In view of this very promising response, bosentan might bea viable option for the treatment of severe arterial cutaneousulcers associated with disorders of the vasculature,4 such asthose characteristic of CUA. Indeed, bosentan seems effectivein the treatment of systemic sclerosis-associated digitalulcers.5 While ‘traditional’ proposed treatments for CUAinclude sodium thiosulfate,6 hyperbaric oxygen,7 and infu-sion of tissue plasminogen activator,8 none of these have besubjected to rigorous randomized controlled trials. While notconstituting an approved use of the drug, CUA, or syndromesclosely resembling CUA, may be amenable to treatment withendothelin-1 receptors antagonists, a prospect which in ourview warrants further investigation.

ACKNOWLEDGEMENTS

The authors thank Luis Sánchez-Grande Alarcón, MD andWesley E. Chang, MD, for helpful insights on the radiologicfindings, and Rafael Abellón-Carsi, MD, for important con-tributions facilitating the writing and translation of themanuscript.

Disclosures: The authors have no conflicts of interest todeclare.

Sources of Funding: None.

Accepted for publication 9 February 2014.

doi:10.1111/nep.12218

REFERENCES

1. Weenig RH, Sewell LD, Davis MD et al. Calciphylaxis: Natural

history, risk factor analysis, and outcome. J. Am. Acad. Dermatol.

2007; 56: 569–79.

2. Ross EA. Evolution of treatment strategies for calciphylaxis. Am. J.

Nephrol. 2011; 34: 460–67.

3. Nigwekar SU, Wolf M, Sterns RH et al. Calciphylaxis from

nonuremic causes: A systematic review. Clin. J. Am. Soc. Nephrol.

2008; 3: 1139–43.

4. Kaoukis A, Deftereos S, Raisakis K et al. The role of endothelin

system in cardiovascular disease and the potential therapeutic

perspectives of its inhibition. Curr. Top. Med. Chem. 2013; 13:

95–114.

5. Humbert M, Cabane J. Successful treatment of systemic sclerosis

digital ulcers and pulmonary arterial hypertension with endothelin

receptor antagonist bosentan. Rheumatology 2003; 42: 191–3.

6. Salmhofer H, Franzen M, Hitzl W et al. Multi-modal treatment of

calciphylaxis with sodium-thiosulfate, cinacalcet and sevelamer

including long-term data. Kidney Blood Press. Res. 2013; 37:

346–59.

7. Podymow T, Wherrett C, Burns KD. Hyperbaric oxygen in the

treatment of calciphylaxis: A case series. Nephrol. Dial. Transplant.

2001; 16: 2176–80.

8. el-Azhary RA, Arthur AK, Davis MD et al. Retrospective analysis of

tissue plasminogen activator as an adjuvant treatment for

calciphylaxis. JAMADermatol. 2013; 149: 63–7.

CHYLOUS PERICARDIAL EFFUSION INGRANULOMATOSIS WITH POLYANGIITIS

Nicholas Larkins,1 Kevin C Harris,2

Kimberly Morishita3 and Douglas G Matsell1,1Division of Nephrology, Department of Pediatrics, 2Divisionof Cardiology, Department of Pediatrics, and 3Division ofRheumatology, Department of Pediatrics, University ofBritish Columbia, Vancouver, Canada

We describe a 15-year-old girl known to have granu-lomatosis with polyangiitis (GPA) who presented duringfollow-up with a large chylous pericardial effusion.

Her initial presentation 2 months prior was with pulmonaryhaemorrhage requiring admission to the intensive care unit.At that time, her urine sediment was active despite a normalserum creatinine (42 μmol/L). An elevated anti neutrophilcytoplasmic antibody titre was demonstrated (1:320), with anelevated proteinase 3 (1:80) and normal myeloperoxidasetitre (1:2). A renal biopsy demonstrated pauci-immune, focalsegmental fibrinoid necrosis with cellular crescents. She wastreated with pulse methylprednisolone and intravenous(IV) cyclophosphamide, to which her pulmonary diseaseresponded well. However, her renal disease remained activedespite continued cyclophosphamide and prednisone.

On review she had symptoms of increased cough andshortness of breath despite normal vital signs. A chest X-ray(CXR) demonstrated significant cardiomegaly (Fig. 1), whichwas not present on CXR performed 2 weeks prior. Anechocardiogram demonstrated a large pericardial effusion

Fig. 1 Marked cardiomegaly due to a large pericardial effusion.

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Correspondence

© 2014 Asian Pacific Society of Nephrology 367

with features of tamponade. Pericardiocentesis drained450 mL of milky white fluid. Fluid chemistry confirmed thediagnosis of chylopericardium with a triglyceride concentra-tion of 9.19 mmol/L, lactate dehydrogenase 354 U/L, andpredominance of lymphocytes. Ultrasound of the internaljugular, subclavian and brachiocephalic veins and magneticresonance imaging of the thorax ruled out thrombosisas a cause of her chylopericardium. There was ongoingpericardial drainage for three days after which IV octreotidewas started. The drainage subsided and the drain wasremoved on day four.

On follow-up the effusion did not recur and there has beenno further cardiac involvement 6 months later. Unfortu-nately, the patient’s renal disease has remained recalcitrantto treatment including rituximab. Her current serumcreatinine is 459 μmol/L. A recent renal biopsy demon-strated widespread glomerulosclerosis.

Cardiac involvement in GPA is uncommon, affecting 6–9%of children and adults with the disease.1,2 Pericarditis is themost common form of cardiac involvement, although coro-nary arteritis is equally common at autopsy.3 Less frequentmanifestations include endocarditis, valvular lesions andarrhythmia.4 We believe this to be the first report of chylous

pericardial effusion in GPA. The case highlights the diversityand seriousness of cardiac disease that may occur in thiscondition. As such patients need to be carefully followed forcardiac involvement, in particular pericarditis, during thecourse of their disease.

No disclaimers or conflict of interest.

Accepted for publication 8 April 2014.

doi:10.1111/nep.12262

REFERENCES

1. Hoffman GS, Kerr GS, Leavitt RY et al. Wegener granulomatosis: An

analysis of 158 patients. Ann. Intern. Med. 1992; 116: 488–98.

2. Rottem M, Fauci AS, Hallahan CW et al. Wegener granulomatosis in

children and adolescents: Clinical presentation and outcome. J.

Peditr. 1993; 122: 26–31.

3. Forstot JZ, Overlie PA, Neufeld GK, Harmon CE, Forstot SL. Cardiac

complications of Wegener granulomatosis: A case report of complete

heart block and review of the literature. Semin. Arthritis Rheum.

1980; 10: 148–54.

4. Grant SCD, Levy RD, Venning MC, Ward C, Brooks NH. Wegener’s

granulomatosis and the heart. Br. Heart J. 1994; 71: 82–6.

Correspondence

© 2014 Asian Pacific Society of Nephrology368