Giant Cell Arteritis
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Kawasaki Disease
Patient and Family Education Kawasaki Disease What is Kawasaki disease? What you need to know about Kawasaki disease (Cow-a-sa-kee) is an illness that young children, usually Kawasaki Disease younger than 5 years old, can get. It causes swelling and inflammation of the small blood vessels in the body. No one knows what causes it. The illness can last up to a few months. How is it diagnosed? We do not have a specific test that can diagnose Kawasaki disease. Symptoms can show up at different times and come and go. The diagnosis is made when doctors see a few or all of these symptoms in a child: • Fever that lasts for at least 4 to 5 days • Red, blood-shot eyes called conjunctivitis (kon-junk-ti-vi-tis) • Swollen lymph nodes of the neck and armpits called lymphadenopathy (lim-fad-e-nop-a-thee) • Rash on different or all parts of the body • Red, cracked lips, very red tongue (strawberry tongue), redness in the mouth and the back of the throat • Swollen and red hands and feet followed by peeling skin on the fingers and toes • Blood tests that show that your child has swelling (inflammation) • Also, children with Kawasaki disease are often very fussy. It can be hard to diagnose because there are other illnesses that can cause these symptoms. To make sure your child gets the correct diagnosis, doctors and specialists from other areas (such as Rheumatology and Infectious Disease) will be involved in your child’s care. Can this disease be serious? Kawasaki disease causes swelling and inflammation of the small blood vessels in the body. -
WO 2017/048702 Al
(12) INTERNATIONAL APPLICATION PUBLISHED UNDER THE PATENT COOPERATION TREATY (PCT) (19) World Intellectual Property Organization International Bureau (10) International Publication Number (43) International Publication Date W O 2017/048702 A l 2 3 March 2017 (23.03.2017) P O P C T (51) International Patent Classification: (81) Designated States (unless otherwise indicated, for every C07D 487/04 (2006.01) A61P 35/00 (2006.01) kind of national protection available): AE, AG, AL, AM, A61K 31/519 (2006.01) AO, AT, AU, AZ, BA, BB, BG, BH, BN, BR, BW, BY, BZ, CA, CH, CL, CN, CO, CR, CU, CZ, DE, DK, DM, (21) International Application Number: DO, DZ, EC, EE, EG, ES, FI, GB, GD, GE, GH, GM, GT, PCT/US20 16/05 1490 HN, HR, HU, ID, IL, IN, IR, IS, JP, KE, KG, KN, KP, KR, (22) International Filing Date: KW, KZ, LA, LC, LK, LR, LS, LU, LY, MA, MD, ME, 13 September 2016 (13.09.201 6) MG, MK, MN, MW, MX, MY, MZ, NA, NG, NI, NO, NZ, OM, PA, PE, PG, PH, PL, PT, QA, RO, RS, RU, RW, SA, (25) Filing Language: English SC, SD, SE, SG, SK, SL, SM, ST, SV, SY, TH, TJ, TM, (26) Publication Language: English TN, TR, TT, TZ, UA, UG, US, UZ, VC, VN, ZA, ZM, ZW. (30) Priority Data: 62/218,493 14 September 2015 (14.09.2015) US (84) Designated States (unless otherwise indicated, for every 62/218,486 14 September 2015 (14.09.2015) US kind of regional protection available): ARIPO (BW, GH, GM, KE, LR, LS, MW, MZ, NA, RW, SD, SL, ST, SZ, (71) Applicant: INFINITY PHARMACEUTICALS, INC. -
With Kawasaki Disease, Time Is Coronary Health
Clinical AND Health Affairs With Kawasaki Disease, Time is Coronary Health BY CLAIRE JANSSON-KNODELL AND RHAMY MAGID, M.D. previously healthy 3-year-old Hmong his shins believed to be an allergic reac- per minute). His blood pressure was boy presented to Children’s Hospitals tion. During a follow-up visit to his pe- 110/66 mm Hg, and he was irritable. He A and Clinics of Minnesota with a his- diatrician, the boy had a low-grade fever, weighed 15.2 kg. His sclerae were injected tory of fever that was unremitting despite swelling in his legs, and an erythematous bilaterally without exudate. His lips ap- antipyretics. Two weeks prior to admission rash. He was referred to the hospital, but peared bright pink with cheilosis with- at Children’s, he presented to an outside his mother chose to keep him home be- out frank cracking. His oropharynx was hospital with a fever accompanied by left- cause she assumed he had improved. He erythematous. He did not have cervical sided neck swelling. A neck ultrasound was “playful and interactive” at home and lymphadenopathy. His hands and feet were showed a lymph node measuring 3.5 x the neck swelling had lessened. A week edematous bilaterally. He had no rash, pe- 2.7 x 2.2 cm, without abscess or fluid col- later, the child returned to the clinic with techiae or ecchymosis. He had nonbleed- lection. He was treated for acute cervical persistent fever and pain in his foot that ing desquamation of his hands circumfer- lymphadenitis with antibiotics (ceftriaxone caused him to limp. -
