Diagnosis Of Vasculitis Research Articles

The role of susceptibility-weighted imaging & contrast-enhanced MRI in the diagnosis of primary CNS vasculitis: a large case series.

Primary CNS Vasculitis (PCNSV) is a rare, diverse, and polymorphic CNS blood vessel inflammatory condition. Due to its rarity, clinical variability, heterogeneous imaging results, and lack of definitive laboratory markers, PCNSV diagnosis is challenging. This retrospective cohort analysis identified patients with histological diagnosis of PCNSV. Demographic data, clinical presentation, neuroimaging studies, and histopathologic findings were recorded. We enrolled 56 patients with a positive biopsy of CNS vasculitis. Most patients had cerebral hemisphere or brainstem symptoms. Most brain MRI lesions were bilateral, diffuse discrete to confluent white matter lesions. Frontal lobe lesions predominated, followed by inferior cerebellar lesions. Susceptibility-weighted imaging (SWI) hemorrhages in 96.4% (54/56) of patients, either solitary microhemorrhages or a combination of micro and macrohemorrhages. Contrast-enhanced T1-WIs revealed parenchymal enhancement in 96.3% (52/54 patients). The most prevalent pattern of enhancement observed was dot-linear (87%), followed by nodular (61.1%), perivascular (25.9%), and patchy (16.7%). Venulitis was found in 19 of 20 individuals in cerebral DSA. Hemorrhages in SWI and dot-linear enhancement pattern should be incorporated as MINOR diagnostic criteria to diagnose PCNSV accurately within an appropriate clinical context. Microhemorrhages in SWI and venulitis in DSA, should be regarded as a potential marker for PCNSV.

Retinal Occlusive Vasculitis after Intravitreal Injection of Brolucizumab

Purpose: To report two instances of retinal arterial occlusive vasculitis following intravitreal injection of brolucizumab.Case summary: Two patients noted decreased visual acuity approximately one month after receiving intravitreal brolucizumab injections to treat wet age-related macular degeneration. In case 1, an 85-year-old male reported a reduction in visual acuity in his left eye, declining from a Snellen measurement of 0.2 to hand motion. In case 2, a 70-year-old female experienced a decline in visual acuity in her right eye from 0.4 to finger count at 50 cm. Both patients presented with anterior chamber reactions, and fluorescein angiography demonstrated delayed retinal artery filling time, leading to the diagnosis of retinal occlusive vasculitis. They were treated with both topical and systemic corticosteroids. For case 1, a subsequent vitrectomy was performed to remove the inflammatory membrane located behind the intraocular lens. However, despite aggressive interventions, neither patient showed visual improvement.Conclusions: Retinal occlusive vasculitis, which can result from intraocular inflammation, may occur after intravitreal brolucizumab injection. This condition can lead to significant visual impairment even with aggressive treatment.

DIAGNOSTIC CHALLENGE IN MIDDLE-AGED WOMAN WITH RECURRENT ISCHEMIC STROKES: A CASE OF PRIMARY CENTRAL NERVOUS SYSTEM VASCULITIS AND LITERATURE REVIEW

Primary vasculitides of the central nervous system are rare vascular disorders whose pathophysiology involves inflammatory infiltration exclusively in the vessels of the central nervous system. Its spectrum extends from primary angiitis of the central nervous system to cerebral amyloid angiopathy. This case portrays a 46-year-old woman with a history of multiple strokes. History of multiple comorbidities such as systemic arterial hypertension, prediabetes, and smoking. During follow-up after the second ischemic event, a patent foramen ovale was detected with positive microbubbles stand. Therefore, she was using oral anticoagulants. Transthoracic echocardiogram and magnetic resonance angiography without relevant changes. However, she resorted to the neurological deficit three more times. Cerebrospinal fluid (CSF) analysis was done twice; both were normal. Cerebral angiography was performed at the time of the last stroke and revealed suggestive signs of vasculitis. Discrimination of angiitis and reversible cerebral vasoconstriction syndrome was challenging and based on the demographic and clinical features of the patient. The characteristics of this case that corroborate the hypothesis of angiitis are the patient's age, which is in the median age range (40-60 years), chronic headache, and focal symptoms not temporally associated with the headache. Intravenous methylprednisolone pulse therapy (one gram/day) was performed for five days. Evolved with progressive improvement of the motor deficit. On hospital discharge, it was prescribed prednisone, and after ten days, Azathioprine 2.5mg/Kg/day was started. Diagnosis of primary central nervous system vasculitis requires high clinical suspicion due to the low specificity of the clinical features. This report reinforces the importance of investigating CNS vasculitis in young patients with a history of recurrent stroke.

