Systemic Vasculitis Hendaoui, Lotfi; Bouhaouala, M. Habib; Joffre, Francis ...
2011, 20111012, 2011-02-01
eBook
The prompt diagnosis of systemic vasculitis is essential as a missed diagnosis can be disastrous. Imaging is of vital importance in achieving a correct diagnosis and in some cases also plays a role ...in endovascular treatment. In this book, the imaging features of the many different types of vasculitis are clearly demonstrated by means of numerous high-quality illustrations. All relevant imaging modalities are considered, and key distinctive characteristics are highlighted. In addition, each chapter discusses the etiology, epidemiology, pathogenesis, clinical presentation, biology, and treatment of the vasculitis in question. This book is the result of cooperation between expert teams from a range of countries. The wealth of illustrations and informative clinical case studies will prove invaluable for all who may be confronted with these problematic disorders.
The third edition of this definitive and highly-regarded reference work provides a comprehensive review of vasculitis, a fascinating array of life-threatening and minor conditions caused by ...inflammatory syndromes and diseases that affect the blood vessels. The text uniquely brings together concepts from both the biological and clinical aspects of vasculitis. Research in clinical immunology now invigorates the entire area of vasculitis and shapes a rational approach to pathogenesis,diagnosis, and treatment, which is the substance of this text. Over 40 chapters cover known vasculitic conditions, and are illustrated with over 250 full-colour photographs of clinical and pathologic findings, diagrams, and tables. Separate sections are devoted to basic science, clinicalmanifestations commonly seen in vasculitis, imaging and percutaneous interventions, individual diseases and syndromes, as well as conditions which can mimic vasculitis. Many of the conditions are rare, and it is the exceptional clinician who has much experience with more than a few of these; thus, the invaluable experience of the international team of authors makes this edition truly indispensible.Now in the Oxford Textbooks in Rheumatology series, this new edition of Vasculitis is published with a concurrent online version, which features access to the full content of the textbook, contains links from the references to primary research journal articles, allows full text searches, and provides access to figures and tables that can be downloaded to PowerPoint.This volume is the definitive reference for rheumatologists, clinical immunologists, and general internists, and will also be of interest to dermatologists, gastroenterologists, cardiologists, pulmonologists, nephrologists, neurologists, pathologists, vascular surgeons, and pediatricians.
Uvod. IgA vaskulitis (IgAV ) najčešći je sistemski vaskulitis dječje dobi. U više od polovice bolesnika s IgAV -om dolazi do zahvaćanja gastrointestinalnog sustava najčešće u vidu bolova u trbuhu, ...mučnine i povraćanja, a u do 5% bolesnika mogu se razviti ozbiljne komplikacije poput intususcepcije, perforacije crijeva i/ili akutnog krvarenja. Cilj istraživanja bio je utvrditi kliničke i laboratorijske značajke zahvaćenosti gastrointestinalnog sustava u IgAV -u. Materijali i metode. Retrospektivna analiza podataka bolesnika s IgAV -om, dijagnosticiranih i liječenih u Referentnom centru za pedijatrijsku i adolescentnu reumatologiju Ministarstva zdravstva RH u razdoblju od 2009. do 2020. godine, koji su imali zahvaćen gastrointestinalni sustav. Razlike između kategorijskih varijabla ispitane su pomoću χ2 testa, a one između numeričkih Mann-Whitneyevim U-testom. Rezultati. IgAV je dijagnosticiran u 216 bolesnika, od toga 116 dječaka i 100 djevojčica. Gastrointestinalni sustav bio je zahvaćen u 94 bolesnika (43,5%), raspon dobi u trenutku dijagnoze bio je 6,75 (5,2–9) godina, a omjer M:Ž 1,68:1. Najčešći klinički znak zahvaćanja gastrointestinalnog sustava u IgA vaskulitisu bila je bol u trbuhu koju je imalo 45 bolesnika (47,9%). Bolovi u trbuhu bili su najčešće locirani periumbilikalno (62,5%). Jedan bolesnik razvio je ileokoličnu invaginaciju crijeva. Učestalost generaliziranoga purpuričnog osipa (p=0,023) i pojave nefritisa (p=0,001) bila je veća u bolesnika sa zahvaćenim gastrointestinalnim sustavom u usporedbi sa skupinom bolesnika bez zahvaćenog gastrointestinalnog sustava. Ta skupina bolesnika imala je statistički
značajno veći broj leukocita (p=0,021) te niže vrijednosti sedimentacije eritrocita (p=0,039) i ukupnih proteina (p=0,002). Bolesnici sa zahvaćenim gastrointestinalnim sustavom češće su bili muškog spola (p=0,019), imali su dulje trajanje hospitalizacije (p<0,001) i veću učestalost relapsa (p=0,011). Zaključak. Zaključno, gastrointestinalni simptomi u IgAV -u najčešće su samoograničavajući, a komplikacije rijetke. Uočili smo da su bolesnici s gastrointestinalnim simptomima IgA vaskulitisa češće muškog spola, imaju duže trajanje hospitalizacije te veću pojavu nefritisa i relapsa.
