Case Of ANCA-associated Vasculitis Research Articles

B-074 Comparative Evaluation of Clinical Utility between Indirect Immunofluorescence and Immunoassay Methods for Antineutrophil Cytopalsmic Antibody Detection

Abstract Background ANCA testing is essential for the diagnosis of vasculitis, commonly using indirect immunofluorescence(IIF) and immunoassay(IA) methods. Recent guidelines recommend IA as the primary test for suspected GPA and MPA, but actual practice varies among physicians. Here, we retrospectively analyzed ANCA test results, focusing on new cases of ANCA-associated vasculitis, and compared the utility of IIF and IA to guide appropriate test selection. Methods From January 2022 to July 2023, 24 new cases of ANCA-associated vasculitis were diagnosed. We checked whether ANCA testing was performed and the results, and compared the turnaround time(TAT) and costs. IIF ANCA tests were performed on a computer-based automated instrument(Helios), and IA ANCA tests were performed on automated instruments(Alegria, Phadia 250) with MPO antigen and PR3 antigen tested simultaneously in a bundled prescription. Results Among the 24 cases, there were 6 GPA, 7 MPA, 7 EGPA, and 4 ANCA-associated GN, with varying diagnostic sensitivities. GPA and EGPA had different ANCA positivity rates than previously reported, suggesting potential ethnic variations in ANCA expression and positivity rates. The test sequence and results, summarized in Table 1, showed 21 out of 22 cases had identical concordance between methods, with an excellent concordance rate (kappa value=0.83). TAT for IIF was 3,452 minutes, and for IA was 1,491 minutes for the whole period, but IA TAT increased to 2,748 minutes after implementing a days-of-the-week testing system. Test costs, with a relative value score of 118.99 for IIF and 156.05 for IA, indicated IA cost approximately 2.6 times higher, considering the bundled prescription of both antigens for IA ANCA testing. Conclusions Evaluating the utility of both methods for ANCA detection, our study highlights high inter-method agreement in ANCA-associated vasculitis. Considering overall cost and turnaround time, IIF ANCA testing remains effective as a screening test, demonstrating standalone effectiveness.

A Case of ANCA-Associated Vasculitis with Glomerulonephritis Unmasked by Electron Microscopy

A Case of ANCA-Associated Vasculitis with Glomerulonephritis Unmasked by Electron Microscopy

A Case of ANCA-Associated Vasculitis with IgG4-Related Disease Treated with the C5a Receptor Inhibitor Avacopan

A Case of ANCA-Associated Vasculitis with IgG4-Related Disease Treated with the C5a Receptor Inhibitor Avacopan

