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Radiology Case Reports Aug 2024This case describes a rare and complex presentation of Takayasu arteritis, a large vessel vasculitis primarily affecting young females. Unlike typical Takayasu arteritis...
This case describes a rare and complex presentation of Takayasu arteritis, a large vessel vasculitis primarily affecting young females. Unlike typical Takayasu arteritis cases characterized by arterial stenosis, this 34-year-old male presented with an unusually high number of aneurysms affecting the aorta, subclavian arteries, and other segments. This unique abundance of aneurysms complicates diagnosis and management. This disease typically manifests as arterial stenosis, with aneurysms occurring in a minority of cases. The most common site for aneurysms is the ascending aorta, making multifocal aneurysms, as seen in this case, exceptionally rare. Managing multiple aneurysms in Takayasu arteritis is complex, necessitating careful consideration of factors like aneurysm size, morphology, and risk of complications. This case underscores the unique challenges posed by multifocal aneurysms in this condition, highlighting the need for a comprehensive approach to treatment.
PubMed: 38832341
DOI: 10.1016/j.radcr.2024.04.056 -
Cureus May 2024This case report describes a complicated case of giant cell arteritis (GCA) with tongue necrosis and bilateral central retinal artery occlusion (CRAO). An 81-year-old...
This case report describes a complicated case of giant cell arteritis (GCA) with tongue necrosis and bilateral central retinal artery occlusion (CRAO). An 81-year-old male patient with a history of recent retinal artery occlusion, ischemic stroke, and hypertensive emergency was evaluated. Clinical examination, including a visual acuity assessment, fundus evaluation, and oral examination, was performed. Laboratory investigations, such as erythrocyte sedimentation rate (ESR), were conducted. A temporal artery biopsy was performed to confirm the diagnosis of GCA. The patient presented with sudden vision loss in the left eye following a prior episode of retinal artery occlusion in the right eye. Ophthalmoscopic examination revealed CRAO in the left eye. Additionally, tongue necrosis, a rare manifestation of GCA, was observed. The ESR was significantly elevated. A temporal artery biopsy supported the diagnosis of GCA. The patient was promptly referred for immunologist consultation and initiated on intravenous methylprednisolone therapy. This case highlights the diverse and potentially devastating nature of GCA, involving ocular and systemic manifestations. Bilateral CRAO and tongue necrosis are rare but significant complications of GCA. Prompt diagnosis and early initiation of corticosteroid therapy are crucial to prevent irreversible visual loss and further complications. A multidisciplinary approach involving ophthalmologists and other specialists is essential for the comprehensive management of GCA.
PubMed: 38826883
DOI: 10.7759/cureus.59554 -
Reumatologia Clinica May 2024Infections in patients with systemic vasculitis represent one of the main causes of mortality. Corticosteroid use, immunosuppressive therapy, age, associated organic...
UNLABELLED
Infections in patients with systemic vasculitis represent one of the main causes of mortality. Corticosteroid use, immunosuppressive therapy, age, associated organic involvement and dialysis dependence are risk factors of infection.
OBJECTIVES
To determine the prevalence of severe infection and associated factors in patients diagnosed with ANCA-associated vasculitis (AAV) and Polyarteritis Nodosa (PAN).
METHODS
retrospective study was conduced in a single rheumatology center (2000-2018). We included patients diagnosed with AAV (Granulomatosis with Polyangiitis (GPA), Eosinophilic Granulomatosis with Polyangiitis (EGPA) and Microscopic Polyangiitis (PAM) and Polyarteritis nodosa (PAN). Serious infectious events requiring hospitalisation or prolonged antibiotic/antiviral treatment, recurrent infection of Herpes Zoster Virus or opportunistic infections were evaluated. Sites of infection, isolated microorganisms and mortality related were analyzed.
RESULTS
105 patients were analyzed, follow-up time median 18 m, 58.7% were women and median age was 52 years. Types of vasculitis: 41.9% PAM, 16.2% EPGA, 40% GPA, 1.9% PAN. Constitutional, pulmonary, renal and otorhinolaryngology manifestations were the most frequent.
