DOCSLIB.ORG

A Primer on Vasculitis

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
A Primer on Vasculitis Clinical AND Health Affairs A Primer on Vasculitis BY KENNETH J. WARRINGTON, M.D., AND ERIC L. MATTESON, M.D., M.P.H. Vasculitides are rare but serious conditions involving inflammation of the body’s blood vessels that can lead to organ damage and myriad complications. This article describes the various forms of vasculitis and their incidence, and discusses diagnosis and treatment of patients with the more common forms. asculitis refers to a heterogenous occasionally, they can be limited to a single The incidence of ANCA-associated vascu- group of conditions characterized by organ. Cutaneous vasculitis, testicular vas- litis is approximately 10 to 20 persons per Vinflammation of blood vessel walls, culitis and primary central nervous system million per year, with granulomatosis with which results in vascular damage. Because vasculitis are examples of single-organ polyangiitis being more common than inflammation can affect vessels of any size vasculitides. Vasculitis may develop in the microscopic polyangiitis and eosinophilic in any location, vasculitides have varying context of an underlying autoimmune dis- granulomatosis with polyangiitis. clinical presentations. ease such as systemic lupus erythematosus Vasculitides are generally classified and rheumatoid arthritis as well as with Etiology based on the predominant type of vessel malignancy, infection or medication use. In the majority of cases, the cause of (large, medium or small) involved. Giant (Table).1 vasculitis is unknown. The prevailing cell arteritis and Takayasu’s arteritis are hypothesis is that vasculitis is initiated by both large-vessel vasculitides that affect Incidence an environmental agent in a genetically the aorta and its primary and second- In general, vasculitis is rare and therefore predisposed individual. Genetic polymor- ary branches. Polyarteritis nodosa and can be difficult to recognize clinically. The phisms in the human leukocyte antigen Kawasaki disease predominantly involve incidence of specific vasculitic disorders (HLA) genes and polymorphisms in genes medium-sized arteries. The most com- varies according to patient age, race and encoding cytokines and other immuno- mon forms of small-vessel vasculitis are geographic location. For example, giant regulatory proteins have been associated granulomatosis with polyangiitis (formerly cell arteritis affects people older than 50 with an increased risk of several types of Wegener’s granulomatosis), microscopic years of age and is most common among vasculitis.2 polyangiitis, and eosinophilic granuloma- people of Northern European descent. It In certain forms of vasculitis, investiga- tosis with polyangiitis (formerly Churg- is one of the most common forms of vas- tors have identified probable etiologies. Strauss syndrome). The latter three condi- culitis in adults, with an average annual About one-third of polyarteritis nodosa tions are often collectively referred to as incidence of about 19 cases per 100,000 cases are caused by chronic hepatitis B the anti-neutrophil cytoplasmic antibody persons 50 years of age and older. Con- infection, while hepatitis C can trigger (ANCA)-associated vasculitides. Other versely, Takayasu arteritis generally occurs cryoglobulinemic vasculitis. Neoplasms, less common forms of small-vessel vasculi- in individuals younger than 40 years of age particularly hematologic malignancies, can tis include cryoglobulinemic vasculitis and and is more common among Asians. In the also cause vasculitis. Drug-induced vas- Henoch-Schönlein purpura. Some forms United States, its annual incidence is about culitis can occur with medications such as of systemic vasculitis such as Behçet’s dis- 2.6 per million population. Polyarteritis antibiotics, hydralazine, propylthiouracil ease can affect vessels of any size and type nodosa affects about five to 10 individuals and allopurinol. (arteries, veins and capillaries). per million per year, and it is becoming Typically, vasculitides are systemic dis- even less common in countries with a de- eases with multi-organ involvement; but creasing prevalence of hepatitis B infection. 