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The Kaohsiung Journal of Medical... Jul 2024
Topics: Humans; Female; Takayasu Arteritis; Adult; Heart Failure; Thrombosis; Heart Ventricles; Empyema; Acute Disease
PubMed: 38686703
DOI: 10.1002/kjm2.12839 -
Cureus Mar 2024This case report describes a rare case of intravascular large B-cell lymphoma (IVLBCL), initially presenting with nonspecific symptoms of fever and fatigue, and...
This case report describes a rare case of intravascular large B-cell lymphoma (IVLBCL), initially presenting with nonspecific symptoms of fever and fatigue, and tentatively diagnosed as disseminated carcinomatosis of the bone marrow originating from urothelial cancer in an 80-year-old woman. The patient's journey began with symptoms treated as common ailments and progressed through multiple differential diagnoses, including giant cell arteritis, TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly) syndrome, and disseminated carcinomatosis of the bone marrow originating from urothelial cancer due to the presence of systemic inflammation, anasarca, and elevated soluble interleukin 2 receptor levels, indicative of an intense immunological response. Despite initial treatments, her condition deteriorated, leading to further investigations that ultimately revealed the presence of malignant cells in the urine and bone marrow, confirming the diagnosis of IVLBCL. This case underscores the diagnostic challenges faced when elderly patients present with systemic inflammation and the critical need for thorough investigation beyond initial impressions. It highlights the importance of considering differentiation between disseminated carcinomatosis of the bone marrow and IVLBCL in the differential diagnosis of persistent inflammation, especially in cases where common causes have been excluded and the primary malignancy is not immediately apparent.
PubMed: 38686250
DOI: 10.7759/cureus.57221 -
Annals of the Rheumatic Diseases Apr 2024Giant cell arteritis (GCA), the most common systemic vasculitis, is characterised by aberrant interactions between infiltrating and resident cells of the vessel wall.... (Review)
Review
Giant cell arteritis (GCA), the most common systemic vasculitis, is characterised by aberrant interactions between infiltrating and resident cells of the vessel wall. Ageing and breach of tolerance are prerequisites for GCA development, resulting in dendritic and T-cell dysfunction. Inflammatory cytokines polarise T-cells, activate resident macrophages and synergistically enhance vascular inflammation, providing a loop of autoreactivity. These events originate in the adventitia, commonly regarded as the biological epicentre of the vessel wall, with additional recruitment of cells that infiltrate and migrate towards the intima. Thus, GCA-vessels exhibit infiltrates across the vascular layers, with various cytokines and growth factors amplifying the pathogenic process. These events activate ineffective repair mechanisms, where dysfunctional vascular smooth muscle cells and fibroblasts phenotypically shift along their lineage and colonise the intima. While high-dose glucocorticoids broadly suppress these inflammatory events, they cause well known deleterious effects. Despite the emerging targeted therapeutics, disease relapse remains common, affecting >50% of patients. This may reflect a discrepancy between systemic and local mediators of inflammation. Indeed, temporal arteries and aortas of GCA-patients can show immune-mediated abnormalities, despite the treatment induced clinical remission. The mechanisms of persistence of vascular disease in GCA remain elusive. Studies in other chronic inflammatory diseases point to the fibroblasts (and their lineage cells including myofibroblasts) as possible orchestrators or even effectors of disease chronicity through interactions with immune cells. Here, we critically review the contribution of immune and stromal cells to GCA pathogenesis and analyse the molecular mechanisms by which these would underpin the persistence of vascular disease.
PubMed: 38684323
DOI: 10.1136/ard-2023-225270 -
Clinical and Experimental Rheumatology Apr 2024Giant cell arteritis (GCA) is one of the most common forms of vasculitis. There is an abundance of studies which are conducted in a randomised controlled trial setting...
OBJECTIVES
Giant cell arteritis (GCA) is one of the most common forms of vasculitis. There is an abundance of studies which are conducted in a randomised controlled trial setting but limited with respect to cohort size and follow-up time. GeVas is the first large-scale registry for vasculitides in German-speaking countries that enables to evaluate this rare disease. Herein we focus on the subgroup of GCA patients including follow-up data up to one year.
