Search

Objective: To present patients with giant cell arteritis (GCA) in our centre over a 10 year period in order to confirm current understanding of giant cell arteritis (GCA) and observe any possible specifities in our cohort. Patients and Methods: In this retrospective study all patients diagnosed with GCA in the Clinical Hospital Centre Rijeka from 1 Jan 2011 to 31 Dec 2021 were included. Data were collected on disease presentation and diagnostic workup at the initial exam, and treatment. Results: A total of 51 patients were included in the study, of which 72.55% patients were female, and 72.55% were over 70. Of 50 patients with available data in medical documentation, 8 (16.00%) had systemic disease (S-GCA), while the rest had cranial disease (C-GCA). Headache, considered also a pathognomonic sign, was the most common initial symptom (88.00% in whole cohort, or 97.62% if S-GCA is excluded). Of 49 patients, all had increased erthyrocyte sedimentation rate. Temporal artery biopsy was positive in 12/16 patients with C-GCA, while temporal artery ultrasound was positive in 12/16 patients. Biopsy or ultrasound was not performed in 18 patients, of which 8 were patients with S-GCA, and data were not available for one patient. No patients had both a biopsy and ultrasound performed. All patients were treated with glucocorticoids, 9.80% were also treated with methotrexate, 7.84% with toclizumab, and one with both methotrexate and azathioprine. Conclusion: These results are comparable to other centres in Croatia and at least one centre abroad., Cilj: Prikazati bolesnike s GCA u našem centru tijekom 10-godišnjeg razdoblja kako bi se potvrdila trenutna saznanja o arteritisu divovskih stanica (GCA) i uočile eventualne specifičnosti naše kohorte. Ispitanici i metode: U retrospektivnu studiju uključeni su svi ispitanici s dijagnosticiranim GCA u Kliničkom bolničkom centru Rijeka od 1. siječnja 2011. do 31. prosinca 2021. Prikupljeni su podatci o njihovoj kliničkoj slici te dijagnostičkoj obradi pri prvom kliničkom pregledu te o terapiji. Rezultati: U studiju je uključen 51 ispitanik, od kojih je 72,55 % ženskog spola te je 72,55 % starije od 70 godina. Od 50 ispitanika za koje postoje podatci u dokumentaciji, 8 (16 %) ih je imalo sistemski oblik (S-GCA), a ostali kranijalni (C-GCA). Glavobolja, koja je ujedno patognomonični znak, bila je najčešći inicijalni simptom (88 % za cijelu kohortu, odnosno 97,62 % ako se isključi S-GCA). Od 49 ispitanika za koje imamo podatke, svi imaju ubrzanu sedimentaciju eritrocita. Biopsija temporalne arterije pozitivna je u 12/16 ispitanika s C-GCA, dok je ultrazvuk bio pozitivan također u 12/16 ispitanika. U 18 bolesnika nije rađena ni biopsija ni ultrazvuk, od kojih su 8 bili bolesnici sa S-GCA te o jednom bolesniku nemamo podatake. Niti jednom pacijentu nisu rađeni i biopsija i ultrazvuk. Svi su ispitanici liječeni s glukokortikoidima, 9,80 % ispitanika je uz to liječeno metotreksatom, 7,84 % s tocilizumabom, dok je jedan od ispitanika liječen s metotreksatom i s azatioprinom. Zaključak: Rezultati su usporedivi s dvama centrima u Hrvatskoj i najmanje jednim inozemnim centrom.

We describe the case of a patient who presented with symptoms of persistent headaches, left-sided facial pain and blurry vision of the left eye. The patient had recovered from a herpes zoster infection of the V1 division of the trigeminal nerve 1 month prior. Serum inflammatory markers were elevated, raising concern for temporal arteritis. Empiric high-dose prednisone was initiated. Bilateral temporal artery biopsies were performed but did not show evidence of vasculitis or multinucleated giant cells. Instead, extracellular material deposits were present within the vessel walls. Congo red staining was diagnostic for amyloidosis. Liquid chromatography and mass spectrometry identified the amyloid fibrils to be transthyretin-type (ATTR) consistent with age-related amyloidosis. Temporal artery involvement of amyloidosis is rare but when present is most often due to light chain amyloidosis. Based on our review of the literature, only a few cases of temporal artery ATTR amyloidosis have been reported.

Background: Takayasu arteritis (TA) is a large vessel vasculitis that may complicate with cerebrovascular ischemic events. The objective was to describe clinical and vascular features of TA patients with cerebrovascular ischemic events and to identify risk factors for these events. Methods: We analyzed the prevalence and type of stroke/transient ischemic attack (TIA), factors associated with cerebrovascular ischemic events, and stroke-free survival in a large cohort fulfilling the American College of Rheumatology or Ishikawa criteria of TA. Results: Among 320 patients with TA (median age at diagnosis, 36 [25–47] years; 261 [86%] women), 63 (20%) had a stroke (n=41; 65%) or TIA (n=22; 35%). Ischemic event localized in the carotid territory for 55 (87%) patients and the vertebral artery territory in 8 (13%) patients. Multiple stenosis were observed in 33 (52%) patients with a median number of stenosis of 2 (minimum, 0 to maximum, 11), and aneurysms were observed in 10 (16%) patients. A history of stroke or TIA before TA diagnosis (hazard ratio [HR], 4.50 [2.45–8.17]; P P =0.05), myocardial infarction history (HR, 0.21 [0.05–0.89]; P =0.039), thoracic aorta involvement (HR, 2.05 [1.30–3.75]; P =0.023), time from first symptoms to diagnosis >1 year (HR, 2.22 [1.30–3.80]; P =0.005), and aspirin treatment (HR, 1.82 [1.04–3.19]; P =0.035) were associated with cerebrovascular ischemic event. In multivariate analysis, time from first symptoms to TA diagnosis >1 year (HR, 2.16 [1.27–3.70]; P =0.007) was independently associated with cerebrovascular ischemic events in patients with TA. The HR for cerebrovascular ischemic event in patients who already experienced a stroke/TIA was 5.11 (2.91–8.99; P Conclusions: Carotid stroke/TIA is frequent in TA. We identified factors associated with cerebrovascular ischemic events.

Post-transplant lymphoproliferative disorder is a growing complication of kidney transplantation and is associated with a poor prognosis. Anti-CD19 chimeric antigen receptor (CAR) T-cell therapy is an important new treatment option modifying the outcome of refractory hematological cancers. Here, we report the case of a 40-year-old kidney transplant recipient who developed a Burkitt-like lymphoma with 11q aberration 5 years after transplantation. After 3 unsuccessful lines of chemotherapy, it was decided to treat the patient with anti-CD19 CAR T cells as a salvage therapy. Three months after CAR T-cell infusion, she experienced a grade IIB T cell-mediated rejection with severe tubulitis (T3), slight interstitial inflammation (I1), and severe intimal arteritis (V2) with blood suffusion. Among T cells infiltrating the graft, some of them expressed the anti-CD19 CAR. CAR T cells within the graft and in blood samples were also detected by droplet digital polymerase chain reaction. Function of the kidney transplant improved after corticosteroid treatment and remained stable. However, lymphoma progressed, with a massive pulmonary mass leading to the patient's death 10 months after CAR T-cell infusion.

Aims Inflammation is involved in various processes of atherosclerosis development. Serum C-reactive protein (CRP) levels, a predictor for cardiovascular risk, are reportedly reduced by statins. However, several studies have demonstrated that CRP is a bystander during atherogenesis. While S100A12 has been focused on as an inflammatory molecule, it remains unclear whether statins affect circulating S100A12 levels. Here, we investigated whether atorvastatin treatment affected S100A12 and which biomarkers were correlated with changes in arterial inflammation. Methods We performed a prospective, randomized open-labeled trial on whether atorvastatin affected arterial (carotid and thoracic aorta) inflammation using 18fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG-PET/CT) and inflammatory markers. Thirty-one statin-naive patients with carotid atherosclerotic plaques were randomized to either a group receiving dietary management (n=15) or one receiving atorvastatin (10mg/day, n=16) for 12weeks. 18F-FDG-PET/CT and flow-mediated vasodilation (FMD) were performed, the latter to evaluate endothelial function. Results Atorvastatin, but not the diet-only treatment, significantly reduced LDL-cholesterol (LDL-C, -43%), serum CRP (-37%) and S100A12 levels (-28%) and improved FMD (+38%). 18F-FDG-PET/CT demonstrated that atorvastatin, but not the diet-only treatment, significantly reduced accumulation of 18F-FDG in the carotid artery and thoracic aorta. A multivariate analysis revealed that reduction in CRP, S100A12, LDL-C, oxidized-LDL, and increase in FMD were significantly associated with reduced arterial inflammation in the thoracic aorta, but not in the carotid artery. Conclusions Atorvastatin treatment reduced S100A12/CRP levels, and the changes in these circulating markers mirrored the improvement in arterial inflammation. Our observations suggest that S100A12 may be an emerging therapeutic target for atherosclerosis.