36 | MINNESOTA MEDICINE | MAY 2013 Clinical AND Health Affairs Complications and Clinical TABLE Manifestations 1 The clinical manifestations and complica- Nomenclature for Vasculitides tions of vasculitis are varied and depend Large-vessel vasculitis on the vascular bed that is involved by the • Takayasu arteritis inflammatory process: • Giant cell arteritis In giant cell arteritis, the lumen may • Medium-vessel vasculitis become occluded through intimal • Polyarteritis nodosa hyperplasia, causing end-organ isch- • Kawasaki disease emia. Since the extracranial arteries are Small-vessel vasculitis typically involved, patients often pres- • Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis ent with headaches and scalp tender- • Microscopic polyangiitis ness. Patients frequently complain of • Granulomatosis with polyangiitis (Wegener’s) symptoms of polymyalgia rheumatica • Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) including pain and stiffness in the neck • Immune complex SVV • Anti–glomerular basement membrane disease and proximal extremities. The serious • Cryoglobulinemic vasculitis clinical consequences that may ensue • IgA vasculitis (Henoch-Schönlein) include jaw claudication, vision loss • Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis) caused by ischemic optic neuropathy, Variable-vessel vasculitis cerebrovascular ischemic events and • Behçet’s disease limb claudication. In the aorta, damage • Cogan’s syndrome to the vessel wall may lead to progres- Single-organ vasculitis sive dilatation, aneurysm formation and • Cutaneous leukocytoclastic angiitis life-threatening events such as aortic • Cutaneous arteritis dissection.3 • Primary central nervous system vasculitis • The inflammatory vasculopathy in • Isolated aortitis Others Takayasu arteritis may lead to complica- • tions such as cerebrovascular events, Vasculitis associated with systemic disease limb claudication, renovascular hyper- • Lupus vasculitis Rheumatoid vasculitis tension and aneurysm formation. Less • • Sarcoid vasculitis often, it involves the coronary arteries, • Others causing ischemic heart disease and Vasculitis associated with probable etiology pulmonary hypertension. Patients often • Hepatitis C-associated cryoglobulinemic vasculitis require surgical revascularization to re- • Hepatitis B-associated vasculitis store vascular patency. • Syphilis-associated aortitis • Polyarteritis nodosa is characterized by • Drug-associated immune complex vasculitis necrotizing inflammation of muscular • ANCA-associated vasculitis arteries, which often produces microan- • Cancer-associated vasculitis Others eurysms of the visceral arteries. Patients • typically present with constitutional SOURCE symptoms and evidence of multi-organ dysfunction. Neurologic manifestations 1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference such as mononeuritis multiplex, cutane- Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1-11. ous lesions (eg, skin ulcerations) and gastrointestinal complications such as mesenteric ischemia, bowel infarction • Because the various forms of ANCA- • Clinical manifestations of granulo- or hemorrhage are characteristic. Renal associated vasculitis primarily involve matosis with polyangiitis may include involvement often leads to arterial hy- small vessels such as arterioles, venules sinusitis, otitis, pulmonary nodules pertension and ischemic nephropathy and capillaries, the main target organs and alveolar hemorrhage. The major- with renal failure. Peripheral arterial are the respiratory system and kidneys, ity of patients will develop glomeru- occlusions can result in ischemia and where extensive capillary networks are lonephritis, which can lead to rapidly gangrene of the digits.4 present. progressive renal failure. Other disease manifestations may include ocular MAY 2013 | MINNESOTA MEDICINE | 37 Clinical AND Health Affairs inflammation (scleritis, proptosis), Laboratory findings suggestive of vas- lung nodules, alveolar infiltrates/hemor- cutaneous vasculitis and mononeuritis culitis may include normocytic anemia, rhage, organ infarcts). Vascular imaging multiplex.5 thrombocytosis, and elevated erythrocyte such as magnetic resonance angiography • Rapidly progressive glomerulonephritis sedimentation rate (ESR) and C-reactive (MRA) or computed tomography angi- and alveolar hemorrhage are the most protein (CRP). Unfortunately, these tests ography (CTA) is particularly useful for common clinical manifestations of mi- are not specific and may be abnormal be- noninvasive imaging of medium-sized and croscopic polyangiitis, which clinically cause of myriad inflammatory, infectious large arteries. Vessel wall edema, contrast can be very similar to granulomatosis or neoplastic disease entities. Typically, enhancement and/or wall thickening are with polyangiitis.6 ESR and CRP are markedly elevated in characteristic findings of large-vessel • Eosinophilic granulomatosis with poly- vasculitis; however, not all patients with vasculitis. In addition, MRA or CTA may angiitis has three main disease features: vasculitis mount an inflammatory re- reveal luminal changes such as long ta- allergic rhinitis and asthma, eosino- sponse—especially those with single-organ pered arterial stenoses of the aortic arch philic infiltrative disease and systemic vasculitis. branches or aneurysms.9 Conventional small-vessel
Load more

Recommended publications
  • 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.
