DOCSLIB.ORG

Bilateral Vertebral Artery Vasculitis—A Rare Manifestation of Giant Cell Arteritis and a Difficult Diagnosis Made Possible by 2-[18F]FDG PET/CT

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Bilateral Vertebral Artery Vasculitis—A Rare Manifestation of Giant Cell Arteritis and a Difficult Diagnosis Made Possible by 2-[18F]FDG PET/CT diagnostics Interesting Images Bilateral Vertebral Artery Vasculitis—A Rare Manifestation of Giant Cell Arteritis and a Difficult Diagnosis Made Possible by 2-[18F]FDG PET/CT Natasja Degn Justesen 1,2,*, Michael Stormly Hansen 1 , Mads Radmer Jensen 3, Oliver Niels Klefter 1, Jane Maestri Brittain 4 and Steffen Hamann 1,2 1 Department of Ophthalmology, Rigshospitalet, University of Copenhagen, 2600 Glostrup, Denmark; [email protected] (M.S.H.); [email protected] (O.N.K.); [email protected] (S.H.) 2 Faculty of Health and Medical Sciences, University of Copenhagen, 2200 Copenhagen, Denmark 3 Department of Clinical Physiology and Nuclear Medicine, Bispebjerg & Frederiksberg Hospital, University of Copenhagen, 2400 Copenhagen, Denmark; [email protected] 4 Department of Clinical Physiology, Nuclear Medicine and PET, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark; [email protected] * Correspondence: [email protected] Abstract: Giant cell arteritis (GCA) is the most common form of large vessel vasculitis. GCA is a medical and ophthalmological emergency, and rapid diagnosis and treatment with high-dose corticosteroids is critical in order to reduce the risk of stroke and sudden irreversible loss of vision. GCA can be difficult to diagnose due to insidious and unspecific symptoms—especially if typical Citation: Justesen, N.D.; Hansen, superficial extracranial arteries are not affected. In these cases, verification of clinical diagnosis using M.S.; Jensen, M.R.; Klefter, O.N.; temporal artery biopsy is not possible. This example illustrates the diagnostic value of hybrid imaging Brittain, J.M.; Hamann, S. Bilateral 18 Vertebral Artery Vasculitis—A Rare with 2-deoxy-2-[ F]fluoro-D-glucose positron emission tomography/computed tomography (2- 18 Manifestation of Giant Cell Arteritis [ F]FDG PET/CT), and the limitations of the temporal artery biopsy in bilateral vertebral GCA, and a Difficult Diagnosis Made causing transient ischemic attack in the visual cortex. In addition it indicates that inflammation in Possible by 2-[18F]FDG PET/CT. the artery wall can be visualized on 2-[18F]FDG PET/CT despite long term and ongoing high dose Diagnostics 2021, 11, 879. https:// glucocorticoid treatment. doi.org/10.3390/diagnostics11050879 Keywords: giant cell arteritis; large vessel vasculitis; 2-[18F]FDG PET/CT; temporal artery biopsy; Academic Editors: Riemer bilateral vertebral artery vasculitis; vertebrobasilar insufficiency H.J.A. Slart and Lars C. Gormsen Received: 22 April 2021 Accepted: 11 May 2021 Published: 14 May 2021 Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in published maps and institutional affil- iations. Copyright: © 2021 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https:// creativecommons.org/licenses/by/ 4.0/). Diagnostics 2021, 11, 879. https://doi.org/10.3390/diagnostics11050879 https://www.mdpi.com/journal/diagnostics Diagnostics 2021,, 11,, 879879 2 2of of 3 Figure 1. Whole body 2-[2-[18F]FDGF]FDG PET/low PET/low dose dose CT CT scan scan from from vertex vertex to to the the knees knees 32 32 days after start of corticosteroid treatment: ( A) Maximum intensity projection showing normal 2-[2-[1818F]FDG uptake in the aorta and its large branches. Poor signal to noise ratio is likely due to ongoingongoing high dosedose steroidsteroid treatment.treatment. Columns Columns depict depict PET PET ( (B),), lowdose