DOCSLIB.ORG

Antineutrophil Cytoplasmic Antibodies, Abnormal

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Antineutrophil Cytoplasmic Antibodies, Abnormal British Journal of Rheumatology 1996;35:958-964 ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES, ABNORMAL ANGIOGRAMS AND PATHOLOGICAL FINDINGS IN POLYARTERTTIS NODOSA AND CHURG-STRAUSS SYNDROME: INDICATIONS FOR THE CLASSIFICATION OF VASCULITIDES OF THE POLYARTERTTIS NODOSA GROUP* L. GUILLEVIN, F. LHOTE, J. AMOUROUX,* R. GHERARDI,t P. CALLARDJ: and P. CASASSUS Service de Medecine Interne, *Service (TAnatomo-pathologie, Hopital Avicenne, Bobigny, ^Service de Neuropathologie, Hopital Henri Mondor, Creteil and %Service a"Anatomo-pathologie, Hopital Tenon, Paris, France Downloaded from https://academic.oup.com/rheumatology/article/35/10/958/1782474 by guest on 30 September 2021 SUMMARY The present study attempted to define the clinical, radiological, immunological and pathological characteristics of microscopic polyangiitis (MPA), and to separate them from classic PAN (c-PAN) and Churg-Strauss syndrome (CSS). In most cases, patients presenting microaneurysms and/or multiple vessel stenoses, which reflect medium-sized vessel involvement, did not have antineutrophil cytoplasmic antibodies (ANCA) (6.6%). Conversely, patients with glomerulonephritis almost never had abnormal angiograms. Furthermore, the clinical characteristics of ANCA-positive patients also indicate small-sized vessel involvement. Skin involvement (73.1 vs 26.7%, P < 0.05), glomcrulonephritis (38.5 vs 0%, P sg 0.001) and the presence of ANCA (34.6 vs 6.7%, P ^ 0.05) were significantly more frequent in patients with normal than abnormal angiograms, respectively. Conversely, hypertension (66.7 vs 23.1%, P sg 0.02), renal vasculitis (46.7 vs 0%, P sg 0.001) and hepatitis B antigenaemia (60 vs 11.5%, P < 0.01) were significantly more common in patients with abnormal angiograms. Stratification of patients according to vessel size showed that, except for skin involvement (P ^ 0.05) and glomerulonephritis (P < 0.01), which are direct manifestations of small-sized vessel diseases, clinical symptoms of PAN or CSS, angiographic findings and ANCA were not correlated to arteriole size. Although at present it is not possible to separate definitively MPA from c-PAN, our results show that ANCA should be considered diagnostic for MPA and, in most cases, should be an exclusion criterion for c-PAN. Conversely, small-sized vessel involvement can be observed in patients presenting characteristics of c-PAN, MPA or CSS and, therefore, is not a sufficient criterion for assigning diagnosis. KEY WORDS: Polyarteritis nodosa, Microscopic polyangiitis, Churg-Strauss syndrome, ANCA. POLYARTERITIS nodosa (PAN) comprises a variety of clinical manifestations are very similar to those of necrotizing angiitides that are not homogeneous. This PAN, but it is characterized by the presence of rapidly vasculitis group includes Churg-Strauss syndrome progressive glomerulonephritis (RPGN), which is (CSS) but, according to a recent classification [1], it nearly constant and pulmonary involvement usually can be subdivided into classic polyarteritis nodosa absent in c-PAN [3]. Davson et al. [4] attempted to (c-PAN), microscopic polyangiitis (MPA), formerly characterize MPA more clearly as early as 1948, by microscopic polyarteritis, and CSS. c-PAN was first noting its constant association with glomerulo- described by Kussmaul and Maier [2]. The main nephritis, unlike patients with c-PAN who only manifestations of this well-known form of necrotizing occasionally develop vascular nephropathy. CSS is a angiitis are weight loss, fever, asthenia, peripheral disorder characterized by hypereosinophilia and neuropathy, renal involvement, musculoskeletal and systemic necrotizing vasculitis similar to that of PAN, cutaneous manifestations, hypertension, gastrointest- and occurring in individuals with asthma and allergic inal tract involvement and cardiac failure [3]. Primary rhinitis. and secondary vasculitides can be distinguished by