Polyarteritis nodosa pan is a systemic necrotizing vasculitis that typically affects mediumsized muscular arteries, with occasional involvement of small muscular arteries 1,2. Disebabkan oleh kompleks imun dalam sirkulasi yang ada dalam membran basalis glomerulus dan menimbulkan kerusakan price, 2006. Lupus eritematosus sistemik, poliarteritis nodosa, sklerosis sistemik progresif systemic lupus erytematosus sle atau lupus eritematosus sistemik les adalah penyakit radang atau inflamasi multisistem yang penyebabnya diduga karena adanya perubahan sistem imun. Pagnoux c, seror r, henegar c, mahr a, cohen p, le guern v, et al. Cutaneous polyarteritis nodosa cpan was first described in 1931. Polyarteritis nodosa genetic and rare diseases information. Started in 1995, this collection now contains 6769 interlinked topic pages divided into a tree of 31 specialty books and 732 chapters. Introduction polyarteritis nodosa pan is a systemic necrotizing vasculitis that predominantly affects mediumsized muscular arteries and often involves small muscular arteries. Deaths occurring during the first year after treatment onset for polyarteritis nodosa, microscopic polyangiitis, and churgstrauss syndrome. Pan most commonly affects vessels related to the skin, joints, peripheral nerves, gastrointestinal tract, heart, eyes, and kidneys. Previously, it had been referred to as infantile polyarteritis nodosa ipan.
Gejala awal yang paling sering ditemukan adalah demam. The most common areas of involvement include the muscles, joints, intestines bowels, nerves, kidneys, and skin. Internationally, the annual estimated incidence of pan ranges from 1. What is cutaneous polyarteritis nodosa cutaneous polyarteritis nodosa pan is a rare form of vasculitis inflammation of blood vessels that involves small and mediumsized arteries of the dermis and subcutaneous tissue. Polyarteritis nodosa nord national organization for.
Stone, md, mph case presentation a 30yearold man was referred for evaluation and treatment of still disease. Polyarteritis nodosa symptoms, diagnosis and treatment. A serious blood vessel disease where small and mediumsized arteries become swollen and damaged and are unable to adequately supply oxygenated blood to various tissues in the body. His illness had begun 12 years earlier when, as a high school senior, he developed daily temperatures of 38. How does it relate to systemic polyarteritis nodosa although identical skin lesions are common in systemic pan. Poliarteritis nodosa klasik adalah vaskulitis sistemik pertama telah dijelaskan sebelumnya. Dec 03, 2018 classic polyarteritis nodosa pan or cpan is a systemic vasculitis characterized by necrotizing inflammatory lesions that affect mediumsized and small muscular arteries, preferentially at vessel bifurcations, resulting in microaneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, and, consequently, organ ischemia or infarctio.
Sep 14, 2015 bourgarit a, le toumelin p, pagnoux c, et al. Penyakit peradangan kronik dimana sistem imun dalam tubu menyerang. Polyarteritis nodosa definition of polyarteritis nodosa by. Pan most commonly affects vessels related to the skin, joints, peripheral nerves. Baseline evaluation prior to treatment for polyarteritis nodosa pan, patients should undergo a careful history, physical examination, and laboratory evaluation. The approach to treatment of pan depends upon the following variables, which require assessment before beginning therapy. Polyarteritis nodosa or pan is a rare form of autoimmune disease characterized by inflammation of small and mediumsized arteries. Apr 18, 2014 polyarteritis nodosa pan usmle step 1 pathology case based discussion duration. Apr 25, 2018 childhood polyarteritis nodosa cpan is a rare and often fatal disease that affects small and medium arteries. Polyarteritis nodosa pan is a rare disease that results from blood vessel inflammation vasculitis causing injury to organ systems.
Clinical features and outcomes in 348 patients with polyarteritis nodosa. Livedo pattern of the lower legs with purpura and subcutaneous nodules on the bilateral legs. Most cases occur in the 4th or 5th decade of life, although it can occur at any age. The first description of polyarteritis nodosa pan was in 1852 by karl rokitansky, a pathologist at the university of vienna. Salah satu contohnya adalah sindrom churgstrauss, yang membedakannya dengan poliarteritis nodosa adalah bahwa pada sindrom ini terjadi asma. Pan affects mediumsized blood vessels that supply the skin, nervous system, joints, kidneys, gastrointestinal gi tract, and heart, among other. Polyarteritis nodosa pan is a systemic inflammatory necrotizing vasculitis that involves small to mediumsized arteries larger than arterioles. Following an extensive evaluation, he was given the diag. It primarily affects small and medium arteries, which can become inflamed or damaged. Polyarteritis nodosa symptoms, diagnosis, treatments and. Blood vessels in any organ or organ system may be affected, including those supplying the kidneys, heart, intestine, nervous system, andor skeletal muscles.
Polyarteritis nodosa pan is a rare disease that makes your blood vessels swell. It can affect the blood vessels that go to almost every part of your body, including your heart, kidneys, and. In polyarteritis nodosa, small aneurysms are strung like the beads of a rosary, therefore. The use of the term infantile is too restrictive, as infancy connotes age 1 year or younger.