#5344 EPIDEMIOLOGY OF GLOMERULAR DISEASES IN THE PRE- AND POST-COVID-19 ERA IN CENTRAL QUEENSLAND, AUSTRALIA

Abstract Background and Aims Development of new onset and recurrent glomerular diseases (GD) following Covid-19 infection or vaccination are increasingly reported in the literature with various proposed pathogenic mechanisms. Australia reported its first case of Covid-19 in January 2020. Currently in Australia, there has been 11.3 million cases of Covid-19 infection and 98% of individuals aged ≥ 16 years received at least 1 dose of Covid-19 vaccination. However, there are no studies in Australia that evaluate the risk of GD after Covid-19 infection or vaccination. We compared kidney biopsy results pre- (01/01/2017-31/12/2019) and post- (01/01/2020-31/12/2022) Covid-19 era to assess for potential changes in the epidemiology of kidney biopsy-proven GD. Method In this single-centred retrospective study, all renal biopsies performed in Central Queensland (CQ) between 01/01/2017 and 31/12/2022 were reviewed. This pilot study is a subset of Queensland Kidney Biopsy Registry which has captured all renal biopsies of adults residing in CQ with a regional population of 220,912 with a land area of 497,714 km2. Results Eighty-seven percent of the CQ population received at least one dose of Covid-19 vaccination and 53,234 cases of Covid-19 infection were confirmed by rapid antigen test (RAT) as of the end of January 2023. The total number of kidney biopsies performed pre- and post-Covid-19 era were 47 vs 58, respectively, and the majority were native kidney biopsies (94% vs 95%) in CQ whereby the number of nephrologists in the respective catchment areas and access availability to kidney biopsies remained similar. The mean age of patients was 55.3 years (standard deviation, SD 16.4) vs 55.6 years (SD 15.6) and the majority were males (70% vs 62%) and Caucasians (77% vs 76%). No case of recurrent GD was diagnosed in pre-Covid-19 era whereas 5 cases of recurrent GD {2 membranous nephropathy, 1 minimal change disease, 1 lupus nephritis class III-IV, and 1 ANCA associated vasculitis (AAV)} in post-Covid-19 era, p = 0.025. The commonest cause of GD in the pre-Covid-19 era was IgA nephropathy (IgAN) whereas diabetic nephropathy and AAV was the most common GD during the post-Covid-19 era. The incidence of IgAN decreased from 12 cases (18.1 cases/million person years) in pre-Covid-19 era to 3 cases (4.5 cases/million person years) in the post-Covid-19 era (incidence rate ratio, IRR of 0.25, 95% CI 0.05-0.93, p = 0.020). The incidence of diabetic nephropathy increased from 1 case (1.5 cases million person years) during the three years pre-Covid-19 era to 13 cases (19.6 cases/million person years) for three years post-Covid-19 era (IRR of 13.1, 95% CI 1.95-552.47, p = 0.001). In the post-Covid-19 era, the mean age of patients with diabetic nephropathy was 51.2 years (SD 11.9) and the majority were indigenous Australian (n = 8, 62%) with poor diabetic control. They had kidney biopsies for nephrotic range proteinuria with or without acute kidney injury and haematuria. The incidence of AAV increased from 3 cases (4.5 cases/million person years) during the three years pre-Covid-19 era to 10 cases (15.1 cases/million person years) for three years post-Covid-19 era (IRR of 3.4, 95% CI 0.86-18.85, p = 0.052). In the post-Covid-19 era, the mean age of patients with AAV was 68.4 years (SD 16.1) with Caucasian (n = 10, 100%). PR3-AAV (n = 5) and MPO-AAV (n = 5) are equally prevalent, and 3 patients (30%) had concomitant pulmonary complications. Three cases of AAV each occurred in the first and second years and 4 cases in the third year during the post-Covid-19 era. There was no increase in the incidence rate for other types of glomerular diseases. Conclusion Our findings suggest the frequency of incident and recurrent GD may vary with the emergence of Covid-19 and steps taken to minimise viral complications that include primary preventative measures via vaccination. Larger epidemiological studies are required to better elucidate the risk of Covid-19 infection and vaccination in relation to GD.

Etiological Aspects of Lower Extremity Peripheral Arterial Disease: Multicentric Prospective Study of 126 Cases

<i>Introduction</i>: Atherosclerosis is the main cause of lower extremity peripheral arterial disease (PAD). However, there are other etiologies little reported. The objective of this work was to study the socio-demographic, clinical and etiological aspects of PAD. <i>Patients and study method</i>: This was a multicentre, descriptive, cross-sectional study conducted from January 1^st to October 30^th, 2021, in Thies and Dakar, Senegal. Any clinically suspected PAD was confirmed by arterial Doppler ultrasound and/or CT angiography. Clinical, biological and ultrasound data were used to establish the etiology and diagnosis criteria of OLIN for the diagnosis of thromboangiitis obliterans (TAO). <i>Results</i>: A total of 126 patients were collected, 31 (24.6%) in Thies and 95 (75.4%) in Dakar. There were 72 men (57.1%) and 54 women (42.9%), a male-to-female sex ratio of 1.3. The average age was 62.5 years 10.8 years. The majority of patients had a low socio-economic profile (52%). Etiologies were dominated by atheromatous disease (n= 120; 95.2%) and the cardiovascular risk factors were in order of frequency: physical inactivity (n=98; 77.8%), type 2 diabetes (n=78; 62%), hypertension (n= 69; 54.8%), smoking (n= 46; 36.5%) and dyslipidemia (n=29; 23%). Thromboangiitis obliterans (Buerger’s disease) was the second etiology found in 5 patients (3.96% of cases) and concerned only men who regularly used tobacco and cannabis. One case of ANCA-associated vasculitis was also found. In addition, there was a considerable delay in diagnosis with 117 patients (92.8%) at the critical ischemia stage at the time of diagnosis and amputation of varying magnitude in 110 patients (87.3%). This delay in diagnosis was at least due to insufficient screening, limited access to arterial Doppler echo and the lack of specialized services in vascular medicine, outside the capital Dakar. <i>Conclusion</i>: The most common etiology of lower extremity PAD was atherosclerosis. Obliterating thromboangiitis was not uncommon. Early detection of PAD and control of risk factors for atherosclerosis must be the rule.

