Giant cell arteritis and therapeutic response: a dual facet of immunotherapy in metastatic clear cell renal carcinoma.

Immune checkpoint inhibitors have emerged as a promising cancer treatment, allowing significant and long-term therapeutic responses. Nivolumab, an anti-programmed cell death protein-1, is one of the molecules of this therapeutic class with known and manageable side effects. Giant cell arteritis is a rare immune-related adverse event most often manifested by headaches poorly released by common antalgics and can result in visual loss. We report its occurrence in an 80-year-old patient on maintenance nivolumab for metastatic clear cell renal carcinoma. Prompt diagnosis and initiation of glucocorticoid therapy led to symptom improvement and visual recovery.

Faster steroid-free remission with tocilizumab compared to methotrexate in giant cell arteritis: a real-life experience in two reference centres.

Glucocorticoids (GCs) are still the mainstay of treatment of giant cell arteritis (GCA). Although GCs are highly effective in GCA, the high burden of toxicity of GCs as well as the disease relapse during GC tapering is well documented. To compare the efficacy and rapidity of TCZ and MTX as steroid-sparing agents in a real-life cohort of GCA patients. A retrospective analysis was conducted including patients with newly diagnosed GCA from the Rheumatology Units of Udine and Rome. The inclusion criterion was the treatment with TCZ or MTX as first steroid-sparing drug. 112 GCA patients (81 females) with a median age of 70 (IQ 65-75) years were collected. Thirty-one out of 112 (27.7%) patients were treated with TCZ (162 mg/week), while 81/112 (72.3%) patients received MTX (up to 20 mg/week) as a GC-sparing agent. At month 6 after GCA onset, 5/31 (16.1%) patients in TCZ group and none in MTX group were in GC-free sustained remission (p value = 0.001). Similarly, at month 12, 64.5% (20/31) and 11.1% (9/81) of patients were in sustained GC-free remission in TCZ and MTX group, respectively (p value <0.001). At month 24 of follow-up, at least one relapse of the disease occurred in 7/31 (22.6%) in TCZ-treated and 28/81 (34.6%) in MTX-treated patients, respectively (p value = 0.22). TCZ allowed a faster discontinuation of steroid therapy than MTX in GCA patients, without increasing the risk of relapse.

New-onset of giant cell arteritis with ischemic optic neuropathy following the seventh-dose of COVID-19 mRNA vaccination: A case report and literature review.

Coronavirus disease (COVID-19) vaccines have demonstrated excellent efficacy in reducing the morbidity and severity of the disease. However, some patients have been reported to develop systemic rheumatic diseases, such as rheumatoid arthritis, myocarditis, Guillain-Barre syndrome, and giant cell arteritis (GCA) following COVID-19 vaccination. We present a case of GCA with ischemic optic neuropathy following COVID-19 mRNA vaccination. A 73-year-old woman developed headache, myalgia, scalp tenderness, and jaw claudication 4 days after her seventh dose of the vaccination; she also developed severe visual disturbances 1 month after the vaccination. The blood examination tests showed an increased serum C-reactive protein level and erythrocyte sedimentation rate. The echogram for the temporal artery showed a halo sign. Ophthalmic examination revealed ischemic optic neuropathy in both eyes. The patient was treated with a high-dose glucocorticoid and tocilizumab under the diagnosis of GCA with ischemic optic neuropathy, obtaining mild improvement of the symptoms. This report underscores the need for clinical vigilance and further data collection regarding GCA cases after COVID-19 vaccination.

Takayasu arteritis manifesting as acute kidney injury and congestion due to renal artery stenosis and myocarditis: a case report.

