BACKGROUND: Pulmonary haemorrhage (PH) is a serious manifestation of systemic vasculitis with high mortality rates yet vasculitis is associated with an increased prevalence of venous thromboembolism (VTE). The concurrent presentation of severe PH and VTE poses a challenge in terms of therapeutic management. METHODS: This is a retrospective case review of the clinical manifestations and response to treatment in vasculitis patients presenting with concurrent pulmonary haemorrhage and VTE (pulmonary embolism and/or deep venous thrombosis). RESULTS: Of 35 patients with severe PH due to systemic vasculitis, 7 (20%) had concurrent VTE. The most common cause was anti-neutrophil cytoplasm antibody-associated vasculitis, followed by anti-glomerular basement membrane disease. Vasculitis responded to conventional therapies and VTE treatment with anticoagulation was uncomplicated in five of six cases. In one case, anticoagulation precipitated the PH and another was not anticoagulated and developed recurrent VTE. All patients survived without further complications after a mean follow-up of 46 months (3-98). CONCLUSIONS: Concurrent VTE occurred in one-fifth of cases with severe PH due to vasculitis. Management of VTE with anticoagulation was effective but led to pulmonary haemorrhage in one patient.
BACKGROUND:Pulmonary haemorrhage (PH) is a serious manifestation of systemic vasculitis with high mortality rates yet vasculitis is associated with an increased prevalence of venous thromboembolism (VTE). The concurrent presentation of severe PH and VTE poses a challenge in terms of therapeutic management. METHODS: This is a retrospective case review of the clinical manifestations and response to treatment in vasculitispatients presenting with concurrent pulmonary haemorrhage and VTE (pulmonary embolism and/or deep venous thrombosis). RESULTS: Of 35 patients with severe PH due to systemic vasculitis, 7 (20%) had concurrent VTE. The most common cause was anti-neutrophil cytoplasm antibody-associated vasculitis, followed by anti-glomerular basement membrane disease. Vasculitis responded to conventional therapies and VTE treatment with anticoagulation was uncomplicated in five of six cases. In one case, anticoagulation precipitated the PH and another was not anticoagulated and developed recurrent VTE. All patients survived without further complications after a mean follow-up of 46 months (3-98). CONCLUSIONS: Concurrent VTE occurred in one-fifth of cases with severe PH due to vasculitis. Management of VTE with anticoagulation was effective but led to pulmonary haemorrhage in one patient.
Authors: Andreas Kronbichler; Johannes Leierer; Jae Il Shin; Peter A Merkel; Robert Spiera; Philip Seo; Carol A Langford; Gary S Hoffman; Cees G M Kallenberg; E William St Clair; Paul Brunetta; Fernando C Fervenza; Duvuru Geetha; Karina A Keogh; Paul A Monach; Steven R Ytterberg; Gert Mayer; Ulrich Specks; John H Stone Journal: Arthritis Rheumatol Date: 2019-09-25 Impact factor: 10.995