CT, bone window ( C) and fused fused PET/CT PET/CT (D (D) )in in axial axial (upper (upper row), row), coronal coronal (middle (middle ro row)w) and and sagittal sagittal (lower (lower row) row) reconstructions reconstructions of theof the head head and and neck. neck. Blue Bluearrows arrows show show visible visible uptake uptake in the invertebral the vertebral artery interpreted artery interpreted compatible compatible with giant with cell giant arteritis. cell A 67-year-old male smoker with chronic obstructive pulmonary disease was referred to the Department of Internal Med- arteritis. A 67-year-old male smoker with chronic obstructive pulmonary disease was referred to the Department of Internal icine due to unspecific symptoms including weight loss and pain in the neck and shoulders for three months. C-reactive Medicine due to unspecific symptoms including weight loss and pain in the neck and shoulders for three months. C-reactive protein was elevated at 47 mg/L (normal < 10 mg/L). A computed tomography (CT) scan of thorax and abdomen showed proteinincidental was adenomas elevated atin 47the mg/L adrenal (normal glands < 10bilaterally mg/L). Abut computed no signs tomographyof malignancy (CT) or scaninfection. of thorax A rhe andumatology abdomen consulta- showed tionincidental revealed adenomas additional in the history adrenal of unspecific glands bilaterally bilateral but visual no signsdisturbances of malignancy as well or as infection.jaw claudication A rheumatology and scalp consultationtenderness. Baserevealedd on additionalthe initial clinical history assessment, of unspecific the bilateral rheumatologist visual disturbances suspected polymyalgia as well as jaw rheumatica claudication (PMR) and with scalp a tenderness.low proba- bilityBased of on cranial the initial involvement. clinical assessment, Treatment the with rheumatologist prednisolone suspected 30 mg polymyalgiaper day was rheumaticainitiated and (PMR) a temporal with a low artery probability biopsy (TAB)of cranial was involvement. requested along Treatment with an withophthalmological prednisolone consultation. 30 mg per day Four was days initiated later the and ophthalmologist a temporal artery found biopsy no expla- (TAB) wasnation requested to the visual along symptoms with an ophthalmological and no signs of retinal consultation. or optic Fournerve days ischemia. later the As ophthalmologistthe retinal and optic found nerve no explanationassessment wasto the unremarkable, visual symptoms the ophthalmolo and no signsgist of retinalsuspected or optica retro nerve chiasmal ischemia. pathology As the of retinal a transient/intermittent and optic nerve assessment nature. In wasthe setting of PMR the ophthalmology consult raised the suspicion of GCA and increased the dosage of prednisolone to 75 unremarkable, the ophthalmologist suspected a retro chiasmal pathology of a transient/intermittent nature. In the setting mg per day resulting in a decrease in CRP and marked symptom relief. The TAB result came back showing unspecific of PMR the ophthalmology consult raised the suspicion of GCA and increased the dosage of prednisolone to 75 mg per day inflammation in the initial incisions of the temporal artery. In the more profound incisions of the artery a well-defined arearesulting with ina atransmural decrease in inflammation CRP and marked emerged symptom involving relief. adventitia The TAB media result cameand intima. back showing A comb unspecificination of inflammationlymphocytes, neutrophils,in the initial eosinophils incisions of and the epithelioid temporal artery.histiocytes In the were more present. profound The internal incisions elastic of the lamina artery was a well-defineddegraded. No areaconvincing