Background: Takayasu arteritis is a large-vessel vasculitis that affects the aorta and its primary branches. Myocarditis is a rare life-threatening complication and potential diagnostic pitfall in patients with Takayasu arteritis. Case summary: A previously healthy 18-year-old woman presenting with fever, back pain, and dyspnoea was admitted to another hospital for acute hypertension (blood pressure, 230/106 mmHg) and congestive heart failure. Intravenous methylprednisolone pulse with antihypertensive and diuretic medications slightly improved her congestion. However, she developed acute kidney injury and was transferred to our hospital. Transthoracic echocardiography indicated a left ventricular ejection fraction of 45% and diffuse left ventricular hypokinesis. Doppler ultrasound test and magnetic resonance angiography revealed severe bilateral renal artery stenosis. Her diagnosis was Takayasu arteritis, and she received high-dose glucocorticoids. She required temporary haemodialysis, but 2 months after admission, her serum creatinine improved to 1.1 mg/dL without surgical or cardiovascular interventions. Although the pre-discharge test with 1.5 T cardiac magnetic resonance initially failed to diagnose myocarditis, 3 T cardiac magnetic resonance imaging revealed increased native T1 values on T1 mapping (1283-1393 ms), moderate pericardial effusion, and systolic left ventricular wall motion abnormality, indicating active myocarditis. During 6-month subcutaneous tocilizumab treatment (162 mg/week), a left ventricular ejection fraction improved to 55-60% without a relapse. Discussion: This case report highlights the benefits of early multimodal imaging tests including cardiac magnetic resonance imaging for myocarditis and renal artery involvement in Takayasu arteritis. Tocilizumab might be an efficient therapeutic option for severe acute manifestations including myocarditis in young women of reproductive age.

Optic nerve sheath measurement to monitor disease activity in giant cell arteritis: a pilot study.

INTRODUCTION/OBJECTIVES: Optic nerve sheath (ONS) enhancement using magnetic resonance imaging of the orbits was observed in patients with giant cell arteritis (GCA). We previously showed that ONS diameter (ONSD) by bedside ultrasound is increased in patient with active GCA. This study aims to assess whether ONSD decreases with clinical remission in patients with GCA. METHODS: A prospective cohort study was conducted from June 2022 to January 2023. Patients who had an optic nerve ultrasound at GCA diagnosis as part of a previous crosssectional study were eligible. Optic nerve ultrasound was performed by the same investigator at diagnosis and month 3. ONSD (includes the optic nerve and its sheath) and optic nerve diameter (OND) were measured. Descriptive statistics for baseline characteristics and paired sample t-test were performed to assess the mean difference in OND and ONSD between diagnosis and month 3. RESULTS: Nine patients with GCA were included. The median age at disease onset was 79 years (interquartile range (IQR) of 79-82 years), and 7 patients were males. All patients were in clinical remission at month 3 on prednisone (median dose of 15 mg/day, IQR of 10-25 mg). The mean ONSD was lower at month 3 (3.76 mm) compared to baseline (5.98 mm), with a paired mean difference of 2.22 mm (95% CI 1.41-3.03 mm, p < 0.001). As anticipated, OND measurements did not vary between diagnosis and month 3. CONCLUSION: ONSD on ultrasound improves after 3 months of therapy in patients with GCA. A longer prospective study is required to determine if ONSD is useful to assess disease activity in GCA. Key Points • ONS ultrasound can identify patients with active GCA. • The ONSD on ultrasound is dynamic and improved after 3 months of GCA therapy. • ONS ultrasound may be useful to monitor disease activity in GCA.

Impact of conventional and biological disease-modifying anti-rheumatic drugs on arterial lesions in Takayasu arteritis.

BACKGROUND: The definition of Takayasu arteritis (TAK) remission and disease activity is still unclear. Vascular imaging is an essential tool for following-up patients. Herein, we aimed to compare the evolution of vascular lesions (ie vessel wall thickening and stenosis) under conventional disease-modifying anti-rheumatic drugs (cDMARDs) relatively to biological DMARDs (bDMARDs) in TAK patients followed with the same CT angiography modalities. METHOD: We compared 75 lines of therapy in TAK patients who received cDMARDs (n = 40 lines) and bDMARDs (n = 35 lines) using CT angiography. We established 1-3 main target vessels with vessel wall thickening and/or stenosis. Every targeted vessel had its thickness and its lumen diameter measured at the initiation of immunosuppressive treatment and at 12 months. RESULTS: We observed an overall reduction of arterial wall thickness in 73% of cases and 31% had >25% of wall thickness relative decrease. Using a linear mixed effects model, first line immunosuppressive therapy (p= 0.012) and bDMARDs relatively to cDMARDs (p= 0.026) were independently associated with vessel wall thickness reduction in TAK. Thirty-eight percent of the stenotic vessels had a > 25% relative increase in lumen diameter under immunosuppressive therapy. The relative increase >25% in lumen diameter was noted in 56% vs 17% with bDMARDs compared with cDMARDs. CONCLUSION: Immunosuppressive treatments can reduce arterial wall thickness and widen lumen diameter in TAK. bDMARDs seems to be more effective than cDMARDs to improve arterial lesions in TAK.