Thrombokinetics in Giant Cell Arteritis, with Special Reference to Corticosteroid Therapy
Ann Rheum Dis: first published as 10.1136/ard.38.3.244 on 1 June 1979. Downloaded from Annals of the Rheumatic Diseases, 38, 1979, 244-247 Thrombokinetics in giant cell arteritis, with special reference to corticosteroid therapy ANNA-LISA BERGSTROM, BENGT-AKE BENGTSSON, LARS-BERTIL OLSSON, BO-ERIC MALMVALL, AND JACK KUTTI From the Departments of Internal Medicine and Infectious Diseases, Eastern Hospital, University of Gothenburg, S-416 85 Gothenburg, Sweden SUMMARY Duplicate platelet survival studies were carried out on 8 patients with giant cell arteritis (GCA), once before the institution of any therapy, and the second time when they were in a com- pletely asymptomatic phase after having received corticosteroid treatment. The time interval be- tween the studies ranged between 5 and 14 months. In the first study the mean peripheral platelet count was 486 ±25 x 109/1 and in the second 326 ±25 x 109/1. The difference between the means was highly significant (P<0 001). The mean life-span of the platelets was normal in the duplicate experiments (6 * 7 ±0 * 3 and 7 *3 ±0 *4 days, respectively). Platelet production rate was significantly (P<0 001) raised in the first experiment but became normal in response to corticosteroid therapy. It is concluded that the thrombocytosis seen in GCA is reactive to the inflammation present in this disease, and it seems reasonable to assume that the reduction in the peripheral platelet count which occurs in response to corticosteroid therapy accurately reflects the clinical improvement of the patient. Thrombocytosis is frequently present in patients Table 1 Eight patients with GCA in whom duplicate platelet sutrvival studies were carried out with untreated giant cell arteritis (GCA) (Olhagen, http://ard.bmj.com/ 1963; Hamrin, 1972; Malmvall and Bengtsson, Patients Age Sex Time interval Maintenance. -
Pulmonary Microscopic Polyangiitis Presenting As Acute Respiratory Failure from Diffuse Alveolar Hemorrhage
Case report SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES 2015; 32; 372-377 © Mattioli 1885 Pulmonary microscopic polyangiitis presenting as acute respiratory failure from diffuse alveolar hemorrhage Katharine K. Roberts1, Michael M. Chamberlin2, Allen R. Holmes3, Jonathan L. Henderson4, Robert L. Hutton3, William N. Hannah1, Michael J. Morris4 1 Internal Medicine Residency, Department of Medicine, San Antonio Military Medical Center; 2 Internal Medicine, United States Army Health Clinic, Vilseck, Germany; 3 Pathology Residency, Department of Pathology, San Antonio Military Medical Center; 4 Pulmonary/ Critical Care Service, Department of Medicine, San Antonio Military Medical Center Abstract. MicrMicroscopicoscopic polyangiitis and granulomatosis with polyangiitis are rare anti-neutrophilic cytoplas-cytoplas- mic antibody-associated systemic vasculitides that predominantly affect small to medium sized vessels of the lungs and kidneys. These syndromes are largely confined to older adults and often present sub-acutely follow- ing weeks to months of nonspecific prodromal symptoms. While both diseases often manifest within multiple organ systems concurrently, the disease spectrum of microscopic polyangiitis almost always includes the kidneys, while granulomatosis with polyangiitis is most commonly associated with pulmonary disease. We present two cases of rapid onset respiratory failure secondary to diffuse alveolar hemorrhage in young active duty military personnel. After serological testing and surgical lung biopsy, both patients were -
ANCA--Associated Small-Vessel Vasculitis
