Heart 1997;77:576-578

CASE REPORT

Coronary involvement in the Churg-Strausssyndrome

S Hellemans, J Dens, D Knockaert

AbstractThe Churg-Strauss syndrome (CSS) is arare systemic disease characterised byvasculitis and peripheral eosinophilia inpatients with an atopic constitution.Cardiac involvement is an importantcause of morbidity and mortality yetcoronary involvement is very rarely docu-mented. We report the case of a 38 yearold man presenting with fulminant heartfailure. Coronary arteriography demon-strated extensive focal vasculopathy con-sistent with vasculitis. The diagnosis ofCSS was established based upon the clas-sical diagnostic criteria and corticos-teroid treatment resulted in a spectacularremission of disease activity.

Department ofCardiology,GasthuisbergUniversity Hospital,Catholic UniversityLeuven, Leuven,BelgiumS HellemansJ DensDepartment ofGeneral InternalMedicineD KnockaertCorrespondence to:Dr Hellemans, Departmentof Cardiology, GasthuisbergUniversity Hospital,Herestraat 49 B3000Leuven, Belgium.Accepted for publication27 March 1997

Figure 1 Renalarteriogram revealingmultiple microaneurysmsat the distal branches of theleft renal artery.

(Heart 1997;77:576-578)

Keywords: Churg-Strauss syndrome; coronary vasculitis

The Churg-Strauss syndrome (CSS) or aller-gic angiitis and granulomatosis is a rare pri-mary systemic vasculitis, typically charac-terised by a history of asthma or allergy, andby the occurrence of eosinophilia.' Seventyseven cases were examined at the Mayo Clinicduring 1950-92 and a recent epidemiologicalstudy estimated the annual incidence of CSSto be 2-4 per million population.2 3 Cardiacinvolvement was shown in up to 60% of thepost-mortem examinations in the original

report of Churg and Strauss, and accountedfor 50% of deaths in another series.1 4 5 Cardiacinvolvement is mostly thought to result froman interstitial myopathic process but vasculitisof the coronary vessels is documentedextremely rarely.4-6 We describe a typical caseof CSS presenting as fulminant cardiac failurewith angiographically documented coronaryvasculitis.

Case reportA 38 year old man with a five year history ofasthma was admitted to another hospital inNovember 1995 because of severe vomitingand diarrhoea. The peripheral blood eosino-phil count was 48% at that time. No underlyingdisease could be detected and he improvedspontaneously. One month later he was read-mitted because of fever. Despite extensiveinvestigation no infectious cause could befound and five days later diffuse pulmonaryinfiltrates developed. Echocardiographyrevealed left ventricular failure. Because of res-piratory distress, mechanical ventilation wasneeded, and inotropic support with dobuta-mine was instituted. At that time, he wastransferred to our hospital with the diagnosisof cardiorespiratory failure due to myocarditis.The referring cardiologist considered thepatient as a possible candidate for cardiactransplantation.On admission, his temperature was 37°C

and white blood cell count was 25-5 x 109/1with a shift to the left but no eosinophilia.Urine analysis revealed proteinuria, the sedi-ment was normal. The electrocardiogramshowed sinus tachycardia, incomplete rightbundle branch block, and diffuse abnormalrepolarisations.On echocardiography, the left ventricular

ejection fraction was 39%, the right ventricu-lar function was normal as were the heartvalves, and no pericardial effusion was pre-sent.

Because of suspicion of vasculitis, treatmentwith corticosteroids (prednisone 1 mg/kg) wasstarted after performing bronchoscopy withbronchoalveolar lavage which was normal.Endomyocardial biopsy documented noduliwith a central amorf necrosis surrounded byhistiocytes resembling Asschoffbodies.The patient was weaned from the ventilator

after 72 hours. Electromyography revealedsensorimotory polyneuropathy, and renal

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Coronary involvement in the Churg-Strauss syndrome

Figure 2 Coronary arteriogram showing vessel wall irregularities, abrupt terminations,and smaUl aneurysms at the posterior descendens and two inferolateral branches of the rightcoronary artery.

Figure 3 Coronary arteriogram after six months of corticosteroid treatment showingimportant regression of the focal vasculopathy.

angiography, performed because of importantproteinuria, revealed multiple microaneurysms(fig 1). Coronary arteriography was performedone week later and showed extensive focal vas-

culopathy with multiple vessel wall irregulari-ties, abrupt terminations, and small aneurysmsat a diagonal branch of the left anteriordescending coronary artery, the posteriordescendens, and two inferolateral branches ofthe right coronary artery (fig 2).