Antiplatelet therapy to prevent ischemic events in giant cell arteritis: protocol for a systematic review and meta-analysis.

BACKGROUND: Giant cell arteritis (GCA) is the most common systemic vasculitis in adults. Presenting features include new-onset headaches, constitutional symptoms, jaw claudication, polymyalgia rheumatica, and visual symptoms. Arterial inflammation with subsequent stenosis and occlusion may cause tissue ischemia, leading to blindness, strokes, and myocardial infarction. Oral antiplatelet therapy has been hypothesized to reduce GCA-related ischemic events. However, previous studies have demonstrated conflicting results regarding the efficacy of antiplatelet agents in GCA. The objective of this systematic review is to assess the safety and efficacy of antiplatelet therapy for the prevention of these events in adults with giant cell arteritis. METHODS: In this systematic review, we will include randomized controlled trials (RTCs), quasi-randomized trials, non-randomized intervention studies, cohort studies, and case-control studies on patients with new-onset or relapsing GCA. The intervention of interest will be pre-existing use or initiation of an oral antiplatelet medication (aspirin, clopidogrel, prasugrel, or ticagrelor) at GCA onset or relapse. The comparator of interest will be the absence of antiplatelet therapy. Endpoints will be evaluated after 6 and 12 months of follow-up. The primary outcome will be GCA-related ischemic events, including permanent blindness, stroke, myocardial infarction, and ischemic event-related deaths. Adverse events such as major bleeding and death caused by a bleeding event will be assessed. DISCUSSION: GCA-related ischemic events are catastrophic, sudden, often irreversible, and lead to significant morbidity. Antiplatelet agents are affordable, accessible, and could be effective for the prevention of these events. Nevertheless, the potential benefits of platelet aggregation inhibition must be weighed against their associated risk of bleeding. Assessing the efficacy and safety of antiplatelet therapy in GCA is therefore clinically important. SYSTEMATIC REVIEW REGISTRATION: Our systematic review protocol was registered with the International Prospective Register of Systematic Reviews (PROSPERO, registration number CRD42023441574.

An iconodiagnosis for Joos Vijd, as painted by the van Eycks in the Ghent Altarpiece.

The Ghent Altarpiece, a jewel of Gothic art painted by the van Eyck brothers in the fifteenth century, is particularly noteworthy for its use of an innovative dilution of oil, giving it a realistic scope that is particularly conducive to iconodiagnostic hypotheses. For the first time in the literature, we are taking a medical look at this masterpiece, and more specifically at the representation of its patron, whose identity is well known: Joos Vijd, a powerful notable from the town of Ghent, in modern-day Belgium. A vascular turgidity of the temporal artery, which can be suggestive of temporal arteritis, Hertoghe's sign and a slight ear crease were observed. These signs might be vascular lesions accentuated by Vijd's age and attest to van Eyck's virtuosity and anatomic accuracy.

Pro-fibrotic effect of the susceptible gene PCSK5 in vascular fibrosis of Takayasu arteritis via TGF-ß and SMAD3 signaling pathway activation.