METHODS
GeVas is a prospective, web-based, multicentre registry for the documentation of organ manifestations, outcomes, and therapy regimens in vasculitides. Recruitment started in June 2019. By April 2023, 15 centres were initiated and have started to enrol patients.
RESULTS
After 4 years, 195 GCA-patients were included in the registry, of which 64% were female and 36% were male. The average age was 76 years at the time of recruitment (IQR=69-82). Seventy-nine percent were included in the registry because of a newly diagnosed GCA and 21% because of a relapse. At the first assessment most of the patients (89%) described general symptoms. Thirty-one percent stated ocular symptoms. Cranial symptoms were documented in 78% of the cases. All patients were documented with immunosuppressive treatment at start, of whom 95% received prednisolone, 16% cyclophosphamide, 20% methotrexate, and 48% tocilizumab. After three months 62% and after one year 91% of the patients achieved remission.
CONCLUSIONS
Regarding demographics, clinical manifestations and diagnostics, our study showed a similar composition compared to other studies. However, our data differed in terms of treatment regimens.
Topics: Humans; Registries; Giant Cell Arteritis; Male; Female; Aged; Aged, 80 and over; Prospective Studies; Immunosuppressive Agents; Germany; Treatment Outcome; Time Factors; Recurrence
PubMed: 38683207
DOI: 10.55563/clinexprheumatol/d3o0gu -
The Malaysian Journal of Pathology Apr 2024Identification of acute funisitis, a sign of foetal inflammatory response (FIR), is crucial as their presence is associated with ominous neonatal outcomes....
INTRODUCTION
Identification of acute funisitis, a sign of foetal inflammatory response (FIR), is crucial as their presence is associated with ominous neonatal outcomes. Recommendation on which part of umbilical cord should be sampled to facilitate optimal identification of acute funisitis is limited.
METHODS
This is a retrospective cross-sectional study over a seven-month duration recruiting all patients with clinical suspicion of chorioamnionitis and/or maternal intrapartum pyrexia. The distribution and the degree of cord inflammation were assessed. The cases were also evaluated for maternal inflammatory response (MIR) and chorionic vasculitis (CV).
RESULTS
Of the 191 placentas, 88 (46.1%) had some degree of cord inflammation. Forty-nine (55.7%) had a differential in cord inflammation, with distal cord section (n = 38) demonstrating significant greater inflammation than that of proximal cord section (n = 11) (p<0.001). There were 20 cases with phlebitis only and 8 cases demonstrated arteritis only in either proximal or distal cord sections. Increasing magnitude of cord inflammation was significantly associated with increasing severity of MIR and the rate of CV (p<0.001). CV was observed in 25 (24.3%) cases showing absence of cord inflammation, while 12 (13.6%) cases with cord FIR demonstrated no CV.
DISCUSSION
Inflammatory reaction can occur variably throughout the length of the umbilical cord and chorionic plate vessels, with greater inflammation seen in the distal cord section. We affirm the current Amsterdam recommendation of submitting at least two cross sections of the cord representing proximal and distal sites and two sections from placental parenchyma to facilitate the identification of FIR.
Topics: Humans; Chorioamnionitis; Female; Pregnancy; Retrospective Studies; Cross-Sectional Studies; Umbilical Cord; Adult; Inflammation; Placenta
PubMed: 38682843
DOI: No ID Found -
Diagnostics (Basel, Switzerland) Apr 2024Systemic vasculitides are a rare and complex group of diseases that can affect multiple organ systems. Clinically, presentation may be vague and non-specific and as... (Review)
Review
Systemic vasculitides are a rare and complex group of diseases that can affect multiple organ systems. Clinically, presentation may be vague and non-specific and as such, diagnosis and subsequent management are challenging. These entities are typically classified by the size of vessel involved, including large-vessel vasculitis (giant cell arteritis, Takayasu's arteritis, and clinically isolated aortitis), medium-vessel vasculitis (including polyarteritis nodosa and Kawasaki disease), and small-vessel vasculitis (granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis). There are also other systemic vasculitides that do not fit in to these categories, such as Behcet's disease, Cogan syndrome, and IgG4-related disease. Advances in medical imaging modalities have revolutionized the approach to diagnosis of these diseases. Specifically, color Doppler ultrasound, computed tomography and angiography, magnetic resonance imaging, positron emission tomography, or invasive catheterization as indicated have become fundamental in the work up of any patient with suspected systemic or localized vasculitis. This review presents the key diagnostic imaging modalities and their clinical utility in the evaluation of systemic vasculitis.