Recently, the Chapel Hill Consensus Conference considering the causes of PAN, because c-PAN can be nomenclature [1] attempted to define a standardized the consequence of hepatitis B virus (HBV) infection nomenclature system for some of the most common and sometimes of other aetiological agents. Micro- forms of non-infectious systemic vasculitis and to scopic polyangiitis, recently individualized from PAN, determine fundamental definitions for the vasculitides is a systemic vasculitis of small-sized vessels whose so named. The conference authors classified 10 selected vasculitic entities of vasculitis into three groups: large-vessel vasculitis, medium-sized vessel vasculitis 'Presented at the 6th International ANCA Workshop, Paris, and small-vessel vasculitis. The proposed distinguish- France, 28 June-1 July 1995. ing feature for c-PAN versus MPA is the absence Submitted 14 September 1995; revised version accepted 19 April versus the presence of vasculitis in arterioles, venules or 1996. capillaries. Small-vessel involvement, when present, is Correspondence to: L. Guillevin, Service de Medecine Interne, Hopital Avicenne, 125 rue de Stalingrad, 93009 Bobigny cedex, the definitive diagnostic criterion of MPA and excludes France. the diagnosis of PAN even if medium-sized artery © 1996 British Society for Rheumatology 958 GUILLEVIN ET AL.: DIAGNOSIS AND CLASSIFICATION OF VASCULITIDES 959 lesions are also seen. Other clinical, biological and PATIENTS AND METHODS histological criteria are not taken into consideration. Patients ANCA positivity and the presence or absence of Patients included in the study had systemic PAN or microaneurysms and/or stenoses were not considered CSS diagnosed clinically by the presence of multiple to be diagnostic criteria. However, correlation be- system involvement and histological evidence of tween vessel size, ANCA and angiograms has not vascular lesions indicative of vasculitis (focal or been established, and classification criteria need to segmental vascular lesions, fibrinoid necrosis and/or be validated. This nomenclature has the advantage pleomorphic inflammatory cell infiltration of the of emphasizing the existence of MPA, but the arterial wall). Only patients with symptoms of recent disadvantage of giving it too large a place in the onset were included and all explorative examinations family of vasculitides. Small-vessel involvement is were made before the initiation of treatment. Patients Downloaded from https://academic.oup.com/rheumatology/article/35/10/958/1782474 by guest on 30 September 2021 present in c-PAN, and the publication of Kussmaul with cutaneous PAN or limited forms of the disease and Maier [2] included a description of capillary were excluded from the study, as were patients with vasculitis existing with small- and medium-sized other forms of systemic vasculitides. vessel vasculitis lesions. Histological findings of vasculitis, in nerve for instance, are common to Methods several necrotizing angiopathies. Small-vessel in- Patients were selected from the database of the volvement is usually found in epineural arteries, and French Polyarteritis Nodosa Study Group. Only may be concurrent with skin nodules and purpura, patients subjected to ANCA detection and angiogra- which are present in patients with classic PAN. In phy, and in whom vaculitis was observed in biopsies, this study, we attempted to correlate pathological, were included in this study. All of them had been angiographic findings and the presence of ANCA in included in prospective trials, the results of which have patients with PAN and CSS, and to establish been published elsewhere [5, 6]. Abdominal aortogra- whether vessel size and/or ANCA and/or other phy with renal, coeliac and superior mesenteric angiographic findings could be considered a criterion arteriography were performed on every patient. The sufficient to diagnose MPA and/or to exclude arterial changes were classified as follows: normal, c-PAN. stenosis, occlusion and/or aneurysm. Both fusiform TABLE I Oinical manifestations and laboratory