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Eye (London, England) Aug 2023Giant Cell Arteritis (GCA) is well known to be a critical ischaemic disease that requires immediate medical recognition to initiate treatment and where one in five... (Review)
Review
Giant Cell Arteritis (GCA) is well known to be a critical ischaemic disease that requires immediate medical recognition to initiate treatment and where one in five people still suffer visual loss. The immunopathophysiology has continued to be characterised, and the influencing of ageing in the development of GCA is beginning to be understood. Recent national and international guidelines have supported the directed use of cranial ultrasound to reduce diagnostic delay and improve clinical outcomes. Immediate high dose glucocorticoids remain the standard emergency treatment for GCA, with a number of targeted agents that have been shown in clinical trials to have superior clinical efficacy and steroid sparing effects. The aim of this review was to present the latest advances in GCA that have the potential to influence routine clinical practice.
Topics: Humans; Giant Cell Arteritis; Delayed Diagnosis; Glucocorticoids; Aging; Treatment Outcome
PubMed: 36788362
DOI: 10.1038/s41433-023-02433-y -
Indian Journal of Ophthalmology Oct 2023Giant cell arteritis (GCA) is a granulomatous inflammation involving medium and large vessels that can lead to serious clinical manifestations associated with tissue... (Review)
Review
Giant cell arteritis (GCA) is a granulomatous inflammation involving medium and large vessels that can lead to serious clinical manifestations associated with tissue ischemia. Temporal artery biopsy (TAB) is currently the gold standard method for the diagnosis of GCA, with a specificity of 100% and a sensitivity of 77%. However, the false-negative rate for TAB ranges from 9% to 61%. False negatives may be related to the timing of biopsy, the length of specimen, and the existence of "skip lesions." We reviewed the relevant evidence for methods to improve the sensitivity and reduce the false-negative rate for TAB. To reduce the false-negative rate for TAB, it is recommended to perform TAB within 1 week of starting corticosteroid therapy. Although there is currently no consensus, we suggest that the temporal artery is cut to a length of 20‒30 mm and to prepare serial pathological sections. It is necessary to attach great importance to patients suspected of having GCA, and complete TAB should be performed as soon as possible while starting corticosteroid therapy promptly. We also discuss the clinical value of non-invasive vascular imaging technologies, such as DUS, CTA, MRA, and 18F-FDG-PET/CT, as auxiliary methods for GCA diagnosis that could partially replace TAB.
Topics: Humans; Giant Cell Arteritis; Temporal Arteries; Positron Emission Tomography Computed Tomography; Sensitivity and Specificity; Biopsy; Adrenal Cortex Hormones; Retrospective Studies
PubMed: 37787225
DOI: 10.4103/IJO.IJO_3163_22 -
Medicine Nov 2023The clinical manifestations of Fabry disease affect the nerves, kidneys, heart, skin, gastrointestinal tract and eyes. Our aim is to familiarize people with the FD... (Review)
Review
RATIONALE
The clinical manifestations of Fabry disease affect the nerves, kidneys, heart, skin, gastrointestinal tract and eyes. Our aim is to familiarize people with the FD diagnostic process by reporting this case.
PATIENT CONCERNS
A 79-year-old-male patient presented with muscle pain and weakness in the extremities, also with an increasing erythrocyte sedimentation rate and C-reactive protein. Further examinations revealed that multiple organ involvement, such as rash, myocardial hypertrophy, peripheral neuropathy.
DIAGNOSES
Cardiac MR demonstrated hypertrophic cardiomyopathy, myocardial fibrosis and low myocardial T1 value. The patient was eventually diagnosed with Fabry disease through proteomics and genetic testing.
INTERVENTIONS
The treatment is enzyme replacement therapy (ERT). But this patient could not afford ERT and was given only general symptomatic treatment, pregabalin, and a gradual reduction in glucocorticoid.
OUTCOMES
The patient's symptoms of joint pain and muscle weakness reduced significantly, and ESR and CRP had decreased to normal.
LESSONS
FD is a rare disease and difficult to diagnose, but rare does not mean invisible. FD may present with signs and symptoms of rheumatic diseases. Rheumatologists should be aware and concerned about this disease.
