Related to this topic: Support | UK Guidelines | Weblinks | Equipment | Books | Your Experience | Other resources | Glossaries
Print options: Printer friendly version of this leaflet (html)     Other options:  AddThis Social Bookmark Button (what's this?)

PatientPlus articles are written for doctors and so the language can be technical. However, some people find that they add depth to the articles found in the other sections of this website which are written for non-medical people.

Churg-Strauss Syndrome

Synonyms: allergic granulomatosis angiitis, granulomatous small-vessel vasculitis

A rare diffuse vasculitic disease affecting coronary, pulmonary, cerebral, abdominal visceral and skin circulations. The vasculitis affects small and medium sized arteries and veins and is associated with asthma. The aetiology is unknown.1

Epidemiology
  • Incidence is approximately 2.5 cases per 100,000 adults per year
  • Males are affected slightly more frequently than females
  • The age at onset varies from 15-70 years
Presentation

The physical findings are specific to organ system involvement. There are three phases:

The vasculitic phase usually develops within 3 years of the onset of asthma, although it may be delayed for several decades. The most prominent symptoms and signs are:

Differential Diagnosis
  • Other causes of systemic vasculitis:2
    • Primary vasculitis, e.g. polyarteritis nodosa, Wegener granulomatous
    • Secondary vasculitis: a complication of a connective tissue disorder (e.g. rheumatoid arthritis), infection, medication, or malignancy.
  • Wegener granulomatosis and microscopic polyangiitis are also vasculitic syndromes involving small and medium-sized vessels that are associated with antibodies to neutrophil cytoplasmic antigens.
Investigations
  • Antineutrophil cytoplasmic antibodies: 70% of patients are perinuclear-ANCA (p-ANCA) positive (antimyeloperoxidase antibodies).
  • Other likely finding include full blood count shows eosinophilia and anaemia; elevated ESR and CRP; elevated serum creatinine; increased serum IgE levels, hypergammaglobulinaemia; proteinuria, microscopic haematuria, and red blood cell casts in the urine.
  • Chest x-ray: pulmonary opacities, transient pulmonary infiltrates, pleural effusions.
  • Pulmonary CT scan: peripheral areas of parenchymal consolidation with ground-glass attenuation similar to chronic eosinophilic pneumonia.
  • Biopsy: The characteristic changes, found especially in the lung, include small necrotizing granulomas, as well as necrotizing vasculitis involving small arteries and venules.
  • Other investigations are indicated for the complications of the disease and specific organ system involvement.
Management
  • High dose steroids are usually adequate for treatment.
  • Cyclophosphamide is administered in patients with severe or life-threatening complications. Azathioprine is also used.
  • Other treatments include intravenous immune globulin, interferon-alpha, and plasma exchange.
Complications
  • Complications of vasculitis depend on the specific organ system involvement.
  • The principal causes of morbidity and mortality are myocarditis and myocardial infarction secondary to coronary arteritis.
Prognosis
  • Although the overall prognosis is good, and treatment with prednisone alone or in combination with immunosuppressive drugs is usually successful, severe asthma typically persists.3
  • Diffuse organ involvement of Churg-Strauss syndrome, especially cardiovascular and rare involvement of the CNS and renal system, suggests a poorer prognosis and can be fatal.3
  • Without treatment, the 5-year survival rate is about 25%.
  • With treatment, the 1-year survival rate is 90% and the 5-year survival rate is 62%.
  • Although treatment regimes are often successful, severe asthma usually persists.


Document References
  1. Farid-Moayer M; Churg-Strauss Syndrome. eMedicine, January 2007.
  2. Gorson KC; Vasculitic neuropathies: an update. Neurologist. 2007 Jan;13(1):12-9. [abstract]
  3. Noth I, Strek ME, Leff AR; Churg-Strauss syndrome. Lancet. 2003 Feb 15;361(9357):587-94. [abstract]

Internet and Further Reading Acknowledgements EMIS is grateful to Dr Colin Tidy for writing this article. The final copy has passed scrutiny by the independent Mentor GP reviewing team. ©EMIS 2007.
DocID: 1962
Document Version: 20
DocRef: bgp24815
Last Updated: 30 Apr 2007
Review Date: 29 Apr 2009


















Disclaimer: Patient UK has no control of the content of the above links. Inclusion does not imply endorsement by Patient UK.

Advertise on this site




Patient Pharmacy





Disclaimer: Patient UK has no control of the content of the above links. Inclusion does not imply endorsement by Patient UK.

Advertise on this site


PS - Health and Poverty

Perhaps the biggest cause of ill health in the world is poverty. Help to Make Poverty History. For example, why not lend some of your money to disadvantaged communities to enable them to trade their way out of poverty through schemes such as Shared Interest.

See also MAKEPOVERTYHISTORY North East for details and links to campaigns against poverty.

^ Top of Page