title: Rheumatic disorders affecting the head and neck: underestimated diseases.
abstract: OBJECTIVE
  To describe the clinical manifestations of rheumatic disorders with isolated head and neck (H&N) affection and to introduce a novel diagnostic pathway.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Technische Universität München, Hals-Nasen-Ohrenklinik und Poliklinik, München, Germany. a.knopf@lrz.tum.de
authors.forename: Andreas
authors.initials: A
authors.lastname: Knopf
authors.forename: Murat
authors.initials: M
authors.lastname: Bas
authors.forename: Adam
authors.initials: A
authors.lastname: Chaker
authors.forename: Ulrich
authors.initials: U
authors.lastname: Strassen
authors.forename: Anja
authors.initials: A
authors.lastname: Pickhard
authors.forename: Thomas
authors.initials: T
authors.lastname: Stark
authors.forename: Tobias
authors.initials: T
authors.lastname: Lahmer
authors.forename: Klaus
authors.initials: K
authors.lastname: Thürmel
mesh_terms: D000328:Adult; D015897:Comorbidity; D005260:Female; D005858:Germany / Q000453:epidemiology; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology; D006257:Head; D006801:Humans; D007596:Joints / Q000473:pathology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009333:Neck; D012189:Retrospective Studies; D012216:Rheumatic Diseases / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology; D012469:Salivary Glands / Q000473:pathology; D012507:Sarcoidosis / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology; D012737:Sex Factors