Maternal and fetal outcomes of pregnancy in women with primary systemic vasculitis: A single-center cohort study of 20 patients and 30 pregnancies

ObjectivesTo analyze pregnancy outcomes of patients with primary systemic vasculitis followed in a third-level referral center. MethodsRetrospective cohort study of all pregnant women with systemic vasculitis followed between 2009 and 2022 at the High-Risk Pregnancy Clinic of the Department of Systemic Autoimmune Diseases of the Hospital Clínic, Barcelona. ResultsTwenty women with primary vasculitis were identified, with a total of 30 pregnancies. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (n = 7) and Behçet disease (n = 4) were the most frequent types of vasculitis. All women had the diagnosis of vasculitis before pregnancy, with a median time between disease diagnosis and pregnancy of 5.8 years (range: 2 months-29 years). Most were in remission at conception (76.7 %). During pregnancy, a vasculitis flare occurred in 4 (13.3 %) patients (one each with Takayasu arteritis, eosinophilic granulomatosis with polyangiitis [EGPA], IgA vasculitis [IgAV], and Behçet disease [BD]). Four (16.7 %) of the successful pregnancies had post-partum relapses (one each with EGPA, granulomatosis with polyangiitis, IgAV, and BD). Eighty percent of pregnancies resulted in live babies. In four cases (13.3 %), medical termination of pregnancy was decided, considering the mother or baby health risk. There were two spontaneous miscarriages, and no stillbirths or neonatal deaths. Preeclampsia was the most frequent maternal complication (25 %). Newborns were preterm in 24 % and low birthweight in 20 % of cases. No maternal deaths occurred. ConclusionsThis cohort study shows that vasculitis relapses during pregnancy and post-partum, together with other pregnancy complications, occur in a considerable number of patients with systemic vasculitides, although a final good pregnancy outcome can be expected in most cases. These findings emphasize the convenience of managing these special situations in expert reference centers.

A predictive mortality score in ANCA-associated renal vasculitis.

Several scores have been developed to predict mortality at anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) diagnosis. Their prognostic value in Caucasian patients with kidney involvement (AAV-GN) remains uncertain as none has been developed in this specific population. We aimed to propose a novel and more accurate score specific for them. This multicentric study included patients diagnosed with AAV-GN since January 2000 in four nephrology centers (recorded in the Maine-Anjou AAV-GN Registry). Existing scores and baseline characteristics were assessed at diagnosis before any therapeutic intervention. A multivariable analysis was performed to build a new predictive score for death. Its prognosis performance (area under receiving operating curve and C-index) and accuracy (Brier score) was compared with existing scores. One hundred and eighty-five patients with AAV-GN from the RENVAS registry were used as a validation cohort. A total of 228 patients with AAV-GN from the Maine-Anjou registry were included to build the new score. It included the four components most associated with death: age, history of hypertension or cardiac disease, creatinine and hemoglobin levels at diagnosis. Overall, 194 patients had all the data available to determine the performance of the new score and existing scores. The new score performed better than the previous ones in the development and in the validation cohort. Among the scores tested, only Five-Factor Score and Japanese Vasculitis Activity Score had good performance in predicting death in AAV-GN. This original score, named DANGER (Death in ANCA Glomerulonephritis-Estimating the Risk), may be useful to predict the risk of death in AAV-GN patients. Validation in different populations is needed to clarify its role in assisting clinical decisions.

