Pulmonary, cardiac and renal syndrome

(Section Editor: T.J. Rabelink)

Jonathan M. Gleadle, David Davies and Paul Altmann

Oxford Kidney Unit, Churchill Hospital, Oxford, UK

A 30-year-old man presented with symptoms of intermittent wheeze and breathlessness. A diagnosis of asthma was made and he received a 5-day course of oral prednisolone and inhaled beta-2 agonists. Five months later he became unwell with fatigue, anorexia, weight loss and was again wheezy. He received a course of amoxycillin without any symptomatic improvement. He was admitted to hospital and was found to have multiple splinter haemorrhages and a vasculitic rash particularly affecting the palms. Investigations included a normal chest radiograph but a substantially elevated blood eosinophil count of 35x109/l. There was evidence of mild renal impairment with a measured creatinine clearance of 69 ml/min and protein excretion of 0.15 g/24 h. An ECG showed widespread T-wave inversion.

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The very pronounced eosinophilia, asthma, cutaneous signs of vasculitis (splinters and vasculitic rash) and evidence of renal and cardiac involvement are highly suggestive of a diagnosis of Churg-Strauss syndrome.

A renal biopsy demonstrated an acute eosinophilic interstitial nephritis (Figure 1aGo) and evidence of vasculitis with intimal proliferation and fibrinoid change (Figure 1bGo). An ANCA was not detected. An echocardiogram revealed a dilated left ventricle with diffuse hypokinesis.



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Fig. 1. Histology of the renal biopsy appearances in this patient with Churg-Strauss Syndrome showing (a) tubules with a prominent interstitial infiltrate including large numbers of eosinophils and (b) an artery showing vasculitis with fibrinoid change in part of its wall and further interstitial infiltrate.

 
The patient was treated with corticosteroids and oral cyclophosphamide and commenced on an angiotensin converting enzyme inhibitor. There was rapid resolution of the cutaneous signs and of the peripheral eosinophilia. A repeat echocardiogram three months after the initiation of treatment showed considerable improvement in left ventricular function.

Discussion

Churg-Strauss syndrome is a rare systemic vasculitis with an incidence of 2–3/million/year and which can cause renal disease [1]. The typical clinical pattern includes a prodrome of late onset asthma, peripheral blood eosinophilia, eosinophilic tissue infiltrates and vasculitis affecting extrapulmonary sites. The renal histological findings include focal necrotizing glomerulonephritis, a vasculitis often accompanied by a marked eosinophilic infiltrate and an eosinophilic interstitial nephritis has also been described. As in other systemic vasculitides, antibodies to neutrophil cytoplasmic antibody (ANCA) may be present, may correlate with disease activity and might be of pathogenetic importance. In Churg-Strauss syndrome such antibodies appear to be predominantly against myeloperoxidase and in one series were detected in 60% of cases [2].

As occurred in this patient, cardiac involvement is well recognized in Churg-Strauss syndrome and may be associated with an adverse prognosis. Other features associated with poor prognosis are renal impairment, proteinuria, gastointestinal tract involvement and CNS signs [3]. Cardiac involvement may manifest as cardiomyopathy, coronary vasculitis, myocardial fibrosis and mitral regurgitation [4].

Churg-Strauss syndrome is usually treated with corticosteroids which often produce significant clinical improvement. As in treatment of other systemic vasculitides, the administration of cytotoxic agents such as cyclophosphamide is recommended in severe disease, whilst the use of plasma exchange is unproven [1].

Notes

Supported by an educational grant from

Suggested reading

  1. Gaskin G, Clutterbuck EJ, Pusey CD. Renal disease in the Churg-Strauss syndrome. Diagnosis, management and outcome. Contrib Nephrol1991; 94: 58–65[Medline]
  2. Guillevin L, Visser H, Noel LH et al. Antineutrophil cytoplasm antibodies in systemic polyarteritis nodosa with and without hepatitis B virus infection and Churg-Strauss syndrome—62 patients. J Rheumatol1993; 20: 1345–1349[ISI][Medline]
  3. Guillevin L, Lhote F, Gayraud M et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore)1996; 75: 17–28[ISI][Medline]
  4. Kozak M, Gill EA, Green LS. The Churg-Strauss syndrome. A case report with angiographically documented coronary involvement and a review of the literature. Chest1995; 107: 578–580[Abstract/Free Full Text]




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