<\/span><\/h2>\nGranulomatosis with polyangiitis is a rare but life-threatening form of vasculitis<\/span>. There is an immune reaction in which antibodies<\/span> small damage blood vessel<\/span> surrounding walls and tissues.<\/p>\nMultiple organs are often involved. The most commonly affected sites are the ears, nose, throat, lungs, eyes, and kidneys. Mild forms can occur without kidney involvement.<\/p>\n
Granulomatosis with polyangiitis was previously known as Wegener's granulomatosis.<\/p>\n
<\/span>Who gets granulomatosis with polyangiitis?<\/span><\/h2>\nGranulomatosis with polyangiitis affects both sexes equally. It occurs in patients of all ages with a mean age of 41 years. It occurs more commonly in white patients than in blacks or Asians.<\/p>\n
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<\/span>What causes granulomatosis with polyangiitis?<\/span><\/h2>\nThe cause of granulomatosis with polyangiitis is unknown. It is classified as a autoimmune<\/span> cytoplasmic cytoplasmic disease and cytoplasm antibody<\/span> (C-HAUNCH<\/span>) directed against serine proteinase three antigen<\/span> (PR3-ANCA) is believed to be pathogen<\/span>. It can be caused by a bacterial<\/span> infection<\/span>.<\/p>\nGenetic<\/span> mutations<\/span> may be involved, but granulomatosis with polyangiitis is not inherited.<\/p>\n<\/span>What are the clinical characteristics of granulomatosis with polyangiitis?<\/span><\/h2>\n<\/span>\nSystemic<\/span> symptoms<\/span><\/h3>\nGranulomatosis patients with polyangiitis may complain of several non-specific symptoms, especially:<\/p>\n
\n- \nFever<\/span> affects 25% \u201350%<\/li>\n
- Weight loss of> 10% from usual body weight affects 15\u201335%.<\/li>\n<\/ul>\n
<\/span>Upper airway disease<\/span><\/h3>\nSymptoms affecting the ear, nose, and throat are common and affect the 70% of patients at baseline and the 92% of patients during granulomatosis with polyangiitis.<\/p>\n
Manifestations of granulomatosis with polyangiitis in the ear, nose, and throat include:<\/p>\n
\n- Sinusitis, nosebleeds, or nasal deformity<\/li>\n
- Earache or hearing loss (otitis media<\/span>)<\/li>\n
- Painful mouth ulcers<\/span>gum disease (strawberry gums),<\/li>\n
- Subglottic stenosis<\/span> (narrowing of the airways), which occurs with hoarseness, stridor (noisy breathing), or life-threatening airway obstruction.<\/li>\n<\/ul>\n\n
<\/span>Lip ulceration in granulomatosis with polyangiitis<\/span><\/h5>\n<\/section>\n\n\n
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Lip ulceration due to granulomatosis with polyangiitis<\/p>\n<\/p><\/div>\n<\/section>\n
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<\/span>Lung disease<\/span><\/h3>\nPulmonary involvement occurs in 45% of patients with granulomatosis with polyangiitis at presentation and in 87% during disease, which often results in:<\/p>\n
\n- Cough<\/li>\n
- Hemoptysis (coughing up blood)<\/li>\n
- Chest pain (pleurisy).<\/li>\n<\/ul>\n
Chest x-rays can be abnormal, either showing diffuse<\/span> disease (67%) or isolated areas (58%).<\/p>\n<\/span>Nephropathy<\/span><\/h3>\nRenal<\/span> Involvement occurs in 20% of patients at presentation and in 85% during granulomatosis with polyangiitis. It can manifest as oliguria (decreased urine output) and hematuria (blood in the urine).<\/p>\nClose monitoring of kidney function is required for all patients (blood and urine tests).<\/p>\n
<\/span>Eye disease<\/span><\/h3>\nOcular symptoms occur in 28 to 58% of patients with granulomatosis with polyangiitis and are part of the initial presentation in 8 to 16% of patients. The most common problems are:<\/p>\n
\n- Proptosis (bulging of the eye)<\/li>\n
- Pain (which can be very severe)<\/li>\n
- Loss of vision<\/li>\n<\/ul>\n
<\/span>Skin illness<\/span><\/h3>\nThe skin may be affected in 40-50% of patients with granulomatosis with polyangiitis. Skin problems include:<\/p>\n
\n- Ulcers<\/li>\n
- \nPalpable<\/span> purple<\/span> (raised dark red spots due to small vessel vasculitis)<\/li>\n
- \nNodules<\/span>, papules<\/span> and vesicles<\/span> (small blisters)<\/li>\n
- \nPyoderma<\/span> gangrenous (rare)<\/li>\n
- \nRaynaud's phenomenon<\/span> (white \/ purple fingers when exposed to cold) is rare.<\/li>\n<\/ul>\n
<\/span>Muscles and joints<\/span><\/h3>\nMusculoskeletal<\/span> problems occur in 30 to 50% of patients at presentation and in 65 to 75% of patients during illness. These include:<\/p>\n\n- Muscle pain (myalgia<\/span>)<\/li>\n
- Aching joints (arthralgia<\/span>)<\/li>\n
- \nArthritis<\/span>.<\/li>\n<\/ul>\n
