Jozef Rovenský and Juraj Payer (eds.) Dictionary of Rheumatology With contributions by Roy B. Clague, Manfred Herold, Milan Bayer, Helena Tauchmannová, Miroslav Ferenčík, Zdenko Killinger SpringerWienNewYork Jozef Rovenský National Institute of Rheumatic Diseases, Piestany, Slovak Republic Juraj Payer 5th Department of Internal Medicine, Medical Faculty of Comenius University Faculty Hospital Bratislava-Ruzinov, Slovak Republic Roy B. Clague – Nobles Hospital, Isle of Man, UK Manfred Herold – Medical University of Innsbruck, Austria Milan Bayer – Faculty of Medicine in Hradec Kralove, Charles University Prague, Czech Republic Helena Tauchmannová – National Institute of Rheumatic Diseases, Piestany, Slovak Republic Miroslav Ferenčík – Institute of Neuroimmunology, Slovak Academy of Sciences and Institute of Immunology, Faculty of Medicine Comenius Universtiy, Bratislava, Slovak Republic Zdenko Killinger – 5th Department of Internal Medicine, Medical Faculty of Comenius University Faculty Hospital Bratislava-Ruzinov, Slovak Republic With financial support by Bundesministerium für Wissenschaft und Forschung in Wien, Austria. Sponsors, who thankfully granted the translation from Slovak into English: Eli Lilly Slovakia s.r.o., Bratislava; Servier Slovensko, spol. s.r.o., Bratislava; Novartis Slovakia s.r.o., Bratislava; Roche Slo- vensko, s.r.o., Bratislava; sanofi-aventis Pharma Slovakia, Bratislava. The printing was sponsored by: Teva Pharmaceutical, Bartislava, Slovakia; Novartis Slovakia s.r.o., Bratislava; Mayor of Piešťany, Slovakia. Parts of the book were translated from Rovenský et al. “Revmatologický výkladový slovník” GRADA Publishing, 2006 and Payer et al. “Lexikón osteoporózy” SAP, 2007. This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifically those of translation, reprinting, re-use of illustrations, broadcasting, reproduction by photocopying machines or similar means, and storage in data banks. Product Liability: The publisher can give no guarantee for all the information contained in this book. This does also refer to information about drug dosage and application thereof. In every individual case the respective user must check its accuracy by consulting other pharmaceutical literature. The use of registered names, trademarks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. This publication is compiled primarily from the publications Revmatologický výkladový slovník, Grada Publi- shing 2006 and Lexikón osteoporózy, Slovak Academic Press, 2007, together with latest findings. Project management, translation and compilation work: Language Sense Ltd. (John Boyd), Bratislava © 2009 Springer-Verlag/Wien Printed in Germany SpringerWienNewYork is a part of Springer Science + Business Media springer.at Typesetting: Composition & Design Services, Minsk 220027, Belarus Printing: Strauss GmbH, 69509 Mörlenbach, Germany Printed on acid-free and chlorine-free bleached paper SPIN: 11953708 Library of Congress Control Number: 2008940520 ISBN 978-3-211-68584-6 SpringerWienNewYork Preface This dictionary of rheumatology has tionary, we have taken into account the been prepared as a quick reference source fact that an integral part of prevention for the clinical, diagnostic and therapeu- and treatment of rheumatological dis- tic aspects of rheumatic disorders and eases includes rehabilitation. We there- related immunology. Rheumatology is a fore provided guidelines on how to pre- well-recognised specialty of medicine. vent the onset of functional damage and Recently, due to many factors (the envi- its progression towards increased dis- ronment, new viral diseases, genetics, ability. We believe this dictionary will increased life expectancy, improved di- serve not only rheumatologists, but also agnostic tests) an increase in the inci- the many related specialists, trainee doc- dence and prevalence of rheumatological tors, health professionals and nurses in- disorders has been witnessed. Rheuma- volved in the management of patients tology has close relationships with a num- with disorders of the human musculo- ber of medical specialties and health pro- skeletal system. fessionals, such as orthopaedic surgery, sports medicine, neurology, immunology, The early diagnosis and treatment of osteoporosis as well as clinical rheumatol- rheumatological disorders can help to ogy nurses, physiotherapists and occupa- improve their prognosis and we hope tional therapists, so we have endeavoured that this modest monograph will con- to collate some basic knowledge from tribute to a successful therapeutic man- these fields. When compiling this dic- agement. Jozef Rovenský On behalf of the collective authors A α1-antitrypsin