many vasculitides carry ahigh risk of morbidity and mortality and the timely detection and treatment are therefore necessary. In this article, similarities and differences in the clinical presentations, treatment, courses and prognosis of vasculitides in children or adolescents and adults are discussed.

A Case of Takayasu Arteritis in a Male Patient

Takayasu arteritis (TA), otherwise called “pulseless disease”, is a category of primary systemic vasculitis involving medium and large arteries, including the aorta, its branches and the pulmonary and the coronary arteries. The presence of TA is often observed in women and the main population of patients develops their first symptoms between 15 years to 30 years. If left untreated, this disease may lead to the development of many comorbid renal and pulmonary obstructions causing lethal consequences. Here we present a case report of the diagnosis of TA in a 25-year-old male, treatment and following procedures. A patient with no anamnesis was hospitalized with paralysis of the right half of body and altered consciousness. He had been experiencing symptoms like fatigue, general weakness and respiratory distress for the past year. After extensive medical analysis patient was treated with oral prednisone and later endarterectomy was performed. After the operation, the patient improved and was discharged. Till date, no new complaints were reported.

Complications and causes of mortality in patients with primary necrotizing systemic vasculitis

The article reviews the clinical symptoms, main complications in patients with ANCA-associated systemic vasculitis (granulomatous with polyangiitis, microscopic polyangiitis, eosinophilic granulomatous with polyangiitis) and polyarteritis nodosa during the first three years of the disease. We evaluated the relationship between complications and long-term glucocorticosteroid therapy. Patients with granulomatosis had the most unfavourable course of the disease with a high risk of complications. A high incidence of ischemic strokes and chronic kidney disease in patients with microscopic polyangiitis has been identified. We also analyzed cases of lethal outcomes in patients with primary systemic vasculitis.

Determination of Red Blood Cell Distribution Width in Patients with Primary Cutaneous Vasculitis Compared to Systemic Vasculitis

Introduction: Red blood cell distribution width (RDW) has been considered as an inflammatory marker in various disorders. Evaluation of RDW value can also be used as a novel and additional marker for differentiating systemic vasculitis from primary cutaneous vasculitis. Objective: To compare RDW value between patients with cutaneous vasculitis with systemic vasculitis, thereafter to find out it's role as an effective indicator to distinguish both forms of vasculitis. Materials and Methods: This cross sectional observational study was conduct between from July 2016 to December 2017. Total of 48 patients were divided into primary cutaneous vasculitis and systemic vasculitis. Blood was collected in EDTA tube to measure RDW value. Patient’s disease activity also scored and plotted according to Birmingham vasculitis activity score. Statistical analysis was performed by using SPSS. Results: Significantly high mean RDW were found in patients with systemic vasculitis compared to primary cutaneous vasculitis (15.09±0.92 vs. 13.48±1.1, p = 0.000). BVAS was significantly greater (13.93±5.10 vs. 4.87±2.69, p = < 0.001) in systemic vasculitis as well as in patients with high RDW group (11.73±5.71 vs. 5.37±3.96, p = < 0.001). Optimal RDW cut off point for differentiating systemic vasculitis from cutaneous vasculitis was 14.2 with 81.3% sensitivity and 81.2% specificity. Conclusion: Present study revealed importance of RDW monitoring along with disease activity in patients with any form of vasculitis. Systemic vasculitis had higher level of RDW. So RDW can be considered as a marker to discriminate systemic vasculitis from primary cutaneous vasculitis. Medicine Today 2021 Vol.33(2): 84-89

Outcomes of COVID-19 in patients with primary systemic vasculitis or polymyalgia rheumatica from the COVID-19 Global Rheumatology Alliance physician registry: a retrospective cohort study.