We aimed to assess the clinical and radiological characteristics of immunoglobulin G4-related coronary periarteritis through a systematic literature review and from our case series. In the systematic literature review, we assessed English language manuscripts on immunoglobulin G4-related coronary periarteritis cases. Additionally, we identified patients with immunoglobulin G4-related coronary periarteritis at St. Luke's International Hospital in Tokyo, Japan, from 2014 to 2020. We summarized patients' demographics, immunoglobulin-G and -G4 titers, site and morphological features of the coronary lesion, and other organ involvements. We identified 38 cases from the literature and four patients from our institute. Coronary lesions were detected using coronary computed tomography in 40 (95.2%) patients. Mass-like or diffuse wall-thickening lesion was the most frequently observed type in 33 (78.6%) patients. No trends at the site of the coronary arteries were identified. Overall, 32 (76.1%) patients had multiple-organ involvement, of which the most common lesion was peri-aortitis in 21 (50.0%) patients. Ten (23.8%) patients with an isolated coronary lesion had significantly lower immunoglobulin-G4 titers than those with other organ involvements (immunoglobulin-G4: 261 [161.0, 564.0] vs. 1355.0 [320.8, 2480.0] mg/dL, p = 0.033). The wall-thickening lesions responded well to immunosuppressive treatments. Mass-like or diffuse wall-thickening on coronary computed tomography is a characteristic radiographic finding of immunoglobulin G4-related coronary periarteritis, which can occur in any branch. Immunoglobulin G4-related coronary periarteritis showed similar characteristics to other organ lesions, including its relatively low serum immunoglobulin-G4 level in patients with a single-organ disease and its high responsiveness to glucocorticoids.

Vascular rejection (VR) is characterized by arteritis, steroid resistance, and increased graft loss but is poorly described using modern diagnostics.We screened 3715 consecutive biopsies and retrospectively evaluated clinical and histological phenotypes of VR (n = 100) against rejection without arteritis (v0REJ, n = 540) and normal controls (n = 1108).Biopsy sample size affected the likelihood of arterial sampling, VR diagnosis, and final Banff v scores ( P0.001). Local v and cv scores were greatest in larger arteries (n = 258). VR comprised 15.6% of all rejection episodes, presented earlier (median 1.0 mo, interquartile range, 0.4-8 mo) with higher serum creatinine levels and inferior graft survival, versus v0REJ ( P0.001). Early VR (≤1 mo) was common (54%) and predicted by sensitization, delayed function, and prior corticosteroid use, with associated acute dysfunction and optimal therapeutic response, independent of Banff v score. Late VR followed under-immunosuppression in 71.4% (noncompliance 38.8%, iatrogenic 32.6%), and was associated with chronic interstitial fibrosis, incomplete renal functional recovery and persistent inflammation using sequential histopathology. The etiology was "pure" antibody-mediated VR (n = 21), mixed VR (n = 36), and "pure" T cell-mediated VR (n = 43). Isolated VR (n = 34, Banff i1 without tubulitis) comprised 24 T cell-mediated VR and 10 antibody-mediated VR, presenting with mild renal dysfunction, minimal Banff acute scores, and better graft survival compared with inflamed VR. Interstitial inflammation influenced acute renal dysfunction and early treatment response, whereas chronic tubulointerstitial damage determined long-term graft loss.VR is a heterogenous entity influenced by time-of-onset, pathophysiology, accompanying interstitial inflammation and fibrosis. Adequate histological sampling is essential for its accurate diagnostic classification and treatment.

Immunoglobulin-G4-related disease (IgG4-RD) is a multi-organ systemic inflammatory disorder. The ideal treatment of coronary artery involvement in IgG4-RD remains uncertain due to its rarity. We herein report a case of coronary artery involvement with IgG4-RD, wherein mass lesions surrounded the coronary arteries with a moderate stenosis lesion in the right coronary artery (RCA). The fractional flow reserve (FFR) of the RCA was 0.76. After steroid therapy, the mass lesions around the coronary arteries improved. The FFR of the RCA also improved from 0.76 to 0.86. These findings suggest the efficacy of using steroid therapy for coronary artery involvement with IgG4-RD.

Takayasu's arteritis is an inflammatory disease of unknown etiology that causes stenosis, occlusion, or dilatation of the aorta and its major branches, the pulmonary arteries, and the coronary arteries. The incidence of extracranial carotid artery aneurysm in patients with Takayasu's arteritis is reportedly 1.8%-3.9%. We herein report a patient with Takayasu's arteritis who presented with transient left hemiplegia immediately after neck massage. Carotid ultrasonography revealed a thrombus within the fusiform aneurysm on the right common carotid artery. We speculated that fragmentation from the intra-aneurysmal thrombus was caused by neck massage.

The association between Takayasu’s arteritis and membranous nephropathy is uncommon. We present the case of a 46-year-old man with Takayasu’s arteritis treated over 10 years by a multidisciplinary medical team. He had an atrophic left kidney due to arterial stenosis, with a basal creatinine of 1.59 mg/dL (140.55 µmol/l). Three years ago, he presented with full nephrotic syndrome, uncontrolled blood pressure, creatinine increases to 4.5 mg/dL (basal: 1.59 mg/dL), severe hypoalbuminaemia (1.4 g/dL) and albuminuria of 24.6 g per day. He underwent percutaneous biopsy of the right kidney that showed membranous nephropathy with negative PLA2R1 and positive IgG 1, 3 and 4 subclasses. After therapy with oral prednisone and cyclophosphamide, the patient’s kidney function improved, without recurrence of disease after 3 years of follow-up. Here, we present this extremely uncommon association of Takayasu’s arteritis and membranous nephropathy.

Background. Rheumatoid arthritis (RA) belongs to the most common inflammatory diseases of the joints, and in its pathogenetic constructions, a certain role is played by the changes in the autonomic nervous system (ANS). Aim of the study: to evaluate the vegetative passport in RA, the incidence and nature of vegetative dysfunction (VD) in different types of the disease, the relationship with the parameters of variation pulsometry. Materials and methods. There were examined 173 patients with RA aged 18 to 79 years (an average of 46 years), among them there were 21 % of males and 79 % of females. The duration of the disease was 10 years, type of RA, seropositive in terms of rheumatoid factor and citrulline antibodies, was detected in 3/4 of cases, extra-articular (systemic) form of the disease was diagnosed in 37 % of observations, osteoporosis was found in 67 % of patients. Results. VD is observed in 20 % of RA patients, mainly in those with seropositive variant of the disease that is associated with higher values of bone mineral density, larger changes in the variation pulsometry, with the influence on the integral nature of articular syndrome and the rate of involvement in the pathological process of elbow and hip joints, and the severity of autonomic disorders is closely linked to the presence of intra-articular arthrocalcifications, digital arteritis and dyscirculatory encephalopathy. Vegetative passport defines the integral clinical and laboratory and instrumental RA symptoms, while the ANS type is affected by the seropositivity of the disease and the bone density, with the highest level in cases of vagotonia, when VD directly correlates with the joint pathology progression rate, and in sympathicotonia — with intial parameters of rheumatoid factor and arthritis activity, also, regardless of the vegetative status, there are variance-correlation ratios of specific parameters of RA course and variation pulsometry. Conclusions. VD occurs in every fifth patient with RA, is associated with the peculiarities of the articular syndrome course and extra-articular signs of the pathological processes, is involved in disease pathogenesis constructions, and in the future it will be useful to identify actively ANS changes for subsequent timely rehabilitation.

