Rodrigues Carlos Ewerton Maia, Callado Maria Roseli Monteiro, Nobre Christiane Aguiar, Moura Francisca Edwiges Araújo, Vieira Rejane Maria Rodrigues de Abreu, de Albuquerque Lucas Alverne Freitas, Vieira Walber Pinto
Serviço de Reumatologia, Hospital Geral de Fortaleza.
Rev Bras Reumatol. 2010 Mar-Apr;50(2):150-64.
To describe the initial clinical manifestations of Wegener's Granulomatosis (WG) in Brazil.
Retrospective analysis of six medical records of WG patients followed-up at the Rheumatology Department of Hospital Geral of Fortaleza (HGF), as well as a bibliographic survey of cases of WG in Brazil on LILACS, SciELO, and MEDLINE databases.
The study identified 49 patients, 15 (31%) males and 34 (69%) females. Systemic disease was observed in 35 patients (73%): 28 adults, 5 children, and 2 teenagers. Limited disease was observed in 13 adults and 1 child. The average age of onset in adults was 42.2 years (18 to 65 years). Acute clinical manifestations, with the onset of symptoms less than three months before the diagnosis, were observed in 41% (20/49) of the patients, and the insidious presentation in 59% (29/49) of the patients. The prevalence of the initial clinical manifestations in adults with systemic disease (n = 28) was 64% (18/28), upper airways, 36% (10/28), lungs, 18% (5/28), kidneys, 25% (7/28), eyes, 11% (3/28) skin, 25% (7/28), musculoskeletal, and 7% (2/28), neurological. In adults (n = 13) with limited disease, prevalent symptoms included: upper airway, 84% (10/13), eyes, 23% (3/13), and lungs, 15% (2/13).
The prevalence of the initial clinical manifestations of WG in Brazil was similar to that reported in the literature. The lack of specific symptoms may delay diagnosis cases with insidious presentation of the disease and increase the morbidity and mortality in acute disease.
描述巴西韦格纳肉芽肿（WG）的初始临床表现。
对福塔莱萨综合医院（HGF）风湿科随访的6例WG患者的病历进行回顾性分析，并对LILACS、SciELO和MEDLINE数据库中巴西的WG病例进行文献调查。
该研究共纳入49例患者，其中男性15例（31%），女性34例（69%）。35例患者（73%）出现全身疾病：28例成人、5例儿童和2例青少年。13例成人和1例儿童出现局限性疾病。成人发病的平均年龄为42.2岁（18至65岁）。41%（20/49）的患者出现急性临床表现，即症状出现至诊断的时间少于3个月，59%（29/49）的患者表现为隐匿性。全身疾病成人患者（n = 28）初始临床表现的患病率为：上呼吸道64%（18/28）、肺部36%（10/28）、肾脏18%（5/28）、眼睛11%（3/28）、皮肤25%（7/28）、肌肉骨骼7%（2/28）、神经7%（2/28）。局限性疾病成人患者（n = 13）的常见症状包括：上呼吸道84%（10/13）、眼睛23%（3/13）、肺部15%（2/13）。
巴西WG初始临床表现的患病率与文献报道相似。缺乏特异性症状可能会延迟隐匿性疾病病例的诊断，并增加急性疾病的发病率和死亡率。