A CASE OF MICROSCOPIC POLYANGIITIS (MPA) FOLLOWING SARS-COV-2 INFECTION

A CASE OF MICROSCOPIC POLYANGIITIS (MPA) FOLLOWING SARS-COV-2 INFECTION

A case of ANCA-associated vasculitis in a 16-year-old female following SARS-COV-2 infection and a systematic review of the literature

BackgroundAntineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare form of vasculitis in children. SARS-CoV-2, the virus that causes COVID-19 infection, seems to trigger autoimmunity and new-onset autoimmune disease in pediatric and adult patients. We present a case of new-onset AAV following COVID-19 infection in an adolescent patient, and we review the literature of AAV following COVID-19 infection.Case presentationAn adolescent female with a history of asthma was diagnosed with mild COVID-19 infection and subsequently developed persistent cough, wheezing, hearing loss, arthralgias, and rash. Her imaging and laboratory workup showed pulmonary nodules and cavitary lesions, elevated inflammatory markers, negative infectious testing, and positive ANCA. She was treated with glucocorticoids, rituximab, and mycophenolate mofetil. At six-month follow-up, she had improvement in her symptoms, pulmonary function tests, imaging findings, and laboratory markers.ConclusionsWe report the second case of new-onset anti-PR3, C-ANCA vasculitis and the fourth case of pediatric-onset AAV following COVID-19 infection. A systematic review of the literature found 6 cases of new-onset AAV in adults after COVID-19 infection. Pediatric and adult patients who develop AAV post COVID-19 infection have few, if any, comorbidities, and show marked radiographic and symptomatic improvement after treatment. There is increasing evidence for COVID-19-induced autoimmunity in children and our case highlights the importance of considering AAV in a child following a recent COVID-19 infection because timely treatment may improve clinical outcomes.

Infection is associated with increased risk of MPO- but not PR3-ANCA-associated vasculitis.

To determine whether development of ANCA-associated vasculitis (AAV) shows a relationship with a prior infection and if prior infection affects disease characteristics and outcome. All incident cases of AAV diagnosed in a defined region of Sweden from 2000 through 2016 were identified. For each case, 10 individuals from the general population, matched for age, sex and area of residence, were selected. Infections occurring in AAV patients and controls prior to the date of AAV diagnosis (index date for respective controls) were identified using an administrative database. Conditional logistic regression models were used to calculate odds ratios (OR) of developing AAV. Occurrence, clinical characteristics and outcome of AAV were analysed with respect to prior infection. Two-hundred and seventy patients with AAV (48% female) and 2687 controls were included. Prior to diagnosis/index date, 146 (54%) AAV patients had been diagnosed with infection vs 1282 (48%) controls, with OR for AAV 1.57 (95% CI 1.18, 2.19) in those with infections of the upper respiratory tract and 1.68 (1.02, 2.77) in those with pneumonia. Difference from controls was significant in patients with MPO-ANCA 1.99 (95% CI 1.25, 3.1) but not in those with PR3-ANCA 1.0 (0.61, 1.52). Patients with prior infection showed higher disease activity at AAV diagnosis. No differences in disease characteristics, comorbidities or outcome in those with and without prior infections were observed. Respiratory tract infections are positively associated with development of MPO- but not PR3-ANCA vasculitis. Prior infection is associated with higher disease activity at AAV diagnosis.

POS-859 Recurrence or reactivation of SARS-CoV-2 infection after immunosuppressive therapy in ANCA-Associated Vasculitis with COVID-19

Not every kidney damage due to COVID-19 is fully understood. Acute tubular injury is the lesion most frequently described in this disease. However, four cases of ANCA-Associated Vasculitis with COVID-19 with immune pauci-glomerulonephritis have recently been described.

A Case of ANCA-Associated Vasculitis (AAV) in Patients with Systemic Sclerosis (SSc)

A Case of ANCA-Associated Vasculitis (AAV) in Patients with Systemic Sclerosis (SSc)

A Case of ANCA-Associated Vasculitis (AAV) in Mixed Connective Tissue Disease (MCTD) Manifesting as Pulmonary Renal Syndrome

A Case of ANCA-Associated Vasculitis (AAV) in Mixed Connective Tissue Disease (MCTD) Manifesting as Pulmonary Renal Syndrome

258 Atypical Case of ANCA-Associated Vasculitis

258 Atypical Case of ANCA-Associated Vasculitis

Incidence and predictors of severe infections in ANCA-associated vasculitis: a population-based cohort study.