International audience; Background: Inflammation of unknown origin (IUO) is a challenging situation in internal medicine.Objectives: To describe the final diagnoses in IUO and assess the helpfulness of 18F-fluorodesoxyglucose positron emission tomography with computerized tomography (18F-FDG-PET/CT) in the diagnosis strategy.Results: A total of 317 IUO patients with 18F-FDG-PET/CT were enrolled. A diagnosis was reached in 228 patients: noninfectious inflammatory diseases (NIID) (37.5%), infectious diseases (18.6%), malignancies (7.9%), and non-systemic-inflammatory miscellaneous diseases (7.9%). The two leading causes of NIID were polymyalgia rheumatica and giant cell arteritis. 18F-FDG-PET/CT results were classified as true positive in 49.8% of patients and contributory in 75.1% of overall IUO patients (after the complete investigation set and a prolonged follow-up). In multivariate analysis, only C-reactive protein minimum level (≥50 mg/L) was associated with the contributory status of 18F-FDG-PET/CT.Conclusion: Within the wide spectrum of IUO underlying diseases, 18F-FDG-PET/CT is helpful to make a diagnosis and to eliminate inflammatory diseases. Obese patients constitute a specific group needing further studies

The decrease of whole protein in synovial fluid, nitrites, uric and lactic acids against the background of the increase of glucose level and C-reactive protein is observed in patients with osteoarthrosis of second synovitis of knee joints (control group), at that, the degree of activity and roentgenological stage of the disease, presence of digital arteritis and the impairment of endocardium influence the integral surfactant condition of the synovial fluid, and the content of whole protein and β2-microglobulin depend accordingly on the indices of its severity and disease progressing, but dynamic interfacial tension, viscous, elastic and viscoelastic adsorption-rheological properties of articular liquor are closely connected with active surface properties., По сравнению с больными остеоартрозом со вторичным синовитом коленных суставов (контрольная группа) при ревматоидном гоните наблюдается уменьшение содержания в синовиальной жидкости общего белка, нитритов, мочевой и молочной кислоты на фоне повышения уровней глюкозы и С-реактивного протеина, причем на интегральное сурфактантное состояние синовии влияют степень активности и рентгенологическая стадия заболевания, наличие дигитального артериита и поражения эндокарда. Кроме того содержание общего белка и β2-микроглобулина зависит соответственно от индексов тяжести течения и прогрессирования заболевания, а с поверхностно-активными веществами тесно связаны динамическое межфазное натяжение, вязкие, упругие и вязкоэластичные адсорб­ционно-реологические свойства суставного ликвора., Порівняно із хворими на остеоартроз із вторинним синовітом колінних суглобів (контрольна група) при ревматоїдному гоніті спостерігається зменшення вмісту в синовіальній рідині загального білка, нітритів, сечової й молочної кислоти на тлі підвищення рівнів глюкози та С-реактивного протеїну, причому на інтегральний сурфактантний стан синовії впливають ступінь активності та рентгенологічна стадія захворювання, наявність дигітального артеріїту й ураження ендокарда, до того ж вміст загального білка та β2-мікроглобуліну залежить відповідно від індексів тяжкості перебігу та прогресування захворювання, а з поверхнево-активними речовинами щільно пов’язані динамічний міжфазний натяг, в’язкі, пружні й вязкоеластичні адсорбційно-реологічні властивості суглобового ліквору.

C-Reactive protein and erythrocyte sedimentation rate are crucial parameters used to monitor giant cell arteritis (GCA). Given that tocilizumab is approved for the treatment of GCA, these parameters are less sensitive because of the effects of interleukin-6 receptor blockade. Thus, the optimal method for monitoring GCA patients undergoing tocilizumab therapy, especially patients exhibiting a persistent thickened vessel wall in large vessels, remains unclear. Contrast-enhanced ultrasonography (CEUS) can increase the visibility of tissue perfusion by slow blood flow, which cannot be detected by power color doppler. We used CEUS to investigate patients with active and inactive GCA of the large vessels (active large vessel arteritis [aLVV]/inactive large vessel arteritis [iLVV]) who were not administered tocilizumab in this proof-of-concept study. After injection of the ultrasound contrast agent, the contrasted area (CA) of large vessels in a transverse section was calculated twice: first when the lumen was contrasted completely and once again 4–8 s later. We investigated the value of increase in CA that exhibited the best sensitivity and specificity for aLVV. Twenty-four patients were included in this study: 15 with aLVV and 9 with iLVV. The CA increased from 32.2 ± 16.8 to 52.5 ± 21.3 mm2 (p

Background: Takayasu’s Arteritis (TA) is a large vessel vasculitis with diverse clinical presentations and arterial vascular bed involvement.It is characterized by chronic, nonspecific inflammation of all layers of the vessel wall which results in stenosis, occlusion, dilatation, or aneurysm formation in the involved blood vessels. Methods: The study included 36 patients of TA. All patients fulfilled the modified Ishikawa’s diagnostic criteria for TA. All patients were evaluated for clinical presentation, angiographic findings, and severity of the disease. The disease activity was assessed based on Erythrocyte Sedimentation Rate (ESR) and C-Reactive Protein (CRP) and also by CT Angiography (CTA)/Magnetic resonance angiography (MRA)imaging. The angiographic types were classified based on the International TA Conference in Tokyo 1994 angiographic classification. Results: A total of 36 patients were included in the study, 86%were females and a mean age of 21.6 years. Hypertension (78%) was the most common clinical presentation. Type V was the most common angiographic type (42%) followed by type III (25%), type IV (14%), type IIb (11%), type I (5%) and type IIa (3%). Among the aortic arch branches the left subclavian artery (50%), right subclavian artery (38.8%), left vertebral artery (33.3%) and left common carotid artery (27.7%) were the most commonly involved arteries. Disease activity based on CT / MR imaging showed a significant statistical correlation with elevated ESR and positive CRP (p < 0.0001). Mediastinal lymphadenopathy was seen in 21 patients out of which 11 had active disease. However, no significant correlation was found between mediastinal lymphadenopathy and disease activity. Conclusions: TA presents with varied symptomatology and differing vascular involvement. CT/MR angiography is effective in diagnosis and accurately predicted the active stage of the disease.

IgG4-related disease (IgG4-RD) is a systemic autoimmune disorder known to affect multiple organs. However, IgG4-RD rarely affects the myocardium. We herein report a case of left ventricular dysfunction due to cardiac involvement of IgG4-RD.

Chronic inflammation can stimulate the formation and progression of atherosclerotic plaques and increase the vulnerability of plaques. However, there are few studies on the changes of carotid inflammatory plaques during treatment. Our study attempted to investigate the use of superparamagnetic iron oxide nanoparticle (SPION) ultrasound imaging to detect the expression of vascular cell adhesion molecule-1 (VCAM-1) in patients with carotid plaques and analyze the effects of SPION ultrasound imaging in inflammatory plaque visualization effect. SPION microbubble contrast agents have good imaging effects both in vivo and in vitro. We conjugated the VCAM-1 protein to the microbubbles wrapped in SPIONs to form SPIONs carrying VCAM-1 antibodies. Observe the signal intensity of SPIONs carrying VCAM-1 antibody to arteritis plaque. The results showed that the SPION contrast agent carrying VCAM-1 antibody had higher peak gray-scale video intensity than the other two groups of contrast agents not carrying VCAM-1 antibody. It shows that SPIONs have excellent imaging effects in ultrasound imaging, can evaluate the inflammatory response of arterial plaque lesions, and are of great significance for the study of carotid inflammatory plaque changes.

BACKGROUND/OBJECTIVE High-dose glucocorticoids (GCs) are required in the initial treatment of systemic vasculitis. However, slow or delayed tapering can lead to unnecessary GC exposure and toxicity. In this quality improvement initiative, we aimed to increase appropriate GC tapering among newly referred patients awaiting specialty consultation at a tertiary vasculitis clinic. METHODS For each patient referred for anti-neutrophil cytoplasm antibody-associated vasculitis (AAV) or large vessel vasculitis (LVV), recommendation-based GC tapering suggestions were faxed to referring physicians. To maximize uptake, the intervention format was modified according to feedback from referring physicians' offices. The proportion of new patients presenting to their first appointment who (1) had started to taper GCs, (2) were taking their target GC dose according to recommendations, (3) experienced a vasculitis flare during tapering were compared before (July 2017-January 2019) and after (February-October 2019) the intervention. RESULTS Among 169 consecutive patients referred for AAV or LVV, the proportion who had started to taper GCs by their first visit increased from 84 of 117 (72%) preintervention to 49 of 52 (94%) postintervention (p < 0.01). Mean daily prednisone dose at first visit decreased from 29.9 (SD, 18) mg to 21.7 (SD, 14) mg (p < 0.01). However, the proportion who were ultimately taking "target" GC doses at their first visit did not significantly increase (72% vs. 77%). Disease flares during tapering were similar before and after the intervention (9% vs. 12%). CONCLUSIONS Patients with AAV and LVV had increased GC tapering and lower GC doses at first visit following a preappointment intervention. Further strategies are needed to improve timely GC tapering in vasculitis.