Clinical and anamnestic features of patients with systemic vasculitis: a single-center retrospective study.

Systemic vasculitides are the most complex and problematic autoimmune rheumatic diseases characterized by affections of large, medium, or small vessels. Although the immunopathogenesis of vasculitides is thoroughly studied, the epidemiology and etiology are poorly explored. The main triggers of vasculitides are environmental, genetic, and various infectious factors. Diagnosis of vasculitides is complicated due to the non-specific nature of their symptoms. Vasculitides affect various organ systems with abrupt or slow (weeks-months) development of symptoms. This study aims to analyze the demographic and clinical-anamnestic characteristics of patients with systemic vasculitides in a single centre before and during the COVID-19 pandemic in Kazakhstan. A single-centre retrospective study of medical records of 80 patients above 18years was conducted in the Almaty City Rheumatology Center. Medical records of 24 males (30%) and 56 females (70%) with systemic vasculitides, diagnosed from January 2019 to December 2021, were analyzed. Age, gender, damaged organ systems, disability, concomitant diseases, disease experience, laboratory data, and other variables were recorded. The records of hospitalized patients with systemic vasculitides were analyzed. Of 80 patients registered in 2019-2021, the most common were those with IgA vasculitis (n = 32, 40%), Takayasu arteritis (n = 17, 21.25%), and granulomatosis with polyangiitis (n = 12, 15%). Behçet disease was diagnosed less frequently (n = 9, 11.25%). Patients with systemic vasculitides had pre-obesity (n = 19), class 1 obesity (n = 13), and class 2 obesity (n = 2). Musculoskeletal affections were present in 52 patients (65%). Gastrointestinal, cutaneous, and cardiovascular affections were recorded in 45 (56.3%), 37 (46.3%), and 39 (48.8%) cases, respectively. Only 8 patients (10%) had affections of the nervous system. Most patients had elevated C-reactive protein (n = 29, 36.3%) and leukocytosis (n = 33, 41.3%). One-third of patients with vasculitides had a history of abortions. Musculoskeletal, cutaneous, gastrointestinal, and cardiovascular affections are common in patients with systemic vasculitides. Obesity is a frequent comorbidity in vasculitides. Comorbidities and abortions complicate the disease course and its management.

Клинический случай тяжелого течения геморрагического васкулита

Diagnosis of systemic vasculitis often causes difficulties in clinical practice. A clinical case characterized by multiple organ and comorbid pathology is presented. The manifestation of intestinal bleeding was the reason for the diagnosis of severe ulcerative colitis. The appearance of a characteristic hemorrhagic rash, followed by renal pathology with hematuria and severe nephrotic syndrome, made it possible to establish the correct diagnosis.

Nailfold Capillaroscopy Analysis Can Add a New Perspective to Biomarker Research in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), all of which are characterised by inflammation of small–medium-sized vessels. Progressive understanding of these diseases has allowed researchers and clinicians to start discussing nailfold video capillaroscopy (NVC) as a future tool for many applications in daily practice. Today, NVC plays a well-established and validated role in differentiating primary from secondary Raynaud’s phenomenon correlated with scleroderma. Nevertheless, there has not been sufficient attention paid to its real potential in the ANCA-associated vasculitis. In fact, the role of NVC in vasculitis has never been defined and studied in a multicentre and multinational study. In this review, we carried out a literature analysis to identify and synthesise the possible role of capillaroscopy for patients with ANCA-associated vasculitis. Methods: Critical research was performed in the electronic archive (PUBMED, UpToDate, Google Scholar, ResearchGate), supplemented with manual research. We searched in these databases for articles published until November 2023. The following search words were searched in the databases in all possible combinations: capillaroscopy, video capillaroscopy, nailfold-video capillaroscopy, ANCA-associated vasculitis, vasculitis, granulomatosis with polyangiitis, EGPA, and microscopic polyangiitis. Results: The search identified 102 unique search results. After the evaluation, eight articles were selected for further study. The literature reported that capillaroscopy investigations documented non-specific abnormalities in 70–80% of AAV patients. Several patients showed neoangiogenesis, capillary loss, microhaemorrhages, and bushy and enlarged capillaries as the most frequent findings. Furthermore, the difference between active phase and non-active phase in AAV patients was clearly discernible. The non-active phase showed similar rates of capillaroscopy alterations compared to the healthy subjects, but the active phase had higher rates in almost all common abnormalities instead. Conclusions: Microvascular nailfold changes, observed in patients affected by vasculitis, may correlate with the outcome of these patients. However, these non-specific abnormalities may help in the diagnosis of vasculitis. As such, new analysis analyses are necessary to confirm our results.