with a indicationtransmural of inflammation giant cells were emerged detected. involving The final adventitia conclusion media from and the intima. pathologist A combination was, that it of was lymphocytes, an arteritis neutrophils,with a sug- gestioneosinophils of a andgranulomatous epithelioid histiocytesimprint, and were although present. no The giant internal cells were elastic detected, lamina wasGCA degraded. could not No be convincingruled out. To indication pursue clinicallyof giant cells suspected were detected. GCA a 2- The[18F]FDG final conclusionPET was performed from the 32 pathologist days after was, start that of corticosteroid it was an arteritis treatment with a(Figure suggestion 1). This of showa granulomatoused faint but visible imprint, uptake and although in the vertebral no giant arteries cells were bilaterally detected, which GCA in the could context not be was ruled interpret out. Toed pursuecompatible clinically with GCA.suspected No Doppler GCA a 2-[ultrasound18F]FDG PETwas wasperformed performed. Hence, 32 daysthe reported after start visual of corticosteroid symptoms can treatment be explained (Figure by1 ).vertebrobasilar This showed insufficiency causing ischemia in visual cortex, a rare manifestation of GCA. GCA is a chronic, idiopathic, granulomatous faint but visible uptake in the vertebral arteries bilaterally which in the context was interpreted compatible with GCA. No vasculitis of large- and medium-sized arteries with variable segmental distribution resulting in heterogeneous symptoms Doppler ultrasound was performed. Hence, the reported visual symptoms can be explained by vertebrobasilar insufficiency and diagnostic challenges. Incidence is higher in people of Northern European heritage and possible predispositions in- cludecausing genetic, ischemia environmental in visual cortex, and autoimmune a rare manifestation factors. ofGCA GCA. occurs GCA exclusively is a chronic, in idiopathic,patients older granulomatous than 50 years vasculitis of age and of islarge- three and times medium-sized more frequent arteries in women with variable (24.2 per segmental 100,000) distribution than in men resulting (8.2 per in100,000) heterogeneous [1]. Patients
Load more

Recommended publications
  • Guideline-Management-Giant-Cell
    Arthritis Care & Research Vol. 73, No. 8, August 2021, pp 1071–1087 DOI 10.1002/acr.24632 © 2021 American College of Rheumatology. This article has been contributed to by US Government employees and their work is in the public domain in the USA. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis Mehrdad Maz,1 Sharon A. Chung,2 Andy Abril,3 Carol A. Langford,4 Mark Gorelik,5 Gordon Guyatt,6 Amy M. Archer,7 Doyt L. Conn,8 Kathy A. Full,9 Peter C. Grayson,10 Maria F. Ibarra,11 Lisa F. Imundo,5 Susan Kim,2 Peter A. Merkel,12 Rennie L. Rhee,12 Philip Seo,13 John H. Stone,14 Sangeeta Sule,15 Robert P. Sundel,16 Omar I. Vitobaldi,17 Ann Warner,18 Kevin Byram,19 Anisha B. Dua,7 Nedaa Husainat,20 Karen E. James,21 Mohamad A. Kalot,22 Yih Chang Lin,23 Jason M. Springer,1 Marat Turgunbaev,24 Alexandra Villa-Forte, 4 Amy S. Turner,24 and Reem A. Mustafa25 Guidelines and recommendations developed and/or endorsed by the American College of Rheumatology (ACR) are intended to provide guidance for particular patterns of practice and not to dictate the care of a particu- lar patient. The ACR considers adherence to the recommendations within this guideline to be voluntary, with the ultimate determination regarding their application to be made by the physician in light of each patient’s individual circumstances. Guidelines and recommendations are intended to promote beneficial or desirable outcomes but cannot guarantee any specific outcome.
