DOCSLIB.ORG

Duke-Elder Lecture Retinal Arteritis, Retinal Vasculitis and Autoimmune

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Duke-Elder Lecture Retinal Arteritis, Retinal Vasculitis and Autoimmune Eye (1987) 1,441-465 Duke-Elder Lecture Retinal Arteritis, Retinal Vasculitis and Autoimmune Retinal Vasculitis M. D. SANDERS London I would like to thank the President and directorship of Professor Arnold Sorsby. This Council for honouring me with the task of Unit was subsequently transferred to St delivering the 4th Duke-Elder Lecture, and Thomas' with the Royal Eye Hospital in 1973. enabling me to pay tribute to one of the most Staffed by physicians, neurologists and oph­ distinguished British Ophthalmologists of this thalmologists, the Unit has provided a base century. for itensive in-patient investigation of compli­ Born near Dundee in 1898, Sir Stewart cated medical and neuro-ophthalmic prob­ Duke-Elder graduated from St Andrew's lems. Secondly, he was instrumental in this University with a BSc and special distinction author obtaining the Alexander Piggott in physiology. After medical training in Dun­ Warner Memorial Fellowship to study at the dee and Edinburgh he graduated, and like University of California, San Francisco, in many of his compatriots took the high road 1967-1968 under Professor W. F. Hoyt. south to London, where he was fortunate in On joining the staff of the Medical Eye Unit gaining the close friendship of one of at St Thomas' Hospital, it became clear to me London's most illustrious scientific ophthal­ that 'Retinal Vasculitis' was a major cause of mologists, Sir Herbert Parsons. His career visual morbidity and occurred particularly in progressed after appointments at Moorfields young people. In America, 10 per cent of Eye Hospital and St George's Hospital, to patients with severe visual handicap were attain the highest accolades available in his noted to have inflammatory eye disease.l In own land, and his international reputation many cases retinal changes occurred in the similarly grew as he received honours abroad. context of a systemic disease and the ocular His phenomenal ability to condense, con­ changes provided specific diagnostic clues to centrate and clarify, enabled him to write the the systemic disease. Retinal veins were pre­ greatest textbook of ophthalmology ever pro­ dominantly involved, although in some condi­ duced. His broad scientific scholarship tions the arteries appeared to bear the brunt enabled him to initiate and direct the Institute of the disease. Complex immunological of Ophthalmology in London, which he investigations were performed in addition to expanded to become an institute of world full ophthalmic examination so that the renown. natural history of the disease has been Sir Stewart had some influence on the observed over a period of 10 years. This paper nature of this lecture by two actions. In 1954 will therefore present clinical and immu­ he supported the establishment of a Medical nological information on 150 cases of retinal Eye Unit at the Royal Eye Hospital under the vasculitis seen at St Thomas' between 1975 From: National Hospital for Nervous Diseases and St Thomas' Hospital, London. Correspondence to: Mr. M. D. Sanders, FRCP, FRCS, Consultant Ophthalmologist, National Hospital for Nervous Diseases, Queen Square, London WCI. Presented at the Annual Congress of the Ophthalmological Society of the United Kingdom, April 1987. 442 M. D. SANDERS and 1985 which have been subdivided into later related to both arthritic and scrofular those with an associated systemic inflamma­ conditions.ll The advent of the ophthalmo­ tory disease and those with isolated 'Auto­ scope allowed advances in the diagnosis of immune Retinal Vasculitis'. All cases were retinal and choroidal conditions and deemed to have immune mediated vasculitis choroiditis became recognised.12 Combined and in all cases inflammatory causes were inflammation in the iris and choroid was actively excluded. The role of auto-immunity termed uveitis, which relates to the uvea· or has been established clinically and by the grape-like coat of the eye, due to its similarity development of an experimental animal to a black grape. Improved examination of the model which has enabled us to make immu­ eye by the slit lamp and indirect ophthalmo­ nological, neurophysiological, pathological scopy resulted in the recognition of peripheral and therapeutic observation. uveitis. Inflammation of the ciliary body has been Historical Aspects termed peripheral uveitis, 13 pars planitis14 and The concept of inflammationof the veins was more recently intermediate uveitis. firstestablished by another outstanding Scots­ Some revision of this classifiation now man in 1784, when John Hunter first pub­ seems appropriate with the realisation that lished a paper on 'Observations on the the retina contains many powerful antigens, Inflammation of the Internal Coats of the and may be more immunologically active than Veins'.2 Inflammation of the retinal vessels the uvea. The recent availability of monoclo­ was noted over 100 years later in pathological nal antibodies and the detection of retinal specimens by Parsons. 