    [Show full text]
  • 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.
    [Show full text]
  • Psoriasis, a Systemic Disease Beyond the Skin, As Evidenced by Psoriatic Arthritis and Many Comorbities
    1 Psoriasis, a Systemic Disease Beyond the Skin, as Evidenced by Psoriatic Arthritis and Many Comorbities – Clinical Remission with a Leishmania Amastigotes Vaccine, a Serendipity Finding J.A. O’Daly Astralis Ltd, Irvington, NJ USA 1. Introduction Psoriasis is a systemic chronic, relapsing inflammatory skin disorder, with worldwide distribution, affects 1–3% of the world population, prevalence varies according to race, geographic location, and environmental factors (Chandran & Raychaudhuri, 2010; Christophers & Mrowietz, 2003; Farber & Nall, 1974). In Germany, 33,981 from 1,344,071 continuously insured persons in 2005 were diagnosed with psoriasis; thus the one year prevalence was 2.53% in the study group. Up to the age of 80 years the prevalence rate (range: 3.99-4.18%) was increasing with increasing age and highest for the age groups from 50 to 79 years The total rate of psoriasis in children younger than 18 years was 0.71%. The prevalence rates increased in an approximately linear manner from 0.12% at the age of 1 year to 1.2% at the age of 18 years (Schäfer et al., 2011). In France, a case-control study in 6,887 persons, 356 cases were identified (5.16%), who declared having had psoriasis during the previous 12 months (Wolkenstein et al., 2009). The prevalence of psoriasis analyzed across Italy showed that 2.9% of Italians declared suffering from psoriasis (regional range: 0.8-4.5%) in a total of 4109 individuals (Saraceno et al., 2008). The overall rate of comorbidity in subjects with psoriasis aged less than 20 years was twice as high as in subjects without psoriasis.
    [Show full text]
  • 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.
    [Show full text]
  • 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.
    [Show full text]
  • Celiac Disease and Nonceliac Gluten Sensitivitya Review
    Clinical Review & Education JAMA | Review Celiac Disease and Nonceliac Gluten Sensitivity A Review Maureen M. Leonard, MD, MMSc; Anna Sapone, MD, PhD; Carlo Catassi, MD, MPH; Alessio Fasano, MD CME Quiz at IMPORTANCE The prevalence of gluten-related disorders is rising, and increasing numbers of jamanetwork.com/learning individuals are empirically trying a gluten-free diet for a variety of signs and symptoms. This review aims to present current evidence regarding screening, diagnosis, and treatment for celiac disease and nonceliac gluten sensitivity. OBSERVATIONS Celiac disease is a gluten-induced immune-mediated enteropathy characterized by a specific genetic genotype (HLA-DQ2 and HLA-DQ8 genes) and autoantibodies (antitissue transglutaminase and antiendomysial). Although the inflammatory process specifically targets the intestinal mucosa, patients may present with gastrointestinal signs or symptoms, extraintestinal signs or symptoms, or both, Author Affiliations: Center for Celiac suggesting that celiac disease is a systemic disease. Nonceliac gluten sensitivity Research and Treatment, Division of is diagnosed in individuals who do not have celiac disease or wheat allergy but who Pediatric Gastroenterology and Nutrition, MassGeneral Hospital for have intestinal symptoms, extraintestinal symptoms, or both, related to ingestion Children, Boston, Massachusetts of gluten-containing grains, with symptomatic improvement on their withdrawal. The (Leonard, Sapone, Catassi, Fasano); clinical variability and the lack of validated biomarkers for nonceliac gluten sensitivity make Celiac Research Program, Harvard establishing the prevalence, reaching a diagnosis, and further study of this condition Medical School, Boston, Massachusetts (Leonard, Sapone, difficult. Nevertheless, it is possible to differentiate specific gluten-related disorders from Catassi, Fasano); Shire, Lexington, other conditions, based on currently available investigations and algorithms.
