Orphanet Encyclopædia 2003. 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. -
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. -
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. -
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. -
Kawasaki Disease Clinical Guideline
Kawasaki Disease Clinical Guideline November 2, 2016 KAWASAKI DISEASE CLINICAL GUIDELINE - NOVEMBER 2, 2016 1 Definition ! Kawasaki disease (KD), also known as Kawasaki syndrome, is an acute febrile illness of unknown cause that primarily affects children younger than 5 years of age. The disease was first described in Japan by Tomisaku Kawasaki in 1967, and the first cases outside of Japan were reported in Hawaii in 1976. Clinical signs include fever, rash, swelling of the hands and feet, irritation and redness of the whites of the eyes, swollen lymph glands in the neck, and irritation and inflammation of the mouth, lips, and throat. Epidemiology Studies of hospital discharge records by the United States Centers for Disease Control (CDC) estimated an overall annual ! incidence of 20 per 100,000 children younger than five years in the United States [11]. Annual incidence was highest among Asians and Pacific Islanders (30 per 100,000), intermediate among non-Hispanic African Americans (17 per 100,000) and Hispanics (16 per 100,000), and lowest among Caucasians (12 per 100,000) [11]. A winter-spring predominance of cases is characteristic, and the peak incidence of illness is at less than one year of age [11]. In contrast to Japan, surveillance in the United States is passive, and many cases may be missed. The overall incidence was 22 per 100,000 children less than five years of age in San Diego County during a six-year period from 1998 to 2003 [3]. The rates based upon ethnicity were 15, 25, 20, and 46 per 100,000 children less than five years of age for non-Hispanic whites, non-Hispanic African Americans, Hispanics, and Asian/Pacific Islanders, respectively. -
Microscopic Polyangiitis1
Microscopic polyangiitis1 Author: Professor J. Charles Jennette2 Creation Date: October 2002 Scientific Editor: Professor Loïc Guillevin 1This text was adapted from: Jennette JC, Thomas DB, Falk RJ. Microscopic polyangiitis (microscopic polyarteritis). Semin Diagn Pathol. 2001;18:3-13. 2Department of Pathology and Laboratory Medicine, University of North Carolina, 303 Brinkhous-Bullitt Building, NC 27599-7525 Chapel Hill, United States. [email protected] Abstract Keywords Disease name Definition Differential diagnosis Frequency Clinical manifestation Diagnostic methods Treatment Unresolved questions References Abstract Microscopic polyangiitis (MPA) refers to a necrotizing systemic vasculitis with few or no immune deposits that affects small vessels (ie, capillaries, venules and arterioles). Arteries, especially small arteries, are often but not always involved. Vessels of any type in any organ can be affected, resulting in a wide variety of signs and symptoms, and nonspecific clinical manifestations. Common signs and symptoms include nephritis, pulmonary hemorrhage, purpura, peripheral neuropathy, abdominal pain, myalgias and arthralgias. MPA is the most common antineutrophil cytoplasmic autoantibodies (ANCA)-associated small-vessel vasculitis. Most patients have positive myeloperoxidase MPOANCA (PANCA), although proteinase 3 PR3 ANCA (CANCA) may be also present. MPA has an incidence of approximately 1:100,000 with a slight predominance in men, and a mean age of onset of about 50 years. Treatment of patients with MPA consists of three -
COVID 19 Vaccine for Adolescents. Concern About Myocarditis and Pericarditis
Opinion COVID 19 Vaccine for Adolescents. Concern about Myocarditis and Pericarditis Giuseppe Calcaterra 1, Jawahar Lal Mehta 2 , Cesare de Gregorio 3 , Gianfranco Butera 4, Paola Neroni 5, Vassilios Fanos 5 and Pier Paolo Bassareo 6,* 1 Department of Cardiology, Postgraduate Medical School of Cardiology, University of Palermo, 90127 Palermo, Italy; [email protected] 2 Department of Medicine, University of Arkansas for Medical Sciences and the Veterans Affairs Medical Center, Little Rock, AR 72205, USA; [email protected] 3 Department of Clinical and Experimental Medicine, University of Messina, 98125 Messina, Italy; [email protected] 4 Cardiology, Cardiac Surgery, and Heart Lung Transplantation Department, ERN, GUAR HEART, Bambino Gesu’ Hospital and Research Institute, IRCCS Rome, 00165 Rome, Italy; [email protected] 5 Neonatal Intensive Care Unit, Department of Surgical Sciences, Policlinico Universitario di Monserrato, University of Cagliari, 09042 Monserrato, Italy; [email protected] (P.N.); [email protected] (V.F.) 6 Department of Cardiology, Mater Misericordiae University Hospital and Our Lady’s Children’s Hospital Crumlin, University College of Dublin, School of Medicine, D07R2WY Dublin, Ireland * Correspondence: [email protected]; Tel.: +353-1409-6083 Abstract: The alarming onset of some cases of myocarditis and pericarditis following the adminis- tration of Pfizer–BioNTech and Moderna COVID-19 mRNA-based vaccines in adolescent males has recently been highlighted. All occurred after the second dose of the vaccine. Fortunately, none of Citation: Calcaterra, G.; Mehta, J.L.; patients were critically ill and each was discharged home. Owing to the possible link between these de Gregorio, C.; Butera, G.; Neroni, P.; cases and vaccine administration, the US and European health regulators decided to continue to Fanos, V.; Bassareo, P.P. -
Incomplete Kawasaki Disease Following Meningococcal Serogroup B Meningitis
Open Access Austin Journal of Microbiology Case Report Incomplete Kawasaki Disease Following Meningococcal Serogroup B Meningitis El Ahmer I1,2*, Ben Haj Khalifa A1, Tlijeni A2, Wannes S2, Boussofara R2, Farhat J3, Khedher M1 Abstract and Sfar MT2 We report a case of incomplete Kawasaki disease following meningococcal 1Laboratory of Microbiology, Tahar Sfar Hospital, Tunisia serogroup B meningitis which we believe is a very rare and unique reported 2Pediatric Service, Tahar Sfar Hospital, Tunisia observation. 3Pharmacy Service, Tahar Sfar Hospital, Tunisia A five-month-old male infant presented with community-acquired meningitis. *Corresponding author: El Ahmer Ikram, Laboratory Gram stain and culture of the cerebrospinal fluid revealedNeisseria meningitidis. of Microbiology and Pediatric Service, Tahar Sfar The patient received antibiotic treatment. However, his recovery was complicated Hospital, Mahdia, Tunisia; Address: 44 Morocco Street by presenting spiking fever with elevated inflammatory markers. Repeated Chebba, Mahdia 5170, Tunisia cerebrospinal fluid and blood cultures were negative. The fever persisted till Received: July 04, 2017; Accepted: September 11, day 12 of treatment. In the absence of the classic mucocutaneous features 2017; Published: September 20, 2017 of Kawasaki disease, the patient was diagnosed with incomplete Kawasaki disease and treated with high-dose acetylsalicylic acid with good effect. We suggest that our observation translates the potential pathogenicity of N. meningitidis, emphasizes the need for strong Kawasaki disease diagnosis criteria and suggests the superantigen-mediated process in Kawasaki disease. Keywords: Neisseria meningitidis; Meningitis; Kawasaki disease Background with 70% neutrophils, a hemoglobin level of 112 g/L and a platelet count of 407x109 /L. C-reactive protein concentration (81 mg/L) was Kawasaki Disease (KD) is an acute multisystem vasculitis.