BACKGROUND: Vascular fibrosis directly causes vascular thickening in Takayasu arteritis (TAK), in which sustained transforming growth factor beta (TGF-ß) activation is critical. Understanding TGF-ß activation regulation and blocking it might yield a therapeutic effect in TAK. Proprotein convertase subtilisin/kexin type 5 (PCSK5) rs6560480 (T/C) is associated with TAK development. In this study, we assessed the association between the PCSK5 rs6560480 genotype and PCSK5 expression in TAK and explored its molecular role in TGF-ß activation and vascular fibrosis development. METHODS: In TAK patients, PCSK5 and TGF-ß expression in plasma and aortic tissue was examined by ELISA and immunohistochemical staining, and PCSK5 rs6560480 was genotyped. The correlation between PCSK5 and extracellular matrix (ECM) expression was examined by Western blotting (WB) and immunohistochemistry staining. Detection by co-immunoprecipitation was performed to detect the interaction between PCSK5 and TGF-ß in adventitial fibroblasts (AAFs). Downstream signaling pathways were detected by WB and validated with appropriate inhibitors. Potential immunosuppressive agents to inhibit the effects of PCSK5 were explored in cell culture and TAK patients. RESULTS: Patients with PCSK5 rs6560480 TT patients had significantly higher PCSK5 levels and more thickened vascular lesions than patients with PCSK5 rs6560480 CT. PCSK5 expression was significantly increased in alpha smooth muscle actin (α-SMA)-positive myofibroblasts in TAK vascular lesions. Overexpressing PCSK5 facilitated TGF-ß and downstream SMAD2/3 activation and ECM expression in AAFs and aorta in in-vitro culture. The mechanistic study supported that PCSK5 activated precursor TGF-ß (pro-TGF-ß) to the mature form by binding the pro-TGF-ß cleavage site. Leflunomide inhibited PCSK5 and pro-TGF-ß binding, decreasing TGF-ß activation and ECM expression, which was also partially validated in leflunomide-treated patients. CONCLUSION: The findings revealed a novel pro-fibrotic mechanism of PCSK5 in TAK vascular fibrosis via TGF-ß and downstream SMAD2/3 pathway activation. Leflunomide might be anti-fibrotic by disrupting PCSK5 and pro-TGF-ß binding, presenting a new TAK treatment approach.

Polymyalgia rheumatica is a risk factor for more recalcitrant disease in giant cell arteritis: A retrospective cohort study.

OBJECTIVES: To evaluate differences in presentation and outcome of giant cell arteritis (GCA) patients with and without polymyalgia rheumatica (PMR) symptoms. METHODS: Consecutive patients diagnosed with GCA between 2000 and 2020 and followed for ≥12 months at the University Hospitals Leuven (Belgium), were included retrospectively. RESULTS: We included 398 GCA patients, of which 181 (45%) with PMR symptoms. Patients with PMR symptoms had a longer symptom duration (11 vs 6 weeks, p < 0.001). They less frequently reported fever (19% vs 28%, p = 0.030) and fatigue (52% vs 64%, p = 0.015) and tended to have less permanent vision loss (12% vs 19%, p = 0.052). There was no difference in the cumulative oral GC dose at 2 years (4.4 vs 4.3 g methylprednisolone, p = 0.571). However, those with PMR symptoms were treated with higher GC doses during subsequent follow-up (p < 0.05 from 38 months after diagnosis) and had a lower probability of stopping GC (62% vs 71%, HR 0.74 [95%CI 0.58-0.94], p = 0.018) with a longer median duration of GC treatment (29 vs 23 months, p = 0.021). In addition, presence of PMR symptoms was associated with an increased risk of relapse (64% vs 51%, HR 1.38 [95%CI 1.06-1.79], p = 0.017) with a higher number of relapses (1.47 [95%CI 1.30-1.65] vs 1.16 relapses [95%CI 1.02-1.31], p = 0.007). Patients with PMR symptoms less frequently developed thoracic aortic aneurysms during follow-up (3% vs 11%, p = 0.005). CONCLUSION: GCA patients with PMR symptoms had more recalcitrant disease with a higher risk of relapse and longer duration of GC treatment with need for higher GC doses.

Clinical characteristics and courses of Korean patients with giant cell arteritis: a multi-center retrospective study.

