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Summary  Some comorbidities, such as hypertension, are associated with higher von Willebrand factor ( VWF ) levels in the general population. No studies have been conducted to assess this association in patients with von Willebrand disease ( VWD ). Therefore, we studied this association in patients with type 1 ( n  = 333) and type 2 ( n  = 203)  VWD  from the ‘WiN” study.  VWF  antigen ( VWF :Ag) was higher in type 1  VWD  patients with hypertension [difference: 0·23 iu/ml, 95% confidence interval ( CI ): 0·11–0·35], diabetes mellitus (0·11 iu/ml, 95%  CI : −0·02 to 0·23), cancer (0·14 iu/ml, 95%  CI : 0·03–0·25) and thyroid dysfunction (0·14 iu/ml, 95%  CI : 0·03–0·26) than in patients without these comorbidities (all corrected for age, sex and blood group). Similar results were observed for  VWF  collagen binding capacity ( VWF : CB ),  VWF  activity as measured by the  VWF  monoclonal antibody assay ( VWF :Ab) and factor  VIII  ( FVIII ) coagulant activity ( FVIII :C). In type 1  VWD , age was associated with higher  VWF :Ag (0·03 iu/ml; 95%  CI : 0·01–0·04),  VWF : CB  (0·02 iu/ml; 95%  CI : 0·00–0·04),  VWF :Ab (0·04 iu/ml; 95%  CI : 0·02–0·06) and  FVIII :C (0·03 iu/ml; 95%  CI : 0·01–0·06) per decade increase. After adjustment for relevant comorbidities, these associations were no longer significant. Despite the higher  VWF  and  FVIII  levels, type 1  VWD  patients with comorbidities had more bleeding episodes, particularly during surgery. There was no association between comorbidities and  VWF / FVIII  levels or bleeding phenotype in type 2  VWD  patients. In conclusion, comorbidities are associated with higher  VWF  and  FVIII  levels in type 1  VWD  and may explain the age‐related increase of  VWF  and  FVIII  levels.

Comorbidities associated with higher von Willebrand factor ( VWF ) levels may explain the age‐related increase of VWF in von Willebrand disease