    [Show full text]
  • A 69-Year-Old Woman with Intermittent Claudication and Elevated ESR
    756 Self-assessment corner Postgrad Med J: first published as 10.1136/pgmj.74.878.756 on 1 December 1998. Downloaded from A 69-year-old woman with intermittent claudication and elevated ESR M L Amoedo, M P Marco, M D Boquet, S Muray, J M Piulats, M J Panades, J Ramos, E Fernandez A 69-year-old woman was referred to our out-patient clinic because of long-term hypertension and stable chronic renal failure (creatinine 132 ,umol/l), which had been attributed to nephroan- giosclerosis. She presented with a one-year history of anorexia, asthenia and loss of 6 kg ofweight. In addition, she complained of intermittent claudication of the left arm and both legs lasting 2 months. This provoked important functional limitation of the three limbs, and impaired ambula- tion. She did not complain of headache or symptoms suggestive of polymyalgia rheumatica. Her blood pressure was 160/95 mmHg on the right arm and undetectable on the left arm and lower limbs. Both temporal arteries were palpable and not painful. Auscultation of both carotid arteries was normal, without murmurs. Cardiac and pulmonary auscultation were normal. Mur- murs were audible on both subclavian arteries. Neither the humeral nor the radial pulse were detectable on the left upper limb. Murmurs were also audible on both femoral arteries, and nei- ther popliteal nor distal pulses were palpable. Her feet were cold although they had no ischaemic lesions. Funduscopic examination was essentially normal. The most relevant laboratory data were: erythrocyte sedimentation rate (ESR) 120 mm/h, C-reactive protein 4.4 mg/dl (normal range <1.5); antinuclear and antineutrophil cytoplasmic antibodies were negative.
    [Show full text]
  • Crohn's Disease
    Harvard-MIT Division of Health Sciences and Technology HST.121: Gastroenterology, Fall 2005 Instructors: Dr. Jonathan Glickman Vascular and Inflammatory Diseases of the Intestines Overview • Vascular disorders – Vascular “malformations” – Vasculitis – Ischemic disease • Inflammatory disorders of specific etiology – Infetious enterocolitis – “Immune-mediated” enteropathy – Diverticular disease • Idiopathic inflammatory bowel disease – Crohn’s disease – Ulcerative colitis Sporadic Vascular Ectasia (Telangiectasia) • Clusters of tortuous thin-walled small vessels lacking muscle or adventitia located in the mucosa and the submucosa • The most common type occurs in cecum or ascending colon of individuals over the age of 50 and is commonly known as “angiodysplasia” • Angiodysplasias account for 40% of all colonic vascular lesions and are the most common cause of lower GI bleeding in individuals over the age of 60 Angiodysplasia Hereditary Vascular Ectasia • Hereditary Hemorrhagic Telangiectasia (HHT) or Osler- Webber-Rendu disease • Systematic disease primarily involving skin and mucous membranes, and often the GI tract • Autosomal dominant disease with positive family history in 80% of cases • After epistaxis which occurs in 80% of individuals, GI bleed is the most frequent presentation and occurs in 10-40% of cases Arteriovenous Malformations (AVM’s) • Irregular meshwork of structurally abnormal medium to large ectatic vessels • Unlike small vessel ectasias, AVM’s can be distributed in all layers of the bowel wall • AVM’s may present anywhere
    [Show full text]
  • Should I Send My Patient with Previous Giant Cell Arteritis for Imaging of The
    Downloaded from ard.bmj.com on January 23, 2013 - Published by group.bmj.com ARD Online First, published on December 22, 2012 as 10.1136/annrheumdis-2012-202145 Clinical and epidemiological research EXTENDED REPORT Should I send my patient with previous giant cell arteritis for imaging of the thoracic aorta? A systematic literature review and meta-analysis Sarah Louise Mackie,1 Elizabeth M A Hensor,1 Ann W Morgan,1 Colin T Pease2 ▸ Additional material is ABSTRACT together has been estimated for the Swedish popula- published online only. To view Objectives To review the literature in order to estimate tion at 0.16 per 1000 person-years in men, and 0.09 please visit the journal online (http://dx.doi.org/10.1136/ how many previously unknown thoracic aortic aneurysms per 1000 person-years in women, with a median annrheumdis-2012-202145). (TAAs) and thoracic aortic dilatations (TADs) might be age at diagnosis of 71 years and 40% overall still detected by systematic, cross-sectional aortic imaging of unruptured at diagnosis.9 Abdominal aortic aneur- 1NIHR-Leeds Musculoskeletal Biomedical Research Unit, patients with giant cell arteritis (GCA). ysm (AAA) is observed in about 5% of men over 65 10 Leeds Institute of Molecular Methods A systematic literature review was performed in ultrasound screening programmes. Medicine, Leeds, West using Ovid Medline, Embase and the Cochrane Library. Patients with GCA appear to have an elevated Yorkshire, UK Studies potentially relevant to TAA/TAD were evaluated incidence of aortic aneurysm, particularly TAA, 2Department of Rheumatology, Leeds Teaching Hospitals NHS by two authors independently for relevance, bias and compared with the general population; the aneur- Trust, Leeds, West Yorkshire, heterogeneity.
