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Science Translational Medicine Sep 2023Autoimmune vasculitis of the medium and large elastic arteries can cause blindness, stroke, aortic arch syndrome, and aortic aneurysm. The disease is often refractory to...
Autoimmune vasculitis of the medium and large elastic arteries can cause blindness, stroke, aortic arch syndrome, and aortic aneurysm. The disease is often refractory to immunosuppressive therapy and progresses over decades as smoldering aortitis. How the granulomatous infiltrates in the vessel wall are maintained and how tissue-infiltrating T cells and macrophages are replenished are unknown. Single-cell and whole-tissue transcriptomic studies of immune cell populations in vasculitic arteries identified a CD4 T cell population with stem cell-like features. CD4 T cells supplying the tissue-infiltrating and tissue-damaging effector T cells survived in tertiary lymphoid structures around adventitial vasa vasora, expressed the transcription factor T cell factor 1 (TCF1), had high proliferative potential, and gave rise to two effector populations, Eomesodermin (EOMES) cytotoxic T cells and B cell lymphoma 6 (BCL6) T follicular helper-like cells. TCF1CD4 T cells expressing the interleukin 7 receptor (IL-7R) sustained vasculitis in serial transplantation experiments. Thus, TCF1CD4 T cells function as disease stem cells and promote chronicity and autonomy of autoimmune tissue inflammation. Remission-inducing therapies will require targeting stem-like CD4 T cells instead of only effector T cells.
Topics: Humans; Tertiary Lymphoid Structures; Vasculitis; Arteries; Inflammation; CD4-Positive T-Lymphocytes
PubMed: 37672564
DOI: 10.1126/scitranslmed.adh0380 -
Ugeskrift For Laeger Aug 2023In this case report, we present a 70-year-old male who was brought to our hospital with signs of upper gastrointestinal bleeding. The patient was diagnosed with aortitis...
In this case report, we present a 70-year-old male who was brought to our hospital with signs of upper gastrointestinal bleeding. The patient was diagnosed with aortitis two and a half months prior. We suspected upper gastrointestinal bleeding, and the patient was taken to the operating room for an acute endoscopy, which showed blood in the oesophagus, ventricle, and duodenum, but no bleeding source. CT angiography showed erosion of aortic aneurism, at the site of known aortitis, with bleeding into the lung and pleura. The patient was transported to the nearest university hospital for thoracic endovascular repair and survived.
Topics: Male; Humans; Aged; Hematemesis; Hemoptysis; Aortitis; Gastrointestinal Hemorrhage; Aortic Aneurysm; Hospitals, University
PubMed: 37767877
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 -
Cerebellum (London, England) Jun 2024We describe a male patient presenting with cerebellar ataxia and behavioural frontotemporal dementia in whom imaging showed cerebellar atrophy. He had significantly low...
We describe a male patient presenting with cerebellar ataxia and behavioural frontotemporal dementia in whom imaging showed cerebellar atrophy. He had significantly low N-acetyl aspartate to creatine (NAA/Cr) area ratio on MR spectroscopy of the cerebellum, primarily affecting the vermis. CT body scan showed extensive abnormal tissue within the mesentery, the retroperitoneum and perinephric areas. PET-CT showed increased tracer uptake within the wall of the aorta suggestive of an aortitis and within the perinephric tissue bilaterally. Biopsy of the perinephric tissue confirmed IgG4 disease. Treatment with steroids and mycophenolate improved his clinical state, but he developed symptoms attributed to pericardiac effusion that necessitated treatment initially with drainage and subsequently with pericardial window. After a course of rituximab, he had an episode of sepsis that did not respond to appropriate treatment and died as a result. Both the imaging findings and neurological presentation with cerebellar ataxia and behavioural frontotemporal dementia are novel in the context of IgG4 disease.