A serum glycoprotein in- cellular processes that exert their action hibiting proteolytic enzymes, such as trypsin, mainly in blood coagulation, fibrinolysis and chymotrypsin and elastase. It also acts as an inflammation. α2M and protease complexes acute-phase protein. Its serum level rises in are proteolytically inactive and are eliminated inflammatory diseases. The coding gene is quickly (in minutes) from the circulation. Its located on the 14th chromosome, where it serum levels are increased especially in neph- can occur in form of 25 alleles. Some of them rotic syndrome, atopic dermatitis, diabetes code for physiological products (PiMM phe- mellitus and ataxia-telangiectasia. notype), while others are related to patho- logical states, e.g. PiZZ phenotype, which is Abatacept Abatacept (Orencia®) is an in- often associated with emphysema, cirrhosis, jectable, synthetic (man-made) soluble fusion and cholelithiasis, where its serum levels are protein that consists of the extracellular do- diminished (α1-antitrypsin deficiency). main of human cytotoxic T-lymphocyte-as- sociated antigen 4 (CTLA-4) linked to the α-fetoprotein An oncofetal antigen that modified Fc portion of human immunoglob- can be found in small concentrations in nor- ulin G1 (IgG1). Abatacept is produced by re- mal human serum. Its level is high in the fetal combinant DNA technology in a mammalian serum, where presumably thanks to its im- cell expression system. munosuppressive effect, it participates in Abatacept belongs to a new class of drugs neonatal immunological tolerance. The called costimulation modulators, shown to α-fetoprotein level is also increased in sera of inhibit T cell activation by binding to CD80 pregnant women when fetal development is and CD86, thereby blocking interaction with defective (central nervous system defects, im- CD28. Blockade of this interaction has been munodeficiency syndromes, gastrointestinal shown to inhibit the autoimmune T-Cell acti- or other abnormalities). An increased serum vation that has been implicated in the patho- level can be found in patients with certain genesis of rheumatoid arthritis Abatacept at- neoplastic disorders, especially hepatic can- taches to a protein on the surface of T-lym- cer and can be used as a marker of hepatocel- phocytes and blocks both the production of lular carcinoma. new T-lymphocytes and the production of the chemicals that destroy tissue and cause α1-microglobulin (α1M) A protein syn- the symptoms and signs of arthritis. Abata- thesised in the liver and present in blood, se- cept slows the damage to joints and cartilage rum and urine. Complexes of α1M with mo- and relieves the symptoms and signs of ar- nomeric immunoglobulin A (IgA) participate thritis. in renal IgA nephropathy where the serum Abatacept is indicated for reducing signs level of α2M is also usually increased. and symptoms, inducing major clinical re- sponse, slowing the progression of structural α2-macroglobulin (α2M) A serum glyco- damage, and improving physical function in protein working as inhibitor of a number of adult patients with moderately to severely ac- proteases including thrombin, plasmin, kal- tive rheumatoid arthritis who have had an in- likrein, trypsin, chymotrypsin, elastase, col- adequate response to one or more DMARDs, lagenase and cathepsin B and G. α2M is pro- such as methotrexate or TNF antagonists. duced mainly by macrophages and regulates Abatacept may be used as monotherapy or the proteolytic balance in a number of extra- concomitantly with DMARDs other than abduction 2 A TNF antagonists, but has not yet been ap- neus, separated from it by the Achilles bursa. proved by NICE. Tendonitis can be part of the clinical picture Approval of abatacept was supported by of spondyloarthritis, especially ankylosing five randomized, double-blind, placebo-con- spondylitis. It can remain thickened (by fi- trolled clinical trials. In all five studies, sub- brosis) after the inflammation has subsided jects received treatments with abatacept or and nodules may be palpable. placebo at weeks 0, 2, and 4, then every 4 weeks thereafter. Studies have found that Achillodynia Pain of the Achilles tendon, abatacept can reduce the signs and symptoms especially of its insertion, most frequently af- of rheumatoid arthritis. It can also reverse ter a trauma or sporting overload. some signs of joint damage. Abatacept/MTX slowed the progression of structural damage Achondroplasia and hypochondro- compared to placebo/MTX alone (Furst et al. plasia An autosomal dominant hereditary 2007). syndrome characterized by a small stature Abatacept is infused over 30 minutes. The with short extremities, in most cases