BackgroundPatients with primary systemic vasculitis or polymyalgia rheumatica might be at a high risk for poor COVID-19 outcomes due to the treatments used, the potential organ damage cause by primary systemic vasculitis, and the demographic factors associated with these conditions. We therefore aimed to investigate factors associated with COVID-19 outcomes in patients with primary systemic vasculitis or polymyalgia rheumatica.MethodsIn this retrospective cohort study, adult patients (aged ≥18 years) diagnosed with COVID-19 between March 12, 2020, and April 12, 2021, who had a history of primary systemic vasculitis (antineutrophil cytoplasmic antibody [ANCA]-associated vasculitis, giant cell arteritis, Behçet's syndrome, or other vasculitis) or polymyalgia rheumatica, and were reported to the COVID-19 Global Rheumatology Alliance registry were included. To assess COVID-19 outcomes in patients, we used an ordinal COVID-19 severity scale, defined as: (1) no hospitalisation; (2) hospitalisation without supplemental oxygen; (3) hospitalisation with any supplemental oxygen or ventilation; or (4) death. Multivariable ordinal logistic regression analyses were used to estimate odds ratios (ORs), adjusting for age, sex, time period, number of comorbidities, smoking status, obesity, glucocorticoid use, disease activity, region, and medication category. Analyses were also stratified by type of rheumatic disease.FindingsOf 1202 eligible patients identified in the registry, 733 (61·0%) were women and 469 (39·0%) were men, and their mean age was 63·8 years (SD 17·1). A total of 374 (31·1%) patients had polymyalgia rheumatica, 353 (29·4%) had ANCA-associated vasculitis, 183 (15·2%) had giant cell arteritis, 112 (9·3%) had Behçet's syndrome, and 180 (15·0%) had other vasculitis. Of 1020 (84·9%) patients with outcome data, 512 (50·2%) were not hospitalised, 114 (11·2%) were hospitalised and did not receive supplemental oxygen, 239 (23·4%) were hospitalised and received ventilation or supplemental oxygen, and 155 (15·2%) died. A higher odds of poor COVID-19 outcomes were observed in patients who were older (per each additional decade of life OR 1·44 [95% CI 1·31–1·57]), were male compared with female (1·38 [1·05–1·80]), had more comorbidities (per each additional comorbidity 1·39 [1·23–1·58]), were taking 10 mg/day or more of prednisolone compared with none (2·14 [1·50–3·04]), or had moderate, or high or severe disease activity compared with those who had disease remission or low disease activity (2·12 [1·49–3·02]). Risk factors varied among different disease subtypes.InterpretationAmong patients with primary systemic vasculitis and polymyalgia rheumatica, severe COVID-19 outcomes were associated with variable and largely unmodifiable risk factors, such as age, sex, and number of comorbidities, as well as treatments, including high-dose glucocorticoids. Our results could be used to inform mitigation strategies for patients with these diseases.FundingAmerican College of Rheumatology and the European Alliance of Associations for Rheumatology.

The sensitivity of temporal artery biopsy in the detection of giant cell arteritis is independent of tissue length

Abstract Introduction/Objective Giant cell arteritis (GCA) is the most common vasculitis of the elderly, and the most common primary systemic vasculitis overall, with an annual incidence of 200/million. The long term sequelae, namely vision loss and stroke, are permanent and devastating. While GCA is often treated empirically based on clinical presentation, panarteritis on temporal artery biopsy is required for diagnosis. However, these biopsies have the tendency to be falsely negative due to skip lesions, a common feature of GCA. Therefore, we set out to determine whether longer biopsy specimens were more sensitive in the detection of GCA. Methods/Case Report A census of temporal artery biopsies performed with the indication of clinical symptoms of GCA was taken at our institution. The patient age, sex, biopsy laterality, biopsy length, and pathological diagnosis were recorded for each cataloged sample. Statistical significance of difference in biopsy length was tested using an unpaired t-test in R 4.1.0. Results (if a Case Study enter NA) A total of 114 temporal artery specimens were biopsied from 94 different patients with the indication of GCA and assigned a definitive positive or negative diagnosis. Of the 94 patients, 54 were female and 40 were male. Of the total pathological specimens, 11 were positive and 103 were negative. The overall average length of biopsy specimens was 2.13 cm with a standard deviation of 0.65 cm. The average positive biopsy was 2.26 cm long, and the average negative was 2.12 cm, an insignificant difference (0.14 cm, t = 0.7, p = 0.43). In 25 patients, biopsies were taken from both the left and right temporal arteries. Of those patients, 2 were positive for GCA and the remaining 23 were negative. Interestingly, the biopsy result in every case was identical between the left and right samples; we found no instances of pathological evidence of GCA in only one of the two samples from the same patient. Conclusion According to data taken at our institution, there is no indication to lengthen the biopsy requirements from the existing 1.5 cm. However, we have demonstrated evidence that it may be unnecessary to biopsy both temporal arteries in a single patient. Larger studies would be required to confirm our findings.

