Brief Communication: High Incidence of Venous Thrombotic Events among Patients with Wegener Granulomatosis: The Wegener's Clinical Occurrence of Thrombosis (WeCLOT) Study

Merkel, Peter A.; Lo, Grace; Holbrook, Janet T.; Tibbs, Andrea K.; Allen, Nancy B.; Davis, John C.; Hoffman, Gary S.; McCune, W. Joseph; Clair, E. William St.; Specks, Ulrich; Spiera, Robert; Petri, Michelle; Stone, John H.

doi:10.7326/0003-4819-142-8-200504190-00010

Cited by 87 publications

(123 citation statements)

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“…A total of 180 patients were recruited. In a time period of 228 person-years, 16 venous thrombotic events (VTE's) occurred in patients with no history of thromboembolic disease [3]. Similar to our case, the onset was 2.1 months from enrollment.…”

Section: Discussionsupporting

confidence: 65%

“…Specifically, the Wegener's Clinical Occurrence of Thrombosis (WeCLOT) study recruited 180 patients during active periods of disease [3]. This was a multi-centre, randomized, double blind, placebo-controlled study [3].…”

Section: Discussionmentioning

confidence: 99%

“…This was a multi-centre, randomized, double blind, placebo-controlled study [3]. Thromboembolic disease (pulmonary emboli and deep vein thrombosis) was confirmed based upon clinical suspicion.…”

Section: Discussionmentioning

confidence: 99%

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Wegener’s granulomatosis complicated by pulmonary embolism: a case report and review of the literature

2010

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We describe the case of a 79-year-old lady who developed pulmonary emboli on a background of active Wegener's granulomatosis (WG). The literature reports an increased association of venous thromboembolic disease and the small vessel vasculitides, including WG. This case report explores the issues surrounding systemic anticoagulation in the context of a systemic vasculitis that predisposes to severe bleeding, in particular from the respiratory tract.

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Section: Discussionsupporting

confidence: 65%

Section: Discussionmentioning

confidence: 99%

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Wegener’s granulomatosis complicated by pulmonary embolism: a case report and review of the literature

2010

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“…An increased risk of thromboembolic disease has been identified in adult patients with AASV [53,54]; children are also at risk [47].…”

Section: Clinical Features Of Aasvmentioning

confidence: 98%

“…Thromboprophylaxis with low-molecular weight heparin, as per local policy, should be instituted during any hospital admission for active vasculitis due to the increased risk of thrombosis in these patients [54].…”

Section: Treatment Of Aasvmentioning

confidence: 99%

ANCA-associated vasculitides—lessons from the adult literature

2010

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Anti-neutrophil cytoplasmic antigen antibody (ANCA)-associated disease is a rare manifestation of primary systemic vasculitis in paediatric patients but one that carries significant morbidity, potential long-term disability and early mortality. It therefore requires a high index of suspicion, targeted investigation, prompt treatment and long-term follow-up with specialist input at every stage. The well-recognised diversity and overlap in clinical, laboratory and histopathological features of the ANCA-associated systemic vasculitides continue to hamper accurate diagnosis, confounding epidemiological data and necessitating a blanket approach to treatment, which is largely extrapolated from studies in adult patients and carries significant side-effects. Herein we summarise current knowledge of the epidemiology, pathogenesis, principal manifestations, investigation and evidence-based management, extrapolated from adult studies, of these disorders. We also discuss recent efforts towards classification of the childhood vasculitides that emphasise the value of histological diagnosis. Progress in our understanding of the pathophysiology underlying ANCA-associated disease should lead to targeted, safer and more effective therapies for these conditions. Nonetheless, many questions remain outstanding, and academic paediatricians face real challenges in identifying and collating the few cases they encounter into study cohorts. Meeting this challenge will require international collaboration, not only among paediatricians but also with the specialists taking over care of these patients as they reach adulthood.

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