Arthropathy in Von Willebrand's Disease

Ahlberg, A; Silwer, Jörgen

doi:10.3109/17453677008991542

Cited by 19 publications

(11 citation statements)

References 4 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…As VWD became better characterized, subsequent case reports began to document the occurrence of haemarthroses. Ahlberg and Silwer investigated all known cases of VWD in Sweden in 1970 and found that 44% of patients with severe VWD had joint bleeds, and half of those patients suffered from chronic arthropathy [7]. Subsequent data from 66 patients with type 3 VWD in an Italian registry showed similarly that 45% of patients experienced joint bleeding [8].…”

Section: Introductionmentioning

confidence: 99%

Bleeding symptoms and laboratory correlation in patients with severe von Willebrand disease

Metjian¹,

WANG²,

Sood³

et al. 2009

Haemophilia

View full text Add to dashboard Cite

Type 3 von Willebrand disease (VWD) is a rare bleeding disorder with markedly decreased or absent von Willebrand factor (VWF) protein, accompanied by a parallel decrease in VWF function and factor VIII (FVIII) activity. The goal of this study was to describe the population of patients enrolled in the USA Centers for Disease Control Universal Data Collection (UDC) study with type 3 VWD, defined as a VWF:Ag of <10%, and to correlate bleeding symptoms with VWF and FVIII levels. Data on 150 patients were analysed. Almost all patients experienced bleeding episodes (98%) and required blood and/or factor product treatment (92%). While oral mucosal bleeding (the site of first bleed in 54%) was most common, subsequent muscle and joint bleeds were also seen (28%, 45%, respectively), and intracranial haemorrhage occurred in 8% of individuals. Mean age of first bleed was lower in those with either a FVIII < or =5% or a VWF:Ag <1%. Univariate marginal model analysis showed lower levels of FVIII and VWF:Ag both predicted a higher risk of joint bleeding. Longitudinal multivariate analysis found a lower FVIII level (P = 0.03), increasing age (P < 0.0001), history of joint bleeding (P = 0.001), higher body mass index (BMI) (P < 0.0001), and use of home infusion (P = 0.02) were all negatively associated with joint mobility. Low levels of VWF:Ag (P = 0.003) and male sex (P = 0.007) were also negatively associated with joint function. This study documents the strong bleeding phenotype in severe VWD and provides data to help target therapy, including prophylaxis, for patients most at risk of bleeding complications.

show abstract

Section: Introductionmentioning

confidence: 99%

Bleeding symptoms and laboratory correlation in patients with severe von Willebrand disease

Metjian¹,

WANG²,

Sood³

et al. 2009

Haemophilia

View full text Add to dashboard Cite

show abstract

“…The ankle joint is the most frequent location for which patients develop severe VWD [9,[52][53][54]. However, VWD is a known reason for haemophilic arthropathy.…”

Section: Discussionmentioning

confidence: 99%

“…However, VWD is a known reason for haemophilic arthropathy. The ankle joint is the most frequent location for which patients develop severe VWD [9,[52][53][54]. Sood et al [8] described similarity in joint function limitations in patients with Type 3 VWD and moderate haemophilia A.…”

Section: Discussionmentioning

confidence: 99%

Total ankle replacement in patients with von Willebrand disease: mid‐term results of 18 procedures

et al. 2015

View full text Add to dashboard Cite

von Willebrand disease (VWD) is a recognized cause of secondary ankle osteoarthritis (OA). Few studies have examined orthopaedic complications and outcomes in VWD patients treated for end-stage ankle OA with total ankle replacement (TAR). To determine the clinical presentation, intraoperative and postoperative complications and evaluate the mid-term outcome in VWD patients treated with TAR. Eighteen patients with VWD with mean age 47.3 years (range = 34.0-68.7) were treated for end-stage ankle OA with TAR. The mean duration of follow-up was 7.5 years (range = 2.9-13.2). Intraoperative and perioperative complications were recorded. Component stability was assessed with weight-bearing radiographs. Clinical evaluation included range of motion (ROM) tests using a goniometer and under fluoroscopy using a lateral view. Clinical outcomes were analysed by a visual analogue scale, the American Orthopaedic Foot and Ankle Society hindfoot score and Short Form (36) Health Survey (SF-36) health survey. One patient sustained an intraoperative medial malleolar fracture. In two patients delayed wound healing was observed. Two secondary major surgeries were performed. Pain level decreased from 8.2 ± 0.9 (range = 7-10) preoperatively to 1.1 ± 1.2 (range = 0-4) postoperatively. Significant functional improvement including ROM was observed. All categories of SF-36 score showed significant improvement in quality of life. Mid-term results of TAR in patients with VWD are encouraging. The total rate of intraoperative and postoperative complications was 33.3%. However, longer term outcomes are necessary to fully understand the clinical benefit of TAR in patients with VWD.

show abstract

“…Radiologically the affected joints show increased trabeculation of bones, sclerosis and irregularity of the joint surfaces with narrowing of the joint space and subchondral cysts (Ahlberg & Silwer 1970). The appearances are those seen in haemophilic joints, except that squaring of the patella and femoral condyles are seldom seen in vWd.…”

Section: Discussionmentioning

confidence: 99%