A study on clinico- epidemiological profile & histopathological profile of cutaneous vasculitis

: The study aimed to analyse the potential etiological factors, clinical presentation, past medical history & laboratory findings in patients with biopsy-proven cutaneous vasculitis to better understand the disease and identify the disease classifications. The objective of this study was to establish a database of the clinic-epidemiological profile and histopathological features of cutaneous vasculitis in a tertiary hospital in Eastern Nepal.: A Descriptive cross-sectional study was planned to analyse and study the clinic-epidemiological profile and histopathological features of cutaneous vasculitis in Nepal. This study has reviewed the facts which were published earlier to determine the current scenario by vast study of statistics and derivation of facts. Proper examination of data was made to evaluate with final conclusion. The study included sample size of 30 consecutive patients with biopsy-proven cutaneous vasculitis attending the Dermatology outpatient department and admitted to the dermatology ward at B.P. Koirala Institute of Health Science, Dharan. : The study population of 30 patients with biopsy-proven cutaneous vasculitis, with an average age of 32.96 years, and a female-to-male ratio of 1.3:1. The majority of patients (66.67%) had lesions lasting less than 6 weeks, and acute disease was observed in 86.66% of the cases. The most common symptoms were itching (73.66%) and arthralgia (66.67%). Palpable purpura was the predominant cutaneous manifestation (70%), mainly distributed on the legs and ankles (93.33%). Elevated ESR was the most common laboratory abnormality (73.33%). Histopathological examination revealed leukocytoclastic vasculitis in 97% of cases, with predominantly neutrophilic infiltrates. Henoch-Schoenlein purpura (HSP) was the most common classification (43.33%), followed by urticarial vasculitis (26.67%).: This study provides valuable insights into the clinic-epidemiological profile and histopathological features of cutaneous vasculitis in the Nepalese population. It highlights the diverse clinical presentation and laboratory abnormalities associated with the condition, as well as potential etiological factors and disease classifications. The findings contribute to improved understanding, diagnosis, and management of cutaneous vasculitis. Further studies with larger cohorts are needed to validate and expand upon these findings.

Persistent intracranial hyper-inflammation in ruptured cerebral aneurysm after COVID-19: case report and review of the literature

BackgroundThe systemic manifestations of coronavirus disease 2019 (COVID-19) include hyperinflammatory reactions in various organs. Recent studies showed evidence for the frequent involvement of central nervous system in affected patients; however, little is known about clinical features of cerebrovascular diseases in childhood-onset COVID-19.Case presentationA 10-year-old boy recovered from SARS-CoV-2 infection without complication. On 14 days after infection, he presented with loss of consciousness. A head computed tomography detected a ruptured cerebral aneurysm at the left posterior cerebral artery accompanying subarachnoid hemorrhage (SAH). Immediate surgical intervention did not rescue the patient, resulting in the demise 7 days after admission. Serological and genetic tests excluded the diagnosis of vasculitis and connective tissue disorders. Retrospective analysis showed markedly higher levels of interleukin (IL)-1β, IL-6 and IL-8 in the cerebrospinal fluid than the serum sample concurrently obtained. A review of literature indicated that adult patients with COVID-19 have a risk for the later development of SAH during the convalescent phase of COVID-19.ConclusionsSAH is a severe complication of COVID-19 in children and adults who have asymptomatic cerebrovascular aneurysms. The markedly high levels of cytokines detected in the cerebrospinal fluid suggested that intracranial hyperinflammatory condition might be one of the possible mechanisms involved in the rupture of a preexisting cerebrovascular aneurysms.