Objective: Giant cell arteritis (GCA) is a large-vessel vasculitis that primarily affects elderly individuals. However, data regarding Korean patients with GCA are scarce owing to its extremely low prevalence in East Asia. This study aimed to investigate the clinical characteristics of Korean patients with GCA and their outcomes, focusing on relapse. Methods: The medical records of 27 patients with GCA treated at three tertiary hospitals between 2007 and 2022 were retrospectively reviewed. Results: Seventeen (63.0%) patients were females, and the median age at diagnosis was 75 years. Large vessel involvement (LVI) was detected in 12 (44.4%) patients, and polymyalgia rheumatica (PMR) was present in 14 (51.9%) patients. Twelve (44.4%) patients had fever at onset. The presence of LVI or concurrent PMR at diagnosis was associated with a longer time to normalization of the C-reactive protein level (p=0.039) or erythrocyte sedimentation rate (p=0.034). During follow-up (median 33.8 months), four (14.8%) patients experienced relapse. Kaplan-Meier analyses showed that relapse was associated with visual loss (p=0.008) and the absence of fever (p=0.004) at onset, but not with LVI or concurrent PMR. Conclusion: Concurrent PMR and LVI were observed in approximately half of Korean patients with GCA, and the elapsed time to normalization of inflammatory markers in these patients was longer. The relapse rate in Korean GCA is lower than that in Western countries, and afebrile patients or patients with vision loss at onset have a higher risk of relapse, suggesting that physicians should carefully monitor patients with these characteristics.

Single-cell transcriptomic analysis to identify endomembrane regulation of metalloproteins and motor proteins in autoimmunity.

TMEM230 promotes antigen processing, trafficking, and presentation by regulating the endomembrane system of membrane bound organelles (lysosomes, proteosomes and mitochondria) and phagosomes. Activation of the immune system requires trafficking of various cargos between the endomembrane system and cell plasma membrane. The Golgi apparatus is the hub of the endomembrane system and essential for the generation, maintenance, recycling, and trafficking of the components of the endomembrane system itself and immune system. Intracellular trafficking and secretion of immune system components depend on mitochondrial metalloproteins for ATP synthesis that powers motor protein transport of endomembrane cargo. Glycan modifying enzyme genes and motor proteins are essential for the activation of the immune system and trafficking of antigens between the endomembrane system and the plasma membrane. Recently, TMEM230 was identified as co-regulated with RNASET2 in lysosomes and with metalloproteins in various cell types and organelles, including mitochondria in autoimmune diseases. Aberrant metalloproteinase secretion by motor proteins is a major contributor to tissue remodeling of synovial membrane and joint tissue destruction in rheumatoid arthritis (RA) by promoting infiltration of blood vessels, bone erosion, and loss of cartilage by phagocytes. In this study, we identified that specific glycan processing enzymes are upregulated in certain cell types (fibroblast or endothelial cells) that function in destructive tissue remodeling in rheumatoid arthritis compared to osteoarthritis (OA). TMEM230 was identified as a regulator in the secretion of metaloproteinases and heparanase necessary tissue remodeling in OA and RA. In dendritic (DC), natural killer and T cells, TMEM230 was expressed at low or no levels in RA compared to OA. TMEM230 expression in DC likely is necessary for regulatory or helper T cells to maintain tolerance to self-antigens and prevent susceptibility to autoimmune disease. To identify how TMEM230 and the endomembrane system contribute to autoimmunity we investigated, glycan modifying enzymes, metalloproteinases and motor protein genes co-regulated with or regulated by TMEM230 in synovial tissue by analyzing published single cell transcriptomic datasets from RA patient derived synovial tissue.

A young woman presenting with dyspnoea and diffuse T-wave inversions: a case report.

