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Respiratory Medicine Dec 2018The hallmark of vasculitis is autoimmune inflammation of blood vessels and surrounding tissues, resulting in an array of constitutional symptoms and organ damage. The... (Review)
Review
The hallmark of vasculitis is autoimmune inflammation of blood vessels and surrounding tissues, resulting in an array of constitutional symptoms and organ damage. The lung is commonly targeted in the more familiar ANCA-associated small vessel vasculitidies, but large and medium vessel vasculitides, including Takayasu arteritis, giant cell arteritis, polyarteritis nodosa, Behcet's disease, and necrotizing sarcoid granulomatosis, may also feature prominent pulmonary involvement. Pulmonary manifestations of these conditions include pulmonary arterial aneurysms, pulmonary hypertension, diffuse alveolar hemorrhage, pulmonary nodules, and parenchymal infiltrates. An understanding of the diverse manifestations of vasculitis and a high index of clinical suspicion are essential to avoid delays in disease recognition that may result in permanent or life threatening morbidity. In this review, we outline the general clinical manifestations, pulmonary manifestations, diagnostic workup, imaging findings, and treatment of medium, large, and variable vessel vasculitides.
Topics: Aneurysm; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Behcet Syndrome; Giant Cell Arteritis; Hemorrhage; Humans; Hypertension, Pulmonary; Lung Diseases; Polyarteritis Nodosa; Pulmonary Alveoli; Pulmonary Artery; Takayasu Arteritis
PubMed: 30509707
DOI: 10.1016/j.rmed.2018.11.003 -
Clinical and Experimental Rheumatology 2018Polyarteritis nodosa (PAN) is a rare form of primary systemic vasculitis with heterogeneous presentations, treatments and disease course. Historical approaches to... (Review)
Review
Polyarteritis nodosa (PAN) is a rare form of primary systemic vasculitis with heterogeneous presentations, treatments and disease course. Historical approaches to classification and diagnostic terminology are reviewed. Since differentiation of PAN from microscopic polyangiitis (MPA) and other ANCA vasculitides by the Chapel Hill conference statements, and with hepatitis associated PAN defined as a secondary vasculitis, the phenotyping and subclassification of PAN has received little attention. Monogenic disorders similar to PAN have been described (familial Mediterranean fever, Adenosine Deaminase-2 deficiency), and cutaneous PAN and single organ vasculitis, discussed. The overlapping phenotypes between PAN and other primary vasculitic syndromes and subphenotypes within PAN are explored. This work will underpin development of newer treatment regimens and future genetic and related aetiologic studies.
Topics: Disease Progression; History, 19th Century; History, 20th Century; History, 21st Century; Humans; Phenotype; Polyarteritis Nodosa; Research
PubMed: 29465365
DOI: No ID Found -
International Journal of Molecular... Nov 2023Polyarteritis nodosa (PAN), also known as panarteritis nodosa, represents a form of necrotizing vasculitis that predominantly affects medium-sized vessels, although it... (Review)
Review
Polyarteritis nodosa (PAN), also known as panarteritis nodosa, represents a form of necrotizing vasculitis that predominantly affects medium-sized vessels, although it is not restricted to them and can also involve smaller vessels. The clinical presentation is heterogeneous and characterized by a significant number of patients exhibiting general symptoms, including asthenia, fever, and unintended weight loss. Although PAN can involve virtually any organ, it preferentially affects the skin, nervous system, and the gastrointestinal tract. Orchitis is a rare but specific manifestation of PAN. The absence of granulomas, glomerulonephritis, and anti-neutrophil cytoplasmic antibodies serves to distinguish PAN from other types of vasculitis. Major complications consist of hemorrhagic and thrombotic events occurring in mesenteric, cardiac, cerebral, and renal systems. Historically, PAN was frequently linked to hepatitis B virus (HBV) infection, but this association has dramatically changed in recent years due to declining HBV prevalence. Current epidemiological research often identifies a connection between PAN and genetic syndromes as well as neoplasia. This article provides a comprehensive review of PAN, specifically focusing on the progression of its clinical manifestations over time.
Topics: Male; Humans; Polyarteritis Nodosa; Vasculitis; Hepatitis B; Hepatitis B virus; Gastrointestinal Tract
PubMed: 38068989
DOI: 10.3390/ijms242316668 -
Techniques in Vascular and... Dec 2014The first description of polyarteritis nodosa (PAN) was in 1852 by Karl Rokitansky, a pathologist at the University of Vienna. The initial report describes a 23-year-old... (Review)
Review
The first description of polyarteritis nodosa (PAN) was in 1852 by Karl Rokitansky, a pathologist at the University of Vienna. The initial report describes a 23-year-old man who had a 5-day history of fever and diarrhea. Since then, the definition of PAN has evolved. The currently accepted definition of PAN comes from the 2012 Chapel Hill Conference, which classified PAN as a necrotizing arteritis not associated with antineutrophil cytoplasmic antibodies of medium or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries, or venules.