Animal Models of Vasculitis.

The diagnosis of vasculitis in rheumatoid arthritis (RV) is associated with considerable mortality; therefore, understanding the basic mechanisms underlying the pathogenesis of vasculitis is very important. Animal models of vasculitis have contributed to elucidating such mechanisms. We here introduce a Candida albicans water-soluble glycoprotein (CAWS)-induced vasculitis model and the methodological approach to evaluate inflammatory vascular change.

Unravelling a Case of Coronary Vasculitis

Background Coronary vasculitis is a rare cause of myocardial infarction through varied mechanisms. We report the case of a 32-year-old female found to have diffuse coronary microaneurysms during treatment for an Inferior ST Elevation Myocardial Infarction (STEMI), with challenges in pinpointing the underlying aetiology. Results Our patient has been diagnosed with a seronegative arthritis and had been initiated on regular Golimumab 4 months prior. She presented with prolonged fever for 4 weeks and inflammatory oligoarthritis with left sacroiliac joint pain. During this admission, Antinuclear antibody was 1:2560 and anti ds DNA was 1:80. She had highly positive Rickettsia conorii IgG titres of 1:128, suggestive of acute infection with Orientia tsustsugamushi. Meropenem and doxycyclin for presumed typhus had no impact on her fever spikes. In the 10th week of the illness, she developed ischaemic chest pain with electrocardiographic changes of inferior STEMI and was thrombolysed. Invasive coronary angiography revealed numerous coronary microaneurysms affecting all major myocardial vessels, with minimal coronary artery disease. Following multidisciplinary discussion, she was treated with intravenous methylprednisolone, antiplatelets, a statin and warfarin for a diagnosis of coronary vasculitis. Repeat angiography showed complete resolution of the microaneurysms. She continues on anticoagulation and has since had no coronary events. Conclusion Typhus and medium vessel vasculitis such as Kawasaki and Polyarteritis Nodosa were potential diagnoses for this presentation. A diagnosis of SLE was established due to the highly specific immunological profile. As there are reports of high Rickettsial titres in autoimmune disease, which alongside the lack of response to doxycyclin makes Typhus unlikely. The possibility of golimumab induced SLE was briefly entertained due to similar reports, but the positivity of ds DNA and major organ involvement made this possibility unlikely. We highlight the cardiac involvement in this case due to its high morbidity on young patients, and the current paucity of knowledge on SLE-related endothelial dysfunction and immune-mediated vascular damage. The role of anticoagulation in this inflammatory milieu too, is controversial. This case highlights a rare presentation of SLE, with an emphasis on coronary vasculitis. It adds to the current knowledge on the complex cardiovascular risk in SLE and highlights ambiguous areas in current management strategies to prompt further research.

Clinical impact of ceruloplasmin levels at ANCA-associated vasculitis diagnosis.

