Polyarteritis nodosa is a rare vasculitis in children characterized by necrotizing inflammation in small and medium size arteries. Outlook prognosis current treatments with steroids and other drugs that suppress the immune system such as azathioprine or cyclophosphamide can improve symptoms and the chance of longterm survival. Polyarteritis nodosa, also known as kussmaulmaier disease, is a rare entity in the pediatric population. Pan affects mediumsized blood vessels that supply the skin, nervous system, joints, kidneys, gastrointestinal gi tract, and heart, among other. Previously, it had been referred to as infantile polyarteritis nodosa ipan. Polyarteritis nodosa is an autoimmune disease that affects arteries. Unlike some other vasculitides eg, microscopic polyarteritis, granulomatosis with polyangiitis, polyarteritis nodosa is not associated with antineutrophil cytoplasmic antibodies anca. Diagnosis and classification of polyarteritis nodosa. Poliarteritis nodosa sistemica y poliarteritis nodosa. Common areas affected include the muscles, joints, intestines bowels, nerves, kidneys, and skin. Stone, md, mph case presentation a 30yearold man was referred for evaluation and treatment of still disease. Polyarteritis nodosa, also known as panarteritis nodosa, periarteritis nodosa, kussmaul disease or kussmaulmaier disease, is a vasculitis of medium and smallsized arteries, which become. The approach to treatment of pan depends upon the following variables, which require assessment before beginning therapy.
It is a rare disease in children and is characterizedby its benign and chronic course. Poliarterite nodosa cutanea infantil associada a infeccao estreptococica. Inflammation of the arteries can lead to inadequate blood supply and permanent damage to organs. Cutaneous pan cpan is limited to the skin, muscles, joints, and peripheral nerves. For polyarteritis nodosa related to hepatitis, treatment may involve plasmapheresis and antiviral medicines. The topic infantile polyarteritis nodosa you are seeking is a synonym, or alternative name, or is closely related to the medical condition kawasaki disease kd quick summary. Polyarteritis nodosa pan is a systemic necrotizing vasculitis predominantly targeting mediumsized arteries. 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. Polyarteritis nodosa pan is a vasculitis of small and medium arteries of the dermis. Most studies have shown no significant gender predominance. Cutaneous polyarteritis nodosa is a form of polyarteritisnodosa. Most cases occur in the 4th or 5th decade of life, although it can occur at any age. Pdf polyarteritis nodosa is a rare vasculitis in children characterized by necrotizing inflammation in small and medium size arteries. Arthritis rheum 20 jun 10 early recognition and prevention of extended morbidity is facilitated by dermatologic examination.
The first description of polyarteritis nodosa pan was in 1852 by karl rokitansky, a pathologist at the university of vienna. The presence of at least three criteria is as sociated with a sensitivity of 82. His illness had begun 12 years earlier when, as a high school senior, he developed daily temperatures of 38. It can affect the blood vessels that go to almost every part of your body, including your heart, kidneys, and. Polyarteritis nodosa pan is a necrotizing vasculitis resulting in aneurysmal formation and organ infarction. The frequent finding of arthritis is a potential cause. The major environmental factor associated with pan is hbv infection. Blood vessels in any organ or organ system may be affected, including those supplying the kidneys. There is no evidence ofhypertension or organ dysfunction. Started in 1995, this collection now contains 6760 interlinked topic pages divided into a tree of 31 specialty books and 732 chapters. Polyarteritis nodosa nord national organization for. The diagnosis of pan was confirmed in this patient.
It is categorized as a mediumvessel vasculitis chapel hill, 2012. The initial report describes a 23 yearold man who had a fiveday history of fever and diarrhea. Polyarteritis nodosa pan is a rare disease that makes your blood vessels swell. Cutaneous polyarteritis nodosa is an inflammatory disease that involves medium sized vessels of skin and, occasionally, nerves and muscles. Childhood polyarteritis nodosa cpan is a rare and often fatal disease that affects small and medium arteries. It primarily affects small and medium arteries, which can become inflamed or damaged. Child, polyarteritis nodosa, streptococcus pyogenes.
Polyarteritis nodosa pan is a systemic necrotizing vasculitis that typically affects mediumsized muscular arteries, with occasional involvement of small muscular arteries. Criteria for the classification of poliarteritis nodosa. 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 is a rare multisystem disorder characterized by widespread inflammation, weakening, and damage to small and mediumsized arteries.
Article polyarteritis nodosa in a pediatric patient. The use of the term infantile is too restrictive, as infancy connotes age 1 year or younger. Small arteries may also be involved but small vessels, including arterioles, capillaries and venules, are characteristically spared 1. Polyarteritis nodosa pan is a condition that causes swollen arteries. 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. It is classified into systemic and cutaneous pan according to the presence of systemic symptoms or visceral involvement. Treatment is directed toward decreasing the inflammation of the arteries. Acute hemiplegia in kawasaki disease and infantile.
Diagnosis of polyarteritis nodosa is confirmed by a biopsy of involved tissue or angiography. Polyarteritis nodosa is a rare vasculitis of childhood. The requirement for negative results for antineutrophil cytoplasmic antibody anca serology test in polyarteritis nodosa pan is a useful new change that allows for discrimination between pan and ancaassociated vasculitides, which otherwise have similar presentations pathologically and clinically. 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.