It is categorized as a mediumvessel vasculitis chapel hill, 2012. Hal ini berdasarkan fakta bahwa kasus poliarteritis nodosa timbul setelah terjadi reaksi alergik terhadap obat sulfa, penisilin, yodium, tiourasil, dan sebagainya antigenemia hbsag dan infeksi bakteri. Patients can present with systemic and focal symptoms. Polyarteritis nodosa is a rare autoimmune disease characterized by spontaneous inflammation of the arteries of the body. Diagnosis and classification of polyarteritis nodosa. Pan is a rare form of vasculitis, and the precise frequency of this disease is difficult to determine. Adolf kussmaul february 22, 1822may 28, 1902 and his relevance to medicine in the 21st century. The initial report describes a 23 yearold man who had a fiveday history of fever and diarrhea. The major environmental factor associated with pan is hbv infection.
The age of onset ranges from childhood to late adulthood but averages 40 years. Unlike some other vasculitides eg, microscopic polyarteritis, granulomatosis with polyangiitis wegeners, polyarteritis nodosa is not associated with. The initial report describes a 23yearold man who had a 5day history of fever and diarrhea. Cutaneous polyarteritis nodosa often starts in childhood or adolescence. Polyarteritis nodosa pan, is a systemic necrotizing inflammation of blood vessels vasculitis affecting mediumsized muscular arteries, typically involving the arteries of the kidneys and other internal organs but generally sparing the lungs circulation. Tell a friend about us, add a link to this page, or visit the webmasters page for free fun content. Poor function or pain in any of these organs can be a symptom. Poliarteritis nodosa vasculitis enfermedades reumaticas. It is found to occur in all age groups but it is more common. The disease can occur in a mild form or a serious, rapidly fatal form. Inflammation of the blood vessels may affect different organs including intestine, heart, kidney and nerves, and may be life threatening in certain cases. Polyarteritis nodosa pan usmle step 1 pathology case based discussion duration. Polyarteritis nodosa is a rare disease resulting from vasculitis, or blood vessel inflammation. Diduga infeksi dan mekanisme hipersensitivitas memegang peran dalam patogenesisnya.
Blefaritis skuamosa adalah blefaritis disertai terdapatnya skuama atau krusta pada pangkal bulu mata yang bila dikupas tidak mengakibatkan terjadinya luka kulit. Poliarteritis nodosa sering menyerupai penyakit lain, di mana terjadi peradangan arteri vaskulitis. Small arteries may also be involved but small vessels, including arterioles, capillaries and venules, are characteristically spared 1. Polyarteritis nodosa nord national organization for rare. Polyarteritis nodosa pan is a condition that causes swollen arteries. In most cases of cutaneous pan, the disease is triggered by certain infections, particularly group a streptococcus, hepatitis b, hepatitis c, human immunodeficiency virus, parvovirus b19 the cause of fifth disease. Gangguan jaringan ikat misalnya lupus eritematosus sistemik, poliarteritis nodosa, sklerosis sistemik progresif.
Polyarteritis nodosa pan is a blood vessel disease characterized by inflammation of small and mediumsized arteries, preventing them from bringing oxygen and food to organs. Polyarteritis nodosa has been associated with active hepatitis b, hepatitis c, or both. Polyarteritis nodosa is a small and mediumsized arteritis affecting multiple organs, especially the skin, peripheral nerve, gut, kidney, and heart. Polyarteritis nodosa pan is a systemic necrotizing vasculitis predominantly targeting mediumsized arteries. Most studies have shown no significant gender predominance. Polyarteritis nodosa may be similar to hypersensitivity angitis, churgstrauss syndrome, cogans syndrome, kawasakis disease and complications associated with methamphetamine addiction, hepatitis b and c infections and other liver conditions. Cutaneous polyarteritis nodosa successfully treated with. Polyarteritis nodosa is an autoimmune disease that affects arteries.
The term periarteritis nodosa was used in the 19th century to describe any form of systemic vasculitis without a known cause. Pdf poliarteritis nodosa cutanea posestreptococica. Classic polyarteritis nodosa pan or cpan is a systemic vasculitis characterized by necrotizing inflammatory lesions that affect mediumsized and small muscular arteries, preferentially at vessel bifurcations, resulting in microaneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, and, consequently, organ ischemia or infarctio. Treatment is directed toward decreasing the inflammation of the arteries.
Unlike some other vasculitides eg, microscopic polyarteritis, granulomatosis with polyangiitis, polyarteritis nodosa is not associated with antineutrophil cytoplasmic antibodies anca. Pan is more common in males and typically presents around the 5 th to 7 th decades. Polyarteritis nodosa pan is a form of vasculitisa family of rare diseases characterized by inflammation of the blood vessels, which can restrict blood flow and damage vital organs and tissues. Polyarteritis nodosa pan is a necrotizing vasculitis resulting in aneurysmal formation and organ infarction. Polyarteritis nodosa pan is defined as necrotizing vasculitis of mediumsmallsized arteries without glomerulonephritis or vasculitis in arterioles, venules or capillaries and it is not.
Common areas affected include the muscles, joints, intestines bowels, nerves, kidneys, and skin. Symptoms are wideranging because many different organ systems may be involved. The level of disease severity the presence of isolated. Polyarteritis nodosa is a rare multisystem disorder characterized by widespread inflammation, weakening, and damage to small and mediumsized arteries. Nov 07, 2016 polyarteritis nodosa pan is a rare disease, with an incidence of about 3 to 4.
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