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Kawasaki disease, also known as Kawasaki syndrome, lymph node syndrome, and mucocutaneous lymph node syndrome, is an autoimmune disease in which the medium-sized blood vessels throughout the body become inflamed. It is largely seen in children under five years of age. It affects many organ systems, mainly those including the blood vessels, skin, mucous membranes, and lymph nodes. Its rarest but most serious effect is on the heart, where it can cause fatal coronary artery aneurysms in untreated children. Without treatment, mortality may approach 1%, usually within six weeks of onset. With treatment, the mortality rate is 0.17% in the U.S.〔(【引用サイトリンク】title=Merck Manual, Online edition: Kawasaki Disease )〕 Often, a pre-existing viral infection may play a role in its pathogenesis. The skin, the conjunctivae of the eyes, and the mucous membranes of the mouth become red and inflamed. Swelling of the hands and feet is often seen and lymph nodes in the neck are often enlarged. A recurrent fever, often 37.8 °C (100.0 °F) or higher, is characteristic of the acute phase of the disease. In untreated children, the fever lasts about 10 days, but may range from five to 25 days.〔 The disorder was first described in 1967 by Tomisaku Kawasaki in Japan. == Classification == Systemic vasculitis is an inflammatory condition affecting arteries and veins throughout the body, and is usually caused by a proliferation of cells associated with an immune response to a pathogen, or autoimmunity.〔 Systemic vasculitides may be classified according to the type of cells involved in the proliferation, as well as the specific type of tissue damage occurring within the vein or arterial walls.〔 Under this classification scheme for systemic vasculitis, Kawasaki disease is considered to be a necrotizing vasculitis (also called necrotizing angiitis), which may be identified histologically by the occurrence of necrosis (tissue death), fibrosis, and proliferation of cells associated with inflammation in the inner layer of the vascular wall.〔(【引用サイトリンク】title=necrotizing vasculitis – definition of necrotizing vasculitis )〕 (Other diseases featuring necrotizing vasculitis include polyarteritis nodosa, granulomatosis with polyangiitis, Henoch-Schönlein purpura, and Churg-Strauss syndrome.〔) Kawasaki disease may be further classified as a medium-sized-vessel vasculitis, affecting medium- and small-sized blood vessels, such as the smaller cutaneous vasculature (veins and arteries in the skin) that range from 50 to 100 µm in diameter. Kawasaki disease is also considered to be a primary childhood vasculitis, a disorder associated with vasculitis that mainly affects children under the age of 18.〔 A recent, consensus-based evaluation of vasculitides occurring primarily in children resulted in a classification scheme for these disorders, to distinguish them and suggest a more concrete set of diagnostic criteria for each.〔 Within this classification of childhood vasculitides, Kawasaki disease is, again, a predominantly medium-sized vessel vasculitis.〔 It is also an autoimmune form of vasculitis,〔 and is not associated with ANCA antibodies, unlike other vasculitic disorders associated with them (such as granulomatosis with polyangiitis, microscopic polyangiitis, and Churg-Strauss syndrome).〔 This categorization is considered essential for appropriate treatment. 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Kawasaki disease」の詳細全文を読む スポンサード リンク
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