data on patients with PAN and CSS classified according to angiography results and size of affected vessels Angiography Histology Abdominal aneurysms Vessel Vessel Normal and/or stenoses P <200 fim >200 jim P Total Parameter (/) = 26) («-15) < (n = 26) (n - 15) < (n-41) Age (yr), mean ± S.D. 56.8 ± 16 45.6 ± 17 51.7 ± 15 54.5 ± 20.6 Sex ratio, M/F 19 M, 7 F 10 M, 5 F 21 M, 5 F 8 M, 7 F Weight loss 19 (73.1) 14 (93.3) ns 19(73.1) 14 (93.3) ns 33 Fever 16 (61.5) 9(60) ns 16(61.5) 9(60) ns 25 Abdominal symptoms 14 (53.8) 10 (66.7) ns 14 (53.8) 10 (66.7) ns 24 Peripheral neuropathy 16 (61.5) 9(60) ns 15 (57.7) 10 (66.7) ns 25 Arthralgias 16 (61.5) 6(40) ns 14 (53.8) 8 (53.3) ns 22 Skin involvement 19 (73.1) 4 (26.6) 0.05 18 (69.2) 5 (33.3) 0.05 23 Hypertension 6 (23.1) 10 (66.7) 0.02 12 (46.2) 4 (26.7) ns 16 Myalgias 17 (65.4) 6(40) ns 14 (53.8) 9(60) ns 23 Kidney involvement 10 (38.5) 7 (46.7) ns 13(50) 4 (26.7) ns 17 Renal vasculitis 0 7(46.7) 0.001 3(11.5) 4 (26.7) ns 7 Glomerulonephritis 10 (38.5) 0 0.001 10 (38.5) 0 0.01 10 Lung involvement 5 (19.2) 1 (6.7) ns 3(11.5) 3(20) ns 6 Asthma 5 (19.2) 1 (6.7) ns 3(11.5) 3(20) ns 6 Alveolar haemorrhage 0 0 ns 0 0 ns 0 Cardiac involvement (except pericarditis) 4 (15.4) 0 ns 2 (7.7) 2 (13.3) ns 4 Orchitis 0 4(40) 0.01 4(19) 0 ns 4 CNS involvement 3(11.5) 0 ns 2 (7.6) 0 ns 5 ANCA (IFA) 7 p, 2 c 1 p-ANCA 0.05 5 p, 2 c-ANCA 3 p-ANCA ns 10 ANCA (ELISA) 6 anti-MPO, 1 anti-MPO 5 anti-MPO, 3 anti-MPO 2 anti-PR3* 1 PR3* Creatininaemia (/imol/1) 148 ±183 151 ± 112 ns 167 ± 196 118 ±73 ns Hepatitis B antigen 3(11.5) 9(60) 0.01 7 (26.9) 5 (33.3) ns 12 p, perinuclear staining pattern; c, cytoplasmic staining pattern; MPO, myeloperoxidase; PR3, proteinase 3. ns, non-significant.
Load more

Recommended publications
  • 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]
  • 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]
  • 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.
    [Show full text]
  • 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
    [Show full text]
  • Giant Cell Arteritis of the Female Genital Tract With
    900 Annals ofthe Rheumatic Diseases 1992; 51: 900-903 CASE REPORTS Ann Rheum Dis: first published as 10.1136/ard.51.7.900 on 1 July 1992. Downloaded from Giant cell arteritis of the female genital tract with temporal arteritis Franqois Lhote, Claire Mainguene, Valerie Griselle-Wiseler, Renato Fior, Marie-Jose Feintuch, Isabelle Royer, Bernard Jarrousse, Jacques Amouroux, Loic Guillevin Abstract are rarely affected by the inflammatory process 2 The clinical and pathological features of a Most examples of giant cell arteritis of the patient with giant cell arteritis of the uterus genital tract are incidental findings in samples and ovaries are described. A 61 year old removed during operations. We describe here woman had fever and weight loss over a the clinical and pathological features of giant period of eight months. A hysterectomy with cell arteritis, fortuitously discovered in the bilateral salpingo-oophorectomy was per- uterus and ovaries, and preceding the diagnosis formed for a large cystic ovarian mass. of temporal arteritis which was proved by Histological examination showed a benign taking a biopsy sample. In view of similar cases ovarian cyst and unexpected giant cell arteritis reported previously, we discuss the relation affecting numerous small to medium sized between giant cell arteritis of the female genital arteries in the ovaries and myometrium. The tract, temporal arteritis, and polymyalgia diagnosis of temporal arteritis was confirmed rheumatica. Service de Medecine Interne, by a random temporal artery biopsy, despite Hopital Avicenne, the absence of symptoms oftemporal arteritis. 125 route de Stalingrad, This observation is compared with previously Case report 93009 Bobigny Cedex, cases was to us in France reported and the relation between A 61 year old white woman referred F Lhote granulomatous arteritis of the genital tract January 1991 because of a fever of unknown V Griselle-Wiseler and temporal arteritis is discussed.