PubMed: 38667483
DOI: 10.3390/diagnostics14080838 -
Journal of Nuclear Cardiology :... Apr 2024Different types of vasculitis can be distinguished according to the blood vessel's size that is preferentially affected: large-vessel, medium-vessel, and small-vessel... (Review)
Review
Different types of vasculitis can be distinguished according to the blood vessel's size that is preferentially affected: large-vessel, medium-vessel, and small-vessel vasculitides. Giant cell arteritis (GCA) and Takayasu's arteritis (TAK) are the main forms of large-vessel vasculitis, and may lead to lumen narrowing. Clinical manifestations of arterial narrowing on the short- and long term include vision loss, stroke, limb ischemia, and heart failure. Imaging tools are well established diagnostic tests for large-vessel vasculitis and may aid therapy monitoring in selected cases while providing important information regarding the occurrence of vascular damage, tissue and organ complications. This review aims to provide the current status of multimodality imaging for the diagnosis and identification of vascular complications in the field of large vessel vasculitis.
PubMed: 38663459
DOI: 10.1016/j.nuclcard.2024.101864 -
Cureus Mar 2024Giant cell arteritis (GCA) is characterized by headaches, but few studies have examined the detailed characteristics of pathologically confirmed cases. We investigated...
INTRODUCTION
Giant cell arteritis (GCA) is characterized by headaches, but few studies have examined the detailed characteristics of pathologically confirmed cases. We investigated the characteristics of GCA patients, particularly headache, and their correlation with pathological findings.
METHODS
We retrospectively analyzed 26 patients (median age: 77.5 years, male: 38.4%) with GCA who underwent superficial temporal artery (STA) biopsy at the Japanese Red Cross Shizuoka Hospital between May 2001 and February 2022. All patients fulfilled the American College of Rheumatology and European League Against Rheumatism classification criteria for GCA. We focused on the relationship between clinical features, especially headache, and pathological findings.
RESULTS
Twenty-four patients had unilateral, nonpulsatile, intermittent headaches. Transmural inflammation (TMI), a characteristic pathology of GCA, was present in 14 patients. Bivariate analysis revealed significant associations between the TMI and STA-related tenderness (odds ratio [OR]=11, 95% confidence interval [CI]=1.14 to 106.43, p=0.046) and the TMI and STA-related chordal thickening (OR=0.19, 95% CI=0.068 to 0.52, p=0.021).
CONCLUSIONS
Headache in GCA patients was often unilateral, nonpulsatile, and intermittent. This study highlights the significant association of TMI with STA tenderness and ligamentous thickening, which has not been reported previously. Abnormal STA findings were significantly associated with pathological changes in GCA patients, emphasizing the importance of these lesions in predicting GCA.
PubMed: 38659551
DOI: 10.7759/cureus.56843 -
Frontiers in Medicine 2024To derive segmental cut-off values and measures of diagnostic accuracy for the intima-media thickness of compressed temporal artery segments for the diagnosis of giant...
Ultrasound intima-media thickness cut-off values for the diagnosis of giant cell arteritis using a dual clinical and MRI reference standard and cardiovascular risk stratification.
OBJECTIVES
To derive segmental cut-off values and measures of diagnostic accuracy for the intima-media thickness of compressed temporal artery segments for the diagnosis of giant cell arteritis (GCA) on the patient level. To examine the influence of cardiovascular risk.