To determine the incidence rate, predictors and outcome of severe infections in a population-based cohort of ANCA-associated vasculitis (AAV). The study included 325 cases of AAV (152 female) diagnosed from 1997 through 2016 from a defined geographic area in Sweden. All severe infection events (requiring hospitalization and treatment with intravenous antimicrobials) were identified. The Birmingham vasculitis activity score (BVAS) was used to evaluate disease activity, and organ damage was assessed using the vasculitis damage index (VDI). Patients were followed from time of AAV diagnosis to death or December 2017. A total of 129 (40%) patients suffered at least one severe infection. In 2307 person-years (PY) of follow-up, 210 severe infections were diagnosed. The incidence rate of severe infections was 9.1/100 PY and was highest during the first year following AAV diagnosis at 22.1/100 PY (P < 0.001). Pneumonia, sepsis and urinary tract infection were the most common infections. Opportunistic infections constituted only 6% of all severe infections. In Cox regression analysis age and BVAS at diagnosis were the only factors independently predicting severe infection [hazard ratio: 1.54 (P < 0.001) and 1.27 (P = 0.001), respectively]. Severe infection was associated with poorer prognosis with respect to median VDI score 12 months post-AAV diagnosis, renal survival and mortality. Severe infections were the cause of death in 32 patients (22% of all deaths). . Severe infection is a common problem in AAV, with the most important prognostic factors being older age and high disease activity at diagnosis. Severe infections are associated with permanent organ damage and high mortality.

An unusual presentation of propylthiouracil-induced anti-MPO and PR3 positive ANCA vasculitis with associated anti-GBM antibodies, IgA nephropathy and an IgG4 interstitial infiltrate: a case report

BackgroundA number of disease processes can culminate in rapidly progressive glomerulonephritis, including pauci-immune focal segmental necrotising glomerulonephritis, usually seen with positive serum antineutrophil cytoplasmic antibodies (ANCA). Propylthiouracil (PTU) has been associated with drug-induced ANCA-associated vasculitis (AAV), with antibodies against myeloperoxidase (MPO) and proteinase 3 (PR3) present individually and together having been recognised. ‘Double-positive’ vasculitis with ANCA and anti-glomerular basement membrane (GBM) antibodies has also been reported in association with PTU treatment. We present a case of PTU-induced anti-MPO and PR3 positive ANCA vasculitis with associated anti-GBM antibodies, IgA nephropathy and an IgG4 interstitial infiltrate.Case presentationA 51-year-old man presented 2 weeks after re-commencing propylthiouracil (PTU) treatment for Graves’ disease, with a severe acute kidney injury and haemato-proteinuria. He demonstrated positive titres for autoantibodies to PR3 (76.9 IU/mL), MPO (28.8 IU/mL) and GBM (94 IU/mL). Renal biopsy demonstrated numerous glomerular crescents, widespread IgG4-positive lymphoplasmacytic infiltrate and mesangial positivity for IgA. PTU was stopped and he was treated with steroids, plasma exchange and cyclophosphamide with sustained improvement in his renal function.ConclusionsThis case of drug-induced AAV presented a unique and intriguing collection of serological and histological features. We propose that the PTU-induced AAV resulted in epiphenomena of anti-GBM antibody production and an IgG4-cell-rich tubulointerstitial infiltrate. It is uncertain whether the mesangial IgA deposition preceded or resulted from the AAV.