3 In 1890 a Birmingham antibodies should allow a more precise cate­ ophthalmologist, Henry Eales4 described 5 gorisation of ocular inflammatory disease. It cases of young men suffering from con­ does appear that retinal vasculitis emerges as stipation, epistaxis and associated retinal a distinct clinical entity though the primary haemorrhages. Though a high arterial tension immunological event may be directed against was described, in no patient was the sphyg­ constituents of the photoreceptor cells. The momanometer used. A tuberculous origin retina is presented to the immune system by was postulated by Axenfeld and Stock5 and it the retinal vessels and thus the predominant is probable that this was the cause of the con­ clinical and inflammatoryevents occur in rela­ dition Eales described though he obtained no tion to these vessels. Many of the conditions aetiological information in any of his cases. producing retinal disease have been pre­ The association of inflammatory changes in viously termed uveitis, though the author the retinal vessels with uveitis and systemic agrees with Maumenee15 who stated that diseases became well-recognised,6.7.s but a inflammation of the uvea as a primary event is strict nosological entity was not established. A rare and the term uveitis as generally applied form of central 'Retinal Vasculitis' was is inappropriate. described by Lyle and Wybar9 but evidence for an inflammatory basis has never been Vasculitis--The Clinical Spectrum established and Hart and otherslO considered Vasculitis is a clinico-pathological process this an haematological disorder, though characterised by inflammationand necrosis of extensive investigations have failed to elicit blood vessels. In some diseases vasculitis may the initiating cause. be a major feature, whereas in other diseases it occurs as a secondary phenomenon. Vessels Terminological Aspects of any size, and situated in any organ may be Confusion has persisted over the terminology involved. An association either direct or of inflammatory conditions of the eye. Many indirect can be established with immuno­ terms evolved on anatomical grounds without pathogenic mechanisms and immune com­ sufficient clinical, experimental pr path­ plexes mediation is being increasingly ological information being available. Iritis recognised as the underlying mechanism.16 was recognised by the ancients as an inflam­ The prevailing theory relates to primary or mation of the anterior portion of the eye and secondary deposition of immune complexes in DUKE-ELDER LECTURE 443 blood vessel walls, and elegant animal models titude of different causes. support these hypotheses.17 Similarly when the kidney is involved there Necrotising vasculitis was firstdescribed by is the potential for histological examination Kussmaul and Maier in 186618 in a 27 year old and evidence has accrued that DNA related man with classic periarteritis nodosa. antibodies have a predeliction for deposition Systemic vasculitis is recognised as an impor­ in the kidney. tant feature of several of the connective tissue Thus there is a wide spectrum of vasculitis or collagen vascular diseases such as rheu­ in the body, and the ophthalmologist must be matoid arthritis and detectable immune aware of some of these conditions in order to system abnormalities such as hypergam­ provide diagnostic advice. However, the reti­ maglobulinaemia, rheumatoid factor, nal vessels are unique in providing a substrate cryoglobulinaemia, circulating immune com­ in which a microcirculation may be studied plexes and hypocomplementaemia also testify with great precision. The ophthalmologist to an immunological aberration. therefore has a major role in clarifying the A relationship with viral induced disease nosological and diagnostic debate in patients such as hepatitis B, antigen-associated nec­ with vasculitis. rotising vasculitis, or to drug induced hyper­ The aim of this lecture is to concentrate the sensitivity reactions raises the question of attentions of ophthalmologists on the retina multiple triggering mechanisms. and the retinal vessels as being 'a priori' source of immunopathogenic activity and to Polyarteritis Classic escape from the conventional view that nodosa Allergic granulomatosis inflammatory ocular disease is labelled under Systemic necrotising the all encompassing blanket term 'uveitis'. Hypersensitivity Churg Strauss Syndrome Retinal vasculitis should be considered Vasculitis Henoch Schonlein when major inflammatory changes occur in purpura relation to the retinal vessels, often with Essential mixed accompanying
Load more

Recommended publications
  • Old and New Challenges in Uveitis Associated with Behçet's Disease
