Synonyms: allergic granulomatosis angiitis, granulomatous small-vessel vasculitis, eosinophillic granulomatosis with polyangiitis (EGPA)
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 although autoimmune and genetic factors have been implicated. Drug-induced Churg-Strauss syndrome (CSS) has been reported. Drugs implicated have included mesalazine, propylthiouracil, methimazole, freebase cocaine and leukotriene receptor antagonists.
The American College of Rheumatology has identified six criteria for the diagnosis of Churg-Strauss syndrome:
- Asthma (wheezing, expiratory rhonchi).
- Eosinophilia of more than 10% in peripheral blood.
- Paranasal sinusitis.
- Pulmonary infiltrates (may be transient).
- Histological confirmation of vasculitis with extravascular eosinophils.
- Mononeuritis multiplex or polyneuropathy.
The presence of four out of six of these features has a high specificity and sensitivity for the diagnosis of CSS.
- Incidence is approximately 2.5 cases per 100,000 adults per year.
- Males are affected slightly more frequently than females.
- The age at onset usually varies from 15-70 years although patients as young as 9 have been reported.
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The physical findings are specific to organ system involvement. There are three phases:
- Allergic rhinitis and asthma.
- Eosinophilic infiltrative disease, such as eosinophilic pneumonia or gastroenteritis.
- Systemic medium and small vessel vasculitis with granulomatous inflammation.
The vasculitic phase usually develops within three years of the onset of asthma, although it may be delayed for several decades. The most prominent symptoms and signs are:
- Pulmonary: asthma, pneumonitis and haemoptysis.
- Upper respiratory tract: allergic rhinitis, paranasal sinusitis, nasal polyposis.
- Cardiac: heart failure, myocarditis and myocardial infarction.
- Skin: purpura, skin nodules, leukocytoclastic angiitis with palpable purpura, livedo reticularis, urticaria, necrotic bullae and digital ischaemia.
- Renal: glomerulonephritis, hypertension and advanced renal failure.
- Peripheral neuropathy: mononeuritis multiplex (the most frequent form, occurring in as many as 77% of patients). Less frequent symptoms include stroke and eye involvement.
- Gastrointestinal: vasculitis and bleeding, bowel ischaemia and perforation, appendicitis and pancreatitis.
- Cholestatic liver dysfunction has been reported.
- Malaise, fatigue, weight loss, fever, myalgia and arthralgia.
- Myositis following unaccustomed exercise has been reported.
- Other causes of systemic vasculitis:
- Wegener's granulomatosis and microscopic polyangiitis are also vasculitic syndromes involving small- and medium-sized vessels that are associated with antibodies to neutrophil cytoplasmic antigens.
- Antineutrophil cytoplasmic antibodies (ANCA): 70% of patients are perinuclear staining (p-ANCA) positive (antimyeloperoxidase antibodies).
- Other likely findings include eosinophilia and anaemia on the FBC; 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.
- Bronchiolar lavage yields eosinophilia in 33% of cases.
- Biopsy: the characteristic changes, found especially in the lung, include small necrotising granulomas, as well as necrotising vasculitis involving small arteries and venules.
- Other investigations are indicated for the complications of the disease and specific organ system involvement.
- 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.
- Successful use of rituximab has been reported.
- Oral tacrolimus in combination with methylprednisolone and cyclophosphamide was used successfully in the treatment of a child severely ill with CSS.
- Gastrointestinal transplantation in a patient with severe gastrointestinal involvement has been reported.
- Complications of vasculitis depend on the specific organ system involvement.
- Cardiac and neurological complications are particularly serious and are more likely in patients with a delayed diagnosis.
- Although the overall prognosis is good, and treatment with prednisone alone or in combination with immunosuppressive drugs is usually successful, severe asthma typically persists.
- Relapses are not uncommon.
- 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.
- Without treatment, the five-year survival rate is about 25%.
- With treatment, the one-year survival rate is 90% and the five-year survival rate is 62%.
Further reading & references
- Vasculitis UK
- Dennert RM, van Paassen P, Schalla S, et al; Cardiac involvement in Churg-Strauss syndrome. Arthritis Rheum. 2010 Feb;62(2):627-34.
- Zwerina J, Eger G, Englbrecht M, et al; Churg-Strauss syndrome in childhood: a systematic literature review and clinical comparison with adult patients. Semin Arthritis Rheum. 2009 Oct;39(2):108-15. Epub 2008 Jul 17.
- Vaglio A, Moosig F, Zwerina J; Churg-Strauss syndrome: update on pathophysiology and treatment. Curr Opin Rheumatol. 2012 Jan;24(1):24-30.
- Farid-Moayer M et al; Churg-Strauss Syndrome, Medscape, Apr 2011
- Man MA, Alexandrescu D, Pop M, et al; Churg Strauss syndrome associated with montelukast--case report. Pneumologia. 2012 Apr-Jun;61(2):113-6.
- Liu J, Xu Y, Chen Z, et al; A possible case of Churg-Strauss syndrome in a 9-year-old child. Clinics (Sao Paulo). 2012 Aug;67(8):977-80.
- Setoguchi M, Okishige K, Sugiyama K, et al; Sudden Cardiac Death Associated With Churg-Strauss Syndrome. Circ J. 2009 Jun 3.
- Harada M, Oe S, Shibata M, et al; Churg-Strauss syndrome manifesting as cholestasis and diagnosed by liver biopsy. Hepatol Res. 2012 Sep;42(9):940-4. doi: 10.1111/j.1872-034X.2012.00993.x.
- Uehara M, Hashimoto T, Sasahara E, et al; Churg-Strauss syndrome presenting as myositis following unaccustomed exercise. J Clin Neurosci. 2009 Jun 2.
- Gorson KC; Vasculitic neuropathies: an update. Neurologist. 2007 Jan;13(1):12-9.
- Dunogue B, Pagnoux C, Guillevin L; Churg-strauss syndrome: clinical symptoms, complementary investigations, prognosis and outcome, and treatment. Semin Respir Crit Care Med. 2011 Jun;32(3):298-309. doi: 10.1055/s-0031-1279826. Epub 2011 Jun 14.
- Donvik KK, Omdal R; Churg-Strauss syndrome successfully treated with rituximab. Rheumatol Int. 2011 Jan;31(1):89-91. Epub 2009 Sep 30.
- Watanabe S, Aizawa-Yashiro T, Tsuruga K, et al; Successful multidrug treatment of a pediatric patient with severe Churg-Strauss syndrome refractory to prednisolone. Tohoku J Exp Med. 2011;225(2):117-21.
- Darius T, Monbaliu D, Aerts R, et al; Living related intestinal transplantation for Churg-Strauss syndrome: a case report. Transplant Proc. 2010 Dec;42(10):4423-4.
- Rana AQ, Adlul A; Delay in diagnosis of Churg-Strauss syndrome: a case report. Scott Med J. 2012 Oct 1.
- Noth I, Strek ME, Leff AR; Churg-Strauss syndrome. Lancet. 2003 Feb 15;361(9357):587-94.
|Original Author: Dr Colin Tidy||Current Version: Dr Laurence Knott||Peer Reviewer: Prof Cathy Jackson|
|Last Checked: 17/11/2012||Document ID: 1962 Version: 22||© EMIS|
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