P0439THE RELATIONSHIP BETWEEN SOCIO-ECONOMIC STATUS AND RENAL VASCULITIS IN SCOTLAND

Abstract Background and Aims Individuals living in areas of multiple socioeconomic deprivation have reduced life expectancy and experience health inequalities. Chronic kidney disease is more common in areas of social deprivation and these patients are more likely to develop end stage kidney disease. Vasculitis is a rare but significant cause of kidney disease. The impact of socioeconomic status on disease activity or outcomes in patients with ANCA Associated Vasculitis (AAV) is yet to be fully explored. The aim of this study was to establish whether there is an association between the incidence of biopsy-proven renal vasculitis and socioeconomic status, as measured by the Scottish Index of Multiple Deprivation (SIMD). Method Using the Scottish renal biopsy registry, we identified all adult native renal biopsies performed across Scotland between 2014 and 2018 with a diagnosis of AAV. Patient’s postcode and SIMD (2016) rank were recorded. Patients were separated into quintiles of SIMD rank. Baseline demographics were recorded. We derived the denominator population from the 2016 SIMD census. Data were calculated per million population (PMP) served. Results 339 biopsy proven cases of AAV were identified. 6 cases were excluded as postcode was unavailable. Overall, mean age was 65.9 (±13.0) years and 45% of patients were male. At time of diagnosis, mean estimated glomerular filtration rate (eGFR) was 61.7 (±25.7) ml/min/1.73m2 and median urinary protein creatinine ratio (uPCR) was 134mg/mmol (IQR 64-21). Microscopic Polyangiits n=205(65%) was more common than Granulomatosis with Polyangiits n=128(35%). The incidences of kidney biopsy proven AAV were similar across all quintiles of deprivation. In the most deprived 20% of population, incidence rate of kidney biopsy proven AAV was 11.2 per million person-years vs 13.0 per million person-years in least deprived 20% of population. Patients in areas of greatest relative deprivation were younger (64.0 (±12.3) vs. 68.1 (±12.7) years) and had slightly less proteinuria at diagnosis (99mg/mmol (IQR 35-211) vs. 138mg/mmol (IQR 78-281)) when compared to patients living in least deprived areas. However, there was no difference in level of renal function at diagnosis (33.8 (±29.6) ml/min/1.73m2 vs 31.5 (±23.2) ml/min/1.73m2). Conclusion Our complete national dataset shows that there is no significant difference in incidence of renal AAV across the spectrum of socioeconomic deprivation. The analysis of renal function at presentation suggests no evidence of an association between deprivation and delay in diagnosis in a healthcare system free at the point of access.

P182 Prevalence and mortality of ANCA-associated vasculitis in England

Abstract Background The contemporary prevalence of ANCA-associated vasculitis (AAV) in England is unknown. Hospital Episode Statistics (HES) contain data on every hospital and day case NHS admission in England since 1997. In collaboration with the National Congenital Anomaly and Rare Disease Registration Service (NCARDRS) we validated the diagnosis of AAV using ICD codes in HES. The positive predictive value of these codes was 86%, which compares favourably to a median coding accuracy of 80% in a recent systematic review of NHS coding studies. This justifies using this novel dataset for population-based epidemiology with coverage of the whole population of England. Methods We worked within NCARDRS enabled by their Section 251 legal permissions (CAG 10-02(d)/2015). We extracted all cases of AAV from HES 2011/12 to 2016/17 using ICD-10 codes M313 Granulomatosis with polyangiitis (GPA), M317 Microscopic polyangiitis (MPA), and M301 Eosinophilic granulomatosis with polyangiitis (EGPA). We used the Summary Care Record to check vital status and record date of death where appropriate. We estimated point prevalence on 1 July 2016 using ONS mid-year population estimates for England in 2016 as the denominator. Standardised mortality ratio (SMR) was calculated using the Office for National Statistics death summary tables 2016 to provide expected number of deaths for each 5-year age-band and sex. Results We identified 9,890 patients who were coded as having AAV during a hospital admission 2011-2017. This included 6,856 (69.3%) with GPA, 964 (9.8%) with MPA and 2,070 (20.9%) with EGPA. On 1 July 2016, our dataset found 8,040 people in England were living with ANCA associated vasculitis. We estimate the prevalence was 14.55 (95% CI: 14.23-14.87)/100,000 adult population. The median age of these patients was 65.3 years (interquartile range 52.3-74.2). 47% were female. The prevalence of GPA was 9.97/100.000 (95% CI: 9.71-10.24), MPA was 1.40/100,000 (95% CI: 1.30-1.50), and EGPA was 3.18/100,000 (95% CI: 3.03-3.33). People with AAV were 4.6 times more likely to die than the background population of the same age and sex (Standardised Mortality Ratio = 4.58). Conclusion There are no recent UK prevalence estimates for all types of ANCA-associated vasculitis. Studies in Australia, Germany, Southern Sweden and the USA have found estimated prevalence to be between 4.6-18.4 cases per 100,000 individuals. Our estimate of 14.6/100,000 in England is consistent with this, and towards the higher end of the range. However, our estimates underestimate the prevalence of MPA compared to other studies, and further work is needed to increase the routine identification of cases of MPA. Further work within NCARDRS using their unique data linkages will enable more specific AAV case ascertainment as well as nationwide population-based studies on cause of death and studies using the database of English prescriptions dispensed in the community. Disclosures F.A. Pearce None. B. Griffiths None. C. Mukhtyar None. R. Al-Jayoussi None. R.A. Watts None. J. Aston None. M. Bythell None. S. Stevens None. P.C. Lanyon None.

