title: Nonsurgical synovectomy in the treatment of arthropathy in Von Willebrand's Disease.
abstract: Von Willebrand's disease is the most common inherited bleeding disorder, with an overall prevalence in the general population of 0.8% to 1.3%. Hemarthrosis occurs mainly in the severest forms of the disease (type III), with a frequency of 3.5% to 11%, and can cause severe arthropathy similar to that seen in hemophilia. We retrospectively reviewed our experience with nonsurgical synovectomy in the treatment of recurrent hemarthrosis with arthropathy in patients with von Willebrand's disease. Four of our six patients had type III disease and the remaining two had type II disease. The age range was 13 to 63 years. The frequency of hemarthrosis prior to synovectomy was one to four per month. One (n = 2) or both (n = 1) knees were treated in 4 cases, one (n = 1) or both (n = 1) ankles in 3 cases and an elbow in one case. We used yttrium 90 in a dose of 5 mCi for one knee, rhenium 186 in a dose of 2 mCi for two ankles and the elbow and osmic acid for two knees and one ankle. Clinical and radiological results were evaluated six months after synovectomy using the World Federation of Hemophilia score. Radiologic lesions remained stable and clinical manifestations improved in every case (p < 0.05). Five patients achieved a complete remission. Safety was satisfactory. The clinical efficacy of synovectomy done, using radiocolloids or osmic acid in arthropathy due to von Willebrand's disease, seems similar to that in hemophilia.
authors.affiliation: Chair of Rheumatology I, Cagliari University Hospital, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Salis
authors.forename: P
authors.initials: P
authors.lastname: Molho
authors.forename: P
authors.initials: P
authors.lastname: Verrier
authors.forename: N
authors.initials: N
authors.lastname: Stieltjes
authors.forename: D
authors.initials: D
authors.lastname: Vassilieff
authors.forename: N
authors.initials: N
authors.lastname: Ounnoughène
authors.forename: J M
authors.initials: JM
authors.lastname: Schacher
authors.forename: G
authors.initials: G
authors.lastname: Perpignano
authors.forename: Y
authors.initials: Y
authors.lastname: Sultan
authors.forename: C J
authors.initials: CJ
authors.lastname: Menkès
mesh_terms: D000293:Adolescent; D000328:Adult; D001168:Arthritis / Q000209:etiology / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D006395:Hemarthrosis / Q000209:etiology / Q000628:therapy*; D006801:Humans; D007596:Joints / Q000187:drug effects / Q000528:radiation effects; D008297:Male; D008875:Middle Aged; D009993:Osmium Tetroxide / Q000627:therapeutic use; D011868:Radioisotopes / Q000627:therapeutic use; D012189:Retrospective Studies; D012211:Rhenium; D013583:Synovial Membrane / Q000187:drug effects* / Q000528:radiation effects*; D016896:Treatment Outcome; D015021:Yttrium Radioisotopes / Q000627:therapeutic use; D014842:von Willebrand Diseases / Q000150:complications*
chemical_list: D011868:Radioisotopes; D015021:Yttrium Radioisotopes; D012211:Rhenium; D009993:Osmium Tetroxide