Predictors of cardiovascular events in patients with primary systemic vasculitis: a 5-year prospective observational study

Abstract Introduction Granulomatosis with polyangiitis (GPA) is one of antineutrophil cytoplasmic autoantibody (ANCA)-associated systemic vasculitis and is characterised by necrotizing inflammation of small/medium-sized blood vessels. Systemic vasculitis exhibits an enhanced cardiovascular morbidity and cardiovascular disease (CVD) has become a leading cause of death in this group of patients. Objectives The aim of the present study was to assess the prevalence of clinical manifestation of atherosclerosis and its relation with classic risk factors for atherosclerosis, echocardiographic parameters and laboratory findings in GPA patients. Patients and methods The group of consecutive patients with GPA were followed in the study. In all patient's echocardiography and laboratory tests were performed. Results One hundred six patients with GPA (mean age 50.4±14.9 yrs, 67 female) were prospectively followed for 5.1±1.6 yrs. In 19 patients (18%) cardiovascular disease (9 acute coronary syndromes, 4 symptomatic peripheral vascular diseases and 6 strokes) occurred in association with GPA. Analysis showed that the levels of hs-CRP on both visits correlated with BVAS, PR3-ANCA, D-dimer and creatinine. In a multivariate model, only age was predictive of cardiovascular events in this group of patients (OR=1.078, 95% CI: 1.025–1.134, p=0.003). During observation in patients without CVD the level of hs-CRP and D-dimer were significantly reduced on the follow-up visit (p=0.041, p=0.0002). On the other hand, in patients with CV events there was no significant differences in both markers' concentrations despite clinical remission. Conclusions Patients with granulomatosis with polyangiitis have high risk of cardiovascular disease. The age was the only independent predictor of cardiovascular events. Persistent elevation of inflammatory and prothrombotic markers despite clinical remission of the disease could be an indicator of premature atherosclerosis development in patients with systemic vasculitis. Funding Acknowledgement Type of funding sources: None.

Cardiac phenotypes in the acute-phase of microscopic polyangiitis involves dilatation of the left atrium caused by LV diastolic dysfunction

Abstract Background Microscopic polyangiitis (MPA) is a type of primary systemic vasculitis that affects various organs, especially the lungs and kidneys. However, few reports regarding cardiac features of MPA patients were found. Purpose We aim to investigate the echocardiographic parameters of acute-phase MPA. Methods and results This single-center retrospective study included 15 patients with MPA (Mean age at 72.2±7.1 years, women 73.3%) who underwent echocardiography within two weeks of commencing steroid therapy for induction or reinduction. The echocardiography parameters of the patients were compared with those of 30 age and sex-matched controls. In the MPA group, the commonly affected organs were kidneys (93.3%) and lungs (46.7%); 5 patients (33.3%) had a history of hypertension, which had a similar frequency as the control group. No significant difference in left ventricular (LV) diameter, LV ejection fraction, e', or inferior vena cava diameter was observed between the two groups. However, the MPA group showed significantly higher left atrial (LA) diameter (p=0.033) and LA volume index (p=0.001), as well as higher early diastolic filling velocity (E-wave, p=0.015; E/A, p=0.043; E/e', p=0.041), diastolic pulmonary venous flow velocity (p=0.013), trans-tricuspid pressure gradient (p=0.019), and shorter deceleration time (p=0.038), associated with mildly thicker ventricular walls of left ventricle (LV) than the control group. Moreover, serum levels of C-reactive protein showed significant correlation between E wave (r=0.58, p=0.023), E/A (r=0.67, p=0.006), and deceleration time (r=−0.69, p=0.005) in the MPA group. These results may indicate that in MPA, increased LV stiffness, rather than impairment of LV relaxation was contributed to LV diastolic function, resulting in LA enlargement. Conclusion Patients with acute-phase MPA had LA dilatation associated with LV diastolic dysfunction. This finding indicates the importance of cardiac assessment in patients with MPA. Funding Acknowledgement Type of funding sources: None.

