Vasculitis Guideline Project Plan
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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. -
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. -
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. -
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. -
KAWASAKI DISEASE Ian K Maconochie
Arch Dis Child Educ Pract Ed: first published as 10.1136/adc.2004.053728 on 20 May 2004. Downloaded from BEST PRACTICE KAWASAKI DISEASE Ian K Maconochie ep3 Arch Dis Child Educ Pract Ed 2004;89:ep3–ep8. doi: 10.1136/adc.2004.053728 r Tomisaku Kawasaki published a case series of 50 children in 19671 who were febrile and all had a rash, non-exudative conjunctivitis, erythema of the palms and soles of the feet, Dand cervical lymphadenopathy. This constellation of signs Dr Kawasaki termed ‘‘acute febrile mucocutaneous syndrome’’; however the eponym Kawasaki disease has been accepted worldwide. We consider the difficulties in diagnosis and treatment presented by this condition and examine a recently published clinical guideline of its management. DIAGNOSIS Kawasaki disease is a systemic vasculitis predominantly affecting children under the age of 5 years. It has a number of classic clinical features required for diagnosis. In 1990 the American Heart Association committee on rheumatic fever, endocarditis, and Kawasaki disease2 gave the case definition that has been generally accepted—ie, a febrile illness of at least five days with at least four of the five following signs and no other reasonable cause for the findings: c Bilateral conjunctival injection – (there is no corneal ulceration but there may be a concomitant anterior uveitis on slit lamp examination) c Oral changes (erythema of lips or oropharynx, strawberry tongue due to prominent papillae, or fissuring of the lips) (fig 1) c Peripheral extremity changes (oedema, erythema, or generalised or periungal desquamation); erythema is seen in the first week whereas desquamation begins about 14–21 days after the onset of the illness (fig 2) c Rash – this starts in the first few days; it is often diffuse and polymorphic and lasts a week before fading. -
Vasculitis: Pearls for Early Diagnosis and Treatment of Giant Cell Arteritis
Vasculitis: Pearls for early diagnosis and treatment of Giant Cell Arteritis Mary Beth Humphrey, MD, PhD Professor of Medicine McEldowney Chair of Immunology [email protected] Office Phone: 405 271-8001 ext 35290 October 2019 Relevant Disclosure and Resolution Under Accreditation Council for Continuing Medical Education guidelines disclosure must be made regarding relevant financial relationships with commercial interests within the last 12 months. Mary Beth Humphrey I have no relevant financial relationships or affiliations with commercial interests to disclose. Experimental or Off-Label Drug/Therapy/Device Disclosure I will be discussing experimental or off-label drugs, therapies and/or devices that have not been approved by the FDA. Objectives • To recognize early signs of vasculitis. • To discuss Tocilizumab (IL-6 inhibitor) as a new treatment option for temporal arteritis. • To recognize complications of vasculitis and therapies. Professional Practice Gap Gap 1: Application of imaging recommendations in large vessel vasculitis Gap 2: Application of tocilizimab in treatment of giant cell vasculitis Cranial Symptoms Aortic Vision loss Aneurysm GCA Arm PMR Claudication FUO Which is not a risk factor or temporal arteritis? A. Smoking B. Female sex C. Diabetes D. Northern European ancestry E. Age Which is not a risk factor or temporal arteritis? A. Smoking B. Female sex C. Diabetes D. Northern European ancestry E. Age Giant Cell Arteritis • Most common form of systemic vasculitis in adults – Incidence: ~ 1/5,000 persons > 50 yrs/year – Lifetime risk: 1.0% (F) 0.5% (M) • Cause: unknown At risk: Women (80%) > men (20%) Northern European ancestry>>>AA>Hispanics Age: average age at onset ~73 years Smoking: 6x increased risk Kermani TA, et al Ann Rheum Dis. -
COVID-19 Inflammatory Syndrome with Clinical Features Resembling Kawasaki Disease Robert Spencer, Ryan C