Topics: Aged; Humans; Male; Enzyme Replacement Therapy; Fabry Disease; Giant Cell Arteritis; Heart; Kidney; Polymyalgia Rheumatica; Skin
PubMed: 37933054
DOI: 10.1097/MD.0000000000034630 -
Journal of Medical Case Reports Jul 2023Takayasu arteritis is a rare and chronic granulomatous vasculitis that affects the large vessels. Takayasu arteritis targets the aorta and its branches and is still of...
BACKGROUND
Takayasu arteritis is a rare and chronic granulomatous vasculitis that affects the large vessels. Takayasu arteritis targets the aorta and its branches and is still of unknown etiology. It often affects female patients under 50 years of age. A relationship between Takayasu arteritis and tuberculosis has been suggested for a long time.
CASE PRESENTATION
We report a severe case of Takayasu arteritis in a 10-year-old Tunisian child revealed by renovascular hypertension with concomitant pulmonary tuberculosis.
CONCLUSIONS
Our patient is among only a few cases of Takayasu arteritis published worldwide affecting young infants and adolescents, which underlines the strong relationship between Takayasu arteritis and tuberculosis.
Topics: Child; Adolescent; Humans; Female; Takayasu Arteritis; Aorta; Tuberculosis
PubMed: 37455309
DOI: 10.1186/s13256-023-04037-2 -
IDCases 2024There is indirect evidence signifying a potential link between tuberculosis and Takayasu's arteritis (TAK); however, the exact mechanism and relationship between TKA and...
There is indirect evidence signifying a potential link between tuberculosis and Takayasu's arteritis (TAK); however, the exact mechanism and relationship between TKA and Mycobacterium tuberculosis (TB) remain to be elucidated. This case intends to highlight the association between TB and TKA, as early detection can avoid devastating consequences.
PubMed: 38618157
DOI: 10.1016/j.idcr.2024.e01938 -
Journal of Autoimmunity Sep 2023The landscape of polyarteritis nodosa (PAN) has substantially changed during the last decades. Recent data regarding causes, characteristics, and prognosis of systemic...
BACKGROUND
The landscape of polyarteritis nodosa (PAN) has substantially changed during the last decades. Recent data regarding causes, characteristics, and prognosis of systemic PAN in the modern era are lacking.
METHODS
This retrospective study included patients with systemic PAN referred to the French Vasculitis Study Group between 2005 and 2019. Characteristics, associated conditions and outcomes were collected, and predictors of relapse and death were analyzed.
RESULTS
196 patients were included. Main clinical symptoms were constitutional (84%), neurological (59%), skin (58%) and musculoskeletal (58%) manifestations. Secondary PAN accounted for 55 (28%) patients, including myelodysplastic syndrome (9%), solid cancer (7%), lymphoma (4%) and autoinflammatory diseases (4%). No patient had active HBV infection. All treated patients (98.5%) received glucocorticoids (GCs), alone (41%) or in combination with immunosuppressants (59%), with remission achieved in 90%. Relapses were independently associated with age >65 years (HR 1.85; 95% CI1.12-3.08), gastrointestinal involvement (1.95; 95% CI1.09-3.52) and skin necrotic lesions (HR 1.95; 95%CI 1.24-3.05). One-, 5- and 10-year overall survival rates were 93%, 87% and 81%, respectively. In multivariate analyses, age >65 years (HR 2.80; 95%CI 1.23-6.37), necrotic purpura (HR 4.16; 95%CI 1.62-10.70), acute kidney injury (HR 4.89; 95% 1.71-13.99) and secondary PAN (HR 2.98; 95%CI 1.29-6.85) were independently associated with mortality.
CONCLUSION
Landscape of PAN has changed during the last decades, with the disappearance of HBV-PAN and the emergence of secondary PAN. Relapse rate remains high, especially in aged patients with gastrointestinal and cutaneous necrosis, as well as mortality.