Takayasu arteritis is a rare type of chronic, granulomatous vasculitis, characterized by inflammation of blood vessels of large caliber, such as the aorta, and its branches. Clinical presentation varies, depending on the severity of symptoms. Onset may be gradual, however at times, presentation may be acute, and life threatening. Herein, we present the case of a 29-year-old female, 3 months post-op, following a right carotid artery stenting procedure. The patient presented with nonspecific symptoms of malaise, arthralgia, and blurry vision. Clinical presentation and imaging findings were consistent with Takayasu's Arteritis.

Kawasaki disease (KD) is an acute self-limited syndrome that predominantly affects children. Coronary sequelae have been identified to be responsible for a small, but significant percentage of young adults who present with myocardial ischemia. In this study, we present a case of an elderly patient with possible coronary sequelae of KD. A 76-year-old man was referred to our outpatient department for silent myocardial ischemia. Axial images of coronary computed tomography showed multiple lumens in the proximal left anterior descending (LAD) artery. Coronary angiography demonstrated braid-like appearance in the proximal and distal segment of the LAD. Coronary intervention was successfully performed for the proximal LAD lesion using directional atherectomy (DCA) catheter. Microscopic examination of the DCA specimens showed the following histological features: tissues in densely hyalinized fibrosis with occasional microcalcification, or those containing a number of smooth muscle cells (SMCs) with myxoid extracellular matrix. There was paucity of cholesterin crystals and aggregation of foamy cells. In addition, scarcely any inflammatory cell filtration was identified. In the section of SMC-containing samples, formation of multiple re-canalized vessels embracing endothelial cells was confirmed. These histopathologic findings indicated that the present coronary artery lesion has a high possibility of very late cardiovascular sequelae caused by arteritis due to KD, rather than arteriosclerosis. This is the oldest adult case with coronary artery disease possibly resulting from KD sequelae. This case highlights that KD sequelae must be considered as a cause of coronary artery lesion even in older patients.

Giant cell arteritis (GCA), frequently associated with polymyalgia rheumatica (PMR), and Takayasu's arteritis (TAK) are characterized by extensive vascular remodeling that results in occlusion and stenosis. The pathophysiological mechanisms underlying the onset of GCA/PMR and TAK are still hypothetical. However, similarities and differences in the immunopathology and clinical phenotypes of these diseases point toward a possible link between them. The loss of tolerance in the periphery, a breakdown of tissue barriers, and the development of granulomatous vasculitis define a disease continuum. However, statistically powered studies are needed to confirm these correlations. In addition to glucocorticoids, inhibition of the interleukin-6 axis has been proposed as a cornerstone in the treatment of GCA/PMR and TAK. Novel biologic agents targeting the pathogenic pathway at various levels hold promise to achieve glucocorticoid-free sustained remission.

Takayasu’s arteritis (TA) is a vasculitis with a predilection for young women. Left ventricular pseudoaneurysm (PSA) in TA is a rare phenomenon. We report a 36 years old Filipina who presented with heart failure symptoms. Years prior, she had a recurrent fever, headache, myalgia and left arm claudication. On workup, a 2D echo revealed a left ventricular PSA with mural thrombus and moderate mitral regurgitation. Cardiac MRI further characterised the PSA with a sac diameter of 8×7.5×8.4 cm (CC×T×AP). Carotid Duplex Scan revealed total occlusion of the mid to distal right common carotid artery and left subclavian artery. She was started on immunosuppresants and guideline-directed medical therapy (GDMT) for heart failure and subsequently underwent successful endoventricular patch closure and mitral valve repair. This case highlights the importance of actively searching for cardiac complications of TA which although very rare, can dominate the clinical picture and may carry a dismal prognosis if left untreated.

Temporal artery biopsy (TAB) is one of the diagnostic tools to confirm the diagnosis of giant cell arteritis (GCA). We aim to evaluate the clinical and technical determinants of a positive biopsy. Demographics, clinical, technical, and laboratory data of all TAB’s performed between 2007 and 2019 at a single academic medical center. 107 biopsies performed for 103 patients were included; 72.9% were female, and 27.1% were male. The mean age at the time of biopsy was 67.1 ± 9.3 years. One biopsy was excluded for lack of arterial tissue content. Of the remaining 106, 19.6% were positive. The length of the biopsy and the number of arterial cross-sections were not significantly associated with its result. A positive biopsy was seen more in patients with low albumin (p = 0.010) and hypothyroidism (p = 0.017) but less in those with prior glucocorticoids treatment (p = 0.028). Predictors of a positive biopsy included male gender [OR 4.029, 95% CI (1.330–12.209), p = 0.014]; elevated ESR [OR 3.998, 95% CI (1.908–6.787), p = 0.023]; polymyalgia rheumatica (PMR) symptoms [OR 5.121, 95% CI (2.094–9.872), p = 0.001]; and advancing in age (6.5% per every additional year), [OR 1.065, 95% CI (1.005–1.130), p = 0.033]. 53.7% of the patients were eventually diagnosed with GCA; 39.2% of them were based on positive biopsy. In conclusion, old age, male gender, elevated ESR, and PMR symptoms increase the odds of positive TAB. Technical factors, such as biopsy length and the number of cross-sections, did not influence eventual biopsy results, highlighting the pivotal role of the clinical presentation of the patients in selecting patients for TAB.

Grosgefasvaskulitiden fuhren zu einer Entzundung groser und mittelgroser Arterien. Zu ihnen zahlen die Riesenzellarteriitis (RZA) und die Takayasu-Arteriitis (TAK). An der RZA erkranken vorwiegend altere Menschen in der 7. und 8. Lebensdekade, wahrend die TAK uberwiegend junge Frauen im Alter zwischen 20 und 40 Jahren betrifft. Die RZA stellt die haufigste Vaskulitisform der westlichen Welt dar; die TAK tritt hingegen deutlich seltener im europaischen Raum auf. Die Entzundung der Gefaswande fuhrt zu ischamischen Komplikationen, wobei bei der RZA haufiger die Kopfarterien inklusive Augenarterien betroffen sind. Es besteht dann die Gefahr einer akuten Erblindung, die nur durch eine fruhzeitige Diagnose und Therapieeinleitung verhindert werden kann. Beide Vaskulitisformen sind zudem durch eine ausgepragte Entzundungskonstellation in Verbindung mit Fieber, Abgeschlagenheit und Gewichtsverlust gekennzeichnet. Zur Diagnosesicherung kommen bildgebende und histopathologische Verfahren zum Einsatz, wobei sich zur fruhen Diagnostik einer RZA vor allem die Sonografie bewahrt hat. Glukokortikoide stellen die Basis der medikamentosen Therapie dar und werden bei der RZA zunehmend haufig und bei der TAK regelmasig um weitere Immunsuppressiva erganzt. The 2 major forms of large-vessel vasculitides are giant-cell arteritis (GCA) and Takayasu arteritis (TAK). The incidence of GCA climbs continuously during the 7th and 8th decades of life, while TAK peaks in individuals younger than 40 years. GCA is the most frequent vasculitis in the Western world, and the risk of developing TAK is higher in individuals of Asian and Central/South American origins. Especially in GCA, vascular stenosis or occlusion of cranial or ocular arteries may result in vision loss, a complication that can only be prevented by early diagnosis and immediate treatment. Both vasculitides share a strong extravascular inflammation manifesting with an intense acute phase response, and constitutional symptoms. Histopathology and diagnostic imaging are required to establish the diagnosis of GCA or TAK. Ultrasound-based methods are very useful for rapid diagnosis of GCA in patients with imminent vision loss. Corticosteroids are the immunosuppressive drug of choice for large-vessel vasculitides, sometimes supplemented by other immunosuppressive drugs.

