DOCSLIB.ORG

Survival After Aortic Dissection in Giant Cell Arteritis

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text

Load more

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. Eight weeks after discharge, he devel- which in some subjects can be complicated Survival after aortic oped headaches, aching thighs and jaw by skin ulcers5 requiring prompt inter- claudication with temporal artery tenderness. vention.3 4 In the present study, isradipine dissection in giant cell Prednisolone 30 mg daily was begun with was able to reduce the frequency, severity, arteritis rapid improvement. Clinical improvement and disabling nature of acute attacks of correlated with his ESR decreasing to normal Raynaud's phenomenon; this result agrees Acute aortic dissection in giant cell arteritis and with gradual resolution of the radio- with previous experience of the use of is rare and usually fatal. It is associated with femoral delay. calcium antagonists in patients with hypertension and is commoner in females. Raynaud's phenomenon,' 3 4 and reflects an We describe two patients who were diagnosed PATIENT 2 appropriate selection of patients to receive in life and who survived on medical treatment A 68 year old white women presented with the treatment. In addition, the clinicosero- alone for a prolonged period: one for two five days of worsening anterior pleuritic chest logical manifestations and changes in capil- years; the other still alive at three years. A pain radiating to the left, sweats, nausea, and laroscopic findings in this series of patients, third patient who required aortic grafting has an increased temperature. She had a history including those with type I and type II survived 14 years. A literature review of osteoarthrosis, euthyroid Graves' ophthal- Raynaud's phenomenon, were relatively revealed 43 other cases, with only three mopathy, Raynaud's phenomenon, and long- homogeneous. survivors on medical care alone. standing hypertension. Temporal arteritis The clinical improvement of our patients had been diagnosed on biopsy eight years with Raynaud's phenomenon in response to Case reports previously, following a characteristic illness isradipine was reflected in a reduction in PATIENT 1 accompanied by an ESR of 121 mm/lst h. plasma concentrations ofET-1. Although the A 68 year old white male presented with Prednisolone for three years had achieved pathophysiological ftmction of ET-1 remains acute chest pain on exertion radiating to the remission. unknown,"3 a number of vascular disorders back, accompanied by dyspnoea and On admission, the patient was pyrexial at are characterised by increased concentrations sweating. He had noted weight loss of more 37 70C. The heart rate was 110 beat/min with of this vasoactive peptide." How isradipine than 7 kg over the previous months. A history no radiofemoral delay, and the blood pressure may influence the titres ofET-1 is difficult to of back and neck ache was ascribed to was 140/90 mm Hg, equal in both arms. There explain but, because ET-l is released in spondylosis, and shoulder pain to a rotator was dullness to percussion at both lung bases response to ischaemic stimuli," we hypothe- cuff lesion. His pulse was 60 beats/min and bronchial breathing at the left base. ESR sise that isradipine is able to induce a change regular, and his blood pressure was 150/70 was 86 mmnlst h, and the leucocyte count I1-4 in ET-l concentrations indirectly, by improv- mm Hg. An ejection murmur was noted at with a neutrophilia. ECG showed left ventri- ing tissue perfusion. The findings of this the left sternal edge. Examination was other- cular hypertrophy. Chest radiograph showed preliminary investigation merit further in- wise unremarkable. bilateral small pleural effusions and a markedly http://ard.bmj.com/ vestigation in larger series of patients with The patient developed intermittent pyrexia widened mediastinum. CT of the thorax and Raynaud's phenomenon. over the next three weeks, and experienced abdomen revealed a dilated thoracic aorta with frequent left sided stabbing chest pain. The a dissection in the descending portion. Trans- LUCA LA CIVITA leucocyte count increased from normal to DIIJA GIUGGIOLI oesophageal echocardiography (TOE) initially MARIA GRAZIA DEL CHICCA 14-5 with a neutrophilia. Erythrocyte sedi- suggested dissection commencing at the aortic GIOVANNI LONGOMBARDO mentation rate (ESR) peaked at 94 mm/lst root; however, repeat CT, arch aortography GIAMPIERO PASERO h and C reactive protein (CRP) at 260 mg/l. and MRI confirmed type B dissection. The CLODOVEO FERRI on September 25, 2021 by guest. Protected copyright. Istituto Patologia Medica and Blood and urine cultures were negative, and patient was treated with a [3 blocker to main- Fisiologia Clinica (CNR), autoantibody screen was normal. The tain normal blood pressure, and prednisolone University ofPisa, Pisa, Italy electrocardiogram (ECG) showed left ven- at a dose of 20 mg. The pain resolved and she tricular hypertrophy, confirmed by trans- was discharged, remaining well and normo- Correspondence to: Dr Luca La Civita, Istituto di Patologia Medica I, University of Pisa, via Roma thoracic echocardiogram, which showed no tensive for two years. Mediastinal widening 67, 56126 Pisa, Italy. other abnormality. Chest radiograph showed then began to accelerate, MRI imaging con- minor atelectasis at both lung bases. Indium finning a grossly dilated dissecting descending 1 Cleophas T J M, Niemeyer M G. Raynaud's labelled leucocyte scan showed some media- aorta and dilatation of the ascending aorta syndrome, an enigma after 130 years. stinal uptake. Computed tomography (CT) (figure). She died soon afterwards. Angiology 1993; 43: 196-209. 2 Biondi M L, Marasini B, Bassani C, Ago- stoni A. Increased plasma endothelin levels in patients with Raynaud's phenomenon. NEngl JMed 1991; 324: 1139-40. 3 Wollersheim H, van Zwieten P A. Treatment of Raynaud's phenomenon. Eur Heart J 1993; 14:147-9. 4 La Civita L, Pitaro N, Rossi M, et al. Amlodipine in the treatment of Raynaud's phenomenon: a double-blind placebo- controlled crossover study. Clin Drug Invest 1996; 11 (suppl): 126-31. 5 Kallenberg C G M, Pastoor G W, Wouda A, The T. Antinuclear antibodies in patients with Raynaud's phenomenon: clinical signifi- cance of anticentromere antibodies. Ann Rheum Dis 1982; 41: 382-7. 6 Allen E V, Brown G E. Raynaud's disease: a critical review of a minimal requisite for diagnosis. Am J Med Sci 1932; 183: 187-200. 7 Ferri C, Bernini L, Cecchetti R, et al. Cutaneous and serological subset of systemic sclerosis. Their relevance in diagnosis, severity and prognosis of the disease. J Tl weighted axial MR images. Left: Normalflow void in the dilated ascending aorta and a complex Rheumatol 1991; 18: 1826-32. signalpattern in the descending aorta at the origin ofthe dissection. Right: At the level ofthe dilated 8 Maricq H R Widefield capillary microscopy: aortic root, showing the two lumina in the descending thoracic aorta. Letters to the editor 333 Summary ofcases ofaortic dissection associated with giant ceUl arteritis reported in the literature Correspondence to: Dr A M L Lever. Source Number Age Sex Hypertension Comments 1 Klein R G, Hunder G G, Stanson A W, ofcases (yr) Sheps S G. Large artery involvement in giant cell (temporal) arteritis. Ann Intern Med 1975; Ann Rheum Dis: first published as 10.1136/ard.55.5.332 on 1 May 1996. Downloaded from Broch 19472 1 68 F + First recorded case (case report) 83: 806-12. Klein 1975' 3 66 F NK Series of 248 cases of GCA. All 3 sudden deaths; 2 Broch A J, Ytrehus 0. Tre nye tilfelle av arteritis 73 F NK diagnosed at postmortem examination temporalis. Nord Med 1947; 34: 1111. 70 F NK 3 Nakashima Y, Kurozumi T, Sueshi K, Larson 19844 2 NK F + Postmortem series of 161 acute dissections; 'healed Tanaka K. Dissecting aneurysm: a NK F + aortitis' found in both clinicopathological and histopathologic study Leonard 1979' 2 62 F NK Postmortem series of 171 acute dissections of 111 autopsied cases. Hum Pathol 1990; 21: 74 F + 291-6. BMJ 19606 1 70 M + Known GCA. Sudden death (case report) 4 Larson E W, Edwards W D. Risk factors for Save-Soderbergh 2 85 F + Postmortem series of 9 sudden deaths in GCA aortic dissection: a necropsy study of 161 19867 79 F + cases. AmJ Cardiol 1984; 53: 849-55. Nordt 1991'4 1 70 F - Acute myocardial infarction caused by dissection; 5 Leonard J C, Hasleton P S.
Recommended publications
  • Cogan's Syndrome