Topics: Humans; Male; Female; Adult; Middle Aged; Aged; Retrospective Studies; Polyarteritis Nodosa; Recurrence; Prognosis
PubMed: 37536165
DOI: 10.1016/j.jaut.2023.103093 -
Arthritis & Rheumatology (Hoboken, N.J.) Aug 2023Tumor necrosis factor (TNF) alpha and interleukin-17 (IL-17) are thought to be involved in the pathogenesis of Takayasu arteritis (TAK), and TNF inhibitors (TNFi) are... (Comparative Study)
Comparative Study
OBJECTIVE
Tumor necrosis factor (TNF) alpha and interleukin-17 (IL-17) are thought to be involved in the pathogenesis of Takayasu arteritis (TAK), and TNF inhibitors (TNFi) are recommended for the treatment of TAK. The present study was undertaken to investigate the efficacy of secukinumab, an IL-17A monoclonal antibody, compared to treatment with TNFi.
METHODS
This was a prospective, single-center, open-label cohort study. Patients with active TAK who did not respond to treatment with glucocorticoids combined with 2 immunosuppressive agents were treated with either secukinumab or TNFi as an add-on therapy without an increased dosage of glucocorticoids. A complete response was defined as complete resolution of signs and symptoms of active disease, normal values of inflammatory markers, no progression on imaging of involved arteries, and dose of glucocorticoid <15 mg/day. A partial response was similarly defined as a complete response except with an erythrocyte sedimentation rate <40 mm/hour and C-reactive protein level of <20 mg/liter.
RESULTS
Nineteen patients in the secukinumab group and 34 patients in the TNFi group were enrolled. The demographic data and inflammatory markers of the 2 groups were comparable at baseline. Complete response and partial response for patients treated with secukinumab and TNFi were 31.6% and 58.8% (P = 0.057), respectively, at 3 months and 52.6% and 64.7%, respectively, at 6 months (P = 0.389).
CONCLUSION
Our findings suggest that secukinumab and TNFi are effective for patients with TAK who do not respond to oral glucocorticoids and conventional immunosuppressive agents, with similar response rates at 3 and 6 months.
Topics: Humans; Cohort Studies; Glucocorticoids; Immunosuppressive Agents; Prospective Studies; Takayasu Arteritis; Treatment Outcome; Tumor Necrosis Factor Inhibitors; Tumor Necrosis Factor-alpha
PubMed: 36916777
DOI: 10.1002/art.42496 -
Frontiers in Immunology 2023Giant Cell Arteritis (GCA) and Polymyalgia Rheumatica (PMR) are overlapping inflammatory diseases. Antigen-presenting cells (APCs), including monocytes and dendritic...
BACKGROUND
Giant Cell Arteritis (GCA) and Polymyalgia Rheumatica (PMR) are overlapping inflammatory diseases. Antigen-presenting cells (APCs), including monocytes and dendritic cells (DCs), are main contributors to the immunopathology of GCA and PMR. However, little is known about APC phenotypes in the peripheral blood at the time of GCA/PMR diagnosis.
METHODS
APCs among peripheral blood mononuclear cells (PBMCs) of treatment-naive GCA and PMR patients were compared to those in age- and sex-matched healthy controls (HCs) using flow cytometry (n=15 in each group). We identified three monocyte subsets, and three DC subsets: plasmacytoid DCs (pDCs), CD141+ conventional DCs (cDC1) and CD1c+ conventional DCs (cDC2). Each of these subsets was analyzed for expression of pattern recognition receptors (TLR2, TLR4), immune checkpoints (CD86, PDL1, CD40) and activation markers (HLA-DR, CD11c).
RESULTS
t-SNE plots revealed a differential clustering of APCs between GCA/PMR and HCs. Further analyses showed shifts in monocyte subsets and a lower proportion of the small population of cDC1 cells in GCA/PMR, whereas cDC2 proportions correlated negatively with CRP (r=-0.52). Classical monocytes of GCA/PMR patients show reduced expression of TLR2, HLA-DR, CD11c, which was in contrast to non-classical monocytes that showed higher marker expression. Additionally, single cell RNA sequencing in GCA patients identified a number of differentially expressed genes related to inflammation and metabolism in APCs.
CONCLUSION
Circulating non-classical monocytes display an activated phenotype in GCA/PMR patients at diagnosis, whereas classical monocytes show reduced expression of activation markers. Whether these findings reflect APC migration patterns or the effects of long-term inflammation remains to be investigated.