    [Show full text]
  • Survival After Aortic Dissection in Giant Cell Arteritis
    332 Letters to the editor pressure or hypotensive episodes were technique and rating scale for abnormalities of the thorax, without intravenous contrast seen in scleroderma and related disorders. recorded during the trial. Among the side Arthritis Rheum 1981; 24: 1159-65. medium (because of asthma), showed a effects, mild flushing (35%) and headache 9 Ferri C, La Civita L, Zignego A et al. Plasma normal mediastinum but a small rim of L, Ann Rheum Dis: first published as 10.1136/ard.55.5.332 on 1 May 1996. Downloaded from (24%) were the most frequent in isradipine levels of endothelin-I in mixed cryoglobulin- pleural fluid on the left. aemia patients. Br J Rheumatol 1994; 33: recipients. 689-90. At follow up, the patient complained of This study has demonstrated the favour- 10 Yanagisawa M, Kurihara H, Kimura S, et al. A right thigh claudication. Radiofemoral delay able effects of isradipine on patients with novel potent vasoconstrictor peptide pro- was noted. CT of the abdomen showed Raynaud's phenomenon; moreover, it is the duced by vascular endothelial cells. Nature internal displacement ofcalcified atheroma in 1988; 332: 411-5. first to demonstrate a significant reduction 11 Levin E R. Endothelins [review]. N Engl J Med the aorta, suggesting aortic dissection. Mag- in plasma concentrations of ET-1 during 1995; 333: 356-63. netic resonance imaging (MRI) confirmed calcium antagonist treatment. The most this as distal to the origin of the left sub- usual manifestations of Raynaud's phenom- clavian artery, extending to the aortic bifur- enon are pain and numbness in the fingers, cation.
    [Show full text]
  • Thrombosis in Vasculitis: from Pathogenesis to Treatment
    Emmi et al. Thrombosis Journal (2015) 13:15 DOI 10.1186/s12959-015-0047-z REVIEW Open Access Thrombosis in vasculitis: from pathogenesis to treatment Giacomo Emmi1*†, Elena Silvestri1†, Danilo Squatrito1, Amedeo Amedei1,2, Elena Niccolai1, Mario Milco D’Elios1,2, Chiara Della Bella1, Alessia Grassi1, Matteo Becatti3, Claudia Fiorillo3, Lorenzo Emmi2, Augusto Vaglio4 and Domenico Prisco1,2 Abstract In recent years, the relationship between inflammation and thrombosis has been deeply investigated and it is now clear that immune and coagulation systems are functionally interconnected. Inflammation-induced thrombosis is by now considered a feature not only of autoimmune rheumatic diseases, but also of systemic vasculitides such as Behçet’s syndrome, ANCA-associated vasculitis or giant cells arteritis, especially during active disease. These findings have important consequences in terms of management and treatment. Indeed, Behçet’syndrome requires immunosuppressive agents for vascular involvement rather than anticoagulation or antiplatelet therapy, and it is conceivable that also in ANCA-associated vasculitis or large vessel-vasculitis an aggressive anti-inflammatory treatment during active disease could reduce the risk of thrombotic events in early stages. In this review we discuss thrombosis in vasculitides, especially in Behçet’s syndrome, ANCA-associated vasculitis and large-vessel vasculitis, and provide pathogenetic and clinical clues for the different specialists involved in the care of these patients. Keywords: Inflammation-induced thrombosis, Thrombo-embolic disease, Deep vein thrombosis, ANCA associated vasculitis, Large vessel vasculitis, Behçet syndrome Introduction immunosuppressive treatment rather than anticoagulation The relationship between inflammation and thrombosis is for venous or arterial involvement [8], and perhaps one not a recent concept [1], but it has been largely investigated might speculate that also in AAV or LVV an aggressive only in recent years [2].