    Cogan's Syndrome

    IMAGING IN MEDICINE COLODETTI R ET AL. Cogan’s syndrome – A rare aortitis, difficult to diagnose but with therapeutic potential RAIZA COLODETTI1, GUILHERME SPINA2, TATIANA LEAL3, MUCIO OLIVEIRA JR4, ALEXANDRE SOEIRO3* 1MD Cardiologist, Instituto do Coração (InCor), Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brazil 2Assistant Physician at the Valvular Heart Disease Outpatient Clinic, InCor, HC-FMUSP, São Paulo, SP, Brazil 3Assistant Physician at the Clinical Emergency Service, InCor, HC-FMUSP, São Paulo, SP, Brazil 4Director of the Clinical Emergency Service, InCor, HC-FMUSP, São Paulo, SP, Brazil SUMMARY Study conducted at Unidade Clínica de Emergência, Instituto do Coração (InCor), The inflammation of aortic wall, named aortitis, is a rare condition that can Hospital das Clínicas, Faculdade de be caused by a number of pathologies, mainly inflammatory or infectious in Medicina da Universidade de São Paulo (HC-FMUSP), São Paulo, SP, Brazil nature. In this context, the occurrence of combined audiovestibular and/or ocular manifestations eventually led to the diagnosis of Cogan’s syndrome, Article received: 8/18/2017 Accepted for publication: 9/9/2017 making it the rare case, but susceptible to adequate immunosuppressive treatment and satisfactory disease control. *Correspondence: Address: Av. Dr. Enéas de Carvalho Aguiar, 44 Keywords: chest pain, aortitis, Cogan’s syndrome. São Paulo, SP – Brazil Postal code: 05403-900 [email protected] http://dx.doi.org/10.1590/1806-9282.63.12.1028 INTRODUCTION four years ago the episodes began to intensify. He was Inflammation of the aortic wall, called aortitis, is an in- admitted to another service a week before for the same frequent clinical condition that manifests itself with sys- reason, where he underwent coronary angiography, show- temic symptoms and may cause precordial pain.1-4 One ing no coronary obstruction, and an echocardiogram, of the rheumatologic causes of aortitis is a rare disease which revealed a slight dilatation of the ascending aorta.
  • Guideline-Management-Giant-Cell

    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.
  • A 69-Year-Old Woman with Intermittent Claudication and Elevated ESR

    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.
  • Crohn's Disease

    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
  • Should I Send My Patient with Previous Giant Cell Arteritis for Imaging of The

    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.
  • Noninfectious Ascending Aortitis: Staying Ahead of the Curve

    Noninfectious Ascending Aortitis: Staying Ahead of the Curve

    Editorial Noninfectious Ascending Aortitis: Staying Ahead of the Curve Aortitis is the general name for a spectrum of disorders aortic aneurysms is common with aortitis, the vast majority involving inflammation of the aorta1. The causes of aortitis of aortic aneurysms, both thoracic and abdominal, are due are most easily sorted into either infectious or noninfectious to noninflammatory disease including cystic medial degen- disease. Infections associated with aortitis include syphilis, eration, atherosclerosis, inherited connective tissue dis- Salmonella, Staphylococcus, other bacteria, and mycobacte- eases, or a combination of these processes8. ria, especially M. tuberculosis. Noninfectious causes of aor- The most common serious clinical sequelae of aortitis titis include several forms of primary and secondary are thoracic aortic aneurysms, especially of the ascending large-vessel vasculitis. Giant cell arteritis (GCA) and aorta. The rate of aortic aneurysms among patients with Takayasu’s arteritis are the most common causes of nonin- aortitis is markedly elevated compared to the general popu- fectious aortitis. At least 20% of patients with GCA and lation, especially for ascending thoracic lesions. The reason 50%–70% of patients with Takayasu’s arteritis will develop for this differential tropism for the proximal versus distal changes consistent with aortitis, many of whom develop aorta in aortitis, which is the opposite of what is seen in aortic aneurysms2-7. Aortic involvement is also seen in noninflammatory aortic aneurysms, is not clear but may be relapsing polychondritis, Cogan’s syndrome, and Behçet’s related to differences in the tissue structure, presence of disease, and has been reported as a rare association with vasa vasorum, blood flow dynamics, and other factors9.
  • Thrombosis in Vasculitis: from Pathogenesis to Treatment

    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].
  • Statistical Analysis Plan

