Elsevier

The Lancet

Volume 361, Issue 9357, 15 February 2003, Pages 587-594
The Lancet

Seminar
Churg-Strauss syndrome

https://doi.org/10.1016/S0140-6736(03)12518-4Get rights and content

Summary

Churg-Strauss syndrome is a rare diffuse vasculitis that is almost invariably accompanied by severe asthma. Although overall prognosis is good, and treatment with prednisone alone or in combination with immunosuppressive drugs is usually successful, severe asthma typically persists. Diffuse organ involvement of Churg-Strauss syndrome, especially cardiovascular and rare involvement of the CNS and renal system, suggests a poorer prognosis than usual, and can be fatal. The cause of Churg-Strauss syndrome is unknown, but its characteristic histological findings and association with asthma distinguish it from other vasculitides. Controversy surrounds the use of asthma drugs—especially antileukotrienes—and development of the disorder. We review the epidemiological evidence for an association of drug treatment with Churg-Strauss syndrome, the diverse diagnostic and pathological criteria for this syndrome, and treatment options.

Section snippets

Pathophysiology

The cause of Churg-Strauss syndrome is unknown. Asthma and atopy are closely linked, and both are associated with blood eosinophilia. An infectious agent or foreign antigen, which might be inhaled, could initiate allergic inflammation in a genetically susceptible individual, resulting in rhinosinusitis and asthma. Blood and tissue eosinophilia then arise, with infiltrative eosinophilic pneumonia or gastroenteritis in some patients. Vascular inflammation may result from endothelial-cell adhesion

Diagnostic criteria

Syndromes are a constellation of symptoms or pathological findings, which vary in accordance with the diagnostic criteria that are applied. Churg-Strauss syndrome is especially complicated in this respect, since asthma—which is itself a syndrome of reversible airflow obstruction and inflammation—almost always is a component of the disorder. Because Churg-Strauss syndrome occurs rarely, prospective diagnosis in a large series of patients is difficult.

Churg and Strauss initially described the

Frequency of Churg-Strauss syndrome

For reasons given above, the prevalence of Churg-Strauss syndrome in the population varies between reports. In patients in the general population, the frequency of the disorder has been estimated at 2·4–6·8 per 10 00 000 patient-years (panel 2).21, 22 In a 10-year study in the UK, the annual frequency of Churg-Strauss syndrome was 2·7 per 10 00 000 patient-years.22 Watts and colleagues23 reported a significant difference in incidence of the disorder between Spain and the UK.

Diagnosis

Despite the continuous redefinition of diagnostic criteria for Churg-Strauss syndrome (see above), diagnosis remains a clinical one. Pathological confirmation of eosinophilic tissue infiltration or vasculitis is desirable whenever possible. Diagnosis of Churg-Strauss syndrome is suggested when a previously healthy individual presents with adult-onset allergic rhinitis or recurrent sinusitis associated with asthma and followed by signs and symptoms of systemic vasculitis. Diagnosis, however, is

Prognosis

Prognosis for Churg-Strauss syndrome is generally good. Early assessments of prognosis included this disorder with polyarteritis nodosa, thus attenuating projected expectations of remission and cure. However, the disorder has a better rate of long-term remission than polyarteritis nodosa.42, 43, 44 With the introduction of corticosteroid treatment, remission and survival have improved greatly. Patients with limited organ involvement respond well to systemic corticosteroids alone. Those needing

Corticosteroid therapy

Corticosteroids are the cornerstone of treatment for Churg-Strauss syndrome. Because these drugs are usually sufficient for treatment of most patients who do not have severe organ involvement, they should be viewed as first-line therapy, without addition of other immunosuppressive agents. Frequent complications of treatment include iatrogenic Cushing's syndrome, steroid-induced diabetes, corticosteroid-induced myopathy, steroid psychosis, osteoporosis with vertebral fractures,46 avascular

Conclusions

There is no one set of criteria for accurate diagnosis of Churg-Strauss syndrome. Confirmation by biopsy of vasculitis is complicated by the unpredictable and often sequential onset of manifestations of the disease in the various organs it involves, and by its incomplete evolution even in organs that are affected. Clinical diagnosis alone is difficult in early stages of Churg-Strauss syndrome because of the substantial overlap between the disorder and the usual symptoms of severe asthma.

Search strategy and selection criteria

Papers were selected for review by Medline search with relevant cross-referenced topics. Keywords used were Churg-Strauss syndrome; vasculitis and drugs; small vessel vasculitis; asthma and vasculitis; and we searched English language articles. In selected cases, further review of cited papers was obtained directly from the references of papers initially reviewed in search. Non-peer-reviewed publications or single case reports were not included in this review. Abstracts not yet published

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