ANCA–Associated Small-Vessel Vasculitis ISHAK A. MANSI, M.D., PH.D., ADRIANA OPRAN, M.D., and FRED ROSNER, M.D. Mount Sinai Services at Queens Hospital Center, Jamaica, New York and the Mount Sinai School of Medicine, New York, New York Antineutrophil cytoplasmic antibodies (ANCA)–associated vasculitis is the most common primary sys- temic small-vessel vasculitis to occur in adults. Although the etiology is not always known, the inci- dence of vasculitis is increasing, and the diagnosis and management of patients may be challenging because of its relative infrequency, changing nomenclature, and variability of clinical expression. Advances in clinical management have been achieved during the past few years, and many ongoing studies are pending. Vasculitis may affect the large, medium, or small blood vessels. Small-vessel vas- culitis may be further classified as ANCA-associated or non-ANCA–associated vasculitis. ANCA–asso- ciated small-vessel vasculitis includes microscopic polyangiitis, Wegener’s granulomatosis, Churg- Strauss syndrome, and drug-induced vasculitis. Better definition criteria and advancement in the technologies make these diagnoses increasingly common. Features that may aid in defining the spe- cific type of vasculitic disorder include the type of organ involvement, presence and type of ANCA (myeloperoxidase–ANCA or proteinase 3–ANCA), presence of serum cryoglobulins, and the presence of evidence for granulomatous inflammation. Family physicians should be familiar with this group of vasculitic disorders to reach a prompt diagnosis and initiate treatment to prevent end-organ dam- age. Treatment usually includes corticosteroid and immunosuppressive therapy. (Am Fam Physician 2002;65:1615-20. Copyright© 2002 American Academy of Family Physicians.) asculitis is a process caused These antibodies can be detected with indi- by inflammation of blood rect immunofluorescence microscopy. -
A Review of Primary Vasculitis Mimickers Based on the Chapel Hill Consensus Classification
Hindawi International Journal of Rheumatology Volume 2020, Article ID 8392542, 11 pages https://doi.org/10.1155/2020/8392542 Review Article A Review of Primary Vasculitis Mimickers Based on the Chapel Hill Consensus Classification Farah Zarka ,1 Charles Veillette ,1 and Jean-Paul Makhzoum 2 1Hôpital du Sacré-Cœur de Montreal, University of Montreal, Canada 2Vasculitis Clinic, Department of Internal Medicine, Hôpital du Sacré-Coeur de Montreal, University of Montreal, Canada Correspondence should be addressed to Jean-Paul Makhzoum; [email protected] Received 10 July 2019; Accepted 7 January 2020; Published 18 February 2020 Academic Editor: Charles J. Malemud Copyright © 2020 Farah Zarka et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Primary systemic vasculitides are rare diseases that may manifest similarly to more commonly encountered conditions. Depending on the size of the vessel affected (large vessel, medium vessel, or small vessel), different vasculitis mimics must be considered. Establishing the right diagnosis of a vasculitis mimic will prevent unnecessary immunosuppressive therapy. 1. Introduction 2. Large-Vessel Vasculitis Mimics Vasculitides are rare heterogenous diseases that affect vessel Large-vessel vasculitis (LVV) is an inflammatory vascu- walls as the main site of inflammation. Organs affected vary lopathy affecting large arteries; giant cell arteritis (GCA) depending on the type and size of blood vessels involved and Takayasu’s arteritis (TAK) are the two main docu- [1]. Autoimmune vasculitis can be primary (idiopathic) or mented variants, each with their own characteristic fea- secondary to an underlying disease. -
PATIENT FACT SHEET Kawasaki Disease (KD)
PATIENT FACT SHEET Kawasaki Disease (KD) Kawasaki disease (KD) is a childhood illness that causes Kawasaki disease is most common in children younger blood vessels to become inflamed (vasculitis) and swell. than 5 years old; however, older children can be affected Kawasaki disease is a serious illness because it can cause as well. KD occurs more often among boys and is more life-threatening inflammation of blood vessels that supply commonly seen in the winter and spring months. The oxygen and nutrients to the heart (the coronary arteries). exact cause of KD is unknown, but it is suspected that CONDITION This complication can usually