title: Characterization of pain in fabry disease.
abstract: OBJECTIVE
  To give a detailed characterization of pain in a large cohort of patients with Fabry disease.
  
  
  METHODS
  In this single-center, retrospective study we performed a detailed analysis of pain prevalence and characteristics of 132 patients with Fabry disease. Data were obtained by screening all medical records and using standardized extraction forms. Missing data were completed by telephone interviews.
  
  
  RESULTS
  We show that men and women with Fabry disease frequently experience pain, mostly starting in early childhood. Pain is typically episodic with pain attacks being the predominant phenotype. The most frequent localization is in the distal extremities, the most often named pain quality is "burning." Fabry pain is triggerable by physical activity and thermal stimuli and improves over time. Frequently used acute pain medication consists of nonsteroidal antirheumatics and nonopioid analgesics. Long-term medications such as drugs used to treat neuropathic pain syndromes are rarely taken by Fabry patients.
  
  
  CONCLUSIONS
  Pain in Fabry disease affects both sexes and has a complex phenotype that requires comprehensive assessment. Current pain questionnaires fail to cover the entire scope of Fabry pain. Although basically neuropathic, some types of Fabry pain may respond to nonsteroidal antirheumatics and nonopioid analgesics.
authors.affiliation: Department of Neurology †Würzburg Fabry Center for Interdisciplinary Therapy (FAZIT), The University of Würzburg, Würzburg, Germany.
authors.forename: Nurcan
authors.initials: N
authors.lastname: Üçeyler
authors.forename: Shalni
authors.initials: S
authors.lastname: Ganendiran
authors.forename: Daniela
authors.initials: D
authors.lastname: Kramer
authors.forename: Claudia
authors.initials: C
authors.lastname: Sommer
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000700:Analgesics / Q000627:therapeutic use; D015331:Cohort Studies; D003863:Depression / Q000175:diagnosis / Q000209:etiology; D000795:Fabry Disease / Q000150:complications* / Q000453:epidemiology / Q000628:therapy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010146:Pain / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D059408:Pain Management; D010147:Pain Measurement; D012737:Sex Factors; D011795:Surveys and Questionnaires; D055815:Young Adult
chemical_list: D000700:Analgesics