    [Show full text]
  • Giant Cell Arteritis
    GIANT CELL ARTERITIS What is giant cell arteritis (GCA)? Giant cell arteritis (GCA) is a form of vasculitis—a family of rare disorders characterized by inflammation of the blood vessels, which can restrict blood flow and damage vital organs and tissues. Also called temporal arteritis, GCA typically affects the arteries in the neck and scalp, especially the temples. It can also affect the aorta and its large branches to the head, arms and legs. GCA is the most common form of vasculitis in adults over the age of 50. The most common symptoms of GCA include persistent, throbbing headaches, tenderness of the temples and scalp, jaw pain, fever, joint pain, and vision problems. Early treatment is vital to prevent serious complications such as blindness or stroke. GCA is typically treated with high doses of corticosteroids such as prednisone, sometimes in combination with other medications that suppress the immune system. Prompt treatment usually relieves symptoms, however GCA is a chronic condition with periods of relapse and remission, so ongoing medical care is usually necessary. Patients with GCA may also have symptoms of polymyalgia rheumatica (PMR), a closely related inflammatory disorder. Causes The cause of GCA is not yet fully understood by researchers. Vasculitis is classified as an autoimmune disorder—a disease which occurs when the body’s natural defense system mistakenly attacks healthy tissues. Researchers believe a combination of factors may trigger the inflammatory process. Studies have linked genetic factors, infectious agents, and a prior history of cardiovascular disease to the development of GCA. Who gets GCA? GCA is the most common form of vasculitis in older adults, affecting people over 50 years of age, with an average onset of 74 years of age.
    [Show full text]
  • Polyarteritis Nodosa
    POLYARTERITIS NODOSA What is polyarteritis nodosa (PAN)? Polyarteritis nodosa (PAN) is a form of vasculitis—a family of rare diseases characterized by inflammation of the blood vessels, which can restrict blood flow and damage vital organs and tissues. PAN affects medium-sized blood vessels that supply the skin, nervous system, joints, kidneys, gastrointestinal (GI) tract, and heart, among other organs. Depending on the form of the disease, PAN may affect only the skin, a single body organ, or multiple organ systems. When PAN is systemic (affecting the whole body), symptoms can be wide-ranging, from fever, fatigue, weakness and weight loss, to muscle and Joint aches, skin lesions, numbness, and abdominal pain. High blood pressure is common due to kidney damage. Individuals with PAN are also at risk for an aneurysm—an abnormal bulge in a weakened artery wall that can rupture or hemorrhage. Prompt diagnosis and treatment are essential to prevent serious complications associated with PAN. The standard course of treatment includes corticosteroids such as prednisone used in combination with other medications that suppress the immune system. Even with effective treatment, PAN can be a chronic condition with periods of relapse and remission, so ongoing medical care is necessary. Causes The exact cause of PAN is not fully understood by researchers. Vasculitis is classified as an autoimmune disorder, a disease which occurs when the body’s natural defense system mistakenly attacks healthy tissue. The inflammatory process may be set in motion by a reaction to certain drugs or vaccines, or a bacterial or viral infection. PAN has been associated with hepatitis B infection.
    [Show full text]
  • Coronary Vasculitis
    biomedicines Review Coronary Vasculitis Tommaso Gori Kardiologie I and DZHK Standort Rhein-Main, Universitätsmedizin Mainz, 55131 Mainz, Germany; [email protected] Abstract: The term coronary “artery vasculitis” is used for a diverse group of diseases with a wide spectrum of manifestations and severity. Clinical manifestations may include pericarditis or my- ocarditis due to involvement of the coronary microvasculature, stenosis, aneurysm, or spontaneous dissection of large coronaries, or vascular thrombosis. As compared to common atherosclerosis, patients with coronary artery vasculitis are younger and often have a more rapid disease progression. Several clinical entities have been associated with coronary artery vasculitis, including Kawasaki’s disease, Takayasu’s arteritis, polyarteritis nodosa, ANCA-associated vasculitis, giant-cell arteritis, and more recently a Kawasaki-like syndrome associated with SARS-COV-2 infection. This review will provide a short description of these conditions, their diagnosis and therapy for use by the practicing cardiologist. Keywords: coronary artery disease; vasculitis; inflammatory diseases 1. Introduction The term vasculitis refers to a group of conditions whose pathophysiology is mediated by inflammation of blood vessels. Most forms of vasculitis are systemic and may present variable clinical manifestations, requiring a multidisciplinary approach. A number of etiologies have been reported; independently of the specific organ involvement, vasculitis Citation: Gori, T. Coronary can be primary or secondary to another autoimmune disease or can be associated with Vasculitis. Biomedicines 2021, 9, 622. other precipitants such as drugs, infections or malignancy [1]. Almost all cases of coronary https://doi.org/10.3390/ vasculitis appear as a manifestation of systemic (primary) vasculitis, which are classified biomedicines9060622 based on the type and size of the vessels affected and the cellular component responsible for the tissue infiltration.