Objective: To describe the characteristics, assessment and management of older emergency department (ED) patients with non-traumatic headache. Methods: Planned sub-study of a prospective, multicentre, international, observational study, which included adult patients presenting to ED with non-traumatic headache. Patients aged ≥75 years were compared to those aged

Background. Among all rheumatologic disea­ses, rheumatoid arthritis (RA) accounts for 3 % of cases, and the number of such patients is increasing. RA is characteri­zed by systemic vascular lesion (angiopathy), it is determined by the international Chapel Hill classification as “vasculitis associated with systemic disease”. The purpose of the study was to improve the quality of diagnosis, to stablish new links in the pathogenesis and to identify the prognostic criteria for the clinical course of vascular disease in RA. Materials and methods. 131 patients were examined. The ratio of men and women was 1 : 2, the mild, moderate and high degree of the disease activity was 1 : 2 : 1, respectively, the average age of the examined patients was 45.70 ± 1.02 years, the duration of the clinical manifestation was 9.40 ± 0.68 years, I, II, III and IV stages were diagnosed in 8, 40, 34 and 19 % of patients. Echocardiography, sonography and ultrasonic dopplerography of vessels, conjunctival biomicroscopy, morphologi­cal examination of kidney biopsy were preformed, integrated indices of clinical and instrumental vascular pathology were determined. Results. Systemic angiopathy is observed in 61 % of patients with RA, more often in cases of high activity with the presence of osteoporosis, the development of skin vasculitis and peripheral vasoneuropathy are closely related to the serum levels of cyclic citrullinated peptide antibodies, which, along with the concentration of C-reactive protein, has a nega­tive prognostic significance for the vascular pathology, and the appearance of digital arteritis is determined by the activity of the joint syndrome, the appearance of glomerulonephritis — by the high content in the blood of circulating immune complexes, and angiopathy presence reflects the pressure increase in the lesser (pulmonary) circulation. Conclusions. In patients with RA, mesangial proliferative and mesangial capillary glomerulonephritis develops in a ratio of 2 : 1, with an appropriate tubulointerstitial component and the immunoglobulins and complement components deposition (in the stroma > glomeruli > tubules > vessels), at that the structural changes in the renal vessels are closely associated with clinical instrumental manifestations of systemic rheumatoid angiopathy. C-reactive protein values in the blood more than 25 mg/L and anti-citrulline antibodies more than 40 U/ml are prognostic negative criteria for systemic vascular pathology and the kidney stroma damage, severe changes in renal tubules are a risk factor for the high rates of the joint syndrome progression.

Concept of No-Reflow (NR) phenomenon is largely studied in cardiological intervention characterised by the failure of myocardial reperfusion despite the absence of mechanical obstruction of large and medium sized vessels. NR negatively affects outcome of procedure, so there is great importance of prediction and management. The objective was to introduce concept of NR in neuro-intervention and have an idea of incidence and independent predictors of NR in patients with post intervention no-reflow in large and medium sized brain arteries.This was a single-centre prospective case– control study between march 2017-march 2021 in the department of Neurosurgery, Dr. R.M.L.I.M.S., Lucknow, India. Cases were subjects who suffered NR, and the control comparators were those who did not. Clinical outcomes were documented. Salient variables relating to the patients and their presentation, history and angiographical findings were documented.Of 153 consecutive patients, 11(7.2%) suffered from NR, with all cases occurring post intervention in the form of coiling with or without stent/balloon support or flow diverter placement with or without coiling. Patients with NR had increased risk of in-hospital death and disability (1 patient/9.1% death, 8 patients/72.72% with one or multiple neurological dysfunction with varying severity) in comparison to patients without NR (4 patients/2.8% deaths and 12 patients/8.5% disabilities, out of 142 patients). From baseline variables available prior to neuro-endovascular surgery, the independent predictors of NR were increased complexity of pathology, admission systolic hypertension, weight of more than 75 kg and history of hypertension, history of DM, history of ischaemic vascular disease/dissection/aneurysm in the brain or elsewhere, history of systemic arteritis, history of autoimmune disease and atherosclerosis, history of smoking, female gender, elderly. Patients with NR have a higher rate of morbidity and mortality following ischaemia and embolism. Predictors of NR include lesion complexity, systolic hypertension and high body weight, female gender, elderly, dyslipidaemia and preexisting arterial disease. Further validation of this risk model is required with lager number of patients and multicenter studies.

Takayasu’s Arteritis is defined as inflammatory disease of the large blood vessels with unknown cause mostly affecting the aorta and its main branches. The patients diagnosed with Takayasu’s arteritis are reported to have complaint of chronic dizziness. However, literature lacks detailed clinical evaluation of the auditory vestibular function in such patients. The current study documents the findings of auditory and vestibular evaluations in a rare syndrome. It was observed that patients with Takayasu’s arteritis had abnormal auditory and vestibular evoked potentials.

Background Renal involvement in ANCA-associated vasculitis (AAV) is associated with poor outcomes. The clinical significance of arteritis of the small kidney arteries has not been evaluated in detail. Methods In a multicenter cohort of patients with AAV and renal involvement, we sought to describe the clinicopathologic characteristics of patients with AAV who had renal arteritis at diagnosis, and to retrospectively analyze their prognostic value. Results We included 251 patients diagnosed with AAV and renal involvement between 2000 and 2019, including 34 patients (13.5%) with arteritis. Patients with AAV-associated arteritis were older, and had a more pronounced inflammatory syndrome compared with patients without arteritis; they also had significantly lower renal survival (P=0.01). In multivariable analysis, the ANCA renal risk score, age at diagnosis, history of diabetes mellitus, and arteritis on index kidney biopsy were independently associated with ESKD. The addition of the arteritis status significantly improved the discrimination of the ANCA renal risk score, with a concordance index (C-index) of 0.77 for the ANCA renal risk score alone, versus a C-index of 0.80 for the ANCA renal risk score plus arteritis status (P=0.008); ESKD-free survival was significantly worse for patients with an arteritis involving small arteries who were classified as having low or moderate risk, according to the ANCA renal risk score. In two external validation cohorts, we confirmed the incidence and phenotype of this AAV subtype. Conclusions Our findings suggest AAV with renal arteritis represents a different subtype of AAV with specific clinical and histologic characteristics. The prognostic contribution of the arteritis status remains to be prospectively confirmed.

Patients undergoing lung resection are often active or former smokers who have chronic disease related to tobacco (coronary artery disease, chronic obstructive bronchitis, arteritis of the inferior limbs…). Those co-morbidities increase the operative risk for surgery in which morbi-mortality is relevant. On top of this, we are witnessing an increasing number of non-small cell lung cancers in an aging population that might show signs of frailty. The challenge for the surgeon is to identify early those co-morbid and/or frail patients by using predictive scores like Thoracoscore, mFI (modified frailty index) or MSK-F1 (Memorial Sloan-Kettering Frailty Index). Screening for those high-risk patients implies adapting surgical management through a multidisciplinary approach. That is the objective of co-managment, which allows geriatricians and surgeons to collaborate, or enhanced recovery after surgery which is more accessible to all group ages. The objective of this article is to review the management of co-morbid patients in thoracic surgery, by detailing certain predictive scores available and the multidisciplinary approaches developed to treat the patients screened.

Background An early and accurate diagnosis of liver antibody-mediated rejection (AMR) followed by timely intervention is important for clinical management but remains challenging. The aim of this study was to assess the clinicopathologic characteristics and outcomes of late acute AMR in pediatric liver transplantation recipients. Methods We performed a retrospective review of 739 ABO-identical/compatible allograft liver biopsies from 199 pediatric transplantation recipients. Results Based on Banff 2016 AMR criteria, 3 recipients fulfilled the criteria for definite for late acute AMR, 2 met the criteria for suspicious for AMR, and 2 were indeterminate for AMR. We further assessed the clinicopathologic characteristics of these 7 patients. All 7 patients had at least 1 biopsy with a histopathologic pattern compatible with acute AMR. Additionally, we observed accompanied moderately to markedly dilated portal/central veins and endothelialitis disproportionate to the degree of bile duct injury in all 7 patients; periportal/perivenular hepatocyte necrosis was seen in 6/7 patients; and arteritis was seen in 3/7 patients. In each case, microvascular C4d deposition was present in at least 1 biopsy. Posttransplant donor specific anti-HLA antibodies were detected in 5 patients. Two of 7 patients were retransplanted, and 2 died after developing refractory AMR. The remaining 5 patients were alive with stable graft function at a median follow-up of 4.1 years. Conclusions Our data suggest that acute AMR in pediatric liver grafts is rare, can develop late, and may be associated with graft loss or patient death. The recurrent histopathologic findings of moderately to markedly dilated portal/central veins and endothelialitis disproportionate to the degree of bile duct injury are features that appear unique to pediatric acute AMR of liver grafts.