Topics: Humans; Giant Cell Arteritis; Polymyalgia Rheumatica; Leukocytes, Mononuclear; Toll-Like Receptor 2; Dendritic Cells; Inflammation
PubMed: 37600779
DOI: 10.3389/fimmu.2023.1201575 -
Annals of the Rheumatic Diseases Feb 2024The aim of the present study was to determine the clinical significance of subclinical giant cell arteritis (GCA) in polymyalgia rheumatica (PMR) and ascertain its...
OBJECTIVE
The aim of the present study was to determine the clinical significance of subclinical giant cell arteritis (GCA) in polymyalgia rheumatica (PMR) and ascertain its optimal treatment approach.
METHODS
Patients with PMR who fulfilled the 2012 European Alliance of Associations for Rheumatology/American College of Rheumatology Provisional Classification Criteria for PMR, did not have GCA symptoms and were routinely followed up for 2 years and were stratified into two groups, according to their ultrasound results: isolated PMR and PMR with subclinical GCA. The outcomes (relapses, glucocorticoid use and disease-modifying antirheumatic drug treatments) between groups were compared.
RESULTS
We included 150 patients with PMR (50 with subclinical GCA) with a median (IQR) follow-up of 22 (20-24) months. Overall, 47 patients (31.3 %) had a relapse, 31 (62%) in the subclinical GCA group and 16 (16%) in the isolated PMR group (p<0.001). Among patients with subclinical GCA, no differences were found in the mean (SD) prednisone starting dosage between relapsed and non-relapsed patients (32.4±15.6 vs 35.5±12.1 mg, respectively, p=0.722). Patients with subclinical GCA who relapsed had a faster prednisone dose tapering in the first 3 months compared with the non-relapsed patients, with a mean dose at the third month of 10.0±5.2 versus 15.2±7.9 mg daily (p<0.001). No differences were found between relapsing and non-relapsed patients with subclinical GCA regarding age, sex, C reactive protein and erythrocyte sedimentation rate.
CONCLUSIONS
Patients with PMR and subclinical GCA had a significantly higher number of relapses during a 2-year follow-up than patients with isolated PMR. Lower starting doses and rapid glucocorticoid tapering in the first 3 months emerged as risk factors for relapse.
Topics: Humans; Giant Cell Arteritis; Polymyalgia Rheumatica; Prednisone; Glucocorticoids; Recurrence
PubMed: 37932008
DOI: 10.1136/ard-2023-224768 -
Innere Medizin (Heidelberg, Germany) Feb 2024Giant cell arteritis (GCA) and Takayasu arteritis (TAK), as the main representatives of large vessel vasculitis, are rheumatological autoimmune disorders associated with... (Review)
Review
BACKGROUND
Giant cell arteritis (GCA) and Takayasu arteritis (TAK), as the main representatives of large vessel vasculitis, are rheumatological autoimmune disorders associated with inflammatory vessel wall changes in the arterial system that can lead to many types of organ damage.
MATERIAL AND METHODS
In this review the current scientific evidence on the diagnostics and treatment of large vessel vasculitis is evaluated and discussed.
RESULTS
In addition to the medical history and clinical presentation, imaging techniques nowadays represent the core of large vessel vasculitis diagnostics and have largely replaced the histological confirmation of GCA. After the diagnosis, acute treatment with glucocorticoids should be initiated as rapidly as possible but in the long term this should be tapered out or replaced by a steroid-sparing basic treatment. In contrast to GCA with already available options and other biologic disease-modifying antirheumatic drugs (DMARDs) about to be approved, there are still no approved biologic DMARD treatment options available for the less common TAK.
CONCLUSION
In contrast to the substantial progress in imaging diagnostics of large vessel vasculitis and with respect to the treatment of GCA, the much rarer TAK still requires intensive research efforts, especially to improve the treatment situation.
Topics: Humans; Giant Cell Arteritis; Takayasu Arteritis; Glucocorticoids; Antirheumatic Agents; Biological Products
PubMed: 38240814
DOI: 10.1007/s00108-023-01656-1