Surgical Repair of a Ruptured Giant Abdominal Aortic Aneurysm in a 16-Year-Old with Takayasu’s Arteritis: Case Report and Etiological Review

Takayasu’s arteritis is a type of primary systemic vasculitis that affects medium and large arteries, including the aorta and its main branches, as well as the pulmonary and coronary arteries. Although rare in children, it is the third most common vasculitis in the pediatric population, often with delayed diagnosis due to the nonspecific presentation of clinical symptoms in its initial phase. This is a case of a 16-year-old girl with a giant ruptured abdominal aortic aneurysm, who needed surgery [...]

Predictors of cardiovascular events in patients with primary systemic vasculitis: A 5 years prospective observational study

Introduction: Granulomatosis with polyangiitis (GPA) is one of antineutrophil cytoplasmic autoantibody (ANCA) – associated systemic vasculitis and is characterised by inflammation of blood vessels. Systemic vasculitis exhibits an enhanced cardiovascular morbidity and cardiovascular disease (CVD) has become a leading cause of death in this group of patients. Objectives: The aim of the present study was to assess the prevalence of clinical manifestation of atherosclerosis and its relation with classic risk factors for atherosclerosis, echocardiographic parameters and laboratory findings in GPA patients. Patients and methods: The group of consecutive patients with GPA were followed in the study. Results: One hundred six patients with GPA (mean age 50.4 ± 14.9 yrs, 67 female) were prospectively followed for 5.1 ± 1.6 yrs. In 19 patients (18%) cardiovascular disease (9 acute coronary syndromes, 4 symptomatic peripheral vascular diseases and 6 strokes) occurred in association with GPA. In a multivariate model, only age was predictive of cardiovascular events in this group of patients (OR=1.078, 95% CI: 1.025–1.134, p = 0.003). During observation in patients without CVD the level of hs-CRP and D-dimer were significantly reduced on the follow-up visit (p = 0.041, p = 0.0002). On the other hand, in patients with CV events there was no significant differences in both markers’ concentrations despite clinical remission. Conclusions: The age was the only independent predictor of cardiovascular events. Persistent elevation of inflammatory and prothrombotic markers despite clinical remission of the disease could be an indicator of premature atherosclerosis development in patients with systemic vasculitis.

A Rare Cause of Young Stroke: What Rheumatologists Should Know about Fabry Disease

Systemic vasculitis is a category of autoinflammatory diseases usually involving multiple organs. Involvement of the central nervous system (CNS) may mimic other disorders, hence resulting in overdiagnosis. This is a case report of a young man who presented with acrodynia, episodic pyrexia, and recurrent strokes. He was treated for systemic vasculitis with glucocorticoids and immunosuppressants, which failed to prevent the stroke. Finally, Fabry disease was diagnosed after [Formula: see text]-galactosidase A gene mutation analysis was done. Rheumatologists should be aware of Fabry disease, which may present with multi-system involvement that mimics vasculitis. To avoid inappropriate treatment, a comprehensive differential diagnosis should be considered before a diagnosis of primary systemic vasculitis is made. Recurrent strokes, especially in males younger than 40, should raise suspicions of Fabry disease.

POS1243 TELEMEDICINE AND MANAGEMENT OF THE PATIENT AFFECTED BY GIANT CELL ARTERITIS DURING THE COVID-19 PANDEMIC - TELEMACOV