    [Show full text]
  • Versus Arthritis Vasculitis Information Booklet
    Condition Vasculitis Vasculitis This booklet provides information and answers to your questions about this condition. Arthritis Research UK booklets are produced and printed entirely from charitable donations. What is vasculitis? Vasculitis means inflammation of the blood vessels. There are several different types of the condition, many with unknown causes, but treatments can be very effective. In this booklet we’ll briefly explain the main types of vasculitis, how they’re diagnosed and treated, what you can do to help yourself and where to get more information. At the back of this booklet you’ll find a brief glossary of medical words – we’ve underlined these when they’re first used. www.arthritisresearchuk.org Page 2 of 32 Vasculitis information booklet Arthritis Research UK What’s inside? 2 Vasculitis at a glance 5 Who diagnoses and treats 15 What is the outlook? vasculitis? 15 How is vasculitis diagnosed? 6 What is vasculitis? – What tests are there? 8 What are the symptoms 17 What treatments are there for vasculitis? of vasculitis? – Drugs 9 What types of vasculitis 20 Self-help and daily living are there? – Exercise – Takayasu arteritis (TA) – Diet and nutrition – Giant cell arteritis (temporal arteritis) – Stop smoking – Polyarteritis nodosa (PAN) – Keep warm – Kawasaki disease 22 Research and new – Granulomatosis with polyangiitis developments (Wegener’s granulomatosis) 23 Glossary – Behçet’s syndrome – Eosinophilic granulomatosis 26 Where can I find out more? with polyangiitis (Churg–Strauss 28 We’re here to help syndrome) – Microscopic polyangiitis – Cryoglobulin-associated vasculitis – Immunoglobulin A vasculitis (Henoch–Schönlein purpura) 14 Who gets vasculitis? 15 What causes vasculitis? Page 3 of 32 Vasculitis information booklet At a glance Vasculitis There are several different types of vasculitis but all are treatable.
    [Show full text]
  • Giant Cell Arteritis
    GIANT CELL ARTERITIS What is giant cell arteritis (GCA)? Giant cell arteritis (GCA) is a form of vasculitis—a family of rare disorders characterized by inflammation of the blood vessels, which can restrict blood flow and damage vital organs and tissues. Also called temporal arteritis, GCA typically affects the arteries in the neck and scalp, especially the temples. It can also affect the aorta and its large branches to the head, arms and legs. GCA is the most common form of vasculitis in adults over the age of 50. The most common symptoms of GCA include persistent, throbbing headaches, tenderness of the temples and scalp, jaw pain, fever, joint pain, and vision problems. Early treatment is vital to prevent serious complications such as blindness or stroke. GCA is typically treated with high doses of corticosteroids such as prednisone, sometimes in combination with other medications that suppress the immune system. Prompt treatment usually relieves symptoms, however GCA is a chronic condition with periods of relapse and remission, so ongoing medical care is usually necessary. Patients with GCA may also have symptoms of polymyalgia rheumatica (PMR), a closely related inflammatory disorder. Causes The cause of GCA is not yet fully understood by researchers. Vasculitis is classified as an autoimmune disorder—a disease which occurs when the body’s natural defense system mistakenly attacks healthy tissues. Researchers believe a combination of factors may trigger the inflammatory process. Studies have linked genetic factors, infectious agents, and a prior history of cardiovascular disease to the development of GCA. Who gets GCA? GCA is the most common form of vasculitis in older adults, affecting people over 50 years of age, with an average onset of 74 years of age.