    Journal of Clinical Medicine Review Old and New Challenges in Uveitis Associated with Behçet’s Disease Julie Gueudry 1,* , Mathilde Leclercq 2, David Saadoun 3,4,5 and Bahram Bodaghi 6 1 Department of Ophthalmology, Hôpital Charles Nicolle, F-76000 Rouen, France 2 Department of Internal Medicine, Hôpital Charles Nicolle, F-76000 Rouen, France; [email protected] 3 Department of Internal Medicine and Clinical Immunology, AP-HP, Centre National de Références Maladies Autoimmunes et Systémiques Rares et Maladies Autoinflammatoires Rares, Groupe Hospitalier Pitié-Salpêtrière, F-75013 Paris, France; [email protected] 4 Sorbonne Universités, UPMC Univ Paris 06, INSERM, UMR S 959, Immunology-Immunopathology-Immunotherapy (I3), F-75005 Paris, France 5 Biotherapy (CIC-BTi), Hôpital Pitié-Salpêtrière, AP-HP, F-75651 Paris, France 6 Department of Ophthalmology, IHU FOReSIGHT, Sorbonne-AP-HP, Groupe Hospitalier Pitié-Salpêtrière, F-75013 Paris, France; [email protected] * Correspondence: [email protected]; Tel.: +33-2-32-88-80-57 Abstract: Behçet’s disease (BD) is a systemic vasculitis disease of unknown origin occurring in young people, which can be venous, arterial or both, classically occlusive. Ocular involvement is particularly frequent and severe; vascular occlusion secondary to retinal vasculitis may lead to rapid and severe loss of vision. Biologics have transformed the management of intraocular inflammation. However, the diagnosis of BD is still a major challenge. In the absence of a reliable biological marker, diagnosis is based on clinical diagnostic criteria and may be delayed after the appearance of the onset sign. However, therapeutic management of BD needs to be introduced early in order to control inflammation, to preserve visual function and to limit irreversible structural damage.
    [Show full text]
  • Hereditary Hemorrhagic Telangiectasia: Diagnosis and Management From
    REVIEW ARTICLE Hereditary hemorrhagic telangiectasia: Ferrata Storti diagnosis and management from Foundation the hematologist’s perspective Athena Kritharis,1 Hanny Al-Samkari2 and David J Kuter2 1Division of Blood Disorders, Rutgers Cancer Institute of New Jersey, New Brunswick, NJ and 2Hematology Division, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA ABSTRACT Haematologica 2018 Volume 103(9):1433-1443 ereditary hemorrhagic telangiectasia (HHT), also known as Osler- Weber-Rendu syndrome, is an autosomal dominant disorder that Hcauses abnormal blood vessel formation. The diagnosis of hered- itary hemorrhagic telangiectasia is clinical, based on the Curaçao criteria. Genetic mutations that have been identified include ENG, ACVRL1/ALK1, and MADH4/SMAD4, among others. Patients with HHT may have telangiectasias and arteriovenous malformations in various organs and suffer from many complications including bleeding, anemia, iron deficiency, and high-output heart failure. Families with the same mutation exhibit considerable phenotypic variation. Optimal treatment is best delivered via a multidisciplinary approach with appropriate diag- nosis, screening and local and/or systemic management of lesions. Antiangiogenic agents such as bevacizumab have emerged as a promis- ing systemic therapy in reducing bleeding complications but are not cur- ative. Other pharmacological agents include iron supplementation, antifibrinolytics and hormonal treatment. This review discusses the biol- ogy of HHT, management issues that face
    [Show full text]
  • Relapsing Polychondritis
    Relapsing polychondritis Author: Professor Alexandros A. Drosos1 Creation Date: November 2001 Update: October 2004 Scientific Editor: Professor Haralampos M. Moutsopoulos 1Department of Internal Medicine, Section of Rheumatology, Medical School, University of Ioannina, 451 10 Ioannina, GREECE. [email protected] Abstract Keywords Disease name and synonyms Diagnostic criteria / Definition Differential diagnosis Prevalence Laboratory findings Prognosis Management Etiology Genetic findings Diagnostic methods Genetic counseling Unresolved questions References Abstract Relapsing polychondritis (RP) is a multisystem inflammatory disease of unknown etiology affecting the cartilage. It is characterized by recurrent episodes of inflammation affecting the cartilaginous structures, resulting in tissue damage and tissue destruction. All types of cartilage may be involved. Chondritis of auricular, nasal, tracheal cartilage predominates in this disease, suggesting response to tissue-specific antigens such as collagen II and cartilage matrix protein (matrillin-1). The patients present with a wide spectrum of clinical symptoms and signs that often raise major diagnostic dilemmas. In about one third of patients, RP is associated with vasculitis and autoimmune rheumatic diseases. The most commonly reported types of vasculitis range from isolated cutaneous leucocytoclastic vasculitis to systemic polyangiitis. Vessels of all sizes may be affected and large-vessel vasculitis is a well-recognized and potentially fatal complication. The second most commonly associated disorder is autoimmune rheumatic diseases mainly rheumatoid arthritis and systemic lupus erythematosus . Other disorders associated with RP are hematological malignant diseases, gastrointestinal disorders, endocrine diseases and others. Relapsing polychondritis is generally a progressive disease. The majority of the patients experience intermittent or fluctuant inflammatory manifestations. In Rochester (Minnesota), the estimated annual incidence rate was 3.5/million.