Background:Giant cell arteritis (GCA) is the most common primary systemic vasculitis in western countries with the highest incidence among persons 70–79 years of age. Treatment has been with glucocorticoids (GCs) alone for many decades but recently Tocilizumab (TCZ) has demonstrated efficacy in reducing GC dose and flare rates in patients with GCA. Therefore, both early diagnosis and regular monitoring are necessary for the correct management of GCA. The COVID-19 pandemic has led to decisions by the governments of the countries involved, aimed above all at reducing the contagion. This has also led to reductions in health activities, limiting them to those of urgency by reducing or canceling checkups involving the risk of a time gap which for the GCA meant the interruption of clinical monitoring and therapeutic adjustment. At the same time, the pandemic situation has stimulated remote monitoring activities, through telephone contacts or video calls carried out by the rheumatologist. EULAR identified a minimal data set aimed at research and for clinical use, which includes the main clinical and instrumental data to be taken into consideration in monitoring the patient. For many data a clinical examination is not necessary but an interview is sufficient. We activated the TELEMACOV protocol (TELEmedicine and Management of the patient affected by giant cell arteritis during the COVid-19 pandemic) monitoring the follow-up of patients affected by GCA through telemedicine tools in order to maintain an effective and risk-free follow-up in a pathology with a high risk of relapse.Objectives:The purpose of the study is to evaluate the effectiveness of telemedicine in the follow-up of the patient with GCA.Methods:We evaluated patients (pts) with a clinical diagnosis of GCA (received in previous periods) who were admitted to the our Rheumatology Unit. They were monitored monthly by telephone from 9 March to 9 June 2020 (during lockdown). All patients were asked questions divided according to the sub-groups listed below:- Onset of new symptoms or their recurrence- Exams carried out- Current therapy- Satisfaction of telephone callResults:We performed 148 remote monitoring visits in 37 pts. The cohort was mainly composed by female (77,8%) and had a mean age of 71,85 ± 9,25 years. They were affected by GCA, with a mean duration of 5,3±2,3 months. The characteristics of these pts and the course of the disease are reported in Table 1. Pts treated with TCZ reduced their GC dose more than patients treated with GC alone (p: 0.032). Only one patient (treated with GC alone) had an ocular flare with the need to increase the dosage of the GC with good response and rapid improvement. Furthermore, all patients considered this type of monitoring very satisfactory according to the Likert scale (1-5) with mean 4,4±0,2.Conclusion:Our study has shown how telemedicine can be well used in pts with GCA as a possible alternative, for a limited period, to traditional visits, especially in a fragile population such as the elderly and more exposed to the risk of SARS-COV2 infection.

AB0384 IMPACT OF TEMPORAL ARTERY ULTRASOUND ON SURVIVAL OF PATIENTS WITH GIANT CELL ARTERITIS

Background:Giant cell arteritis (GCA) is the most common primary systemic vasculitis in adults over 50 years of age. Its incidence increases with age, with a peak between 70-80 years and predominates in women, 3:1. It is a medical emergency that, if not diagnosed, can lead to irreversible complications. The delay in time from diagnosis to start of treatment is crucial to avoid possible serious outcomes on short, medium and long term. Survival in GCA is estimated between 60-90% at 5 years and 48-81% at 10 years. Efforts have been made to implement rapid diagnostic circuits to assess patients and initiate treatment without delay with good results both in reducing permanent vision loss and in reducing the costs of these patients due to emergency visits and admissions. The morbidity and mortality of this disease is high, but the use of efficient diagnostic strategies, such as ultrasound of superficial temporal arteries, has proven to be a useful, practical, cost-effective and, above all, quick tool to make the diagnostic approach.Objectives:Analyze the impact of early temporal artery ultrasound on survival for patients with GCA.Methods:Survival study of 48 patients with GCA, in two different “stages” in terms of diagnostic approach: Group A (n = 27), patients diagnosed between 2002 - 2011 using only ACR 1990 criteria and Group E (n = 21) diagnosed between 2010-2015 using ACR criteria and TAUS. TAUS was performed by Rheumatologists with extensive experience in ultrasound and within a period of no more than 7 days for these patients. The definitive diagnosis of GCA was based on the clinical criteria of the Rheumatologist within the clinical and analytical context and with the specific complementary examinations for each case (Ultrasound, PET-CT, biopsy). Demographic data, comorbidities, signs and symptoms at debut, analytical data, complementary examinations, treatment and evolution were obtained retrospectively through the electronic medical record of the patient, based on the database of our GCA cohort. A survival analysis was performed considering death as the main outcome. The statistic used was the Kaplan-Meier test. In addition, other complications related to treatment or pathology are collected.Results:The mean age at diagnosis of our patients was 79 + - 6 years, with a female: male ratio of 3: 1. The follow-up was between 2 and 16 years with a mean of 5.8 + - 3 years, until the last visit collected or until the outcome of death. Group A had a survival at 5 and 10 years of 53.4% and 36.7% respectively, while group E of 79.5% at both cut-off points. (Figure 1).There is a significant difference between the survival of both groups, p <0.01, this being better in the group in which TAUS was implemented for rapid diagnosis (group E). The main causes of death were cardiovascular events, 30%, predominantly in group E (75%), and infection, 30%, predominantly in group A. The median from diagnosis to death was 3 years (range 1 - 13).Figure 1.Group A (red line) according to ACG 1990 criteria and Group B (green line) according to ACG criteria and implementing TAUS for rapid diagnosisConclusion:The implementation of temporal artery ultrasound (TAUS) is associated with a significant improvement in the survival rate of patients with GCA and a reduction in treatment-related complications in patients who were diagnosed with ultrasound in less than 7 days compared to those diagnosed by the conventional healthcare attention routes.

