<\/span><\/h2>\nEosinophilic granulomatosis with polyangiitis is a rare disease that affects the lungs, skin, and sometimes other organs. The name comes from the presence of eosinophils<\/span>, granulomas<\/span> and inflamed blood vessels<\/span>. Also known as Churg-Strauss syndrome<\/span>Churg-Strauss granulomatosis and allergic granulomatosis.<\/p>\nThe main characteristics of eosinophilic granulomatosis with polyangiitis are:<\/p>\n
\n- Asthma<\/li>\n
- \nEosinophils<\/span> infiltration<\/span> In the lungs<\/li>\n
- Eosinophils in the blood<\/li>\n
- \n
Small container vasculitis<\/span> (inflamed blood vessels) and granulomas (mixed chronic<\/span> inflammatory<\/span> cells) in biopsy<\/span>.<\/li>\n<\/ul>\n<\/span>What are eosinophils?<\/span><\/h2>\nEosinophils are a type of white blood cell. They often increase in number in allergic disorders.<\/p>\n
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<\/span>What is the cause of eosinophilic granulomatosis with polyangiitis?<\/span><\/h2>\nThe cause of eosinophilic granulomatosis with polyangiitis is uncertain, but it is currently believed to be a autoimmune<\/span> process.<\/p>\nIt has been associated with pharmacological sensitivities to penicillin, penicillamine, iodides, leukotrienes<\/span> modifiers and mesalazine in some patients.<\/p>\n<\/span>What are the clinical characteristics of eosinophilic granulomatosis with polyangiitis?<\/span><\/h2>\nEosinophilic granulomatosis with polyangiitis has been divided into three distinct phases, which may or may not be sequential. Symptoms depend on the phase and the organs involved.<\/p>\n
\n\n\n| \nProdromal<\/span> phase<\/td>\n | \n\n- Respiratory symptoms dominate, especially asthma.<\/li>\n
- Allergic rhinitis can also occur<\/li>\n<\/ul>\n<\/td>\n<\/tr>\n
\n| Second stage<\/td>\n | \n\n- \nPeripheral<\/span> blood eosinophilia<\/span>\n<\/li>\n
- Infiltration of eosinophilic tissue in the lungs and intestine.<\/li>\n
- Weightloss, fever<\/span>, sweats, abdominal pain and diarrhea can occur<\/li>\n<\/ul>\n<\/td>\n<\/tr>\n
\n| Vasculitic phase<\/td>\n | \n\n- It can involve any organ: the heart is most often involved, but the lung, the kidney, lymph nodes<\/span> and the muscle can also be affected<\/li>\n
- Symptoms may include bloody diarrhea, blood in the urine, joint pain, inflammation<\/span> of the heart, seizures, coma and skin damage nerves<\/span>\n<\/li>\n
- Asthma can decrease as the disease progresses<\/li>\n
- Skin involvement occurs in about half of patients:\n
\n- Blisters, petechiae<\/span>, purple<\/span> and nodules<\/span>, often with central necrosis<\/span> due to decomposition of the tissue.<\/li>\n
- It affects the back of the arms or the front of the legs, especially the elbows and knees. The scalp or trunk may be involved.<\/li>\n
- Cultures of new lesions can appear for months.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n\n
<\/span>Churg-Strauss cutaneous vasculitis before and after debridement<\/span><\/h5>\n<\/section>\n\n\n \n  \n<\/div>\n Eosinophilic granulomatosis with polyangiitis<\/p>\n \n  \n<\/div>\n Eosinophilic granulomatosis with polyangiitis<\/p>\n<\/p><\/div>\n<\/section>\n <\/div>\n <\/span>How is eosinophilic granulomatosis with polyangiitis diagnosed?<\/span><\/h2>\nThere is no specific blood test for eosinophilic granulomatosis with polyangiitis. Investigations generally include:<\/p>\n \n- Complete blood count<\/li>\n
- Rheumatoid factor (RhF), antinuclear antibody<\/span> (ANA), antineutrofilo antibodies<\/span> (HAUNCH<\/span>)<\/li>\n
- Kidney, liver and muscle function tests, as indicated by symptoms and signs.<\/li>\n<\/ul>\n
Imaging studies may include x-rays of the lungs and sinuses and an electrocardiogram (ECG).<\/p>\n Skin biopsy and kidney biopsy can demonstrate the diagnostic combination of tissue eosinophilia, vasculitis, and granulomas. See eosinophilic granulomatosis with polyangiitis pathology<\/span>.<\/p>\n\n \n \n \n<\/div><\/div><\/div><\/div>\n <\/span>What is the treatment for eosinophilic granulomatosis with polyangiitis?<\/span><\/h2>\nSystemic<\/span> Corticosteroids are the first-line therapy for eosinophilic granulomatosis with polyangiitis. Mild disease can be initially treated with oral corticosteroids, but most authors suggest starting therapy for extensive disease with intravenous (IV) corticosteroids. They are usually prescribed in the long term.<\/p>\nThe patient's response to corticosteroids is often dramatic. Within 1-2 weeks, eosinophilia, the ESR<\/span> and muscle enzyme<\/span> levels can be normalized. Corticosteroids can be sharpened and remission<\/span> is normally sustained Low doses of prednisone can be continued for residual asthma or other symptoms.<\/p>\nIf vasculitic symptoms are not controlled or if large doses of steroids are required, cyclophosphamide can be used. This appears to improve outcome in severe disease and may reduce the chance of relapse<\/span>.<\/p>\nOther medications reported useful in eosinophilic granulomatosis with polyangiitis include:<\/p>\n \n- Methotrexate<\/li>\n
- \nTumor<\/span> necrosis factor (TNF) blockers, such as infliximab and etanercept\n<\/li>\n
- Recombinant interferon (IFN) alpha<\/li>\n
- Rituximab<\/li>\n<\/ul>\n
Antibiotic prophylaxis<\/span> Sulfamethoxazole-trimethoprim may be recommended because intense immunosuppression increases the risk of infection<\/span>.<\/p>\n<\/span>What is the result of eosinophilic granulomatosis with polyangiitis?<\/span><\/h2>\nTreatment of eosinophilic granulomatosis with polyangiitis with systemic steroids has improved the survival of patients from 50% at three years to 75% at eight years.<\/p>\n At least half of the deaths in eosinophilic granulomatosis with polyangiitis are due to cardiac involvement.<\/p>\n<\/section>","protected":false},"excerpt":{"rendered":" Que es eosinof\u00edlica granulomatosis con poliangitis? La granulomatosis eosinof\u00edlica con poliange\u00edtis es una enfermedad rara que afecta los pulmones, la piel y, a veces, otros \u00f3rganos. El nombre proviene de…<\/p>","protected":false},"author":8,"featured_media":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","format":"standard","meta":{"footnotes":""},"categories":[204],"tags":[],"class_list":["post-6867","wiki","type-wiki","status-publish","format-standard","category-glosario-definiciones"],"_links":{"self":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/6867","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki"}],"about":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/types\/wiki"}],"author":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/users\/8"}],"replies":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/comments?post=6867"}],"version-history":[{"count":0,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/6867\/revisions"}],"wp:attachment":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/media?parent=6867"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/categories?post=6867"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/tags?post=6867"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}} | | |