SESSION TITLE: Critical Care Case Report Posters II
SESSION TYPE: Affiliate Case Report Poster
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: Non-specific symptoms with multisystem involvement of ENT, lungs, kidneys, skin, or pulmonary hemorrhage classically can prompt the clinician to consider ANCA-associated vasculitides (AAV). In addition, increased incidence of venous thromboembolism (VTE) has been described. We present a case with initial severe presentation noted to have VTE.
CASE PRESENTATION: A 61 year-old male had recently travelled to California from Australia for wine research. Upon arrival to the US, he was hospitalized with a near syncopal episode and was found to have deep venous thrombosis in left lower extremity and was started on intravenous heparin. He had mild hematuria and renal dysfunction initially attributed to dehydration. Over the next 24 hours, he developed hypoxemic respiratory failure and progressive renal failure. He developed ARDS and significant bloody secretions. Heparin drip was stopped and IVC filter placed. Computerized Topographic angiogram showed pulmonary embolism (PE) and extensive ground glass opacities. Bronchoscope showed diffuse alveolar hemorrhage (DAH). Studies were sent for vasculitides and hemodialysis was initiated for progressive renal failure. Serological testing demonstrated elevated myeloperoxidase (MPO) and renal biopsy showed pauci-immune necrotizing glomerulonephritis. A diagnosis of myeloperoxidase ANCA disease/ microscopic polyangitis (MPA) was made. He was treated with Therapeutic plasma exchange (TPE), Glucocorticoids and Rituximab. He was extubated five days later and radiographic infiltrates eventually cleared after 3 weeks.
DISCUSSION: AAV may present with life threatening clinical scenarios and prompt diagnosis is essential and potentially lifesaving. The diagnosis is best made by clinical findings and presence of positive ANCA test followed by histologic examination of biopsy tissue. Treatment consists of immunosuppressant, glucocorticoids and in severe cases TPE as well.
CONCLUSIONS: There is increased awareness of incidence of VTE in AAV likely related to endothelial changes present during active disease. The incidence of VTE in AAV is <1%, with 90% mortality. If associated with DAH, it is recommended to place an IVC filter followed by treatment with TPE, Glucocorticoids and Rituximab. Anticoagulation may be initiated only when ANCA titer and Birmingham Vasculitis Activity Score (BVAS) improves
Reference #1: QJM. 1994 Nov;87(11):671-8. Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. Luqmani RA et al
Reference #2: Rheumatology (2008) 47 (4): 530-534. Venous thromboembolism in ANCA-associated vasculitis—incidence and risk factors Authors: P. M. Stassen, R. P. H. Derks, C. G. M. Kallenberg C. A. Stegeman
DISCLOSURE: The following authors have nothing to disclose: Leena Gupta, Bruce Dreyfuss, Srilakshmi Vemulakonda, Cyrus Shariat, Koorosh Shariat, Gill Narinder, Francisco Brun
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