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European Journal of Echocardiography 2004 5(1):65-67; doi:10.1016/S1525-2167(03)00037-4
© 2004 by European Society of Cardiology
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Copyright © 2003, The European Society of Cardiology

Churg–Strauss syndrome as an unusual cause of spontaneous atraumatic intra-pericardial thrombosis

E.W Laua,*, D.M Carruthersb and N Prasada

aDepartment of Cardiology, City Hospital, Dudley Road, Birmingham B18 7QH, UK
bDepartment of Rheumatology, City Hospital, Dudley Road, Birmingham B18 7QH, UK

Received 23 December 2002; received in revised form 8 April 2003; accepted after revision 10 April 2003.

* Corresponding author. University of Ottawa Heart Institute, Room H145, 40 Ruskin Street, Ottawa, Ontario, Canada K1Y 4W7. Tel.: +1-613-761-4914; fax: +1-613-761-4407. elau{at}ottawaheart.ca


   Abstract
Top
 Abstract
1. Introduction
 2. Case details
 3. Discussion
 References
 
Intra-pericardial thrombosis is usually due to haemorrhage into the pericardial space or after traumatic pericardiocentesis. An unusual case of spontaneous atraumatic intra-pericardial thrombosis due to Churg–Strauss syndrome, which responded well to immunosuppressive therapy, is presented.

Keywords: intra-pericardial thrombosis; Churg–Strauss syndrome


   1. Introduction
Top
 Abstract
 1. Introduction
2. Case details
 3. Discussion
 References
 
Even though a pericardial effusion is a common echocardiographic finding, intra-pericardial thrombosis is not. Most of the cases of intra-pericardial thrombosis encountered clinically are due to haemorrhage into the pericardial space (e.g. aortic dissection, cardiac rupture) or after traumatic pericardiocentesis.1–3 An unusual case of spontaneous atraumatic intra-pericardial thrombosis due to Churg–Strauss syndrome (CSS) is presented.


   2. Case details
Top
 Abstract
 1. Introduction
 2. Case details
3. Discussion
 References
 
A 31-year-old woman presented with a 3-month history of progressive nodular rash on the hands and feet, a dry cough and pleuritic chest pain. Her past medical history included alopecia and asthma. On examination, she had a low-grade pyrexia at 37.4 °C, splinter haemorrhages in the fingers and a palpable purpuric rash on the hands and feet, but no other remarkable features. Her eosinophil count was grossly elevated at 20x109/l. Her C-reaction protein and erythrocyte sedimentation rate were moderately elevated at 72 mg/l and 50 mm/h, respectively. She was weakly positive for rheumatoid factor (titre 1:80), but negative for antinuclear antibodies and antineutrophil cytoplasmic antibodies. Her chest X-ray showed cardiomegaly without pulmonary oedema or pulmonary infiltrates. An echocardiogram showed a structurally normal heart with no evidence of any vegetation or valvular dysfunction. The left ventricle had normal systolic and diastolic functions and its cavity was free of thrombus. However, there was a moderate-sized global effusion measuring 1.5 cm in thickness and containing prominent clump-like structures, which were interpreted as thrombi (Fig. 1). No features of tamponade were present. Histology of her skin nodules showed eosinophilic vasculitis.


Figure 1
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  		Figure 1 Pericardial effusion containing clump-like structures in a transthoracic echocardiogram taken from the back of the chest. An unorthodox posterior view was used, as it best displayed the clump-like structures in the pericardial effusion, which accumulated mostly posteriorly. A left pleural effusion was also noticed.

 
On the basis of the history and investigation results, the diagnosis of CSS was made. Pericardiocentesis was not performed because the pericardial effusion accumulated mostly posteriorly, which made it technically difficult to drain, and was not causing any haemodynamic compromise. Instead, she was treated with high-dose steroid therapy (intravenous methylprednisolone 500 mg twice, followed by oral prednisolone 40 mg o.d.) and experienced a dramatic clinical improvement within 2 days. A repeat echocardiogram 6 weeks after the initial one showed complete resolution of her pericardial effusion and intra-pericardial thrombosis (Fig. 2). The patient was successfully maintained in remission on oral steroid and azathioprine.