PREVALENCE OF INFECTION
34.2%, with a median of 3 months from diagnosis of vasculitis to the infectious event. Low respiratory tract (42.8%), sepsis (31.4%), and urinary tract (14.3%) were the most common sites of infections. Bacterial aetiology was the most prevalent (67.7%). Mortality at the first event was 14.3% and a 72.2% of patients were in the induction phase of treatment. Infectious events were significantly associated with age > 65 years (p = 0.030), presence of lung (p = 0.016) and renal involvement (p = 0.001), BVASv3 > 15, mortality (p = 0.0002).
CONCLUSIONS
The prevalence of infection was 34.2%. Lower airway infections, septicemia and urinary tract infections were the most prevalent. Infections were associated with renal and pulmonary involvement, age older than 65 years and score BVAS > 15. Severe infections were associated with mortality, especially in elderly patients.
Topics: Humans; Female; Male; Middle Aged; Retrospective Studies; Adult; Aged; Prevalence; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Polyarteritis Nodosa; Risk Factors; Infections; Opportunistic Infections
PubMed: 38821740
DOI: 10.1016/j.reumae.2024.05.004 -
Turkish Journal of Medical Sciences 2024The aim of this study is to evaluate the baseline F18-FDG PET/CT findings of individuals diagnosed with giant cell arteritis (GCA) and to explore its association with...
BACKGROUND/AIM
The aim of this study is to evaluate the baseline F18-FDG PET/CT findings of individuals diagnosed with giant cell arteritis (GCA) and to explore its association with clinical findings and classification criteria.
MATERIALS AND METHODS
We analysed data from patients who underwent F18-FDG PET/CT scans to investigate large vessel (LV) involvement between 2010 and 2019. Only patients with a clinical diagnosis of GCA and at least 6 months of follow-up were included. We compared initial clinical features and laboratory findings based on the presence of LV vasculitis on PET/CT and the maximum standard uptake value (SUVmax) of vascular territories.
RESULTS
Twenty-nine patients (median age at diagnosis: 70, F/M: 24/5) were included in the study. Among them, 21 patients (72.4%) presented with cranial symptoms, while 8 patients (27.5%) had isolated LV-GCA. Twenty-two patients (75.9%) met the ACR/EULAR 2022 GCA classification criteria. LV vasculitis was detected on PET/CT in 23 patients (79.3%). A positive correlation was observed between SUVmax in the thoracic aorta and both CRP and ESR levels (r = 0.50, p = 0.026 and r = 0.63, p = 0.002, respectively). PET/CT positive patients were found to be younger (p = 0.016) and more frequently female (p = 0.017). They also exhibited fewer headaches (56.5% vs. 100%, p = 0.04), experienced fewer flares during follow-up (p = 0.03), and had a lower cumulative glucocorticoid dose at the 6th month (p = 0.036). Comparison of PET/CT-positive patients (n = 23) based on the fulfilment of the ACR/EULAR 2022 classification criteria revealed that patients who met these criteria were older (p = 0.02) and had significantly lower CRP levels at diagnosis (p = 0.02).
CONCLUSION
The performance of F18-FDG PET/CT in diagnosing LV involvement in GCA is favourable, and the severity of FDG uptake in the vessel wall correlates with the acute phase response. Patients with extracranial involvement on PET/CT exhibit distinct features, including a younger age and female predominance. Additionally, these patients appear to experience fewer relapses and require lower doses of glucocorticoids. However, the clinical significance of PET/CT in patients who met ACR/EULAR classification criteria, predominantly consisting of patients with ischemic cranial symptoms, could not be determined in our study.