METHODS
Retrospectively, patients evaluated for GCA with an ultrasound of the temporal arteries and an MRI of the head, including a T1-fatsat-black blood (T1-BB) sequence, were identified and classified based on cardiovascular risk and a dual reference standard of T1-BB on the segmental level and the clinical diagnosis on the patient level. Intima-media thickness of the common superficial temporal artery (CSTA), frontal and parietal branches (FB, PB) were measured by compression technique. Statistically and clinically optimal (specificity of approx. 90% for the patient level) cut-offs were derived. Diagnostic accuracy was evaluated on the patient level.
RESULTS
The population consisted of 144 patients, 74 (51.4%) with and 70 (48.6%) without GCA. The cut-offs were 0.86 mm, 0.68 mm and 0.67 mm for the CSTA, the FB and PB, respectively. On the patient level sensitivity and specificity were 86.5 and 81.4%. cut-offs were 1.01 mm, 0.82 mm and 0.69 mm and showed a sensitivity of 79.7% and a specificity of 90.0%. For patients high cardiovascular risk, statistically optimal cut-offs showed a sensitivity of 89.6% and a specificity of 90.5%.
CONCLUSION
Newly derived ultrasound intima-media thickness cut-offs with a dual reference standard show high diagnostic accuracy on the patient level for the diagnosis of GCA, particularly in patients without high cardiovascular risk.
PubMed: 38654833
DOI: 10.3389/fmed.2024.1389655 -
Journal of Autoimmunity Jun 2024The IL-12-IFNγ-Th1 and the IL-6-IL-23-Th17 axes are considered the dominant pathogenic pathways in Giant Cell Arteritis (GCA). Both pathways signal via activation of...
INTRODUCTION
The IL-12-IFNγ-Th1 and the IL-6-IL-23-Th17 axes are considered the dominant pathogenic pathways in Giant Cell Arteritis (GCA). Both pathways signal via activation of the downstream JAK/STAT proteins. We hypothesized that phosphorylated STAT (pSTAT) signatures in circulating immune cells may aid to stratify GCA-patients for personalized treatment.
METHODS
To investigate pSTAT expression, PBMCs from treatment-naive GCA-patients (n = 18), infection controls (INF, n = 11) and age-matched healthy controls (HC, n = 15) were stimulated in vitro with IL-6, IL-2, IL-10, IFN-γ, M-CSF or GM-CSF, and stained with CD3, CD4, CD19, CD45RO, pSTAT1, pSTAT3, pSTAT5 antibodies, and analyzed by flow cytometry. Serum IL-6, sIL-6-receptor and gp130 were measured by Luminex. The change in percentages of pSTAT3+CD4+T-cells was evaluated at diagnosis and at 3 months and 1-year of follow-up. Kaplan-Meier analyses was used to asses prognostic accuracy.
RESULTS
Analysis of IL-6 stimulated immune cell subsets revealed a significant decrease in percentages of pSTAT3+CD4+T-cells of GCA-patients and INF-controls compared to HCs. Following patient stratification according to high (median>1.5 pg/mL) and low (median<1.5 pg/mL) IL-6 levels, we observed a reduction in the pSTAT3 response in GCA-patients with high serum IL-6. Percentages of pSTAT3+CD4+T-cells in patients with high serum IL-6 levels at diagnosis normalized after glucocorticoid (GC) treatment. Importantly, we found that patients with low percentages of pSTAT3+CD4+T-cells at baseline require longer GC-treatment.
CONCLUSION
Overall, in GCA, the percentages of in vitro IL-6-induced pSTAT3+CD4+T-cells likely reflect prior in vivo exposure to high IL-6 and may serve as a prognostic marker for GC-treatment duration and may assist improving personalized treatment options in the future.
Topics: Humans; Giant Cell Arteritis; Interleukin-6; Female; CD4-Positive T-Lymphocytes; Male; Signal Transduction; Aged; Janus Kinases; Middle Aged; Phosphorylation; STAT3 Transcription Factor; Aged, 80 and over; STAT Transcription Factors; Receptors, Interleukin-6; Biomarkers; Cytokine Receptor gp130
PubMed: 38653164
DOI: 10.1016/j.jaut.2024.103215