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
SeminarChurg-Strauss syndrome
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
References (67)
- et al.
Workshop summary report: the relationship of asthma therapy and Churg-Strauss syndrome
NIH Workshop Summ Rep
(2001) - et al.
Involvement of soluble CD95 in Churg-Strauss syndrome
Am J Pathol
(1999) - et al.
Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis
Kidney Int
(1998) - et al.
Churg-Strauss angiitis: arguments favoring the responsibility of inhaled antigens
Chest
(1991) - et al.
Churg-Strauss syndrome in patients receiving montelukast as treatment for asthma
Chest
(2000) - et al.
Churg-Strauss syndrome after corticosteroid withdrawal in an asthmatic patient treated with pranlukast
J Allergy Clin Immunol
(1999) - et al.
The leukotriene C4 transporter MRPI regulates CCL19 (MIP-3beta, ELC)-dependent mobilization of dendritic cells to lymph nodes
Cell
(2000) - et al.
Churg-Strauss syndrome
Ann Allergy Asthma Immunol
(2001) - et al.
Thoracic manifestation of Churg-Strauss syndrome: radiologic and clinical findings
Chest
(2000) - et al.
Longterm followup study of periarteritis nodosa
Am J Med
(1967)
Immunosuppressive and corticosteroid therapy of polyarteritis nodosa
Am J Med
Sputum eosinophilia in Churg Strauss syndrome
Respir Med
Cyclophosphamide hemorhagic cystitis requiring urinary diversion
J Urol
The incidence of bladder cancer after cyclophosphamide therapy
J Urol
Treatment of systemic vasculitis with pooled intravenous immunoglobulin
Lancet
Pathogenesis of ANCA-associated systemic vasculitis
J Pathol
Churg-Strauss syndrome: serum markers of lymphocyte activation and endothelial damage
Arthritis Rheum
Clinicopathological features of Churg-Strauss syndrome-associated neuropathy
Brain
Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome
Medicine(Baltimore)
Churg-Strauss syndrome: clinical study and long-term follow-up of 96 patients
Medicine
Anti-neutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitro
Proc Natl Acad Sci USA
Nomenclature of systemic vasculitides: proposal of an international consensus conference
Arthritis Rheum
Small-vessel vasculitis
N Engl J Med
Allergic granulomatosis, allergic angiitis, and periarteritis nodosa
Am J Pathol
Churg-Strauss syndrome in a district hospital
QJM
The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis)
Arthritis Rheum
Churg-Strauss syndrome: report of a case without pre-existing asthma
J Dermatol
Allergic granulomatosis and angiitis (Churg-Strauss syndrome): report of an autopsy case in a nonasthmatic patient
Acta Pathol Jpn
Churg-Strauss syndrome presenting with marked eosinophilia and pericardial effusion
Can J Cardiol
Nonasthmatic case of Churg-Strauss syndrome with rapidly progressive glomerulonephritis
Intern Med
Gastrointestinal presentation of Churg Strauss syndrome
Sarcoidosis
Prevalence of Churg-Strauss syndrome, vasculitis, eosinophila and associated conditions: retrospective analysis of 58 prescription-event monitoring cohort studies
Pharmacoepidemiol Drug Safety
Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom
Arthritis Rheum
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