Technische Universität München, Hals-Nasen-Ohrenklinik und Poliklinik, München, Germany.
Rheumatology (Oxford). 2011 Nov;50(11):2029-34. doi: 10.1093/rheumatology/ker213. Epub 2011 Aug 24.
To describe the clinical manifestations of rheumatic disorders with isolated head and neck (H&N) affection and to introduce a novel diagnostic pathway.
From 2004 to 2010, 90 patients presented with isolated H&N symptoms of a rheumatic disorder were included in the study. Rheumatic disorders were classified according to the ACR criteria. In 2008, we introduced a novel diagnostic pathway to reduce under-diagnosis of primary rheumatic disorders in the H&N. Disease-related data were assessed retrospectively and set into clinical context.
The majority of patients suffered from SS (n = 42), granulomatosis with polyangiitis (Wegener's) (n = 13) and sarcoidosis (n = 18) with predominance for female patients (n = 65). Enlargement of the major salivary glands (n = 47), sicca symptoms (n = 41) and cervical lymphadenopathy (n = 25) represented the most frequent symptoms. Interestingly, 3% of all enlargements of salivary glands and 4% of all cervical lymphadenopathy could be contributed to rheumatic disorders. The mean time to diagnosis was 20.71 months for SS, 8.4 months for granulomatosis with polyangiitis and 57.5 months for sarcoidosis. After implementation of the newly developed diagnostic pathway in 2008, the annually diagnosed rheumatic disorders increased 5-fold.
The majority of rheumatic diseases of the H&N can be related to SS, granulomatosis with polyangiitis and sarcoidosis. However, the lack of specific symptoms and the clinical variability of H&N manifestation may contribute to a prolonged time to diagnosis. Our retrospective study points out the variability of symptoms and suggests a diagnostic pathway to reduce the cases of undetected H&N affection in rheumatic disorders.
描述以孤立性头颈部（H&N）表现为特征的风湿性疾病的临床表现，并介绍一种新的诊断途径。
2004 年至 2010 年，我们共纳入 90 例以风湿性疾病孤立性 H&N 症状就诊的患者。根据 ACR 标准对风湿性疾病进行分类。2008 年，我们引入了一种新的诊断途径，以减少头颈部风湿性疾病的漏诊。回顾性评估疾病相关数据，并将其置于临床背景下进行分析。
大多数患者患有 SS（n=42）、肉芽肿性多血管炎（韦格纳氏）（n=13）和结节病（n=18），以女性患者为主（n=65）。大唾液腺肿大（n=47）、干燥症状（n=41）和颈部淋巴结肿大（n=25）是最常见的症状。有趣的是，3%的唾液腺肿大和 4%的颈部淋巴结肿大可归因于风湿性疾病。SS 的平均诊断时间为 20.71 个月，肉芽肿性多血管炎为 8.4 个月，结节病为 57.5 个月。2008 年新的诊断途径实施后，每年诊断的风湿性疾病增加了 5 倍。
H&N 的大多数风湿性疾病可归因于 SS、肉芽肿性多血管炎和结节病。然而，缺乏特异性症状和 H&N 表现的临床变异性可能导致诊断时间延长。我们的回顾性研究指出了症状的可变性，并提出了一种诊断途径，以减少风湿性疾病中未被发现的 H&N 受累病例。