Background Hypertension associated with hyperhomocysteinemia (HHcy) is correlated with a high risk of vascular diseases. Studies found that folic acid (FA) supplementation can reduce the risk of cardiovascular and cerebrovascular events. The aim of the present study was to explore the potential mechanisms of FA attenuating HHcy-related arterial injury in spontaneously hypertensive rats (SHRs). Methods 24 SHRs were randomized into the control group, the HHcy group, and the HHcy + FA group (8 per group). The SHRs in the HHcy group and the HHcy + FA group were given DL-Hcy intraperitoneally to mimic hypertension associated with HHcy. The SHRs in the HHcy + FA group were given FA by gavage to mimic an FA-fortified diet. The histopathology and immunohistochemistry of rat aorta and carotid artery were analyzed, and the relative expression levels of immune/inflammation and oxidative stress molecules in arterial tissue were detected by quantitative real-time polymerase chain reaction (qRT-PCR) and Western blot. Results FA significantly reduced the expression levels of nuclear factor-κ-gene binding (NF-κB) p65/Rela and interleukin-6 (IL-6) in rat arterial tissues, as well as the levels of plasma HHcy and serum malondialdehyde (MDA) in hypertension associated with HHcy rats ( p < 0.05). At the same time, FA significantly increased the serum superoxide dismutase (SOD) level in hypertension associated with HHcy rats, and even the SOD level of the HHcy + FA group was higher than that of the control group ( p < 0.05). However, HHcy induced the opposite results of the above indicators in SHRs compared with the control group ( p < 0.05). Conclusions The arterial protection mechanisms of FA are related to reducing the concentration of HHcy to eliminate the tissue toxicity of HHcy, inhibiting NF-κBp65/Rela/IL-6 pathway molecules to regulate inflammatory response, and promoting the potential anti-oxidative stress pathway molecules to reduce oxidative stress level.

Background: Neurological manifestation of Takayasu’s Arteritis (TA) in pregnancy presenting as convulsive syncope is extremely rare, and poses a serious diagnostic dilemma due to other vast causes of fits in pregnancy. Objective: We aimed to present and shed more light on a case of TA with convulsive syncope in pregnancy refractory to anticonvulsants for seven weeks, and review the literature on the management of TA in pregnancy. Case presentation: A gravida 4 para 3+0 at 28 weeks of amenorrhea presented with repeated episodes of the sudden loss of consciousness, followed by a fall and jerking of the limbs. These were refractory to anticonvulsants that she had used for seven weeks. Physical examination revealed undetectable pulse and blood pressure (BP) in the upper limbs but elevated BP in the lower limbs. Further investigations confirmed TA and she improved on steroids and antihypertensives. Conclusion: This case typically describes the unexpected presentation of TA with convulsive syncope. It calls for meticu- lous clinical assessment of epileptic seizures in pregnancy to avoid a late diagnosis of TA and its potential poor outcomes. Keywords: Takayasu’s Arteritis in pregnancy; convulsive syncope; case report.

OBJECTIVES Predictive data for the development of aortic dilatation (AD) in giant-cell arteritis (GCA) are controversial. The aim was to investigate by computed tomography (CT) the prevalence of AD in a consecutive cohort of GCA patients and controls, and to identify possible predictors for AD. METHODS GCA patients and controls were identified by electronic search and underwent aortic contrast enhanced CT defining AD by aortic diameter adjusted to age, gender and body surface area. Pulse-wave velocity, intima-media thickness (IMT) and laboratory studies including lymphocyte subsets were conducted identifying potential factors associated with AD. Clinical and laboratory parameters at disease onset, occurrence of aortic rupture/dissection before and up to five years after study visit were retrieved by chart review. RESULTS 144 GCA patients and 115 controls were included. GCA patients developed more frequently AD of the ascending and thoracic descending aorta compared to controls (OR 2.60, p = 0.016; OR 3.65, p = 0.005, respectively). Factors associated with AD development of thoracic descending aorta, but not of the ascending aorta, were higher percentages of circulating CD3+CD4+ cells, higher CD4/CD8 ratio, presence of polymyalgia rheumatica and increased carotid IMT at disease onset (OR range 1.10-3.11, all with p

The present study compares disease characteristics, imaging modalities used, and patterns of treatment in two large cohorts of Takayasu arteritis (TAK) from Italy and India. Clinic files were retrospectively reviewed to retrieve information about initial choices of vascular imaging and immunosuppressive therapies. Unpaired t-tests compared means, and proportions were compared using Fisher’s exact test or Chi square test [Odds ratios (OR) with 95% confidence intervals (95%CI) calculated where appropriate]. The cohorts comprised 318 patients [Italy (n = 127), India (n = 191)] with similar delays to diagnosis. Ultrasound (OR Italy vs. India 9.25, 95%CI 5.02–17.07) was more frequently used in Italy and CT angiography in India (OR 0.32, 95%CI 0.20–0.51). Corticosteroid use was more prevalent and for longer duration in Italy. TAK from Italy had been more often treated with methotrexate, leflunomide or azathioprine, as opposed to tacrolimus in TAK from India (p < 0.05). Biologic or targeted synthetic disease-modifying agents were almost exclusively used in Italy. Survival on first immunosuppressive agent was longer from Italy than from India (log rank test p value 0.041). Considerable differences in the choice of initial vascular imaging modality and therapies for TAK from Italy and India could relate to prevalent socio-economic disparities. These should be considered while developing treatment recommendations for TAK.

ObjectiveTo evaluate the randomized controlled trials (RCTs) of Curcumin and Curcuma longa Extract in the treatment of autoimmune diseases.MethodsDatabases such as Embase, Web of Science, PubMed and The Cochrane Library were searched from the database establishment to February 2022 to collect RCTs of Curcumin and Curcuma longa Extract in the treatment of autoimmune diseases. Then the literature was screened and the data were extracted. Meta-analysis was performed using RevMan 5.3 software.ResultsA total of 34 records were included, involving 31 RCTs and 10 types of autoimmune disease. Among them, ankylosing spondylitis (AS) involves one RCT, Behcet ‘s disease (BD) involves one RCT, Crohn ‘s disease involves two RCTs, multiple sclerosis (MS) involves two RCTs, oral lichen planus involves six RCTs, psoriasis involves two RCTs, rheumatoid arthritis (RA) involves five RCTs, systemic lupus erythematosus (SLE) involves two RCTs, arteritis involves one RCT, ulcerative colitis (UC) involves nine RCTs. Among them, most of the RCTs of ulcerative colitis (UC), oral lichen planus, RA showed that curcumin and curcumin extracts improved clinical or laboratory results. Crohn ‘ s disease, MS, SLE, psoriasis included two RCTs; they all showed improvements (at least one RCT reported improvements in clinical outcomes). AS, BD and arteritis included only one RCT, and the clinical results showed improvement. However, due to the small number of RCTs and the small number of patients involved in each disease, there is still a need for more high-quality RCTs.ConclusionCurcumin and Curcuma longa Extract had good clinical efficacy in the treatment of Psoriasis, UC and RA, so Curcumin and Curcuma longa Extract could be used in the treatment of the above diseases in the future. The results of Meta-analysis showed that Curcumin and Curcuma longa Extract did not show efficacy in the treatment of oral lichen planus, while Takayasu arteritis, SLE, MS, AS, BD and CD did not report sufficient clinical data for meta-analysis. Therefore, large-sample, multi-center clinical trials are still needed for revision or validation.