Ceruloplasmin is an inhibitor of myeloperoxidase (MPO) activity that plays an important role in the pathophysiology of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). This study aimed to evaluate the prognostic impact of serum level of ceruloplasmin at diagnosis in patients with anti-MPO antibody-positive AAV. This retrospective monocentric study in Caen University Hospital involved all consecutive adult anti-MPO antibody-positive patients with microscopic polyangiitis or granulomatosis with polyangiitis, diagnosed between January 2010 and January 2022 with available serum sample at inclusion. Patients outcomes were analyzed from two subgroups constituted according to the median serum level of ceruloplasmin. The same analyses were then performed in anti-proteinase 3 (PR3) antibody-positive patients. Within the 92 patients analyzed, 50 patients had anti-MPO antibodies with a median ceruloplasmin level of 0.44 [quartiles 1-3, 0.40-0.49] g/L and a median Birmingham Vasculitis Activity Score of 19 [14-22]. After a median follow-up period of 40 [22-86] months, 13 (26%) patients had died: 10 (40%) in the low ceruloplasmin group and 3 (12%) in the high ceruloplasmin group (p = 0.03), with a significantly worse survival rate in the low ceruloplasmin group (p = 0.021). No significant differences in relapse rate or renal failure was observed between the two groups. The same analyses performed in the group of AAV patients with anti-PR3 antibody did not show any differences. In anti-MPO AAV patients, serum level of ceruloplasmin at diagnosis seems to be associated with a significant impact on survival.

Primary angiitis of the central nervous system presenting as sudden vision loss in a young patient

Primary angiitis of the central nervous system is a rare disorder that primarily affects the cerebral vessels and can prove to be a diagnostic challenge to the clinicians. It can present with a variety of clinical manifestations and lacks the evidence of any systemic disease. Digital subtraction angiography is often necessary to confirm the diagnosis of cerebral vasculitis but in cases where it may fail to do so, brain biopsy is eventually required. Obliviousness to this condition may lead to false management strategies and hence it is imperative to make an accurate diagnosis to facilitate proper treatment that mainly includes a combination of steroids and immunosuppressive agents. We report a case of a 27-year-old male with sudden bilateral vision loss who underwent extensive workup and was ultimately diagnosed with primary angiitis of the central nervous system. He was treated with steroids and responded well to it.
 

Chinese guidelines for the diagnosis and management of childhood IgA vasculitis (2023)

Chinese guidelines for the diagnosis and management of childhood IgA vasculitis (2023)

Clinical value of 18F-fluorodeoxyglucose positron emission tomography/computed tomography combined with computed tomography angiography in large-vessel vasculitis

Abstract OBJECTIVES: The objectives of this study were to investigate the clinical application of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG-PET/CT) combined with CT angiography (CTA) fusion images at diagnosis and assessment in large-vessel vasculitis (LVV). MATERIALS AND METHODS: Forty-six patients with LVV who underwent both 18F-FDG-PET/CT and CTA procedures were studied in the Second Hospital of Harbin Medical University from September 2019 to June 2022, and the clinical disease activity of patients was judged by the Physician Global Assessment. Clinical data, acute-phase reactants (APRs), and imaging data were collected. Meanwhile, the APRS must be obtained within 1 week of 18F-FDG-PET/CT. 18F-FDG-PET/CT was primarily used to evaluate LVV activity, while CTA was primarily used to observe morphological changes in arteries, including arterial wall thickening, narrowing, and corresponding complications. PET/CT images were evaluated by two nuclear medicine physicians, both of them unaware of the patients’ laboratory tests and clinical signs. Two nuclear medicine specialists evaluated the PET/CT images and PET/CTA images, who were blinded to the patients’ information. The concordance of two physicians in the LVV visual grading scale was studied by calculating the Cohen’s kappa index (k) which evaluates the power of concordance. The paired t-test was used to analyze the differences between PET/CTA images and PET/CT images. RESULTS: The sensitivity and specificity of the semi-quantitative analysis to assess LVV activity was 94.1% and 93.1%, respectively, when a cutoff of the mean SUVmax/SUVmeanliver of 1.15. It was found that the images obtained after delayed phase were clearer and the contrast between the arterial wall and the lumen was higher in 19 patients. We also concluded that PET/CTA examinations were able to detect more lesion sites compared to PET/CT examinations in 28 patients (P < 0.001), especially for patients with long-term treatment, and the interpretation of PET/CTA images took less time than PET/CT images(P < 0.001), ultimately achieving a shorter time, more comprehensive and accurate interpretation. CONCLUSION: Although 18F-FDG-PET/CT can assess the activity of LVV, it is poor at observing morphological changes in arteries. The use of 18F-FDG-PET/CTA imaging scans in LVV can accurately assess disease activity while at the same time providing a comprehensive, accurate, and efficient determination of disease severity, allowing patients to receive comprehensive diagnostic information from PET/CTA examination.