    [Show full text]
  • 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa
    Arthritis Care & Research Vol. 73, No. 8, August 2021, pp 1061–1070 DOI 10.1002/acr.24633 © 2021 American College of Rheumatology. This article has been contributed to by US Government employees and their work is in the public domain in the USA. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa Sharon A. Chung,1 Mark Gorelik,2 Carol A. Langford,3 Mehrdad Maz,4 Andy Abril,5 Gordon Guyatt,6 Amy M. Archer,7 Doyt L. Conn,8 Kathy A. Full,9 Peter C. Grayson,10 Maria F. Ibarra,11 Lisa F. Imundo,2 Susan Kim,1 Peter A. Merkel,12 Rennie L. Rhee,12 Philip Seo,13 John H. Stone,14 Sangeeta Sule,15 Robert P. Sundel,16 Omar I. Vitobaldi,17 Ann Warner,18 Kevin Byram,19 Anisha B. Dua,7 Nedaa Husainat,20 Karen E. James,21 Mohamad Kalot,22 Yih Chang Lin,23 Jason M. Springer,4 Marat Turgunbaev,24 Alexandra Villa-Forte, 3 Amy S. Turner,24 and Reem A. Mustafa25 Guidelines and recommendations developed and/or endorsed by the American College of Rheumatology (ACR) are intended to provide guidance for particular patterns of practice and not to dictate the care of a particular patient. The ACR considers adherence to the recommendations within this guideline to be volun- tary, with the ultimate determination regarding their application to be made by the physician in light of each patient’s individual circumstances. Guidelines and recommendations are intended to promote beneficial or desirable outcomes but cannot guarantee any specific outcome. Guidelines and recommendations developed and endorsed by the ACR are subject to periodic revision as warranted by the evolution of medical knowledge, technology, and practice.
    [Show full text]
  • EULAR/PRINTO/PRES Criteria for Henoch–Schönlein
    Criteria Ann Rheum Dis: first published as 10.1136/ard.2009.116657 on 22 April 2010. Downloaded from EULAR/PRINTO/PRES criteria for Henoch–Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classifi cation criteria Seza Ozen,1 Angela Pistorio,2 Silvia M Iusan,3 Aysin Bakkaloglu,1 Troels Herlin,4 Riva Brik,5 Antonella Buoncompagni,3 Calin Lazar,6 Ilmay Bilge,7 Yosef Uziel,8 Donato Rigante,9 Luca Cantarini,10 Maria Odete Hilario,11 Clovis A Silva,12 Mauricio Alegria,13 Ximena Norambuena,14 Alexandre Belot,15 Yackov Berkun,16 Amparo Ibanez Estrella,17 Alma Nunzia Olivieri,18 Maria Giannina Alpigiani,19 Ingrida Rumba,20 Flavio Sztajnbok,21 Lana Tambic-Bukovac,22 Luciana Breda,23 Sulaiman Al-Mayouf,24 Dimitrina Mihaylova,25 Vyacheslav Chasnyk,26 Claudia Sengler,27 Maria Klein-Gitelman,28 Djamal Djeddi,29 Laura Nuno,30 Chris Pruunsild,31 Jurgen Brunner,32 Anuela Kondi,3 Karaman Pagava,33 Silvia Pederzoli,3 Alberto Martini,3,34 Nicolino Ruperto3; for the Paediatric Rheumatology International Trials Organisation (PRINTO) ▶ Additional data are published ABSTRACT Paediatric Rheumatology European Society propose online only at http://ard.bmj. Objectives To validate the previously proposed validated classifi cation criteria for HSP, c-PAN, c-WG and com/content/vol69/issue5 classifi cation criteria for Henoch–Schönlein purpura c-TA with high sensitivity/specifi city. For numbered affi liations see (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener end of the article granulomatosis (c-WG) and c-Takayasu arteritis (c-TA). Methods Step 1: retrospective/prospective web- INTRODUCTION Correspondence to data collection for children with HSP, c-PAN, c-WG and In 1990 the American College of Rheumatology Professor Seza Ozen, Hacettepe ≤ University Children’s Hospital, c-TA with age at diagnosis 18 years.
