title: Disease burden, disease manifestations and current treatment regimen of the SAPHO syndrome in Germany: results from a nationwide patient survey.
abstract: BACKGROUND
  Due to diagnostic and therapeutic advances, quality of life of patients with spondyloarthritides (SpA) has improved substantially in recent years. However, little is known about how patients with the SAPHO syndrome, a heterogeneous disease counted among the SpAs, profit from these advances.
  
  
  OBJECTIVE
  To investigate current aspects of patient care in a nationwide SAPHO cohort.
  
  
  METHODS
  Patients were recruited in a university centre and via a nationwide SAPHO patient support group. Medical records were reviewed and patients were asked to complete a questionnaire on the course of diagnosis, disease burden and treatment regimen.
  
  
  RESULTS
  A total of 64 patients were included in the analysis. The mean time from disease onset to diagnosis was 3.8 ± 5.3 years. The patients' overall satisfaction with the course of diagnosis was 23.0 ± 28.9 on a visual analogue scale (VAS) from 0 to 100. Musculoskeletal symptoms had the highest impact on the patients' wellbeing. The mean overall disease burden on a VAS for pain was 45.4 ± 25.9. Limitations in the quality of life were reported mainly in the general health, bodily pain and vitality dimensions of the SF-36 questionnaire. Current treatments consisted of NSAIDs (77%), DMARDs (27%), glucocorticoids (23%), TNF-inhibitors (16%) and bisphosphonates (11%).
  
  
  CONCLUSIONS
  The SAPHO syndrome has a high impact on the patients' general health and quality of life. Establishing the diagnosis still takes years and expends multiple medical resources. Effective treatments such as TNF-inhibitors are rarely prescribed and current disease burden is not acceptable.
authors.affiliation: Department of Internal Medicine IV, Division of Rheumatology, University of Munich, Munich, Germany. Electronic address: Matthias.witt@med.uni-muenchen.de.
authors.forename: Matthias
authors.initials: M
authors.lastname: Witt
authors.affiliation: Department of Internal Medicine IV, Division of Rheumatology, University of Munich, Munich, Germany.
authors.forename: Johanna
authors.initials: J
authors.lastname: Meier
authors.affiliation: Department of Internal Medicine IV, Division of Rheumatology, University of Munich, Munich, Germany.
authors.forename: Ariane
authors.initials: A
authors.lastname: Hammitzsch
authors.affiliation: Department of Internal Medicine IV, Division of Rheumatology, University of Munich, Munich, Germany.
authors.forename: Fabian
authors.initials: F
authors.lastname: Proft
authors.affiliation: Department of Internal Medicine IV, Division of Rheumatology, University of Munich, Munich, Germany.
authors.forename: Hendrik
authors.initials: H
authors.lastname: Schulze-Koops
authors.affiliation: Department of Internal Medicine IV, Division of Rheumatology, University of Munich, Munich, Germany.
authors.forename: Mathias
authors.initials: M
authors.lastname: Grunke
mesh_terms: D020083:Acquired Hyperostosis Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology*; D000328:Adult; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D018501:Antirheumatic Agents / Q000627:therapeutic use; D050071:Bone Density Conservation Agents / Q000627:therapeutic use; D015331:Cohort Studies; D017281:Cost of Illness; D057210:Delayed Diagnosis / Q000706:statistics & numerical data; D004164:Diphosphonates / Q000627:therapeutic use; D005260:Female; D005858:Germany; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008297:Male; D008875:Middle Aged; D017060:Patient Satisfaction; D011788:Quality of Life; D012189:Retrospective Studies; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D016896:Treatment Outcome; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D018501:Antirheumatic Agents; D050071:Bone Density Conservation Agents; D004164:Diphosphonates; D005938:Glucocorticoids; D014409:Tumor Necrosis Factor-alpha
keywords: Diagnosis; Disease burden; Nationwide cohort; SAPHO; Treatment