    [Show full text]
  • 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.
    [Show full text]
  • 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.
    [Show full text]
  • Conditions Related to Inflammatory Arthritis
    Conditions Related to Inflammatory Arthritis There are many conditions related to inflammatory arthritis. Some exhibit symptoms similar to those of inflammatory arthritis, some are autoimmune disorders that result from inflammatory arthritis, and some occur in conjunction with inflammatory arthritis. Related conditions are listed for information purposes only. • Adhesive capsulitis – also known as “frozen shoulder,” the connective tissue surrounding the joint becomes stiff and inflamed causing extreme pain and greatly restricting movement. • Adult onset Still’s disease – a form of arthritis characterized by high spiking fevers and a salmon- colored rash. Still’s disease is more common in children. • Caplan’s syndrome – an inflammation and scarring of the lungs in people with rheumatoid arthritis who have exposure to coal dust, as in a mine. • Celiac disease – an autoimmune disorder of the small intestine that causes malabsorption of nutrients and can eventually cause osteopenia or osteoporosis. • Dermatomyositis – a connective tissue disease characterized by inflammation of the muscles and the skin. The condition is believed to be caused either by viral infection or an autoimmune reaction. • Diabetic finger sclerosis – a complication of diabetes, causing a hardening of the skin and connective tissue in the fingers, thus causing stiffness. • Duchenne muscular dystrophy – one of the most prevalent types of muscular dystrophy, characterized by rapid muscle degeneration. • Dupuytren’s contracture – an abnormal thickening of tissues in the palm and fingers that can cause the fingers to curl. • Eosinophilic fasciitis (Shulman’s syndrome) – a condition in which the muscle tissue underneath the skin becomes swollen and thick. People with eosinophilic fasciitis have a buildup of eosinophils—a type of white blood cell—in the affected tissue.
    [Show full text]
  • Comorbidities in Psoriasis: the Recognition of Psoriasis As a Systemic Disease and Current Management
    Review Derleme 71 DOI: 10.4274/turkderm.09476 Turkderm-Turk Arch Dermatol Venereology 2017;51:71-7 Comorbidities in psoriasis: The recognition of psoriasis as a systemic disease and current management Psoriazisde komorbiditeler: Psoriazisin sistemik hastalık olarak kabul edilmesi ve güncel yaklaşım Göknur Kalkan Ankara Yıldırım Beyazıt University Faculty of Medicine, Department of Dermatology, Ankara, Turkey Abstract Psoriasis, with a worldwide prevalence of 2-3%, is now assumed as a systemic chronic inflammatory disease accompanied by comorbidities while it was accepted as a disease limited only to the skin in the past. There are several classifications of the comorbidities which are more common in patients with moderate to severe psoriasis. Simply, comorbidities can be classified as classic, emerging, related to lifestyle, related to treatment. They can also be categorized as medical comorbidities, psychiatric/psychologic comorbidities, and behaviors contributing to medical and psychiatric comorbidities. In this review, providing early diagnosis and treatment of comorbidities, learning screening recommendations for early detection and long-term disease control and improvement in life quality by integrated, multidisciplinary approach were targeted. Keywords: Psoriasis, comorbidity, systemic disease Öz Tüm dünyada %2-3 oranında görülme sıklığına sahip psoriazis, geçmişte sadece deriye sınırlı kabul edilirken, günümüzde birçok komorbiditenin eşlik ettiği kronik sistemik enflamatuvar bir hastalık olarak ele alınmaktadır. Orta-şiddetli düzeyde
    [Show full text]
  • 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.
    [Show full text]
  • 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.
    [Show full text]