Th1-type and Th2-type cytokine profiles and adhesion molecules in the serum of patients suffering from systemic lupus erythematosus and the cytokine production by peripheral blood mononuclear cells ...(PBMC) were studied. Tumor necrosis factor-alpha (TNF-α), interferongamma (IFN-γ), interleukin-1β (IL-1β), IL-4, IL-10, IL-13, intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1) were measured using ELISA technique in the sera of 16 systemic lupus erythematosus patients without vasculitis (SLE), 30 SLE patients with vasculitis (LV), and in 18 healthy controls. The cytokines were also measured in the culture media of unstimulated, concana valin-A (Con-A) and phorbol-12-myristate-13-acetate (PMA) stimulated PBMC. TNF-α serum levels were significantly elevated in both SLE and LV patients and those of IL-1β in SLE patients. TNF-α was also significantly increased in SLE compared to LV patients. Serum levels of all three Th-2 cytokines were significantly elevated in both SLE and LV patients compared to healthy controls. Serum IFN-γ and Th2 cytokine levels were significantly increased in patients with more active disease. Both ICAM-1 and VCAM-1 were significantly increased in SLE patients and only VCAM-1 in LV patients. ICAM-1 showed a significant correlation with IL-1β, IFN-γ, IL-4 and IL-10 in both patient groups. In the SLE group VCAM-1 correlated significantly only with ICAM-1, but in the LV group only with IL-1β and IFN-γ. Compared to healthy controls, basal TNF-α and IL-4 production by unstimulated PBMC derived from SLE patients were significantly increased. Con-A-stimulated PBMC of both SLE groups produced significantly more IFN-γ, IL-4 and IL-13 than Con-A-stimulated control cells. Con-A-stimulated cells derived from LV patients produced much more INF-γ than cells from SLE patients. PMA strongly stimulated INFγ, TNFα and IL-13 production by cells derived from both SLE groups but had no effect on IL-4 production. In addition, it had little if any effect on the production of INFγ and IL-13 by PBMC derived from healthy donors. These findings suggest that the altered pattern of cytokine production by PBMC may play an important role in the SLE pathophysiology, accounting for differences in the clinical expression of the disease. The differences in adhesion molecules production and their correlation with cytokines suggest ICAM-1 and VCAM-1 as useful markers in SLE patients stratification.
U serumu bolesnika sa sistemskim lupusom eritematodesom određivani su Th1 i Th2 citokini i adhezioni molekuli a u kulturi mononuklearnih ćelija periferne krvi (PBMC) produkcija citokina. Faktor tumorske nekroze-alfa (TNF-α), interferon-gama (IFN-γ), interleukin-1b (IL-1β), IL-4, IL-10, IL-13, interćelijski adhezioni molekul-1 (ICAM-1) i vaskularni ćelijski adhezioni molekul-1 (VCAM-1) mereni su ELISA tehnikom u serumu 16 bolesnika sa sistemskim lupusom eritematodesom bez vaskulita (SLE), 30 bolesnika sa SLE koji su imali vaskulitis i 18 zdravih kontrola. Citokini su, takođe, određivani u kulturi nestimulisanih kao i konkanavalinom-A (Con-A) i forbol-12-miristat-13-acetatom (PMA) stimulisanih PBMC. Serumski nivoi TNF-α su značajno povišeni kod obe grupe bolesnika, dok je IL-1β povišen samo u grupi sa SLE. Serumski TNF-α je, takođe, značajno viši kod bolesnika sa SLE u poređenju sa LV grupom. Sva tri Th2 citokina su značajno viša kod obe grupe bolesnika u poređenju sa zdravim ispitanicima. Nivoi IFN-γ i Th2 citokina značajno su viši kod bolesnika sa težim oblikom bolesti. Kod bolesnika sa SLE značajno su povišene koncentracije oba adheziona molekula, dok je kod bolesnika sa LV povišen samo VCAM-1. Kod obe grupe bolesnika postoji značajna korelacija ICAM-1 i IL-1β, IFN-γ, IL-4 i IL-10. U grupi sa SLE VCAM-1 značajno korelira samo sa ICAM-1, a u grupi sa LV sa IL-1β i IFN-γ. U poređenju sa kontrolama, bazalna produkcija TNF-a i IL-4 od strane nestimulisanih PBMC bolesnika sa SLE je značajno povišena. Konkanavalinom-A stimulisane PBMC obe grupe bolesnika proizvodile su značajno više IFN-γ, IL-4 i IL-13 nego Con-A stimulisane ćelije kontrolne grupe. Konkanavalinom-A stimulisane ćelije bolesnika sa LV produkovale su mnogo više IFN-γ nego ćelije bolesnika sa SLE. U ćelijama obe grupe bolesnika PMA je izazvao jaku stimulaciju produkcije IFN-γ, TNF-α i IL-13, ali nije pokazao uticaj na produkciju IL-4. Isti stimulator pokazao je mali ili nikakav efekat na produkciju IFN-g i IL-13 u PBMC zdravih osoba. Ovi nalazi sugerišu da poremećeni profil produkcije citokina u PBMC može imati značajnu ulogu u patofiziologiji SLE, čime se mogu objasniti razlike u kliničkom ispoljavanju bolesti. Razlike u produkciji adhezionih molekula i njihovoj korelaciji sa citokinima sugerišu da ICAM-1 i VCAM-1 mogu biti korisni markeri u stratifikaciji bolesnika sa SLE.