COVID-19 Inflammatory Syndrome With Clinical Features Resembling Kawasaki Disease Robert Spencer, MD,a Ryan C. Closson, MD,a Mark Gorelik, MD,b Alexis D. Boneparth, MD,b Rebecca F. Hough, MD, PhD,c Karen P. Acker, MD,d Usha Krishnan, MDa We describe 2 patients with coronavirus disease who had multiple clinical abstract features suggestive of Kawasaki disease (KD). Both patients presented with fever lasting .5 days and were found to have rash, conjunctival injection, and swollen lips. One patient also had extremity swelling, whereas the other Divisions of aPediatric Cardiology, bAllergy, Immunology, and fi Rheumatology, and cPediatric Critical Care Medicine, developed desquamation of the ngers. In both cases, laboratory results were Department of Pediatrics, Columbia University Irving similar to those seen in KD. These patients had highly unusual but similar Medical Center and Morgan Stanley Children’s Hospital, New d features, and both appeared to respond favorably to treatment. It remains York, New York; and Division of Pediatric Infectious Diseases, Department of Pediatrics, Weill Cornell Medicine, unclear whether these patients had true KD or manifestations of coronavirus New York, New York disease that resembled KD. Dr Spencer was the consulting cardiology fellow who evaluated 1 of the 2 patients, and he was the primary author of this case report; Dr Closson was the consulting cardiology fellow who evaluated the As of May 18, 2020, nearly 1.5 million of his hands and feet developed. other patient, and he contributed references and cases of coronavirus disease (COVID- Two days later, he developed revisions to the manuscript; Dr Gorelik was the fi consulting rheumatology attending who helped 19) have been con rmed in the a polymorphous rash on his torso, manage one of these patients, and he contributed 1 United States. -
Audio Vestibular Gluco Corticoid General and Local Or Cytotoxic Agents
Global Journal of Otolaryngology ISSN 2474-7556 Case Report Glob J Otolaryngol Volume 13 Issue 5 - March 2018 Copyright © All rights are reserved by Cristina Otilia Laza DOI: 10.19080/GJO.2018.13.555871 Autoimmune Granulomatosis with Polyangiitis or Wegener Granulomatosis Cristina Otilia Laza1*, Gina Enciu2, Luminita Micu2 and Maria Suta3 1Department of ENT, County Clinical Emergency Hospital of Constanta, Romania 2Department of Anatomo pathology, County Clinical Emergency Hospital of Constanta, Romania 3Department of Rheumatology, County Clinical Emergency Hospital of Constanta, Romania Submission: February 19, 2018; Published: March 14, 2018 *Corresponding author: Cristina Otilia Laza, Department of ENT, County Clinical Emergency Hospital of Constanta, Romania, Email: Abstract Granulomatosis with polyangiitis, formerly known as Wegener granulomatosis, is a disease that typically consists of a triad of airway necrotizing granulomas, systemic vasculitis, and focal glomerulonephritis. If the disease does not involve the kidneys, it is called limited granulomatosis with polyangiitis. The etiology and pathogenesis of WG are unknown. Infectious, genetic, and environmental risk factors and combinations thereof have been proposed. The evidence to date suggests that WG is a complex, immune-mediated disorder in which tissue production of ANCA, directed against antigens present within the primary granules of neutrophils and monocytes; these antibodies produce tissueinjury damageresults from by interacting the interplay with of primedan initiating neutrophils inflammatory and endothelial event and cells a highly The purposespecific immune of this article response. is to Part present of this 4 patients response all consists diagnosed of the in our department ,with head and neck lesions ,every case with his manifestation and response to the treatment .We consider that a well trained ENT specialist must be able to diagnose and recognize such a disease but this requires knowledge and hard work. -
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. -
Understanding the Cryoglobulinemias
Current Rheumatology Reports (2019) 21:60 https://doi.org/10.1007/s11926-019-0859-0 VASCULITIS (L ESPINOZA, SECTION EDITOR) Understanding the Cryoglobulinemias Alejandro Fuentes1 & Claudia Mardones1 & Paula I. Burgos1 # Springer Science+Business Media, LLC, part of Springer Nature 2019 Abstract Purpose of the Review Cryoglobulins are immunoglobulins with the ability to precipitate at temperatures <37 °C. They are related to hematological disorders, infections [especially hepatitis C virus (HCV)], and autoimmune diseases. In this article, the state of the art on Cryoglobulinemic Vasculitis (CV), in a helpful and schematic way, with a special focus on HCV related Mixed Cryoglobulinemia treatment are reviewed. Recent Findings Direct – acting antivirals (DAA) against HCV have emerged as an important key in HCV treatment to related Cryoglobulinemic Vasculitis, and should be kept in mind as the initial