    [Show full text]
  • 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]
  • Birdshot Chorioretinopathy: Clinical Characteristics and Evolution*
    Br J Ophthalmol: first published as 10.1136/bjo.72.9.646 on 1 September 1988. Downloaded from British Journal of Ophthalmology, 1988, 72, 646-659 Birdshot chorioretinopathy: clinical characteristics and evolution* HILDE A PRIEM'2 AND JENDO A OOSTERHUIS2 From the 'Eye Clinic, University ofGent, Belgium, and the 2Eye Clinic, University ofLeiden, The Netherlands SUMMARY During the period 1980-6 102 patients from 14 European eye clinics were diagnosed as having birdshot chorioretinopathy (BSCR). All were Caucasian, and the series consisted of 47 men and 55 women, with a mean age of 52*5 years. The major findings in this rare disorder concern the ocular fundus. Most marked are the patterned distribution of depigmented spots without hyperpigmentation, radiation from the optic disc in association with vitritis, retinal vasculopathy with frequent cystoid macular oedema, and involvement of the optic nerve head. The distribution and appearance of the lesions suggest that they are related to the major choroidal veins. Complications of the disease were epiretinal membranes, retinal neovascularisation, recurrent vitreous haemorrhage, subretinal neovascular membranes occurring both in the juxtapapillary and macular regions, and optic atrophy. The medical history was not contributory. HLA testing showed very strong disease association with HLA A29 (95.8%). The evidence suggests that it is a single disease a entity rather than group of disorders because of the remarkable similarity in the copyright. ophthalmological appearance and the clinical course, combined with the exceptionally high association with HLA A29. In 1980 a rare ocular disease was described by Ryan and Maumenee.' In the absence of a known aetiology, they chose the descriptive name 'birdshot http://bjo.bmj.com/ retinochoroidopathy' inspired by the unusual picture, which showed multiple small, cream coloured lesions, scattered mainly around the optic disc and radiating towards the equator, without hyperpigmentation at the level of the retinal pigment _ * epithelium.
    [Show full text]
  • Relapsing Polychondritis Jozef Rovenský1* and Marie Sedláčková2
    Rovenský et al. J Rheum Dis Treat 2016, 2:043 Volume 2 | Issue 4 Journal of ISSN: 2469-5726 Rheumatic Diseases and Treatment Review Article: Open Access Relapsing Polychondritis Jozef Rovenský1* and Marie Sedláčková2 1National Institute of Rheumatic Diseases, Piešťany, Slovak Republic 2Department of Rheumatology and Rehabilitation, Thomayer Hospital, Prague 4, Czech Republic *Corresponding author: Jozef Rovenský, National Institute of Rheumatic Diseases, Piešťany, Slovak Republic, E-mail: [email protected] of patients; in the systemic vasculitis subgroup survival is similar to Abstract that of patients with polyarteritis (up to about five years in 45% of Relapsing polychondritis (RP) is a rare immune-mediated disease patients). The period of survival is reduced mainly due to infection that may affect multiple organs. It is characterised by recurrent and respiratory compromise. episodes of inflammation of cartilaginous structures and other connective tissues, rich in glycosaminoglycan. Clinical symptoms Etiology and Pathogenesis concentrate in auricles, nose, larynx, upper airways, joints, heart, blood vessels, inner ear, cornea and sclera. The most prominent RP manifestation is inflammation of cartilaginous structures resulting in their destruction and fibrosis. Diagnosis of the disease is based on the Minnesota diagnostic criteria of 1986 and RP has to be suspected when the inflammatory It is characterised by a dense inflammatory infiltrate, composed bouts involve at least two of the typical sites - auricular, nasal, of neutrophil leukocytes, lymphocytes, macrophages and plasma laryngo-tracheal or one of the typical sites and two other - ocular, cells. At the onset, the disease affects only the perichondral area, statoacoustic disturbances (hearing loss and/or vertigo) and the inflammatory process gradually leads to loss of proteoglycans, arthritis.
