Mosheimer Birgit A, Oppl Bastian, Zandieh Shahin, Fillitz Michael, Keil Felix, Klaushofer Klaus, Weiss Günter, Zwerina Jochen
Department of Internal Medicine VI, Infectious Diseases, Immunology, Rheumatology, Pneumology, Medical University of Innsbruck, Anichstrasse 35, Innsbruck, Austria.
Ludwig Boltzmann Institute of Osteology at the Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling, 1st Medical Department, Hanusch Hospital, Vienna, Austria.
Curr Rheumatol Rep. 2017 May;19(5):29. doi: 10.1007/s11926-017-0656-6.
Rosai-Dorfman disease (RDD) is a rare histiocytic disorder typically presenting as painless cervical lymphadenopathy. Extranodal involvement is common and may also affect bones. Here, we present a patient with typical nodal disease and multifocal bone manifestations. Further, a systematic literature review was performed to better understand the phenotype, clinical course and treatment options of such patients.
RDD is a nonmalignant, classically sporadic histiocytosis. Nevertheless, increasing evidence also suggests familial forms of the disease. According to our literature review, bone involvement is exceedingly rare and heterogeneous. Clinical outcome in terms of mortality seems to be favorable in most cases. Currently, therapy strategies include surgical and immunosuppressive treatments, but the optimal treatment of osseous RDD remains to be defined. Patients with osseous RDD may present to rheumatologists with arthralgia or arthritis. Due to the rarity of the disease, diagnosis and treatment remain challenging.
罗萨伊-多夫曼病（RDD）是一种罕见的组织细胞疾病，通常表现为无痛性颈部淋巴结病。结外受累很常见，也可能累及骨骼。在此，我们报告一名具有典型淋巴结疾病和多灶性骨表现的患者。此外，进行了系统的文献综述，以更好地了解此类患者的表型、临床病程和治疗选择。
RDD是一种非恶性的、典型的散发性组织细胞增多症。然而，越来越多的证据也表明该疾病存在家族性形式。根据我们的文献综述，骨受累极其罕见且具有异质性。在大多数情况下，就死亡率而言临床结局似乎良好。目前，治疗策略包括手术和免疫抑制治疗，但骨RDD的最佳治疗方法仍有待确定。骨RDD患者可能因关节痛或关节炎就诊于风湿病学家。由于该疾病罕见，诊断和治疗仍然具有挑战性。
