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 Table of Contents  
CASE REPORT
Year : 2012  |  Volume : 26  |  Issue : 1  |  Page : 11-13

Churg Strauss syndrome


Department of Pulmonary Medicine, R. D. Gardi Medical College, Ujjain (MP), India

Date of Web Publication10-Dec-2012

Correspondence Address:
Hanmant G Varudkar
B5/12 Staff Quarters, R D Gardi Medical College, Ujjain (MP)
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-6691.104439

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  Abstract 

The patient is a known case of Asthma with marked peripheral eosinophilia developed recurrent haemoptysis and lung infiltrates. Bronchoscopic lavage and FNAC of lung showed pulmonary eosinophilia. Due to raised IgE levels and strongly positive pANCA, the case was diagnosed as Churg Strauss syndrome. He responded well to corticosteroids with complete resolution and is presently on the tapering doses.

Keywords: Bronchial asthma, haemoptysis, infiltrates, IgE, pANCA


How to cite this article:
Sharma A, Agrawat J, Julka A, Varudkar HG, Sharma P. Churg Strauss syndrome. Indian J Allergy Asthma Immunol 2012;26:11-3

How to cite this URL:
Sharma A, Agrawat J, Julka A, Varudkar HG, Sharma P. Churg Strauss syndrome. Indian J Allergy Asthma Immunol [serial online] 2012 [cited 2017 Mar 22];26:11-3. Available from: http://www.ijaai.in/text.asp?2012/26/1/11/104439


  Introduction Top


Pulmonary eosinophilia can be due to large number of diseases as varied as allergic disorders, parasitic, fungal infections, vasculitis, drugs, lymphoma, idiopathic etc. [1] As the course is mild in some and life threatening in other conditions a detailed history and relevant investigations are required for correct diagnosis. This is not only important for the correct management but also for prognostic forecast. Discussed below is a rare case of pulmonary eosinophilia that is Churg Strauss Syndrome. [2]


  Case Report Top


A 27 year, smoker, old male was referred to our department for evaluation of minimal recurrent haemoptysis, cough and cold with breathlessness; provisionally diagnosed bronchial asthma responding well bronchodilators. He had been fully treated with anti-Tuberculosis treatment for lung consolidation empirically as his sputum was negative for AFB. He had also developed a right sided pneumothorax for which an intercostal drain had been inserted.

This well built, febrile patient had BP was 120/70 mm of Hg, pallor, facial puffiness and pedal edema. Nasal mucosa was edematous and inflamed with post nasal drip but no crusts or bleeding points. He also had urticaria.

Clinical examination revealed crepitations in left axillary and infra mammary area, bilateral wheezes and hepatomegaly. Investigations revealed-Hb was 10.7gm%, TLC-8800, DLC N-40%, L-28%, E-30%, M-2% ESR-92 mm in one hour, absolute eosinophil count −2640/mm. [3] Rest of blood investigations were unremarkable. X-ray Chest showed opacity in the left lower zone [Figure 1]. Sputum for AFB was negative. Stool examination did not reveal any ova or cyst. USG abdomen showed hepato-spleenomagaly but no ascites or lymphadenopathy. Echocardiography revealed mild Tricuspid regurgitation.
Figure 1: Radiograph showing pleural thickening on right side with consolidation left lower lobe of lung

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Contrast enhanced CT Scan of chest revealed a peripheral consolidation with ground glass opacities and fibrosis in left upper lobe. There was pleural thickening on the right. Fiber optic bronchoscopy revealed an inflamed mucosa. Bronchoalveolar lavage showed eosinophillic inflammation. Fine needle aspiration cytology of affected lung too showed inflammation with dense eosinophils.

Anti-nuclear antibody was found to be positive. Total IgE levels were markedly raised-517 IU/ml. The pANCA (perinuclear anti neutrophilic cytoplasmic antibodies) were found to be strongly positive and cANCA was negative. These investigations supported diagnosis of Churg Strauss. Predisolone 40mg once a day along with supportive therapy was started.