Seit der Entdeckung der ANCA vor 30 Jahren haben sich die Testmethoden weiter verfeinert. Aktuellen Standards ist die Testung in der Indirekten Immunfluoreszenz (IFT) gefolgt von ...Antigen-spezifischen-Tests. Diese konnten in ihrer Sensitivität und Spezifität weiter deutlich gesteigert werden, insbesondere durch den Einsatz von “capture” und “anchor” ELISA-Techniken, wobei die Qualität der verfügbaren kommerziellen Testsysteme gemessen an internationalen Standards z.T. unzureichend ist. Ihr alleiniger Einsatz unter Verzicht auf die IFT wird weiter nicht empfohlen. Proteinase3- und Myeloperoxidase-ANCA haben die größte gesicherte diagnostische Bedeutung für die ANCA-assoziierten Vaskulitiden. Der Steigerung der Sensitivität und Spezifität steht ein zunehmend ungezielter klinischer Einsatz gegenüber, der, bei sehr niedriger Prävalenz der infrage stehenden Erkrankungen, zu einer dennoch hohen Rate an “falsch” positiven Befunden führt. Neue Testmethoden, die eventuell den Einsatz von zwei Methoden ablösen könnten, befinden sich in der Entwicklung, bedürfen aber der Evaluation in geeigneten internationalen multizentrischen Studien.
Since the discovery of ANCA 30 years ago, there has been a substantial improvement in this detection technique. Current state of the art is indirect immunofluorescence (IFT) followed by antigen-specific assays. The performance of the available Proteinase 3- and Myeloperoxidase- (PR3- and MPO-) ANCA assays concerning sensitivity and specificity has been continuously improved, especially due to the use of capture and anchor ELISA techniques. However, the quality of several commercially available ANCA assays is low when tested with an international standard. The sole use of ELISA and abolition of IFT is not recommended. PR3- and MPO-ANCA have well-established significance in the diagnosis of ANCA-associated vasculitides. Despite the achieved increase in sensitivity and specificity of ANCA testing, increasing unselected and broad clinical use leads to a rise in “false” positive results. New detection methods, that might replace the currently used combination of IFT and ELISA, are under development but will need evaluation in international multicenter trials.
Zusammenfassung
Influenzainfektionen gehen auch heute noch mit einer beträchtlichen Morbidität und nicht zu unterschätzenden Mortalität einher. Es werden daher in vielen Ländern der Welt ...Schutzimpfungen für Risikogruppen empfohlen. Immer wieder allerdings werden im klinischen Alltag neu oder wieder aufgetretene Erkrankungen der Haut mit einer vorangegangenen Grippeschutzimpfung in Verbindung gebracht. In der vorliegenden Arbeit geben wir einen systematischen Literaturüberblick zu dieser Thematik und stellen Daten des Paul‐Ehrlich‐Instituts vor, das in Deutschland Impfnebenwirkungen erfaßt. Neben Lokalreaktionen an der Injektionsstelle, überwiegend urtikariellen Exanthemen und anaphylaktischen Reaktionen auf Komponenten des Grippeimpfstoffs, wurden Fälle von Vaskulitiden dokumentiert, die mit Wahrscheinlichkeit auf eine vorangegangene Impfung zurückzuführen waren. Für weitere Hauterkrankungen konnte ein wahrscheinlicher oder sicherer Zusammenhang nicht überzeugend aufgezeigt werden. Das sehr geringe Risiko dermatologischer Komplikationen rechtfertigt nicht den Verzicht auf eine indikationsgerecht eingesetzte Grippeschutzimpfung.
Summary
Influenza remains an important cause of morbidity and mortality worldwide; therefore, vaccination of risk groups is recommended by health authorities in many countries of the world. Vaccination against influenza has been implicated occasionally induce or trigger skin diseases. We review the literature and present data from the German registry for vaccination‐associated adverse effects at the Paul‐Ehrlich‐Institut. In addition to local reactions at the injection site, exanthema, urticaria and anaphylactic reactions to vaccine components, vasculitis has been documented to be likely induced or triggered by influenza vaccination. Other skin diseases do not appear to be associated with vaccination to influenza. Overall, the risk of vaccination‐associated skin disease is very low and is not a reason to avoid of influenza vaccination.
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