    [Show full text]
  • Idiopathic Peripheral Retinal Telangiectasia in Adults: a Case Series and Literature Review
    Journal of Clinical Medicine Article Idiopathic Peripheral Retinal Telangiectasia in Adults: A Case Series and Literature Review Maciej Gaw˛ecki Dobry Wzrok Ophthalmological Clinic, 80-822 Gdansk, Poland; [email protected]; Tel.: +48-501-788-654 Abstract: Idiopathic peripheral retinal telangiectasia (IPT), often termed as Coats disease, can present in a milder form with the onset in adulthood. The goal of this case series study and literature review was to describe and classify different presenting forms and treatment of this entity and to review contemporary methods of its management. Six cases of adult onset IPT were described with the following phenotypes based on fundus ophthalmoscopy, fluorescein angiography, and optical coherence tomography findings: IPT without exudates or foveal involvement, IPT with peripheral exudates without foveal involvement, IPT with peripheral exudates and cystoid macular edema, and IPT with peripheral and macular hard exudates. Treatments applied in this series included observation, laser photocoagulation, and anti-vascular endothelial growth factor (VEGF) treatment with variable outcomes depending upon the extent of IPT, the aggressiveness of laser treatment, and the stringency of follow-up. The accompanying literature review suggests that ablative therapies, especially laser photocoagulation, remain the most effective treatment option in adult-onset IPT, with anti-VEGF therapy serving as an adjuvant procedure. Close follow-up is necessary to achieve and maintain reasonable good visual and morphological results. Keywords: Coats disease; peripheral retinal telangiectasia; laser photocoagulation; anti-VEGF treatment Citation: Gaw˛ecki,M. Idiopathic Peripheral Retinal Telangiectasia in 1. Introduction Adults: A Case Series and Literature Review. J. Clin. Med. 2021, 10, 1767. Idiopathic peripheral retinal telangiectasia (IPT), usually referred to as Coats disease, https://doi.org/10.3390/jcm10081767 has been well-described in the medical literature since its discovery in 1908 [1].
    [Show full text]
  • Coronary Vasculitis
    biomedicines Review Coronary Vasculitis Tommaso Gori Kardiologie I and DZHK Standort Rhein-Main, Universitätsmedizin Mainz, 55131 Mainz, Germany; [email protected] Abstract: The term coronary “artery vasculitis” is used for a diverse group of diseases with a wide spectrum of manifestations and severity. Clinical manifestations may include pericarditis or my- ocarditis due to involvement of the coronary microvasculature, stenosis, aneurysm, or spontaneous dissection of large coronaries, or vascular thrombosis. As compared to common atherosclerosis, patients with coronary artery vasculitis are younger and often have a more rapid disease progression. Several clinical entities have been associated with coronary artery vasculitis, including Kawasaki’s disease, Takayasu’s arteritis, polyarteritis nodosa, ANCA-associated vasculitis, giant-cell arteritis, and more recently a Kawasaki-like syndrome associated with SARS-COV-2 infection. This review will provide a short description of these conditions, their diagnosis and therapy for use by the practicing cardiologist. Keywords: coronary artery disease; vasculitis; inflammatory diseases 1. Introduction The term vasculitis refers to a group of conditions whose pathophysiology is mediated by inflammation of blood vessels. Most forms of vasculitis are systemic and may present variable clinical manifestations, requiring a multidisciplinary approach. A number of etiologies have been reported; independently of the specific organ involvement, vasculitis Citation: Gori, T. Coronary can be primary or secondary to another autoimmune disease or can be associated with Vasculitis. Biomedicines 2021, 9, 622. other precipitants such as drugs, infections or malignancy [1]. Almost all cases of coronary https://doi.org/10.3390/ vasculitis appear as a manifestation of systemic (primary) vasculitis, which are classified biomedicines9060622 based on the type and size of the vessels affected and the cellular component responsible for the tissue infiltration.