Objective The activity and risk of relapse of Takayasu’s arteritis are low during pregnancy. Up to 40% of patients may have unfavorable obstetric outcomes, therefore it is important to know their clinical behavior. We describe the clinical characteristics and obstetric outcome of pregnant women with Takayasu arteritis treated in a hospital of high complexity. Materials and methods A retrospective evaluation of medical records of pregnant patients with Takayasu’s arteritis treated at Hospital Universitario San Vicente Fundacion in Medellin, Colombia between 2011 and 2018. Results Six patients with a median age at diagnosis 17.5 years (IQR 9.25), at delivery 24 years (IQR 8.25), disease duration 5.5 years (IQR 10.5). Three patients had extensive aortic involvement; at delivery, three patients were active and required immunosuppressants, five had high blood pressure, one developed preeclampsia in the second trimester, one had severe mitral and tricuspid insufficiency with a decreased ejection fraction of the left ventricle; two had aneurysms (left subclavian artery and ascending aorta). No pregnancy resulted in abortion or preterm birth; there were two fetal deaths, one due to intrauterine growth restriction and placental insufficiency and another of unknown etiology; both patients with disease activity, extensive aortic condition, and arterial hypertension. Five deliveries were by cesarean section by maternal indication; there was no aortic dissection, aneurysmal rupture or cerebral hemorrhage. Conclusion Patients with active disease and extensive aortic compromise presented unfavorable obstetric results, suggesting that inadequate control of vasculitis leads to greater maternal-fetal complications.

Kawasaki disease (KD) is an acute systemic vascular disease that affects mostly medium sized and small vessels. The most serious of which is coronary artery disease. Vasculitis involves proliferative granulomatous inflammation and resolves by cicatrization if not treated. It is generally a self-limited disease and its highest incidence is in children under five years. The diagnostic criteria include the presence of fever for at least 5 days along with four of five other clinical features (rash, mucositis, conjunctival injection, cervical lymphadenopathy or extremity changes). Atypical Kawasaki disease includes patients who meet only 2 or 3 of the 5 criteria for diagnosis. We here report a 4- year old male patient who presented with fever, mucositis, perineal rash and evidence of de novo femoral vasculitis leading to spontaneously leaking femoral arteritis. His complete blood count revealed thrombocytopenia. The patient fulfilled the criteria for the diagnosis of atypical Kawasaki Disease. He received intravenous immunoglobulins and aspirin in anti-inflammatory doses and his condition improved. We report this unusual presentation of Kawasaki disease with spontaneous femoral artery leak, rash and thrombocytopenia. Diagnosis of atypical Kawasaki disease is very challenging but essential as initiation of specific management is life-saving.

Összefoglaló. Bevezetés: Az antitest közvetítette kilökődés a graftvesztés gyakori oka a vesetranszplantáltak körében. Célkitűzés: Célul tűztük ki, hogy ismertetjük a centrumunkban biopsziával igazolt humorális kilökődéssel rendelkező betegeknek a kezelésre (standard kezelés: plazmaferézis, immunglobulin, rituximab) adott válaszát, valamint hogy vizsgáljuk a proteinuria grafttúlélésre kifejtett hatását és azt, hogy ezt a DSA-tól függetlenül teszi-e. Vizsgáltuk az eGFR-, a DSA-MFI-értéknek az antirejekciós terápia hatására bekövetkező változásait is. Módszer: 85 beteg retrospektív analízisét végeztük el. A szövettani elemzésben a Banff-klasszifikációt vettük alapul. A csoportok összehasonlításához kategorikus változók esetén a Fisher-féle egzakt próbát, folyamatos változók esetén a Kruskal–Wallis-próbát használtuk. Eredmények: A biopsziával igazolt humorális rejekciós csoportba (ABMR-csoport) 19, a DSA-pozitív csoportba 14, a DSA-negatív csoportba 52 beteget választottunk be. A DSA-érték az ABMR-csoportban 61,16%-kal csökkent, a DSA-pozitív csoportban 42,86%-kal redukálódott (Fisher-féle egzakt: p = 0,1). Az ABMR-csoportban 9 betegnek a jelentős, 4-nek a nephroticus mértékű proteinuriája csökkenthető volt (az ABMR-csoport 68%-a). A legjobb grafttúlélés a legalacsonyabb fehérjeürítésnél adódott. Az antirejekciós terápiát követően készült biopsziákban: a glomerulitis, az interstitialis gyulladás, az arteritis mértéke csökkent az antihumorális kezelés hatására, azonban krónikus elváltozások jelentek meg. Következtetés: Az ABMR-csoportban az antirejekciós terápiát követően a fehérjeürítés monitorizálása javasolt, hiszen becsülhető vele a grafttúlélés. Orv Hetil. 2021; 162(26): 1029–1037. Summary. Introduction: Antibody-mediated rejection is a common cause of graft loss among kidney transplant recipients. Objective: We aimed to describe the response of patients with biopsy-proven humoral rejection to treatment (standard treatment: plasmapheresis, immunoglobulin, rituximab) in our center. We also analyzed the effect of proteinuria on graft survival and whether this effect is independent of donor-specific antibodies (DSAs). Changes of eGFR and level of DSA following rejection treatment were examined. Method: In this study, laboratory data of 85 patients were analysed. Histological analysis was based on the Banff classification. Fisher’s exact test was used for statistical analysis, and Kruskal–Wallis test was used to compare patient groups per variable. Results: Data from 85 patients were processed retrospectively. 19 patients were selected for the biopsy-confirmed humoral rejection group (ABMR group), 14 for the DSA-positive group, and 52 for the DSA-negative group. DSA titer decreased by 61.16% in the ABMR group after treatment and by 42.86% in the DSA-positive group (Fisher’s exact test: p = 0.1). In the ABMR group, significant nephrotic proteinuria in 4 patients and severe proteinuria in 9 patients were reduced (68% of ABMR group). The patients with the lowest protein excretion had the best graft survival. In biopsies performed after antirejection therapy, the extent of glomerulitis, interstitial inflammation, arteritis decreased with antihumoral treatment, but chronic lesions appeared. Conclusion: Following treatment of biopsy-proven ABMR, reduction of proteinuria predicts graft survival and should be monitored as an important factor-predicting prognosis. Orv Hetil. 2021; 162(26): 1029–1037.

The study rationale was to assess the performance of qualitative and semi-quantitative scoring methods for 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) assessment in large-vessel vasculitis (LVV). Methods: Patients with giant cell arteritis (GCA) or Takayasu's arteritis (TAK) underwent clinical and imaging assessment, blinded to each other, within a prospective observational cohort. FDG-PET-CT scans were interpreted for active vasculitis by central reader assessment. Arterial FDG uptake was scored by qualitative visual assessment using the PET vascular activity score (PETVAS) and by semi-quantitative assessment using standardized uptake values (SUV) and target-to-background ratios (TBR) relative to liver/blood activity. Performance of each scoring method was assessed by intra-rater reliability using the intra-class coefficient (ICC) and area under receiver-operator characteristic curves (AUC), using physician assessment of clinical disease activity and reader interpretation of vascular PET activity as independent reference standards. Wilcoxon signed-rank test was used to analyze change in arterial FDG uptake over time. Results: Ninety-five patients (GCA=52; TAK=43) contributed 212 FDG-PET studies. The ICC for semi-quantitative evaluation [0.99 (range 0.98-1.00)] was greater than the ICC for qualitative evaluation [0.82 (range 0.56-0.93)]. PETVAS and TBR metrics were more strongly associated with reader interpretation of PET activity than SUV metrics. All assessment methods were significantly associated with physician assessment of clinical disease activity, but the semi-quantitative metric TBRLiver¬ achieved the highest AUC (0.66). Significant but weak correlations with C-reactive protein were observed for SUV metrics (r = 0.19, p

The presence of intimal arteritis (v) in renal allograft biopsy specimens establishes the presence of acute T-cell mediated rejection (TCMR), Grade IIa-III, according to the Banff classification of rejection. The clinical significance of isolated v1 lesions (v1), characterized by arteritis alone, compared with lesions of arteritis with tubulointerstitial inflammation (i-t-v) has been controversial.We performed a retrospective review of 280 patients undergoing renal transplantation between 2005 and 2015 who received a "for cause" transplant biopsy using the Banff 2013 classification. Patients with TCMR grade IIa (n = 83) were subdivided into groups with isolated v1 arteritis and i-t-v. Pre- and postoperative renal function, graft survival, and overall survival were evaluated in all patients.Donor and recipient demographics were similar between groups. One month following treatment of rejection, patients with v1 disease had superior recovery of glomerular filtration rate vs patients with i-t-v (P.002). At a median follow-up of 41 months from transplant, death-censored graft survival was 92% vs 79% (P = .04), and overall survival was 98% vs 79% (P.004) in the isolated v1 and i-t-v groups, respectively.Despite having identical Banff classification of TCMR IIa, our results indicate that graft survival in patients with isolated v1 rejection is superior to those with i-t-v. Following corroboration with data from other centers, modification of the Banff classification scheme should be considered.

