title: Therapeutic implications of coexisting severe pulmonary hemorrhage and pulmonary emboli in a case of Wegener granulomatosis.
abstract: Wegener granulomatosis classically involves the renal, respiratory, and ear, nose, and throat systems. Pulmonary hemorrhage is recognized as a severe respiratory complication. Untreated, the mortality rate approaches 90% at 2 years. We describe a case of Wegener granulomatosis with coexistent severe lung hemorrhage and pulmonary and deep vein thromboses. A 31-year-old man presented with features of vasculitis, including epistaxis, fever, and acute kidney injury with an increased serum creatinine level (3.27 mg/dL). Kidney biopsy confirmed pauci-immune crescentic glomerulonephritis, and antineutrophil cytoplasmic antibody showing a cytoplasmic staining pattern was strongly positive. Standard immunosuppression therapy (prednisolone and cyclophosphamide) was started. Eleven days later, the patient developed sudden dyspnea. A computed tomographic pulmonary angiogram showed pulmonary emboli, and ultrasound of the limbs showed ileofemoral thrombi bilaterally. Subcutaneous enoxaparin and warfarin therapy was started, but 8 days later, the patient had a massive pulmonary hemorrhage. Anticoagulation therapy was stopped, and plasma exchange was started to prevent further life-threatening hemorrhage. An inferior vena cava filter was inserted to prevent further pulmonary emboli during the period when anticoagulation was withheld. Kidney function improved, and pulmonary hemorrhage resolved after 5 plasma exchanges. Reintroduction of intravenous heparin and subsequently warfarin caused no further bleeding. We discuss the difficult management dilemma this combination of disease manifestations presents and review the current literature.
authors.affiliation: Renal Unit, Royal London Hospital, London, UK. gavin.dreyer@bartsandthelondon.nhs.uk
authors.forename: Gavin
authors.initials: G
authors.lastname: Dreyer
authors.forename: Stanley
authors.initials: S
authors.lastname: Fan
mesh_terms: D000328:Adult; D000925:Anticoagulants / Q000008:administration & dosage*; D004359:Drug Therapy, Combination; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000175:diagnosis; D006469:Hemoptysis / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D006493:Heparin / Q000008:administration & dosage*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007279:Injections, Subcutaneous; D008297:Male; D010951:Plasma Exchange / Q000379:methods*; D011655:Pulmonary Embolism / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D016306:Vena Cava Filters
chemical_list: D000925:Anticoagulants; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D006493:Heparin