P096 Pregnancy outcomes in women attending a joint obstetric and rheumatology clinic in a tertiary centre over a 2-year period

Abstract Background/Aims The Lupus in Pregnancy Scanning (LIPS) clinic, a joint obstetrics and rheumatology clinic was established in 2010 at Saint Mary’s Hospital, Manchester, UK for women with systemic lupus erythematosus (SLE) and other complex rheumatological conditions. We aimed to describe pregnancy outcomes of women attending this clinic to establish a baseline for future changes aimed at improving the service. Methods Data were collected retrospectively from electronic records of patients who attended the LIPS clinic at least once between 1st January 2018 and 31st December 2019. Results Pregnancy outcomes were available in 105/125 (84%) women (Table). The median age [inter-quartile range] was 30.6 years [IQR 27.7 - 33.6] and 40 (38%) were of non-Caucasian background. Sixty-one (58%) had a connective tissue disease (CTD) of whom 36 (59%) had SLE. Other rheumatological diagnoses included inflammatory arthritis, primary anti-phospholipid syndrome (APS) and systemic vasculitis. Anti-Ro was found in 32 (31%) and anti-phospholipid antibodies in 25 (24%). During pregnancy, 65 (62%) received aspirin and 40 (38%) had low molecular weight heparin (LMWH). In the antenatal period, 43 (41%) took steroids, 52 (50%) had conventional disease modifying anti-rheumatic drugs and 8 (8%) received biologics. Active disease in the antenatal period was noted in 14 (13%) women. Regarding pregnancy outcomes (Table), still-births were low (0.95%). The rate of C-sections (45%) and assisted deliveries (19.6%) was comparable to previously published data from similar clinics. P096 Table 1:Pregnancy outcomes over a 2-year period in women attending the LIPS clinic at St. Mary's HospitalPregnancy Outcomen (%)Miscarriage3 (2.9)Deliveries• Live101 (96.2)• Still-birth1 (0.95)Median Gestation (weeks) [inter-quartile range (IQR)]38 [37 - 39]Sex of Neonate• Female57 (56)• Male45 (44)Mode of Delivery• Normal vaginal36 (35.3)• Assisted20 (19.6)• Elective Caesarean Section15 (14.7)• Emergency Caesarean Section31 (30.4)Median Neonatal Birth Weight (grams) [IQR]3137 [2724 - 3428]• Low Birth Weight <2500g15 (15)Maternal Complications (Antenatal and Peri-Partum)• Infection22 (22)• Pre-Eclampsia1 (1)• Post-Partum Haemorrhage56 (55)Neonatal Intensive Care Admission7 (7)Neonatal Complications• Sepsis1 (1)• Congenital Heart Block2 (2)• Prematurity (<36 weeks)39 (38) Conclusion In this cohort we report a high live birth rate and comparable rates of assisted delivery to similar cohorts. Infection and post-partum haemorrhage are maternal complications that are common, and reflect the complex clinical presentations and therapeutic regimes in these conditions. Overall this specialist clinic achieves favourable maternal and foetal outcomes in this high-risk population. Disclosure T. David: None. R. Hum: None. Y. Lau: None. S. Thornber: None. L. Simcox: None. I. Bruce: None. C. Tower: None. P. Ho: None.

Trend and Geographic Disparities in the Mortality Rates of Primary Systemic Vasculitis in the United States from 1999 to 2019: A Population-Based Study.