Topics: Adult; Diagnostic Imaging; Female; Humans; Male; Middle Aged; Polyarteritis Nodosa
PubMed: 25770638
DOI: 10.1053/j.tvir.2014.11.005 -
Pediatric Nephrology (Berlin, Germany) Sep 2010Medium-size-artery vasculitides do occur in childhood and manifest, in the main, as polyarteritis nodosa (PAN), cutaneous PAN and Kawasaki disease. Of these, PAN is the... (Review)
Review
Medium-size-artery vasculitides do occur in childhood and manifest, in the main, as polyarteritis nodosa (PAN), cutaneous PAN and Kawasaki disease. Of these, PAN is the most serious, with high morbidity and not inconsequential mortality rates. New classification criteria for PAN have been validated that will have value in epidemiological studies and clinical trials. Renal involvement is common and recent therapeutic advances may result in improved treatment options. Cutaneous PAN is a milder disease characterised by periodic exacerbations and often associated with streptococcal infection. There is controversy as to whether this is a separate entity or part of the systemic PAN spectrum. Kawasaki disease is an acute self-limiting systemic vasculitis, the second commonest vasculitis in childhood and the commonest cause of childhood-acquired heart disease. Renal manifestations occur and include tubulointerstitial nephritis and renal failure. An infectious trigger and a genetic predisposition seem likely. Intravenous immunoglobulin (IV-Ig) and aspirin are effective therapeutically, but in resistant cases, either steroid or infliximab have a role. Greater understanding of the pathogenetic mechanisms involved in these three types of vasculitis and better long-term follow-up data will lead to improved therapy and prediction of prognosis.
Topics: Child; Child, Preschool; Disease Progression; Female; Heart Diseases; Humans; Kidney Diseases; Male; Mucocutaneous Lymph Node Syndrome; Polyarteritis Nodosa; Treatment Outcome
PubMed: 19946711
DOI: 10.1007/s00467-009-1336-1 -
Proceedings of the Royal Society of... Apr 1953
Topics: Child; Humans; Infant; Polyarteritis Nodosa
PubMed: 13055885
DOI: No ID Found -
Journal of the National Medical... Mar 1948
Topics: Humans; Polyarteritis Nodosa
PubMed: 18857080
DOI: No ID Found -
British Medical Journal May 1947
Topics: Humans; Polyarteritis Nodosa
PubMed: 20248101
DOI: 10.1136/bmj.1.4507.738 -
Proceedings of the Royal Society of... Nov 1949
Topics: Humans; Polyarteritis Nodosa
PubMed: 15394808
DOI: No ID Found -
BMC Cardiovascular Disorders Jan 2021Cardiac vasculitis is recognized as a heterogeneous disease process with a wide spectrum of manifestations including pericarditis, myocarditis, valvular heart disease... (Review)
Review
Cardiac vasculitis is recognized as a heterogeneous disease process with a wide spectrum of manifestations including pericarditis, myocarditis, valvular heart disease and less frequently, coronary artery vasculitis (CAV). CAV encompasses an emerging field of diseases which differ from conventional atherosclerotic disease and have a proclivity for the younger population groups. CAV portends multiple complications including the development of coronary artery aneurysms, coronary stenotic lesions, and thrombosis, all which may result in acute coronary syndromes. There are several aetiologies for CAV; with Kawasaki's disease, Takayasu's arteritis, Polyarteritis Nodosa, and Giant-Cell Arteritis more frequently described clinically, and in literature. There is a growing role for multi-modality imaging in assisting the diagnostic process; including transthoracic echocardiography, cardiac magnetic resonance imaging, computed tomography coronary angiography, fluorodeoxyglucose-positron emission tomography and conventional coronary angiogram with intravascular ultrasound. Whilst the treatment paradigms fundamentally vary between different aetiologies, there are overlaps with pharmacological regimes in immunosuppressive agents and anti-platelet therapies. Interventional and surgical management are is a consideration in select populations groups, within a multi-disciplinary context. Further large-scale studies are required to better appropriately outline management protocols in this niche population.
Topics: Cardiac Imaging Techniques; Coronary Artery Disease; Giant Cell Arteritis; Humans; Mucocutaneous Lymph Node Syndrome; Multimodal Imaging; Polyarteritis Nodosa; Predictive Value of Tests; Prognosis; Takayasu Arteritis
PubMed: 33407141
DOI: 10.1186/s12872-020-01813-6