Background: T-wave inversions on electrocardiograms (ECGs) indicate a variety of conditions, such as coronary artery disease, myocarditis, and cardiomyopathy. Pulmonary artery stenosis (PAS) and pulmonary hypertension (PH) may cause right ventricular enlargement and ischaemia, which are reflected as T-wave inversions on ECGs. Continuous ECG monitoring is crucial for detecting dynamic changes indicative of PAS progression and reversal in right heart remodelling. Case summary: This report presents the case of a young woman who experienced exertional dyspnoea for 5 years with ECG findings showing T-wave inversions across multiple leads. The patient was diagnosed with PAS and PH caused by Takayasu arteritis (TA). Following three successful balloon pulmonary angioplasty sessions, the patient exhibited significant clinical improvement, including the remission of PAS and PH. Throughout a 59-month cumulative follow-up period, the sustained effectiveness of the treatment was evidenced by the regression of right heart remodelling, as manifested in the normalization of the initially inverted T-waves on the ECG. Discussion: Electrocardiogram changes, including right axis deviation, right bundle branch block, a deep S wave in lead I (R/S < 1), and a prominent R wave in lead aVR (R/Q > 1), have been termed PAS syndrome, often linked to TA-associated PAS, especially in young East Asian females. Early diagnosis is crucial but challenging due to atypical symptoms. The non-invasive ECG is vital for detection, with balloon pulmonary angioplasty serving as an effective treatment for TA-induced PAS when surgery is not an option, improving outcomes and potentially reversing right heart remodelling.

Clinical and molecular spectrum of v-lesion.

Isolated v-lesion presents diagnostic stratification and clinical challenges. We characterized allograft outcomes for this entity based on post-transplant time (early: ≤1 month vs. late: >1 month) and compared its molecular phenotype with other v+ rejection forms. Using the NanoString® B-HOT panel, we analyzed 92 archival FFPE kidney biopsies from three centers: isolated v-lesion (n=23), ABMR v+ (n=26), TCMR v+ (n=10), mixed rejection v+ (n=23), and normal tissue (n=10). Six gene sets (ABMR, DSAST, ENDAT, TCMR, early/acute injury, late injury) were assessed. Early isolated v-lesions had the poorest one-year death-censored graft survival compared to late isolated v-lesions or other rejections (p=0.034). Gene set analysis showed lower TCMR-related gene expression in isolated v+ groups than TCMR and mixed rejection (p<0.001). Both early and late isolated v-lesions had lower ABMR-related gene expression than ABMR, mixed rejection, and TCMR (p≤0.022). Late isolated v-lesions showed reduced DSAST and ENDAT gene expression versus ABMR (p≤0.046); and decreased early/acute injury gene expression than early isolated v+, ABMR, TCMR, and mixed rejection (p≤0.026). In conclusion, isolated v-lesions exhibit distinct gene expression patterns versus other rejection v+ forms. Early isolated v+ is associated with poorer prognosis and increased early/acute injury gene expression than late isolated v+, suggesting distinct etiologies.

Positron Emission Tomography/Computed Tomography in Polymyalgia Rheumatica: When and for What-A Critical Review.

Polymyalgia rheumatica (PMR) is an inflammatory disease common in people aged 50 years and older. This condition is characterized by the presence of pain and stiffness involving mainly the shoulder and pelvic girdle. Besides the frequent association with giant cell arteritis (GCA), several conditions may mimic PMR or present with PMR features. Since the diagnosis is basically clinical, an adequate diagnosis of this condition is usually required. Positron emission tomography/computed tomography (PET-CT) has proved to be a useful tool for the diagnosis of PMR. The use of 18F-FDG-PET imaging appears promising as it provides detailed information on inflammatory activity that may not be evident with traditional methods. However, since PET-CT is not strictly necessary for the diagnosis of PMR, clinicians should consider several situations in which this imaging technique can be used in patients with suspected PMR.

Relapses in giant cell arteritis: Updated review for clinical practice.

Giant cell arteritis (GCA), the most common primary vasculitis in adults, is a granulomatous systemic vasculitis usually affecting the aorta and its major branches, particularly the carotid and vertebral arteries. Although remission can be achieved in most patients with GCA using high-dose glucocorticoids (GC), relapses are frequent, occurring in >40% of GC-only treated patients, mostly during the first two years after diagnosis. Relapsing courses lead to high GC exposure, increasing the risk of treatment-related adverse effects. Although tocilizumab is an efficacious GC-sparing therapy that allows increased sustained remission and reduced cumulative GC doses, relapses are common after drug discontinuation. This narrative review examines the most relevant features of relapses in GCA, including its definition, classification, frequency, clinical, laboratory, and imaging characteristics, chronology, probable pathophysiology, and predictive factors. In addition, we discuss treatment options for relapsing patients and the effect of relapses on patient outcomes.