    [Show full text]
  • Review Polyarteritis Nodosa Revisited
    Review Polyarteritis nodosa revisited: a review of historical approaches, subphenotypes and a research agenda O. Karadag1,2, D.J. Jayne1 1Department of Medicine, University of ABSTRACT However, the majority of PAN cases Cambridge, United Kingdom; Polyarteritis nodosa (PAN) is a rare reflect a variety of phenotypes of un- 2 Hacettepe University Vasculitis Centre, form of primary systemic vasculitis known cause and lack a system of phe- Ankara, Turkey. with heterogeneous presentations, treat- notypic subclassification. PAN can oc- Omer Karadag, MD ments and disease course. Historical cur at any age with wide variability in David J. Jayne, MD approaches to classification and diag- organ involvement, although survival Please address correspondence to: nostic terminology are reviewed. Since and recommended treatment strategies Prof. Omer Karadag, differentiation of PAN from microscopic Hacettepe Universitesi Tip Fakultesi, broadly reflect severity. We propose that Romatoloji Bilim Dali, polyangiitis (MPA) and other ANCA vas- subphenotypes within PAN having dif- 06100 Sihhiye-Altındag, culitides by the Chapel Hill conference ferent clinical courses may be different Ankara, Turkey. statements, and with hepatitis associated diseases rather than a spectrum of the E-mail: [email protected]; PAN defined as a secondary vasculitis, same disease. There is a need to better [email protected] the phenotyping and subclassification of understand these subphenotypes to un- Received on December 27, 2017; PAN has received little attention. Mono- derpin further clinical and translational accepted on January 17, 2018. genic disorders similar to PAN have been research and improve patient outcomes. Clin Exp Rheumatol 2018; 36 (Suppl. 111): described (familial Mediterranean fever, This paper aims to assess the heteroge- S135-S142.
    [Show full text]
  • Cutaneous Vasculitis
    Cutaneous Vasculitis Authors: Lorinda Chung, M.D. and David Fiorentino1, M.D., Ph.D. Creation date: March 2005 Scientific editor: Prof Loïc Guillevin 1Department of Dermatology, Division of Rheumatology and Immunology, Stanford University School of Medicine, 900 Blake Wilbur Drive W0074, Stanford, CA. USA. [email protected] Abstract Keywords Definition Classification / Etiology Approach to the Patient Treatment Future Directions References Abstract Cutaneous vasculitis is a histopathologic entity characterized by neutrophilic transmural inflammation of the blood vessel wall associated with fibrinoid necrosis, termed leukocytoclastic vasculitis (LCV). Clinical manifestations of cutaneous vasculitis occur when small and/or medium vessels are involved. Small vessel vasculitis can present as palpable purpura, urticaria, pustules, vesicles, petechiae, or erythema multiforme-like lesions. Signs of medium vessel vasculitis include livedo reticularis, ulcers, subcutaneous nodules, and digital necrosis. The frequency of vasculitis with skin involvement is unknown. Vasculitis can involve any organ system in the body, ranging from skin-limited to systemic disease. Although vasculitis is idiopathic in 50% of cases, common associations include infections, inflammatory diseases, drugs, and malignancy. The management of cutaneous vasculitis is based on four sequential steps: confirming the diagnosis with a skin biopsy, evaluating for systemic disease, determining the cause or association, and treating based on the severity of disease. Keywords Cutaneous vasculitis, leukocytoclastic vasculitis Definition Vasculitis is inflammation of the blood vessel Classification / Etiology wall that leads to various clinical manifestations The classification schemes for the vasculitides depending on which organ systems are involved. are based on several criteria, including the size Cutaneous vasculitis is a histopathologic entity of the vessel involved, clinical and characterized by neutrophilic transmural histopathologic features, and etiology.
    [Show full text]