203 From Boom to Bust: The Use of Charting to Help Assess Causative Relationships Through a Case of ANCA-Associated Vasculitis

203 From Boom to Bust: The Use of Charting to Help Assess Causative Relationships Through a Case of ANCA-Associated Vasculitis

The Association of Airway Comorbidities With the Clinical Phenotypes and Outcomes of Patients With Antineutrophil Cytoplasmic Autoantibody-associated Vasculitis.

We investigated the association of airway comorbidities with the clinical phenotypes and outcomes of myeloperoxidase (MPO)-antineutrophil cytoplasmic antibodies (ANCA)-positive ANCA-associated vasculitis (AAV). An AAV patient multicenter cohort trial was established in 13 hospitals in western Japan between 2012 and 2018. We examined 143 of the new-onset MPO-ANCA-positive AAV patients. Their clinical characteristics and comorbidities at disease onset were compared based on clinical phenotypes. Multivariate analysis was performed to identify factors predictive of remission and death. Twenty-seven cases with granulomatosis with polyangiitis (GPA), 10 with eosinophilic GPA (EGPA), 81 with microscopic polyangiitis (MPA), and 25 with unclassified AAV were identified. The average age of MPO-ANCA-positive patients was 71.4 years. Comorbidity (87.4%) and airway comorbidity (70.6%) were frequently observed in these patients. Examination of the clinical phenotypes revealed that the cases of GPA were frequently accompanied by infectious airway comorbidity (upper airway disease, bronchiectasis, pulmonary infections), and most of the cases of MPA and unclassified AAV were accompanied by fibrotic interstitial lung disease (fILD) or emphysema. Among MPO-ANCA-positive patients, infectious airway comorbidity was predictive of both remission (HR 1.58, P = 0.03) and mortality (HR 2.64, P = 0.04), and fILD was predictive of mortality (HR 7.55, P = 0.008). The combination of infectious airway comorbidities and fILD caused the worst survival outcomes in patients. MPO-ANCA-positive AAV was frequently accompanied by airway comorbidities. In addition to fILD, infectious airway comorbidities were closely associated with those clinical phenotypes and outcomes.

158. INCIDENCE AND PREDICTORS OF SEVERE INFECTIONS IN ANCA-ASSOCIATED VASCULITIS IN A POPULATION-BASED COHORT – PRELIMINARY RESULTS

Background: Infectious complications in ANCA associated vasculitis (AAV) are a major cause of morbidity and mortality. The aim of this study was to determine the incidence rates, predictors and outcome of severe infections in AAV. Methods: We conducted a population-based cohort study in Southern Sweden with 326 incident cases of AAV diagnosed between 1997 and 2016. Diagnosis of vasculitis was confirmed by case record review and patients were classified according to the European Medicines Agency algorithm. Demographics, clinical, andlaboratory data was collected from time of diagnosis and follow-up. All events of severe infection (required hospitalization and treated with intravenous antimicrobials) were identified. Vasculitis disease activity was evaluated using the Birmingham Vasculitis Activity Score (BVAS) and the extent of organ damage was assessed using the vasculitis damage index (VDI). Patients were followed from time of AAV-diagnosis to death or end of study, December 2017Results: Data on 262 patients (122 women) was collated and are presented in this report. Total time of follow-up was 1368 person-year. In total 104 (39.7%) patients experienced at least one severe infection during the follow-up, 33 (12.5%) suffered 2 infections and 14 (5%) suffered 3 severe infections or more. The incidence rate of severe infection was higher during the first year after diagnosis compared to that during the whole follow-up time (24.3/100 year vs. 7.6/100, p<0.001). Patients with severe infection were older at diagnosis, had higher serum creatinine, higher BVAS at diagnosis and higher VDI after 12 months (Table 1). They were also more likely to be MPO-ANCA positive. Age and BVAS at diagnosis were the only factors that independently predicted severe infection. Severe infection was associated with worse prognosis in terms of renal and patient’s survival. Conclusion: In this cohort the incidence rate of severe infection is comparable to earlier published data in AAV. The rate of severe infection is higher early in the disease course. Severe infection is still a major clinical problem and is associated with high age, increased disease activity at diagnosis, renal disease and MPO-ANCA positivity. Severe infection is associated with a worse prognosis.

A Case of ANCA Associated Vasculitis with Hemobilia due to Rupture of the Hepatic Artery Aneurysm

A Case of ANCA Associated Vasculitis with Hemobilia due to Rupture of the Hepatic Artery Aneurysm