Topics: Humans; Giant Cell Arteritis; Female; Fluorodeoxyglucose F18; Male; Aged; Positron Emission Tomography Computed Tomography; Middle Aged; Radiopharmaceuticals; Aged, 80 and over; Retrospective Studies
PubMed: 38812633
DOI: 10.55730/1300-0144.5767 -
Journal of Autoimmunity May 2024In polymyalgia rheumatica (PMR), glucocorticoids (GCs) relieve pain and stiffness, but fatigue may persist. We aimed to explore the effect of disease, GCs and PMR...
OBJECTIVE
In polymyalgia rheumatica (PMR), glucocorticoids (GCs) relieve pain and stiffness, but fatigue may persist. We aimed to explore the effect of disease, GCs and PMR symptoms in the metabolite signatures of peripheral blood from patients with PMR or the related disease, giant cell arteritis (GCA).
METHODS
Nuclear magnetic resonance spectroscopy was performed on serum from 40 patients with untreated PMR, 84 with new-onset confirmed GCA, and 53 with suspected GCA who later were clinically confirmed non-GCA, and 39 age-matched controls. Further samples from PMR patients were taken one and six months into glucocorticoid therapy to explore relationship of metabolites to persistent fatigue. 100 metabolites were identified using Chenomx and statistical analysis performed in SIMCA-P to examine the relationship between metabolic profiles and, disease, GC treatment or symptoms.
RESULTS
The metabolite signature of patients with PMR and GCA differed from that of age-matched non-inflammatory controls (R > 0.7). There was a smaller separation between patients with clinically confirmed GCA and those with suspected GCA who later were clinically confirmed non-GCA (R = 0.135). In PMR, metabolite signatures were further altered with glucocorticoid treatment (R = 0.42) but did not return to that seen in controls. Metabolites correlated with CRP, pain, stiffness, and fatigue (R ≥ 0.39). CRP, pain, and stiffness declined with treatment and were associated with 3-hydroxybutyrate and acetoacetate, but fatigue did not. Metabolites differentiated patients with high and low fatigue both before and after treatment (R > 0.9). Low serum glutamine was predictive of high fatigue at both time points (0.79-fold change).
CONCLUSION
PMR and GCA alter the metabolite signature. In PMR, this is further altered by glucocorticoid therapy. Treatment-induced metabolite changes were linked to measures of inflammation (CRP, pain and stiffness), but not to fatigue. Furthermore, metabolite signatures distinguished patients with high or low fatigue.
PubMed: 38797046
DOI: 10.1016/j.jaut.2024.103260 -
Frontiers in Immunology 2024It has been well documented that Takayasu arteritis (TAK) and ulcerative colitis (UC) coexist in the same patients. characterizes the co-occurrence, which is one of the...
BACKGROUND
It has been well documented that Takayasu arteritis (TAK) and ulcerative colitis (UC) coexist in the same patients. characterizes the co-occurrence, which is one of the common genetic features between these two diseases, indicating shared underlying pathologic mechanisms. Anti-integrin αvβ6 antibody (Ab) is present in sera of UC patients in a highly specific manner. We investigated if there were any associations between anti-integrin αvβ6 Ab and TAK, considering the risk HLA alleles.
METHODS
A total of 227 Japanese TAK patients were recruited in the current study and their serum samples were subjected to measurement of anti-integrin αvβ6 Ab by ELISA. The clinical information, including the co-occurrence of UC, was collected. The HLA allele carrier status was determined by Luminex or genotype imputation.
RESULTS
The information about the presence of UC was available for 165 patients, among which eight (4.84%) patients had UC. Anti-integrin αvβ6 antibody was identified in 7 out of 8 TAK subjects with UC (87.5%) while only 5 out of 157 (3.18%) TAK subjects without UC had the antibody (OR 121, p=7.46×10). A total of 99 out of 218 (45.4%) patients were carriers. There was no significant association between the presence of anti-integrin αvβ6 Ab and carrier status in those without UC (OR 2.01, 95% CI 0.33-12.4, p = 0.189).