<\/span>Nervous system<\/span><\/h3>\nNeurological <\/span>Symptoms are seldom a presenting feature, but may occur in 22-50% of patients during granulomatosis with polyangiitis. Peripheral<\/span> nerves<\/span> They are mainly affected, leading to loss or abnormal sensations or weakness of some muscles. Central nervous system compromise (i.e. the brain and spinal cord cable<\/span>) occur in <10% de los casos.<\/p>\n<\/span>Gastrointestinal tract<\/span><\/h3>\nThe exact frequency of gastrointestinal symptoms in granulomatosis with polyangiitis is unknown. The symptoms are more frequent:<\/p>\n
\n- Abdominal pain<\/li>\n
- Diarrhea<\/li>\n
- Bleeding<\/li>\n<\/ul>\n
<\/span>Heart<\/span><\/h3>\nHeart problems occur in 12-30% of granulomatosis patients with polyangiitis. These include:<\/p>\n
\n- Inflamed cardiac sac (pericarditis<\/span>)<\/li>\n
- \nAngina pectoris<\/span> (cardiac ischemia<\/span>)<\/li>\n
- Palpitations (irregular heartbeat).<\/li>\n<\/ul>\n
<\/span>How is granulomatosis with polyangiitis diagnosed?<\/span><\/h2>\nDiagnosis of granulomatosis with polyangiitis is not always straightforward.<\/p>\n
Blood count abnormalities in granulomatosis with polyangiitis may include:<\/p>\n
\n- High white blood cell count (leukocytosis)<\/li>\n
- Reduced hemoglobin<\/span> (normocytic normochromic anemia<\/span>)<\/li>\n
- High platelet<\/span> count (thrombocytosis)<\/li>\n
- High erythrocytes<\/span> sedimentation rate (ESR<\/span>)<\/li>\n<\/ul>\n
Antineutrophil cytoplasmic antibody (ANCA) is the most useful blood test. One particular type, c-ANCA is highly specific for polyangiitis granulomatosis and affects 70% in patients with the disease. It is recognized by the cytoplasmic staining pattern and is directed against a enzyme<\/span>, proteinase-3. Protoplasm staining perinuclear<\/span> neutrophil cytoplasmic antibodies<\/span>Called p-ANCA, it occurs in the 70% of people with granulomatosis with polyangiitis.<\/p>\nA positive test result for rheumatoid factor occurs in 50-60% of patients with polyangiitis granulomatosis and can be misleading because this normally indicates rheumatoid arthritis.<\/p>\n
A skin biopsy<\/span> can be useful if you demonstrate granulomatous<\/span> vasculitis (inflamed blood vessels<\/span> with a certain pattern of inflammation<\/span>) Necrotizing<\/span> vasculitis, extravascular palisade granulomas<\/span>and leukocytoclastic<\/span> Vasculitis can also occur.<\/p>\n\n
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<\/span>What is the treatment for granulomatosis with polyangiitis?<\/span><\/h2>\nGranulomatosis with polyangiitis is usually treated with glucocorticoids<\/span> and cyclophosphamide. Other drugs used include:<\/p>\n\n- Methotrexate<\/li>\n
- Azathioprine<\/li>\n
- Cyclosporine<\/li>\n
- Antibiotics<\/li>\n
- Intravenous immunoglobulin<\/span><\/li>\n
- \nPlasma<\/span> to exchange<\/li>\n
- Mycophenolate<\/li>\n
- \n
Rituximab<\/li>\n<\/ul>\n
Skin lesions can be treated symptomatically with current<\/span> steroids<\/p>\nSurgery may be necessary to repair the tissue after fibrosis<\/span> or necrosis<\/span>.<\/p>\n<\/span>What is the prognosis for granulomatosis with polyangiitis?<\/span><\/h2>\nGranulomatosis with polyangiitis can be slow or fast. progressive<\/span>. There is often no way to predict which patients will continue to have limited and milder forms of the disease and which will continue with more severe progressive progression, generalized<\/span> forms of the disease. Without treatment, severe disease has a high mortality rate due to infection, heart or lung disease, kidney failure, or malignancy<\/span>.<\/p>\nDisease activity must be regularly monitored. Close monitoring of kidney function is especially important. Many different specialists may need to be involved in patient care. Careful and long-term monitoring is required.<\/p>\n<\/section>","protected":false},"excerpt":{"rendered":"
\u00bfQu\u00e9 es la granulomatosis con poliangitis? La granulomatosis con poliangitis es una forma poco frecuente pero potencialmente mortal de vasculitis. Hay una reacci\u00f3n inmune en la que anticuerpos da\u00f1o peque\u00f1o…<\/p>","protected":false},"author":8,"featured_media":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","format":"standard","meta":{"footnotes":""},"categories":[],"tags":[],"class_list":["post-8264","wiki","type-wiki","status-publish","format-standard"],"_links":{"self":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/8264","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=8264"}],"version-history":[{"count":0,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/8264\/revisions"}],"wp:attachment":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/media?parent=8264"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/categories?post=8264"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/tags?post=8264"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}