Figure 2
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  		Figure 2 Resolution of pericardial effusion after immunosuppressive therapy.

 

   3. Discussion
Top
 Abstract
 1. Introduction
 2. Case details
 3. Discussion
References
 
CSS is a systemic vasculitic disorder characterised by asthma and eosinophilia4 that can affect the lungs (pulmonary infiltrates and nodules, hilar lymphadenopathy and pleural effusion), skin (rashes and subcutaneous nodules), nervous system (mononeuritis multiplex and polyneuropathy), gastrointestinal tract (obstruction and haemorrhagic diarrhoea) and the heart.5,6 Cardiac involvement is common in CSS, occurring in 40% of cases,5,6 and can take the form of coronary vasculitis,7 pericardial effusion,6,8 acute pericarditis, myocarditis, intra-cardiac thrombosis,9 endomyocardial fibrosis and cardiac failure.10 However, spontaneous atraumatic intra-pericardial thrombosis has not previously been reported in association with CSS.

The thrombotic process in CSS may be due to a combination of local hypercoagulability and stasis from the vasculitic process and subsequent fibrosis involving the underlying endomyocardium.10 When a pericardial effusion is present in CSS, the fluid usually is an exudate with a low glucose content but marked eosinophilia.6 Extrapolating from the knowledge on the hypereosinophilic syndrome, another condition associated with intense hypereosinophilia, prominent intra-cardiac thrombosis and endomyocardial fibrosis,11 release of toxic granules by eosinophils may also contribute to intra-pericardial thrombosis in CSS.12,13 It is possible that without intense steroid therapy, pericardial fibrosis and constriction could have ensued. Constrictive pericarditis has previously been reported in association with the hypereosinophilic syndrome.14

There was no easy way to obtain a histological proof that the clump-like intra-pericardial structures observed in this case were actually thrombi. Pericardiocentesis would not have helped, as the procedure itself could have induced intra-pericardial thrombosis, and surgical pericardiotomy could not have been justified on clinical grounds. On this basis, the assertion that the intra-pericardial structures represented thrombi must remain speculative. For example, it is possible that they were made up of agglutinated proteins. But regardless of their exact chemical and biological compositions, what is clinically relevant is that the morphological appearance of these intra-pericardial structures on two-dimensional echocardiogram is suggestive of intra-pericardial thrombosis and should alert the attending clinician to the possible underlying diagnosis of CSS.

In conclusion, spontaneous atraumatic intra-pericardial thrombosis in the presence of hypereosinophilia should raise suspicion about CSS. This is important as prompt diagnosis and early treatment of the condition can lead to rapid clinical improvement and avoid long-term complications.


   References
Top
 Abstract
 1. Introduction
 2. Case details
 3. Discussion
 References
 

  1. Preis L.K., Taylor G.J., Martin R.P. Traumatic pericardiocentesis: two-dimensional echocardiographic visualization of an unfortunate event. Arch Intern Med (1982) 142:2327–2329.[CrossRef][Web of Science][Medline]
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  5. Reid A.J., Harrison B.D., Watts R.A., Watkin S.W., McCann B.G., Scott D.G. Churg–Strauss syndrome in a district hospital. QJM (1998) 91:219–229.[Abstract/Free Full Text]
  6. Solans R., Bosch J.A., Perez-Bocanegra C., Selva A., Huguet P., Alijotas J., et al. Churg–Strauss syndrome: outcome and long-term follow-up of 32 patients. Rheumatology (2001) 40:763–771.[Abstract/Free Full Text]
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  14. Lui C.Y., Makoui C. Severe constrictive pericarditis as an unsuspected cause of death in a patient with idiopathic hypereosinophilic syndrome and restrictive cardiomyopathy. Clin Cardiol (1988) 11:502–504.[Web of Science][Medline]

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