CONCLUSIONS
The prevalence of anti-integrin αvβ6 Ab was high in TAK patients with UC, but not in the absence of concomitant UC. The effect of on anti-integrin αvβ6 Ab production would be minimal.
Topics: Humans; Colitis, Ulcerative; Takayasu Arteritis; Female; Integrins; Male; Adult; Middle Aged; Antigens, Neoplasm; HLA-B52 Antigen; Alleles; Young Adult; Japan; Genotype; Autoantibodies
PubMed: 38784377
DOI: 10.3389/fimmu.2024.1387516 -
Cureus Apr 2024The primary treatment of choice for polymyalgia rheumatica (PMR) is corticosteroids, which are better avoided for elderly patients susceptible to PMR. The cases of five...
The primary treatment of choice for polymyalgia rheumatica (PMR) is corticosteroids, which are better avoided for elderly patients susceptible to PMR. The cases of five patients cured with only a small dosage of 600 mg/day ibuprofen without steroids or methotrexate are reported. Their clinical features were compared with those of the 26 PMR patients who had steroids and/or methotrexate in addition to ibuprofen. PMR was diagnosed based on the 2015 EULAR/ACR criteria. They were all females aged 73-80. They all had no giant cell arteritis or autoantibodies. Nonsteroidal anti-inflammatory drugs (NSAIDs) other than ibuprofen had not worked in four cases; for the one, ibuprofen was the first NSAID. Their serum CRP levels were 1.57-12.8 mg/dL at ibuprofen introduction. Colchicine was co-administered in two patients. At the next visit three to seven days after ibuprofen introduction, they all showed a clear recovery with a CRP level decrease. Ibuprofen tapering was started within three months, and no relapse was until two to five years' follow-up. Comparison with the 26 patients who had additional steroid and/or methotrexate showed that the disease duration until ibuprofen introduction was statistically significantly shorter in the five patients (1.40±0.65 vs 3.28±2.98 months). Ibuprofen would be the first-line drug for PMR, and its earliest use would be beneficial.
PubMed: 38784367
DOI: 10.7759/cureus.58778 -
Autoimmunity Reviews May 2024Large-vessel vasculitides (LVV) comprise a group of chronic inflammatory diseases of the aorta and its major branches. The most common forms of LVV are giant cell... (Review)
Review
Large-vessel vasculitides (LVV) comprise a group of chronic inflammatory diseases of the aorta and its major branches. The most common forms of LVV are giant cell arteritis (GCA) and Takayasu arteritis (TAK). Both GCA and TAK are characterized by granulomatous inflammation of the vessel wall accompanied by a maladaptive immune and vascular response that promotes vascular damage and remodeling. The inflammatory process in LVV starts in the adventitia where fibroblasts constitute the dominant cell population. Fibroblasts are traditionally recognized for synthesizing and renewing the extracellular matrix thereby being major players in maintenance of normal tissue architecture and in tissue repair. More recently, fibroblasts have emerged as a highly plastic cell population exerting various functions, including the regulation of local immune processes and organization of immune cells at the site of inflammation through production of cytokines, chemokines and growth factors as well as cell-cell interaction. In this review, we summarize and discuss the current knowledge on fibroblasts in LVV. Furthermore, we identify key questions that need to be addressed to fully understand the role of fibroblasts in the pathogenesis of LVV.