The current data on rates and geographic distribution of vasculitis mortality are limited. We aimed to estimate the mortality rates of primary systemic vasculitis and its geographic distribution using recent population data in the United States. The mortality rates of vasculitis from 1999 to 2019 were obtained from the Center for Disease Control (CDC) Wonder Multiple Cause of Death (MCD). The age-adjusted rates per million for vasculitis as MCD and as an underlying cause of death (UCD) were calculated by state using demographics. A joinpoint regression analysis was applied to evaluate trends over time. The age-adjusted mortality rate of vasculitis as MCD was 4.077 (95% CI: 4.029–4.125) and as a UCD was 1.888 per million (95% CI: 1.855–1.921). Since 1999, mortality rates have progressively decreased. The age-adjusted mortality rate was higher in females than in males. The highest mortality rate for vasculitis as MCD was in White patients (4.371; 95% CI: 4.317–4.424). The northern states and areas with lower populations had higher mortality rates. We found a trend of progressive decreases in the mortality rates of vasculitis, as well as gender, racial, and geographic disparities. Further analyses are warranted to better understand the factors associated with these disparities in order to implement targeted public health interventions to decrease them.

Immune Axonal Neuropathies Associated With Systemic Autoimmune Rheumatic Diseases.

Immune axonal neuropathies are a particular group of immune-mediated neuropathies that occasionally accompany systemic autoimmune rheumatic diseases such as connective tissue dissorders and primary systemic vasculitides. Apart from vasculitis of vasa nervorum, various other mechanisms are involved in their pathogenesis, with possible therapeutic implications. Immune axonal neuropathies have highly heterogeneous clinical presentation and course, ranging from mild chronic distal sensorimotor polyneuropathy to severe subacute mononeuritis multiplex with rapid progression and constitutional symptoms such as fever, malaise, weight loss and night sweats, underpinning a vasculitic process. Sensory neuronopathy (ganglionopathy), small fiber neuropathy (sensory and/or autonomic), axonal variants of Guillain-Barré syndrome and cranial neuropathies have also been reported. In contrast to demyelinating neuropathies, immune axonal neuropathies show absent or reduced nerve amplitudes with normal latencies and conduction velocities on nerve conduction studies. Diagnosis and initiation of treatment are often delayed, leading to accumulating disability. Considering the lack of validated diagnostic criteria and evidence-based treatment protocols for immune axonal neuropathies, this review offers a comprehensive perspective on etiopathogenesis, clinical and paraclinical findings as well as therapy guidance for assisting the clinician in approaching these patients. High quality clinical research is required in order to provide indications and follow up rules for treatment in immune axonal neuropathies related to systemic autoimmune rheumatic diseases.

Pulmonary involvement in primary systemic vasculitides.

This study describes the spectrum and initial impact of pulmonary manifestations in the primary systemic vasculitides. Description and comparison of pulmonary manifestations in adults with Takayasu's arteritis (TAK), GCA, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic GPA (EGPA), polyarteritis nodosa (PAN) and IgA vasculitis (IgAV), using data collected within the Diagnostic and Classification Criteria in Vasculitis study. Data from 1952 patients with primary vasculitides were included: 170 TAK, 657 GCA, 555 GPA, 223 MPA, 146 EGPA, 153 IgAV and 48 PAN. Pulmonary manifestations were observed in patients with TAK (21.8%), GCA (15.8%), GPA (64.5%), MPA (65.9%), EGPA (89.0%), PAN (27.1%) and IgAV (5.9%). Dyspnoea occurred in patients with TAK (14.7%), GCA (7.8%), GPA (41.8%), MPA (43.5%), EGPA (65.8%), PAN (18.8%) and IgAV (2.6%). Cough was reported in TAK (7.6%), GCA (9.3%), GPA (34.8%), MPA (37.7%), EGPA (55.5%), PAN (16.7%) and IgAV (3.3%). Haemoptysis occurred mainly in patients with ANCA-associated vasculitis (AAV). Fibrosis on imaging at diagnosis was documented in GPA (1.9%), MPA (24.9%) and EGPA (6.3%). Only patients with AAV (GPA 2.7%, MPA 2.7% and EGPA 3.4%) required mechanical ventilation. At 6 months, the presence of at least one pulmonary item in the Vasculitis Damage Index was observed in TAK (4.1%), GCA (3.3%), GPA (15.4%), MPA (28.7%), EGPA (52.7%), PAN (6.2%) and IgAV (1.3%). Pulmonary manifestations can occur in all primary systemic vasculitides, but are more frequent and more often associated with permanent damage in AAV.