be prevented by early it may be triggered by an infection. It may also occur in DESCRIPTION diagnosis and treatment. children who have a genetic predisposition to the disease. Kawasaki disease is not contagious. The most common symptoms include prolonged fever, There is no specific test to diagnose Kawasaki disease. rash, bloodshot eyes, red cracked lips and tongue, Rather, doctors diagnose Kawasaki disease based on a and lymph node swelling. Children with Kawasaki child’s symptoms and physical exam. A prolonged fever disease may also have painful or swollen joints, extreme (i.e., more than five days) is often the first symptom that fussiness especially in younger children, and swelling of alerts a doctor to consider Kawasaki disease. the gallbladder that can cause belly pain and vomiting. Lab tests may help with diagnosis. This may include: (1) The symptoms of KD often go away on their own and the blood and urine tests, (2) Electrocardiogram, also known child recovers. -
Review Article
J Clin Pathol: first published as 10.1136/jcp.36.7.723 on 1 July 1983. Downloaded from J Clin Pathol 1983;36:723-733 Review article Granulomatous inflammation- a review GERAINT T WILLIAMS, W JONES WILLIAMS From the Department ofPathology, Welsh National School ofMedicine, Cardiff SUMMARY The granulomatous inflammatory response is a special type of chronic inflammation characterised by often focal collections of macrophages, epithelioid cells and multinucleated giant cells. In this review the characteristics of these cells of the mononuclear phagocyte series are considered, with particular reference to the properties of epithelioid cells and the formation of multinucleated giant cells. The initiation and development of granulomatous inflammation is discussed, stressing the importance of persistence of the inciting agent and the complex role of the immune system, not only in the perpetuation of the granulomatous response but also in the development of necrosis and fibrosis. The granulomatous inflammatory response is Macrophages and the mononuclear phagocyte ubiquitous in pathology, being a manifestation of system many infective, toxic, allergic, autoimmune and neoplastic diseases and also conditions of unknown The name "mononuclear phagocyte system" was aetiology. Schistosomiasis, tuberculosis and leprosy, proposed in 1969 to describe the group of highly all infective granulomatous diseases, together affect phagocytic mononuclear cells and their precursors more than 200 million people worldwide, and which are widely distributed in the body, related by http://jcp.bmj.com/ granulomatous reactions to other irritants are a morphology and function, and which originate from regular occurrence in everyday clinical the bone marrow.' Macrophages, monocytes, pro- histopathology. A knowledge of the basic monocytes and their precursor monoblasts are pathophysiology of this distinctive tissue reaction is included, as are Kupffer cells and microglia. -
Orphanet Encyclopædia 2003. Giant Cell Arteritis
Giant cell arteritis Author: Doctor José María Calvo-Romero1 Creation Date: June 2003 Scientific Editor: Professor Loic Guillevin 1Department of Internal Medicine, Hospital de Zafra, Antigua Ctra. Nacional 432, 06300 Zafra (Badajoz), Spain. [email protected] Abstract Key-words Disease names Definition Epidemiology Etiology Clinical manifestations Laboratory findings Diagnosis Treatment Prognosis References Abstract Giant cell arteritis (GCA) or temporal arteritis is a systemic vasculitis which involves large and medium- sized vessels, especially the extracranial branches of the carotid artery, usually in persons older than 50 years. Feared complications of GCA are permanent visual loss, ischaemic strokes and thoracic and abdominal aortic aneurysms. The treatment consists of high-dose steroids. Mortality in patients with GCA seems to be similar to that of controls, probably due to a correct diagnosis and management. GCA is the most common systemic vasculitis in Western countries. The incidence rates described in European countries are around 20:100 000 persons older than 50 years. Key-words vasculitis, giant cell arteritis, temporal arteritis, Horton’s arteritis, large and medium-sized vessels disorder. Disease