treatment in non–severe manifestations. On the other hand, a recent consensus panel has published their recommendations for treatment in severe and life threatening manifestations of Mixed Cryoglobulinemias. Summary HCV-Cryoglobulinemic vasculitis is the most frequent form of CV. There are new treatment options in HCV-CV with DAA, with an important number of patients achieving complete response and sustained virologic response (SVR). In cases of severe forms of CV, treatment with Rituximab and PLEX are options. The lack of data on maintenance therapy could impulse future studies in this setting. Keywords HCV . Mixed Cryoglobulinemia . Type I Cryoglobulinemia . gC1qR . Direct-acting antivirals . Rituximab Introduction and Definitions tion of the total pool of cryoprecipitable immunocomplexes in targeted vessels and due to false negative results owing to im- Cryoglobulins are immunoglobulins (Ig) that precipitate in vitro proper blood sampling or inadequate laboratory processes [4]. -
Syndrome Resembling Kawasaki Disease in COVID-19 Asymptomatic Children
Journal of Infection and Public Health 13 (2020) 1830–1832 Contents lists available at ScienceDirect Journal of Infection and Public Health j ournal homepage: http://www.elsevier.com/locate/jiph Review Article Syndrome resembling Kawasaki disease in COVID-19 asymptomatic children a,∗ b a c,∗ Suriya Rehman , Tariq Majeed , Mohammad Azam Ansari , Ebtesam A. Al-Suhaimi a Department of Epidemic Diseases Research, Institute of Research and Medical Consultations (IRMC), Imam Abdulrahman Bin Faisal University, 31441 Dammam, Saudi Arabia b Department of General Pediatric, Maternity and Children Hospital, Dammam, Saudi Arabia c Department of Biology, College of Science and Institute of Research and Medical Consultations (IRMC), Imam Abdulrahman Bin Faisal University, 31441 Dammam, Saudi Arabia a r t i c l e i n f o a b s t r a c t Article history: The current knowledge about the COVID-19 (Coronavirus Disease-2019) pandemic is still limited and is Received 16 June 2020 unravelling with the passing days, especially clinical data, and research in pediatric age group. Recently, Received in revised form 7 August 2020 there is a new and crucial development reported recently among the COVID-19 asymptomatic children, Accepted 13 August 2020 a novel syndrome affecting asymptomatic COVID-19 children, presenting as a hyperinflammatory syn- drome which is like Kawasaki disease shock syndrome. The purpose of this correspondence is to discuss Keywords: some important findings of the syndrome for the better understanding of the disease. COVID-19 © 2020 The Authors. Published by Elsevier Ltd on behalf of King Saud Bin Abdulaziz University for Children Health Sciences. -
Microscopic Polyangiitis
Supplementary material Ann Rheum Dis Supplementary Methods Polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA, Wegener’s), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss) were defined according to the Chapel Hill definitions. In ANCA-associated vasculitis patients, serum samples were tested for ANCA by means of indirect immunofluorescence and tested for anti–proteinase 3 ANCA and anti-myeloperoxidase ANCA with an enzyme-linked immunosorbent assay. Virus-associated vasculitides were excluded from all these trials and patients with a previous history of cancer of less than 3 or 5 years were ineligible for all trials. Patients were prospectively evaluated at diagnosis, at randomization, every 2-3 months thereafter during the first two years of follow-up, and then every 6 months until the end of follow-up. Long-term follow-up after the end of the protocol was planned for each protocol using the FVSG database. Severe adverse events, including malignancy, were prospectively collected. Lifestyle factors such as smoking were not included in this analysis Association of baseline characteristics and therapeutic strategies with the occurence of malignancy was investigated by univariate analyses using Cox regression models. Variables associated with malignancies with a P value <0.05 in univariate analysis were included in a backward-selection Cox regression analysis. Person-years of follow-up were calculated using the total follow-up and stratified by sex and 5-years age groups. Incidence rates were calculated for all cancers and compared to the French National registry, with indirect standardization on sex and 5-years age groups. Standardized incidence ratios were calculated as the ratio between the observed and the expected number of cancer.