    Statistical Analysis Plan

    Cover Page for Statistical Analysis Plan Sponsor name: Novo Nordisk A/S NCT number NCT03061214 Sponsor trial ID: NN9535-4114 Official title of study: SUSTAINTM CHINA - Efficacy and safety of semaglutide once-weekly versus sitagliptin once-daily as add-on to metformin in subjects with type 2 diabetes Document date: 22 August 2019 Semaglutide s.c (Ozempic®) Date: 22 August 2019 Novo Nordisk Trial ID: NN9535-4114 Version: 1.0 CONFIDENTIAL Clinical Trial Report Status: Final Appendix 16.1.9 16.1.9 Documentation of statistical methods List of contents Statistical analysis plan...................................................................................................................... /LQN Statistical documentation................................................................................................................... /LQN Redacted VWDWLVWLFDODQDO\VLVSODQ Includes redaction of personal identifiable information only. Statistical Analysis Plan Date: 28 May 2019 Novo Nordisk Trial ID: NN9535-4114 Version: 1.0 CONFIDENTIAL UTN:U1111-1149-0432 Status: Final EudraCT No.:NA Page: 1 of 30 Statistical Analysis Plan Trial ID: NN9535-4114 Efficacy and safety of semaglutide once-weekly versus sitagliptin once-daily as add-on to metformin in subjects with type 2 diabetes Author Biostatistics Semaglutide s.c. This confidential document is the property of Novo Nordisk. No unpublished information contained herein may be disclosed without prior written approval from Novo Nordisk. Access to this document must be restricted to relevant parties.This
  • Versus Arthritis Vasculitis Information Booklet

    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.
  • An Elevated Igg4 Response in Chronic Infectious Aortitis Is Associated with Aortic Atherosclerosis Zakir Siddiquee1, R Neal Smith1 and James R Stone1,2

    An Elevated Igg4 Response in Chronic Infectious Aortitis Is Associated with Aortic Atherosclerosis Zakir Siddiquee1, R Neal Smith1 and James R Stone1,2

    Modern Pathology (2015) 28, 1428–1434 1428 © 2015 USCAP, Inc All rights reserved 0893-3952/15 $32.00 An elevated IgG4 response in chronic infectious aortitis is associated with aortic atherosclerosis Zakir Siddiquee1, R Neal Smith1 and James R Stone1,2 1Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA and 2Center for Systems Biology, Massachusetts General Hospital, Boston, MA, USA Recently, it was shown that infectious bacterial aortitis can stimulate an elevated IgG4+ plasma cell response in the vessel wall, which could mimic IgG4 aortitis/periaortitis. However, the factors that are associated with an elevated IgG4+ plasma cell response in infectious aortitis are unclear. To ascertain these factors, 17 cases of infectious aortitis and 6 cases of non-infectious severe abdominal aortic atherosclerosis were assessed for the magnitude of IgG4+ plasma cell response. The degree of IgG4+ plasma cell infiltration was determined by immunohistochemistry. Infectious cases were subcharacterized as chronic (43 weeks duration) or acute (o3 weeks duration) based on the duration of symptoms, and as involving either the ascending aorta or the distal aorta, ie, the descending thoracic and/or abdominal aorta. There was a 5–16-fold greater degree of IgG4+ plasma cell infiltration in the chronic distal infectious aortitis group compared with the other three infectious aortitis groups (P ≤ 0.0007), and compared with non-infectious severe abdominal aortic atherosclerosis (Po0.0008). This resulted in a greater IgG4/IgG ratio in the chronic distal infectious aortitis group compared with the acute ascending and acute distal infectious aortitis groups (Po0.03). The degree of IgG4+ plasma cell infiltration in chronic distal infectious aortitis overlaps with that seen in the aortitis and periaortitis of IgG4-related disease.
  • Peripheral Vascular Disease Imaging Guidelines

    Peripheral Vascular Disease Imaging Guidelines

    Cigna Medical Coverage Policies – Radiology Peripheral Vascular Disease (PVD) Imaging Guidelines Effective October 1, 2021 ____________________________________________________________________________________ Instructions for use The following coverage policy applies to health benefit plans administered by Cigna. Coverage policies are intended to provide guidance in interpreting certain standard Cigna benefit plans and are used by medical directors and other health care professionals in making medical necessity and other coverage determinations. Please note the terms of a customer’s particular benefit plan document may differ significantly from the standard benefit plans upon which these coverage policies are based. For example, a customer’s benefit plan document may contain a specific exclusion related to a topic addressed in a coverage policy. In the event of a conflict, a customer’s benefit plan document always supersedes the information in the coverage policy. In the absence of federal or state coverage mandates, benefits are ultimately determined by the terms of the applicable benefit plan document. Coverage determinations in each specific instance require consideration of: 1. The terms of the applicable benefit plan document in effect on the date of service 2. Any applicable laws and regulations 3. Any relevant collateral source materials including coverage policies 4. The specific facts of the particular situation Coverage policies relate exclusively to the administration of health benefit plans. Coverage policies are not recommendations for treatment and should never be used as treatment guidelines. This evidence-based medical coverage policy has been developed by eviCore, Inc. Some information in this coverage policyy m nota ap ply to all benefit plans administered by Cigna. These guidelines include procedures eviCore does not review for Cigna.
  • Giant Cell Arteritis

    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.