title: Bone Involvement in Rosai-Dorfman Disease (RDD): a Case Report and Systematic Literature Review.
abstract: OBJECTIVE
  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.
  
  
  RESULTS
  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.
authors.affiliation: Department of Internal Medicine VI, Infectious Diseases, Immunology, Rheumatology, Pneumology, Medical University of Innsbruck, Anichstrasse 35, Innsbruck, Austria. birgit.mosheimer@i-med.ac.at.
authors.forename: Birgit A
authors.initials: BA
authors.lastname: Mosheimer
authors.affiliation: Ludwig Boltzmann Institute of Osteology at the Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling, 1st Medical Department, Hanusch Hospital, Vienna, Austria.
authors.forename: Bastian
authors.initials: B
authors.lastname: Oppl
authors.affiliation: Institute of Radiology and Nuclear Medicine, Hanusch Hospital, Vienna, Austria.
authors.forename: Shahin
authors.initials: S
authors.lastname: Zandieh
authors.affiliation: 3rd Medical Department, Hanusch Hospital, Vienna, Austria.
authors.forename: Michael
authors.initials: M
authors.lastname: Fillitz
authors.affiliation: 3rd Medical Department, Hanusch Hospital, Vienna, Austria.
authors.forename: Felix
authors.initials: F
authors.lastname: Keil
authors.affiliation: Ludwig Boltzmann Institute of Osteology at the Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling, 1st Medical Department, Hanusch Hospital, Vienna, Austria.
authors.forename: Klaus
authors.initials: K
authors.lastname: Klaushofer
authors.affiliation: Department of Internal Medicine VI, Infectious Diseases, Immunology, Rheumatology, Pneumology, Medical University of Innsbruck, Anichstrasse 35, Innsbruck, Austria.
authors.forename: Günter
authors.initials: G
authors.lastname: Weiss
authors.affiliation: Ludwig Boltzmann Institute of Osteology at the Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling, 1st Medical Department, Hanusch Hospital, Vienna, Austria.
authors.forename: Jochen
authors.initials: J
authors.lastname: Zwerina
mesh_terms: D000328:Adult; D018771:Arthralgia / Q000209:etiology; D001847:Bone Diseases / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006225:Hand / Q000000981:diagnostic imaging; D015618:Histiocytosis, Sinus / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D011859:Radiography
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents
keywords: Bone involvement; Rosai-Dorfman disease; Treatment strategies