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Sua apresenta\u00e7\u00e3o cl\u00ednica \u00e9 bastante ampla, variando de casos mais brandos a casos graves, com acometimento multissist\u00eamico, caracteristicamente com exacerba\u00e7\u00f5es e remiss\u00f5es. Suas causas ainda s\u00e3o desconhecidas; entretanto, h\u00e1 evid\u00eancias gen\u00e9ticas, ambientais e imunol\u00f3gicas, como a associa\u00e7\u00e3o com o alelo HLA-B51. 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