PubMed: 38782083
DOI: 10.1016/j.autrev.2024.103574 -
Emerging Infectious Diseases Jun 2024Scedosporium spp. and Lomentospora prolificans are emerging non-Aspergillus filamentous fungi. The Scedosporiosis/lomentosporiosis Observational Study we previously... (Observational Study)
Observational Study Review
Scedosporium spp. and Lomentospora prolificans are emerging non-Aspergillus filamentous fungi. The Scedosporiosis/lomentosporiosis Observational Study we previously conducted reported frequent fungal vascular involvement, including aortitis and peripheral arteritis. For this article, we reviewed 7 cases of Scedosporium spp. and L. prolificans arteritis from the Scedosporiosis/lomentosporiosis Observational Study and 13 cases from published literature. Underlying immunosuppression was reported in 70% (14/20) of case-patients, mainly those who had solid organ transplants (10/14). Osteoarticular localization of infection was observed in 50% (10/20) of cases; infections were frequently (7/10) contiguous with vascular infection sites. Scedosporium spp./Lomentospora prolificans infections were diagnosed in 9 of 20 patients ≈3 months after completing treatment for nonvascular scedosporiosis/lomentosporiosis. Aneurysms were found in 8/11 aortitis and 6/10 peripheral arteritis cases. Invasive fungal disease--related deaths were high (12/18 [67%]). The vascular tropism of Scedosporium spp. and L. prolificans indicates vascular imaging, such as computed tomography angiography, is needed to manage infections, especially for osteoarticular locations.
Topics: Humans; Scedosporium; France; Male; Middle Aged; Aged; Female; Mycoses; Adult; Antifungal Agents; Aged, 80 and over; Invasive Fungal Infections
PubMed: 38781681
DOI: 10.3201/eid3006.231409 -
Revista Da Associacao Medica Brasileira... 2024In this study, we aimed to investigate the role of erythrocyte sedimentation rate, C-reactive protein, neutrophil/lymphocyte ratio, platelet/lymphocyte ratio,...
OBJECTIVE
In this study, we aimed to investigate the role of erythrocyte sedimentation rate, C-reactive protein, neutrophil/lymphocyte ratio, platelet/lymphocyte ratio, monocyte/lymphocyte ratio, red blood cell distribution width, mean platelet volume, monocyte/HDL ratio, and C-reactive protein/albumin ratio in the diagnosis and treatment follow-up of active and remission Takayasu arteritis patients compared with healthy control group.
METHODS
This is a retrospective case-control study in which 56 Takayasu arteritis patients and 40 age- and sex-matched healthy control were included. The blood values of Takayasu arteritis patients were analyzed during their active period and post-treatment remission periods, after comparing them with the healthy control. Furthermore, all parameters were evaluated by receiver operating characteristic analysis.
RESULTS
Erythrocyte sedimentation rate, C-reactive protein, neutrophil/lymphocyte ratio, platelet/lymphocyte ratio, monocyte/lymphocyte ratio, monocyte/HDL ratio, and C-reactive protein/albumin ratio values were significantly higher in active Takayasu arteritis patients compared with healthy control and remission Takayasu arteritis groups. In the receiver operating characteristic analysis performed in active Takayasu arteritis and Takayasu arteritis patients in remission, C-reactive protein had the highest power to indicate disease activity, followed by C-reactive protein/albumin ratio, erythrocyte sedimentation rate, and monocyte/HDL ratio. When Takayasu arteritis in remission was compared with the healthy control, a significant difference was found between erythrocyte sedimentation rate, C-reactive protein, red blood cell distribution width, and C-reactive protein/albumin ratio, while no significant difference was found between monocyte/HDL ratio values.
CONCLUSION
C-reactive protein/albumin ratio and red blood cell distribution width can be used in the diagnosis of Takayasu arteritis, and C-reactive protein/albumin ratio, red blood cell distribution width, and monocyte/HDL ratio measurements can be used in the follow-up. As C-reactive protein/albumin ratio is more powerful than C-reactive protein in differentiating the Takayasu arteritis group from the healthy control group, evaluation of C-reactive protein/albumin ratio together with albumin instead of evaluation of C-reactive protein alone when diagnosing the disease may help us to obtain more accurate results in daily practice.
Topics: Humans; Takayasu Arteritis; Female; C-Reactive Protein; Retrospective Studies; Adult; Male; Case-Control Studies; Blood Sedimentation; Monocytes; Young Adult; ROC Curve; Follow-Up Studies; Biomarkers; Middle Aged; Serum Albumin; Adolescent
PubMed: 38775535
DOI: 10.1590/1806-9282.20231683