names Table 1: The Chapel Hill Consensus Giant cell arteritis Conference on the Nomenclature of Systemic Temporal arteritis Vasculitis Horton’s arteritis Large Vessel Vasculitis Giant cell arteritis Definition Takayasu arteritis Giant cell arteritis (GCA) is a relatively common Medium Vessel Vasculitis systemic vasculitis in Europe. GCA involves Polyarteritis nodosa large and medium-sized vessels in patients Kawasaki’s disease usually older than 50 years. It is a Small Vessel Vasculitis granulomatous arteritis of the aorta and its major Wegener’s granulomatosis branches, especially the extracranial branches of Churg-Strauss syndrome Microscopic polyangiitis the carotid artery. -
(Mabthera) Maintenance Therapy for Granulomatosis with Polyangiitis (GPA) and Microscopic Polyangiitis (MPA) NIHRIO (HSRIC) ID: 12979 NICE ID: 9284
NIHR Innovation Observatory Evidence Briefing: August 2017 Rituximab (MabThera) maintenance therapy for granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) NIHRIO (HSRIC) ID: 12979 NICE ID: 9284 LAY SUMMARY Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis is a rare condition in which abnormal antibodies attack the body’s own cells, causing inflammation. Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are two different types of ANCA-associated vasculitis. These conditions can cause serious organ damage and severely impact quality of life. Following initial treatment, these conditions frequently return. Rituximab is a medicine, delivered as an infusion into the vein. It destroys B cells, the part of the immune system thought to be involved in this type of vasculitis. It is already licensed for use (and recommended by NICE) as a treatment for people with GPA or MPA. There has however not been sufficient evidence to consider the continued use of rituximab as maintenance therapy, although this is already commissioned by NHS England in some instances. The current clinical trial examines the use of rituximab as a maintenance treatment in patients with GPA or MPA. If licensed, rituximab would offer another option for maintenance therapy in this patient cohort. This briefing is based on information available at the time of research and a limited literature search. It is not intended to be a definitive statement on the safety, efficacy or effectiveness of the health technology covered and should not be used for commercial purposes or commissioning without additional information. This briefing presents independent research funded by the National Institute for Health Research (NIHR). -
Biomaterials and the Foreign Body Reaction: Surface Chemistry Dependent Macrophage Adhesion, Fusion, Apoptosis, and Cytokine Production
BIOMATERIALS AND THE FOREIGN BODY REACTION: SURFACE CHEMISTRY DEPENDENT MACROPHAGE ADHESION, FUSION, APOPTOSIS, AND CYTOKINE PRODUCTION by JACQUELINE ANN JONES Submitted in partial fulfillment of the requirements For the degree of Doctor of Philosophy Dissertation Advisor: James Morley Anderson, M.D., Ph.D. Department of Biomedical Engineering CASE WESTERN RESERVE UNIVERSITY May, 2007 CASE WESTERN RESERVE UNIVERSITY SCHOOL OF GRADUATE STUDIES We hereby approve the dissertation of ______________________________________________________ candidate for the Ph.D. degree *. (signed)_______________________________________________ (chair of the committee) ________________________________________________ ________________________________________________ ________________________________________________ ________________________________________________ ________________________________________________ (date) _______________________ *We also certify that written approval has been obtained for any proprietary material contained therein. Copyright © 2007 by Jacqueline Ann Jones All rights reserved iii Dedication This work is dedicated to… My ever loving and ever supportive parents: My mother, Iris Quiñones Jones, who gave me the freedom to be and to dream, inspires me with her passion and courage, and taught me the true meaning of friendship. My father, Glen Michael Jones, a natural-born teacher who taught me to be an inquisitive student of life, inspires me with his strength and perseverance, and gave me his kind, earnest heart that loves deeply and always