    [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]
  • Generalized Essential Telangiectasia in a Patient with Graves
    Generalized Essential Telangiectasia in a Patient with Graves’ Disease: Should the Spectrum of Autoimmune Diseases Associated with Generalized Telangiectasia be Expanded? LCDR Ronald Buckley, MC, USN, Bethesda, Maryland COL Kathleen J. Smith, MC, USA, Bethesda, Maryland Henry G. Skelton, MD, Hurndon, Virginia Generalized essential telangiectasia (GET), as orig- rhagic telangiectasia, metastatic carcinoid syndrome, inally described, is not associated with any under- angiokeratoma corporis diffusum, ataxia-telangiecta- lying disease. Although patients with GET lack the sia, portal cirrhosis, and congenital dysplastic an- typical periungual telangiectases associated with giopathies.1 In addition, an entity known as general- autoimmune collagen vascular diseases, these pa- ized essential telangiectasia (GET) has been tients may have an underlying autoimmune described without associated disease.2 process. We present a patient with a history of The majority of the patients with GET are female, Graves’ disease and low-titer anti-nuclear antibod- with onset usually around the fourth decade of life.2 ies, who developed rapidly progressive general- In general, telangiectatic vessels first appear on the ized telangiectases. The gender and age of the ma- lower extremities, and over a few years to decades, jority of patients with GET fit well within the there is progressively more diffuse skin involvement.2-6 demographics of most autoimmune diseases. The Although lack of an association with systemic au- documented occurrence of an autoimmune disease toimmune disease was a criterion for this diagnosis in several of the limited number of patients previ- in the original report, our patient, as well as some ously diagnosed with GET provides additional evi- others who carry this diagnosis, have a documented dence that GET may be associated with an under- or probable underlying autoimmune disease.
    [Show full text]
  • Skin Manifestations of Systemic Disease
    THEME WEIRD SKIN STUFF Adriene Lee BSc(Med), MBBS(Hons), FACD, is visiting dermatologist, St Vincent's Hospital and Monash Medical Centre, and Lecturer, Department of General Practice, Monash University, Victoria. [email protected] Skin manifestations of systemic disease Dermatologic complaints are a common reason for Background presentation to a general practitioner. In such cases, one needs Dermatologic complaints are a common reason for presentation to determine if the complaint may be a manifestation of a more to a general practitioner. In some cases, one needs to determine serious underlying systemic disease. Disorders of the every if the complaint may be a manifestation of a more serious underlying systemic disease. organ system may cause skin symptoms and signs, some of which are due to treatment of these conditions. It is beyond the Objective scope of this review to cover all potential skin manifestations of This article aims to highlight common dermatologic systemic disease. This article highlights the more common, presentations where further assessment is needed to exclude classic and important manifestations in three different groups: an underlying systemic disease, to discuss classic cutaneous features of specific systemic diseases, and to outline rare • ‘When to look further’ – where dermatologic presentations cutaneous paraneoplastic syndromes. require further assessment to exclude underlying systemic disease, and guide appropriate management Discussion • ‘What to look for’ – where certain systemic diseases have Skin manifestations of systemic disease are wide, varied, classic cutaneous findings specific and nonspecific. Generalised pruritus and cutaneous • ‘What not to miss’ – where specific cutaneous signs might be vasculitis are more common cutaneous presentations where an underlying systemic disease may be present and will the initial presentation of an underlying malignancy.
    [Show full text]