Using an artificial intelligence tool incorporating natural language processing to identify patients with a diagnosis of ANCA-associated vasculitis in electronic health records

BackgroundBecause anti-neutrophil cytoplasmatic antibody (ANCA)-associated vasculitis (AAV) is a rare, life-threatening, auto-immune disease, conducting research is difficult but essential. A long-lasting challenge is to identify rare AAV patients within the electronic-health-record (EHR)-system to facilitate real-world research. Artificial intelligence (AI)-search tools using natural language processing (NLP) for text-mining are increasingly postulated as a solution. MethodsWe employed an AI-tool that combined text-mining with NLP-based exclusion, to accurately identify rare AAV patients within large EHR-systems (>2.000.000 records). We developed an identification method in an academic center with an established AAV-training set (n = 203) and validated the method in a non-academic center with an AAV-validation set (n = 84). To assess accuracy anonymized patient records were manually reviewed. ResultsBased on an iterative process, a text-mining search was developed on disease description, laboratory measurements, medication and specialisms. In the training center, 608 patients were identified with a sensitivity of 97.0 % (95%CI [93.7, 98.9]) and positive predictive value (PPV) of 56.9 % (95%CI [52.9, 60.1]). NLP-based exclusion resulted in 444 patients increasing PPV to 77.9 % (95%CI [73.7, 81.7]) while sensitivity remained 96.3 % (95%CI [93.8, 98.0]). In the validation center, text-mining identified 333 patients (sensitivity 97.6 % (95%CI [91.6, 99.7]), PPV 58.2 % (95%CI [52.8, 63.6])) and NLP-based exclusion resulted in 223 patients, increasing PPV to 86.1 % (95%CI [80.9, 90.4]) with 98.0 % (95%CI [94.9, 99.4]) sensitivity. Our identification method outperformed ICD-10-coding predominantly in identifying MPO+ and organ-limited AAV patients. ConclusionsOur study highlights the advantages of implementing AI, notably NLP, to accurately identify rare AAV patients within large EHR-systems and demonstrates the applicability and transportability. Therefore, this method can reduce efforts to identify AAV patients and accelerate real-world research, while avoiding bias by ICD-10-coding.

Abstract 17436: A Diagnostic Copycat: Culture Negative Endocarditis of A Bioprosthetic Valve Presenting as ANCA Vasculitis

A 59-year-old female with a history of bicuspid aortic valve (AV) complicated by aortic stenosis and ascending aortic aneurysm s/p bioprosthetic AV and ascending aorta replacement presented with a 6-month history of weight loss, fatigue, and lower extremity rash. Initial laboratory workup identified anemia, leukopenia, and acute kidney injury with proteinuria and microscopic hematuria. Further workup included negative blood cultures, elevated inflammatory markers, elevated rheumatoid factor, hypocomplimentemia, and ANCA positivity (PR3 Ab positive). Biopsy of her palpable purpuric rash demonstrated vascular immune deposition pattern consistent with leukocytoclastic vasculitis. Renal biopsy revealed focal and crescentic glomerulonephritis with immune complex deposition. CT abdomen noted mesenteric lymphadenopathy with fluid surrounding the thoracic aortic graft. Surface echocardiogram showed bioprosthetic AV replacement with diffuse cusp thickening without stenosis or regurgitation. At this point, a multidisciplinary team offered a broad differential diagnosis of inflammatory vasculitis versus culture-negative endocarditis with plan for further testing. Follow-up whole-body and cardiac PET scan identified FDG-avid abnormalities involving the aortic graft and AV with hypermetabolic soft tissue thickening surrounding the aortic root. Transesophageal echocardiogram confirmed AV thickening with a 1 cm mobile echodensity. Additional serologic testing identified positive Bartonella henselae IgG (1:8192) and IgM (>1:20) titers. The final diagnosis was B. henselae prosthetic AV endocarditis and ascending aortic graft infection. She underwent repeat AV and ascending aortic graft replacement, with graft material positive for B. henselae PCR and was treated with a course of doxycycline and rifampin. B. henselae is a fastidious gram-negative bacterium that can cause culture negative endocarditis but is very rarely associated with aortic graft infection. This case highlights (1) a rare example of B. henselae aortic graft infection, (2) the difficulty in differentiating B. henselae endocarditis from inflammatory vasculitis, and (3) the importance of engaging multidisciplinary teams to solve difficult diagnostic cases.

