Rheumatology Advance Access originally published online on March 27, 2007
Rheumatology 2007 46(5):890; doi:10.1093/rheumatology/kem027
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A case of Budd-Chiari Syndrome presenting in a lady with newly diagnosed Churg-Strauss Syndrome
Rheumatology Department, Inverclyde Royal Hospital, Greenock, United Kingdom
Correspondence to: Claire D. Campbell, Email: clairestrang{at}doctors.org.uk
SIR, We present a case of Budd-Chiari Syndrome presenting in a lady with Churg-Strauss Syndrome (CSS). A 39-yr-old asthmatic lady presented in 2001 with a 6 week history of rash on her lower limbs. She was pyrexial and her peripheral blood film showed marked eosinophilia (56.9% of total white cell count). She was nauseated and had evidence of a lower limb peripheral neuropathy. On admission, ESR was 4 mm/h; coagulation screen, ANA and ANCA were normal. C3 was normal and C4 was low at 0.04 (normal range 0.19–0.45 g/l). Liver function tests were abnormal with raised transaminases. Skin biopsy confirmed an acute leukocytoclastic vasculitis within the dermis and a bone marrow aspirate showed increased eosinophil production with discreet areas of bone marrow necrosis. A diagnosis of CSS was made; she was commenced on high dose oral corticosteroids and intravenous immunoglobulin to reverse the thrombocytopaenia. Her clinical condition improved rapidly and her peripheral eosinophil count returned to normal within 2 days.
Four days following admission, she developed abdominal discomfort and swelling, confirmed by ultrasound to be ascites. A diagnostic paracentesis revealed a low eosinophil count in the ascitic fluid and no organisms. A Doppler ultrasound showed absent flow in several hepatic veins due to thrombosis and a diagnosis of Budd-Chiari syndrome was made. She was commenced on heparin and warfarin resulting in a dramatic improvement in symptoms and repeated ultrasound showed resolution of both thrombus and ascites. Anticoagulation was continued for 6 months after which there was no recurrence of thrombus or ascites. Following discharge she received two intravenous pulses of cyclophosphamide (stopped after development of a macular-papular rash), and subsequently oral azathioprine. Prednisolone dose was gradually reduced and she was discharged from clinic after 1 year. She was re-referred in 2004 after developing joint pains, tiredness and further vasculitic rash. ESR was 81 mm/h and eosinophil count 10.5%. Her symptoms responded to 10 mg of prednisolone, which was reduced and stopped over a period of 4 months. She is currently well and has had no further relapses.
Hepatic vein obstruction (Budd-Chiari syndrome) has been reported in association with idiopathic hypereosinophilic syndrome [1], but this is the first reported case in the context of CSS. This association may have been a coincidence, however thrombosis has been documented in CSS previously [2, 3]. Reported cases have been associated with either normal or raised eosinophil counts at the time of thrombus detection. Ames et al. [2] reported three patients with CSS who developed thrombosis; subclavian vein thrombosis, deep vein thrombosis and middle cerebral artery territory ischaemic stroke. In all three cases, eosinophilia was present at time of thrombosis and the patients all had raised levels of fibrinogen. Garcia et al. [3] reported three cases of venous thromboembolism (deep vein thrombi and pulmonary embolus). Two of these patients had had normal eosinophil counts at presentation, and the third had a blood count with 19% eosinophils.
Thrombosis has also previously been reported in patients with hypereosinophilia [1], and in a patient with venulitis [4]. It has been hypothesized that there may be a role for eosinophil granule proteins, such as Major Basic Protein (MBP) in the pathogenesis of thrombosis in conditions in which eosinophilia is a feature [5]. Wang et al. [6] hypothesize that the HOSCN, produced by eosinophils, may lead to a pro-thrombotic and pro-inflammatory state occurring in cases of thrombosis in conditions associated with raised eosinophils counts. These authors found that HOSCN induces tissue factor activity in human umbilical vein endothelial cells and the pro-inflammatory pathway p65/p50 NF-
B [6]. Young et al. [4] report a case of Budd-Chiari syndrome in which liver biopsy revealed multiple non-caseating granulomas, and a necrotizing granulomatous venulitis involving the hepatic vein, and a venogram showed hepatic venous obstruction. No systemic cause for thrombosis was found, and the patient had normal eosinophil count and coagulation. This patient improved with oral prednisolone 40 mg/day. Ames et al. [2] state that increased release of von Willebrand factor (vWF) from endothelial cells in vasculitis may contribute to thrombosis. Although not measured in our patient, raised levels of vWF may have been a contributing factor.
In conclusion, as far as we are aware, this is the first reported case of Budd-Chiari Syndrome presenting in association with CSS. Thrombosis has been previously documented, and the underlying mechanism may be related to raised eosinophil count or underlying vasculitis.
The authors have declared no conflicts of interest.
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[Abstract/Free Full Text] - Ames PRJ, Roes L, Lupoli S, et al. (1996) Thrombosis in Churg-Strauss Syndrome. Beyond vasculitis? Br J Rheumatol 35:1181–3.
[Abstract/Free Full Text] - Garcia G, Achouh L, Cobarzan D, Fichet D, Humbert M. (2005) Severe thromboembolic disease in Churg-Strauss syndrome. Allergy 60:409–10.[CrossRef][Web of Science][Medline]
- Young ID, Clark RN, Manley PN, Groll A, Simon JB. (1988) Response to steroids in Budd-Chiari Syndrome caused by idiopathic granulomatous venultis. Gastroenterology 94:503–7.[Web of Science][Medline]
- Maukai HY, Ninomiya H, Ohtani K, Nagasawa T, Abe T. (1995) Major basic protein binding to thrombomodulin potentially contributes to thrombosis in patients with eosinophilia. Br J Haematol 90:892–9.[Web of Science][Medline]
- Wang JG, Mahmud SA, Thompson JA, Geng JG, Kkey NS, Slungaard A. (2006) The principal eosinophil peroxidise product, HOSCN, is a uniquely potent phagocyte oxidant inducer of endothelial cell tissue factor activity: a potential mechanism for thrombosis in eosinophilic inflammatory states. Blood 107:558–65.
[Abstract/Free Full Text]
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