    [Show full text]
  • NIH Public Access Author Manuscript Circulation
    NIH Public Access Author Manuscript Circulation. Author manuscript; available in PMC 2009 October 9. NIH-PA Author ManuscriptPublished NIH-PA Author Manuscript in final edited NIH-PA Author Manuscript form as: Circulation. 2008 June 10; 117(23): 3039±3051. doi:10.1161/CIRCULATIONAHA.107.760686. Aortitis Heather L. Gornik, M.D., M.H.S. and Assistant Professor of Medicine, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Medical Director, Non-Invasive Vascular Laboratory Department of Cardiovascular Medicine The Cleveland Clinic Foundation 9500 Euclid Avenue/Desk S60 Cleveland, Ohio 44120 (216) 445-3689 [email protected] Mark A. Creager, M.D.* Professor of Medicine, Harvard Medical School Simon C. Fireman Scholar in Cardiovascular Medicine Director, Vascular Center Brigham and Women's Hospital 75 Francis Street Boston, Massachusetts 02115 (617) 732-5267 [email protected] Keywords Aortitis; vasculitis; giant cell arteritis; Takayasu arteritis; aortic aneurysm Aortitis is the all-encompassing term ascribed to inflammation of the aorta. The most common causes of aortitis are the large vessel vasculitides, giant cell arteritis (GCA) and Takayasu arteritis, although it is also associated with several other rheumatologic diseases. Infectious aortitis is a rare but potentially life-threatening disorder. In some cases, aortitis is an incidental finding at the time of histopathologic examination following surgery for aortic aneurysm. As the clinical presentation of aortitis is highly variable, the cardiovascular clinician must have a high index of suspicion to establish an accurate diagnosis of this disorder in a timely fashion. In this manuscript, we review the pathophysiology, epidemiology, diagnostic approach, and management of aortitis.
    [Show full text]
  • Giant Cell Arteritis: Immune and Vascular Aging As Disease Risk Factors Shalini V Mohan, Y Joyce Liao, Jonathan W Kim, Jörg J Goronzy and Cornelia M Weyand*
    Mohan et al. Arthritis Research & Therapy 2011, 13:231 http://arthritis-research.com/content/13/4/231 REVIEW Giant cell arteritis: immune and vascular aging as disease risk factors Shalini V Mohan, Y Joyce Liao, Jonathan W Kim, Jörg J Goronzy and Cornelia M Weyand* GCA is a complex disorder with multiple pathogenic Abstract factors. An instigator initiating the infl am matory process Susceptibility for giant cell arteritis increases with has not been identifi ed; however, over whelming evidence chronological age, in parallel with age-related has accumulated that abnormalities in innate and restructuring of the immune system and age-induced adaptive immunity play a critical role in the initiation and remodeling of the vascular wall. Immunosenescence the perpetuation of the vasculitis. results in shrinkage of the naïve T-cell pool, contraction Several unique factors of GCA have been informative of T-cell diversity, and impairment of innate immunity. in dissecting its immunopathogenesis. Th e disease is Aging of immunocompetent cells forces the host to characterized by a stringent tissue tropism; meaning that take alternative routes for protective immunity and granulomatous wall infi ltrates typically appear in arteries confers risk for pathogenic immunity that causes of selected vascular beds. Th is pathogenic feature chronic infl ammatory tissue damage. Dwindling strongly suggests that vessel-wall-specifi c factors drive immunocompetence is particularly relevant as the GCA. Dendritic cells (DCs), similar to skin-residing aging host is forced to cope with an ever growing Langerhans cells, have been implicated in providing infectious load. Immunosenescence coincides initial signals that break the immune protection of with vascular aging during which the arterial wall arterial walls [2,3].
    [Show full text]