Six months later the patient was asympto-matic on treatment of 6 mg prednisone once a

day. A repeat coronary arteriography showedspectacular regression of the lesions (fig 3),and ventriculography showed normal left ven-tricular function.

DiscussionThe patient presented with angiographicallyproven vasculitis of the coronary and kidneyarteries. The multiple vessel wall irregularities,

multiple abrupt terminations, and the smallaneurysms (fig 2) were diagnostic for vasculi-tis. This can also be found in rheumatoidarthritis, polyarteritis nodosa, Kawasaki dis-ease, and systemic lupus erythematosus.Because of the history of asthma, the docu-mented eosinophilia of 48% on differentialwhite blood cell count, the presence of pul-monary infiltrates, and polyneuropathy, thiscase met both sets of criteria for the diagnosis ofCSS, recently developed by the AmericanCollege of Rheumatology.7 CSS typically hasthree phases and this was also the case in ourpatient. The initial prodromal phase consistsof allergic rhinitis, nasal polyposis or bronchialasthma. This is followed by a period of periph-eral and tissue eosinophilia commonly associ-ated with pulmonary infiltrates. The thirdphase is characterised by a systemic vasculitisthat can be fulminant and life threatening.5This vasculopathy preferentially affects thelungs, skin, heart, and peripheral nerves.Renal involvement is less frequent but waspresent in our case. Indeed the proteinuriasuggested glomerulonephritis but, because ofsuspicion of vasculitis, we performed renalangiography before considering kidney biopsy.The presence of multiple microaneurysms wasconsidered a contraindication for biopsy.The cardiac involvement in CSS consists of

pericarditis and occasionally tamponade,myocarditis, which can lead to terminalrestrictive or congestive heart disease, andmyocardial infarction.46 Endomyocardialbiopsy rarely shows acute inflammatorychanges and the presence of eosinophils,necrotising vasculitis or extravascular granu-loma is often non-diagnostic. To evaluate thecause of myocardial infarction, chest pain orheart failure in patients with CSS, coronaryarteriography has been performed in somecases and usually no abnormalities werefound. Coronary vasculitis was found atnecropsy in six of 10 cases originally reportedby Churg and Strauss.' 6 Coronary involve-ment has rarely been found premortem and toour knowledge this case represents the firstabnormal coronary arteriogram reported in apatient with CSS who survived. Another casewith an abnormal coronary arteriogram wasrecently reported but this patient died.6Cardiologists are not familiar with vasculitissyndromes because major cardiac problemsare rarely presenting manifestations. This caseunderlines the role of an aggressive invasivediagnostic approach in patients with evidenceof cardiac involvement by a vasculitis syn-drome. It also demonstrates the possible dra-matic reversal of severe cardiac disease withsteroid therapy.89 In our patient no additionalimmunosuppressive therapy was required.Delay in recognising the cardiac involvementprobably accounts for the important cardiacmortality in CSS.' 6 8

1 Churg J, Strauss L. Allergic granulomatosis. Allergic angi-itis and periarteritis nodosa. Am J Pathol 1951;27:277-301.

2 Watts R, Carruthers D, Scott D. Epidemiology of systemicvasculitis: changing incidence or definition? SeminArthritis Rheum 1995;25:28-34.

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Hellemans, Dens, Knockaert

3 Sehgal M, Swanson JW, Desemee RA, Colby TV.Neurologic manifestations of Churg-Strauss syndrome.Mayo Clin Proc 1995;70:337-41.

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7 Masi AT, Hunger GG, Lie JT, Michel BA, Bloch DA,

Arend WP, et al. The American College ofRheumatology 1990 criteria for the classification ofChurg-Strauss syndrome (Allergic granulomatosis andangiitis). Arthritis Rheum 1990;33:1094-100.

8 Abu-Shakra M, Smythe H, Lewtas J, Badley E, Weber D,Keystone E. Outcome of polyarteritis nodosa andChurg-Strauss syndrome. Arthritis Rheum 1994;37:1798-803.

9 Guillevin L, Lhote F, Cohen P, Jarrousse B, Loztholary 0,

Genereau T, et al. Corticosteroids plus pulse cyclophos-phamide and plasma exchanges versus corticosteroidsplus pulse cyclophosphamide alone in the treatment ofpolyarteritis nodosa and Churg-Strauss syndromepatients with factors predicting poor prognosis. ArthnitisRheum 1995;38:1638-45.

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