Genetics of vasculitis.

Systemic vasculitis encompasses a wide range of conditions characterized by varying degrees of inflammation in blood vessels. Although the etiology of vasculitis remains unclear, accumulated data suggest that it is triggered in genetically predisposed individuals by the concurrence of certain environmental factors. The importance of the genetic component has been consistently supported by evidence of familial aggregation, differential prevalence by ethnicity, and multiple genetic associations with disease susceptibility and severity reported in recent years. The strongest association signals in most vasculitides correspond to genetic variants within the HLA region, suggesting an important role of the immune system in its pathophysiology. However, each type of vasculitis has distinct defining HLA association markers, likely due to disease-specific differences in antigenic drivers. Furthermore, other genetic polymorphisms located outside the HLA region play an important role in susceptibility to different vasculitides. More recent research has assessed the shared genetic susceptibility evident across different vasculitides. Future studies should focus on the identification of genetic markers that can serve as reliable biomarkers for early diagnosis, prognosis, and treatment response in systemic vasculitis.

[Inflammatory causes of stroke-Diagnostics and treatment]. / Entzündliche Ursachen von Schlaganfällen ­ Diagnostik und Therapie.

Inflammatory causes of stroke are frequent and often pose diagnostic and therapeutic challenges due to the scarcity of randomized trials and the absence of clear guideline recommendations for many scenarios. Following the publication of the recommendations of the European Stroke Organization on primary angiitis of the central nervous system (PACNS) last year, the German Neurological Society (DGN) has issued very clear guidelines this year on the diagnostics and treatment of PACNS and updated the recommendations for systemic vasculitides; however, stroke often occurs not only as a result of primary vascular inflammation but also as a complication of another organ infection. Approximately 5% of all patients with sepsis, ca. 20% of patients with bacterial meningitis and up to 40% of patients with bacterial endocarditis suffer from a stroke as a complication. This article summarizes the key characteristics of these inflammatory causes of stroke and particularly focuses on the current recommendations for diagnostic and therapeutic management.

High-frequency ultrasound with superb microvascular imaging: a potential tool for ultrasound assessment in patients with giant cell arteritis.

Objective: The objective of this study was 2-fold: first, to evaluate whether superb microvascular imaging (SMI) could be used to visualize neovascularization in temporal arteries, and, second, to evaluate the diagnostic performance of high frequency ultrasound with SMI using an extended protocol in patients with suspected giant cell arteritis (GCA). Methods: This retrospective study comprised 120 patients consecutively examined with an extended CDU protocol (temporal, facial, axillary, subclavian, brachiocephalic, and carotid arteries) between 2020 and 2022. Of all patients, 107 had no previous GCA diagnosis and 13 had a previous GCA diagnosis. SMI was used to evaluate neovascularization in the temporal arteries. Arteritis were characterized as low- or medium-echogenic, homogeneous wall thickening, with or without a positive compression sign in the temporal arteries. The Halo count, i.e., the number of temporal and axillary artery segments with signs of arteritis, was evaluated. The reference was clinically diagnosed GCA confirmed after ≥6-month follow-up. Results: Of the eligible 107 patients with new suspected GCA, 33 (31%) received a clinical GCA diagnosis. Neovascularization was detected in 14 patients (43%). Patients with neovascularization displayed a higher halo count [median 6 (25th-75th percentile 4.75-7) vs. 3 (2-4-4), p = 0.005]. CDU of only the temporal arteries showed sensitivity and specificity (95% confidence intervals) of 94% (80-100%) and 100% (95-100%), respectively. The addition of extra-cranial arteries increased the sensitivity to 100%. Of the 13 patients investigated for suspected relapse, three had a clinically confirmed relapse. One of them displayed neovascularization together with other signs of inflammation. Conclusions: We show for the first time that inflammatory neovascularization of the temporal arteries can be detected by SMI. Neovascularization is associated with a more-widespread cranial disease. The value of neovascularization should be further investigated, especially for the detection of GCA relapse.