We aimed to construct and validate a risk assessment model to identify risk factors for heart failure (HF) in patients with Takayasu’s arteritis (TAK). Three hundred sixty-five patients with TAK were recruited in the East China Takayasu Arteritis Cohort from January 2012 to December 2019. Patients were assigned into training and validation sets following a 2:1 ratio according to the date of enrollment. Clinical characteristics were compared between heart failure (HF) and non-HF subgroups in the training set, and a risk assessment model for HF and its scoring algorithm was established based on logistic regression, which was tested in the validation set. Among total of 74 (20.27%) TAK patients exhibited HF, and 55 cases (74.32%) were in the training set. The risk factors for HF of TAK patients included onset age >38 years, serum tumor necrosis factor (TNF)-α concentration >10 pg/ml, aortic valve involvement, coronary artery involvement, and pulmonary hypertension. We constructed the model without TNF-α (Model 1) and with TNF-α (Model 2). Patients in the training set with the score ≥ 3 appeared to be associated with an increased risk of HF with an area under curve (AUC) of 0.88 and 0.90 in Model 1 and Model 2 respectively. The AUC reached to 0.88 and 0.89 in the validation set that proved the accuracy of the model. We presented a risk assessment model of HF in TAK, which may help clinicians alert the complication of HF in the patients with specifically cardiac impairments.

This series describes three adolescent females who presented with chest pain and ventricular dysfunction related to acute coronary ischemia secondary to Takayasu’s arteritis with varied courses of disease progression leading to a diverse range of therapies including cardiac transplantation. While Takayasu’s arteritis is rare in childhood, it should be strongly considered in any adolescent female presenting with systemic inflammation and chest pain consistent with myocardial infarction. A high index of suspicion can lead to early detection and aggressive management of the underlying vasculitis reducing associated morbidity and mortality. The purpose of this report is to describe the challenges in the clinical diagnosis and management of Takayasu’s arteritis with myocardial infarction. We also seek to enhance awareness about unique presentations of Takayasu’s arteritis within the paediatric community.

A 45-year-old woman with no known medical history died suddenly shortly after complaining of anterior chest discomfort. The autopsy revealed a dissection at the anterior descending branch of the left coronary artery, and eosinophilic adventitial inflammation was observed both in the right coronary artery and in the vicinity of the dissection. Furthermore, there was degeneration of the tunica media in the right coronary artery, and this was thought to be a predissection lesion. In the degenerated area of the tunica media, probable apoptosis of smooth muscle cells was noted, suggesting that the degeneration was not due only to the effect of eosinophilic lytic enzymes. These findings also indicated that eosinophilic infiltration preceded the dissection. Eosinophilic infiltration around the coronary arteries is occasionally observed in cases of sudden death, but although it might be associated with the death, the pathological mechanism is yet to be elucidated. Eosinophilic periarteritis has also been observed around the site of spontaneous coronary artery dissection, although a causal relationship is unproven. The histopathology of this case indicated that the eosinophilic infiltration preceded the dissection. Detailed pathological findings are presented, together with a review of the literature.

Purpose Monitoring disease activity in patients with large vessel vasculitis (LVV) can be challenging. [18F]FDG-PET/CT is increasingly used to evaluate treatment response in LVV. In this systematic review and meta-analysis, we aimed to summarize the current evidence on the value of [18F]FDG-PET/CT for treatment monitoring in LVV. Methods PubMed/MEDLINE and the Cochrane library database were searched from inception through October 21, 2020. Studies containing patients with LVV (i.e. giant cell arteritis, Takayasu arteritis and isolated aortitis) that received treatment and underwent [18F]FDG-PET/CT were included. Screening, full-text review and data extraction were performed by 2 investigators. The risk of bias was examined with the QUADAS-2 tool. Meta-analysis of proportions and diagnostic test accuracy was performed by a random-effects model and bivariate model, respectively. Results Twenty-one studies were included in the systematic review, of which 8 studies were eligible for meta-analysis. Arterial [18F]FDG uptake decreased upon clinical remission in longitudinal studies. High heterogeneity (I2 statistic 94%) precluded meta-analysis of the proportion of patients in which the scan normalized during clinical remission. Meta-analysis of cross-sectional studies indicated that [18F]FDG-PET/CT may detect relapsing/refractory disease with a sensitivity of 77% (95%CI 57–90%) and specificity of 71% (95%CI 47–87%). Substantial heterogeneity was observed among the cross-sectional studies. Both variation in clinical aspects and imaging procedures contributed to the heterogeneity. Conclusion Treatment of LVV leads to reduction of arterial [18F]FDG uptake during clinical remission. [18F]FDG-PET/CT has moderate diagnostic accuracy for detecting active LVV. [18F]FDG-PET/CT may aid treatment monitoring in LVV, but its findings should be interpreted in the context of the clinical suspicion of disease activity. This study underlines the relevance of published procedural recommendations for the use of [18F]FDG-PET/CT in LVV.

BACKGROUND: Although a number of studies have reported on the vascular abnormalities detected by magnetic resonance imaging (MRI) in patients with late-phase acute pancreatitis (AP), few have studied those occurring in the early phase of the disease. The aim of this research was to investigate the MRI findings of early vascular abnormalities in AP and to analyze the correlation of the prevalence of vascular involvement with the severity of AP based on the MR severity index (MRSI) and Acute Physiology and Chronic Health Evaluation (APACHE) II scores. METHODS: A retrospective analysis was conducted of 301 consecutive AP patients who were admitted to our institution between March 2013 and June 2019. All patients underwent initial MRI during the early phase of pancreatitis and one or more repeat MRI scans in the late phase. Peripancreatic vascular conditions and pancreatitis were assessed using T1-/T2-weighted imaging and dynamic-enhanced MRI. The association between the prevalence of vascular involvement and AP severity graded according to the MRSI or APACHE II score was analyzed using Spearman’s rank correlation. RESULTS: Among 301 AP patients, 75 (24.9%) had at least one MRI-detected vascular abnormality. Overall, vascular involvement on MRI was higher in necrotizing pancreatitis than in edematous pancreatitis [43.2% (54/125) vs. 11.9% (21/176), χ(2)=38.2, P

Background Patients with Takayasu's arteritis (TA) experience important changes in lifestyle, quality of life, and functional status due to ischemic symptoms or treatment toxicity. Purpose To describe the clinical characteristics and the patient-reported outcomes (PROs), such as quality of life, disability, fatigue, and perception/impact of the disease in Mexican patients with TA. Methods Cross-sectional study including patients with established diagnosis of TA recruited at a tertiary care center. Demographics, comorbidities, clinical characteristics, laboratory, imaging, and treatment were retrieved. Disease activity (the Indian Takayasu Clinical Activity Score (ITAS) 2010), damage (Vasculitis Damage Index (VDI)), quality of life (Short Form 36 (SF-36)), disability (Health Assessment Questionnaire Disability Index (HAQ-DI)), fatigue (Multidimensional Fatigue Inventory-20), and patient's disease perceptions were assessed. Results Fifteen women were included, with a median age of 41 years (interquartile range (IQR) 30–45) and disease duration of 108 months (IQR 55–197). Median ITAS 2010 and VDI scores were 0 (IQR 0–2) and 3 points (IQR 2–6), respectively. Mean SF-36 score was 71.38 ± 13.39, with mean physical and mental component summaries of 66.52 ± 13.37 and 76.24 ± 14.89, respectively. HAQ-DI mean score was 0.48 ± 0.62, being grip the most affected domain. Among fatigue subscales, the higher scores were present in the physical fatigue (16.3 ± 5.8). Correlations between the HAQ-DI and the VDI score (r = 0.64, P = 0.03); between the general fatigue, score, and disease duration (r = −0.71, P = 0.01); and between the SF-36 total score and the HAQ-DI (r = −0.87, P = 0.0004) were found. Conclusions It is important to identify disease-specific outcomes of interest to the patients to develop tools that assess them with a holistic approach.