Symposium 10: Spotting EGPA from Asthma Populations and How Co-Care Works Between Rheumatology and Respiratory Physicians: A Case Sharing

Eosinophilic Granulomatosis with Polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic vasculitis characterized by asthma, eosinophilia, and vasculitic organ involvement. This rare disease poses diagnostic challenges within the domain of severe asthma. Recognizing EGPA within the asthmatic cohort is therefore crucial for appropriate management and improved patient outcomes. Moreover, effective collaboration between rheumatologists and respiratory physicians is vital for the comprehensive care of EGPA patients. This scientific lecture aims to discuss the identification of EGPA in individuals with asthma and highlight the importance of co-care between rheumatology and respiratory medicine. This lecture will begin by providing an overview of EGPA, its clinical manifestations, and the challenges associated with differentiating it from severe asthma. The crucial identification of distinctive clinical features, such as refractory asthma, peripheral eosinophilia, and systemic symptoms, which serve as beacons for the presence of EGPA, will be discussed. Diagnostic tools encompassing imaging, laboratory tests, and biopsy techniques are explored, focusing on their utility in distinguishing EGPA from asthma. Concomitantly, the lecture will then shift its focus to the collaborative efforts required from rheumatologists and respiratory physicians in managing EGPA patients. The complementary roles of both specialties in achieving optimal patient outcomes will be discussed. Rheumatologists play a crucial role in the diagnosis and treatment of systemic vasculitis, including EGPA, while respiratory physicians provide expertise in managing asthma and respiratory complications. The case will highlight the importance of open communication, shared decision-making, and coordinated treatment plans to ensure comprehensive care for EGPA patients, addressing both their rheumatologic and respiratory needs. Real-life case will be shared to illustrate successful co-management models between rheumatology and respiratory medicine. These examples will underscore the benefits of a multidisciplinary approach, including improved disease management, reduced treatment-related complications, and enhanced patient satisfaction. In conclusion, identifying EGPA within the asthma population is crucial for appropriate management, and effective collaboration between rheumatology and respiratory physicians is essential for comprehensive care. This scientific lecture aims to provide insights into spotting EGPA among asthma patients and emphasize the significance of co-care between these specialties, ultimately improving patient outcomes and quality of life.

The Role of PET in the Diagnosis and Disease Activity Assessment in Large Vessel Vasculitis

The role of 18F-fluorodeoxyglucose (FDG) positron emission tomography (18F-FDG PET) in the diagnosis of large vessel vasculitis (LVV) is well established. It permits us to assess the extent and the grade of vascular involvement and to rule out the other causes in clinical scenarios characterized by less specific symptoms. The advantages of 18F-FDG PET are far less clear in monitoring disease activity over time. Studies looking for the role of 18F-FDG PET as a potential biomarker had conflicting results and whether and when to repeat it during follow-up is based on clinical experience. A comprehensive assessment, including clinical, laboratory and morphological imaging is still required to monitor patients with large-vessel vasculitis over time. The aim of this review is to present more recent data about the utility of 18 F-FDG PET in the diagnosis and follow-up of LVV.