Topics: Humans; Male; Cerebellar Ataxia; Immunoglobulin G4-Related Disease; Middle Aged; Frontotemporal Dementia
PubMed: 37558930
DOI: 10.1007/s12311-023-01592-8 -
Cureus Aug 2023While the acute phase of the COVID-19 pandemic has largely come to pass, the chronic physiologic effects of the coronavirus continue to unfold. Specifically, the number... (Review)
Review
While the acute phase of the COVID-19 pandemic has largely come to pass, the chronic physiologic effects of the coronavirus continue to unfold. Specifically, the number of COVID-19-associated vasculitis cases has steadily increased since the onset of the pandemic. Data have shown that vasculitis may develop less than two weeks after COVID-19 or during a later onset of the disease. At this time, research has demonstrated that the novel coronavirus invades more than just the lungs; it can also attack the nervous system, cardiovascular system, and kidneys. In addition, there is a greater understanding of the pathogenesis regarding COVID-19-induced vasculitis via humoral immunity and immune complex disease. Recent case reports have shown an association between COVID-19 and secondary vasculitis. This review paper discusses case reports and data that suggest that COVID-19 may lead to specific vasculitis diseases such as giant cell arteritis, ophthalmic arteritis, aortitis, and Kawasaki-like disease. More research needs to be performed on this association to aid in diagnosis and treatment.
PubMed: 37638271
DOI: 10.7759/cureus.44119 -
Cureus Jan 2024Ankylosing spondylitis (AS) is a chronic inflammatory condition primarily affecting the axial bone and sacroiliac joints. Its etiology is complicated and involves... (Review)
Review
Ankylosing spondylitis (AS) is a chronic inflammatory condition primarily affecting the axial bone and sacroiliac joints. Its etiology is complicated and involves genetic variables, demographic factors (age of onset, gender, ethnicity, family history), and environmental variables. It typically manifests in males in their third decade. Galen is credited with first recognizing it, according to historical traditions, but it was not until the 19th century that specific diagnostic criteria were developed. The human leukocyte antigen B27 (HLA-B27) variation, around 20% of the genetic risk, is currently the most significant gene associated with AS susceptibility. Over 100 genes have been connected to AS susceptibility. Clinical signs of AS include stiffness and inflammation in the back, eye inflammation, aortitis (inflammation of the aorta), and spinal ankylosis that impacts posture and fatigue. The dagger sign and sacroiliitis on radiographs, in particular, are crucial for diagnosis. Early inflammatory alterations can be found using modern diagnostic tools such as MRI, and the HLA-B27 gene can help confirm the diagnosis. Overall, 80-95% of people with AS have the HLA-B27 marker. Furthermore, although non-specific, elevated inflammatory markers, such as C-reactive protein and erythrocyte sedimentation rate, offer supporting evidence. Over time, treatment paradigms have seen significant change. First-line treatments such as non-steroidal anti-inflammatory drugs are no longer the only options, even though disease-modifying anti-rheumatic drugs and biologics, especially tumor necrosis factor blockers, have been developed. Physical therapy, which emphasizes consistent exercise, stretches, and posture maintenance, is extremely helpful in managing AS. Surgical interventions can be required in extreme situations. The significance of the interleukin 23/17 axis in the disease cascade has been demonstrated by recent research. Furthermore, a deeper comprehension of the genetic landscape, mainly the functions of non-HLA-B27 loci, may open the door for more specialized therapies. Early diagnosis and interdisciplinary therapies can improve patient outcomes and quality of life as our understanding of AS grows.
PubMed: 38371049
DOI: 10.7759/cureus.52559 -
Revista Da Sociedade Brasileira de... 2024
Topics: Humans; Aortitis; Tomography, X-Ray Computed
PubMed: 38324817
DOI: 10.1590/0037-8682-0595-2023 -
Internal Medicine (Tokyo, Japan) Nov 2023
Topics: Humans; Aortitis; Granulocyte Colony-Stimulating Factor; Filgrastim
PubMed: 36948622
DOI: 10.2169/internalmedicine.1357-22