There was marked improvement in symptoms and complete radiological clearance within fortnight [Figure 2]. The steroids were tapered after 8 weeks. However because of recurrence of symptoms the patient is on low dose prednisolone for 8 months.
Figure 2: Marked regression of lesions

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  Discussion Top


Churg Strauss Syndrome (CSS) is pulmonary eosinophilia characterised by vasculitis, an inflammation of the blood vessels, first by American physicians Churg and Strauss in 1951. [3] The incidence CSS of is about 2.4 per million population in England. [4] Allergy, atopy, and elevated levels of IgE point towards a hypersensitivity vasculitic response to external and internal antigen. A strong association has been noted between the use of leukotriene receptor antagonists (LTRA) and CSS. LTRAs reduce the requirement of corticosteroids thereby the pre-existing CSS might get unmasked. [5],[6] Hepatitis B positivity, conceived as initiating etiology, is demonstrated in some studies. [7]

The natural history of the disease can be divided in three main, usually successive, phases: 1) prodromic phase, consisting of asthma associated with rhinosinusitis; 2) the eosinophilic phase, with peripheral and eosinophilic infiltration into tissues, particularly the lung, myocardium and gastrointestinal tract etc; and 3) systemic phase, with the development of necrotizing vasculitis affecting the skin, peripheral nerves and kidneys. [8]

There is eosinophillic infiltration of the tissues and extra vascular granulomas as well as peripheral eosinophilia. The small blood vessels, particularly in lungs, upper respiratory tract, skin as well as peripheral nerves, heart become inflamed. Blood circulation deteriorates and organs may be damaged.

Cardiac manifestations generally are not present on initial presentation of CSS but are a major source of morbidity and the principal cause of death. Progressive congestive heart failure (CHF), or ischemic cardiomyopathy are the results of necrotizing vasculitis of the coronary arteries, fatal up to 60 % of times. [9]

A wide array of neurological manifestations may develop in CSS. Mono- or polyneuropathy is present in 69 to 75 % of cases. Skin findings are present in approximately two-thirds of cases developing in localized crops. Non-thrombocytopenic purpura, tender cutaneous or subcutaneous nodules (which may ulcerate), urticaria, a maculopapular rash, petechiae, ecchymoses, or live do reticularis are different presentations.

In 1990, the American College of Rheumatology published diagnostic criteria for CSS. Presence of at least four out of six of criteria yielded 85% sensitivity and 99.7% specificity in establishing diagnosis. The Criteria are:- (1) asthma, (2) peripheral eosinophilia greater than 10%, (3) mono- or polyneuropathy, (4) migratory or transient pulmonary infiltrates,(5) paranasal sinus abnormality, and (6) extravascular eosinophils in a blood vessel on a biopsy specimen. [10] The presence of asthma or allergy as well as more than 10% eosinophilia was 95% sensitive and 99% specific in distinguishing CSS among the subgroup of patients with well-documented systemic vasculitis. Subsequently, the Chapel Hill consensus conference recommended that diagnostic criteria for CSS include (1) appropriate clinical setting and histopathology and (2) eosinophil-rich and granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small and medium vessels with associated asthma and eosinophilia. However, these criteria require tissue biopsy and are less sensitive for CSS than other. [11]

The differential diagnosis of CSS includes polyarteritis nodosa, microscopic angiitis, WG (Wegeners Granulomatosis), CEP, ABPA, idiopathic hypereosinophilic syndrome, Loeffler's syndrome, asthma, fungal or parasitic infection, drug-induced vasculitis, sarcoidosis, and Hodgkin's lymphoma. CSS can be distinguished from WG since compared with WG, patients with CSS have nasal polyps and allergic rhinitis but lack significant necrotizing upper-airway lesions and cavitation of lung nodules, and are more likely to have pANCA (in contrast to the cANCA seen in WG). Also, patients with CSS are less likely to develop renal failure, and vasculitic neuropathy and asthma/eosinophilia are not typical features of WG.

Management

Patients in whom CSS goes untreated have a poor prognosis; up to 50% die within 3 months after the onset of vasculitis. Corticosteroid treatment generally leads to dramatic clinical improvement, with disease stabilization or cure. Prednisone, 0.5 to 1.5 mg/kg/day (or 60 mg/day in adults) is given for 6 to 12 weeks or longer, aiming to eliminate constitutional symptoms and cardiac, renal, neurological, or other vasculitic manifestations. Higher doses are occasionally required. Severe hypertension and mononeuritis multiplex often require prolonged steroid treatment, and may be difficult to eliminate. Once the vasculitic phase is controlled, steroids may be tapered, with doses titrated to maintain disease control. Low-dose prednisone is often given every day or every other day. Treatment with immunosuppressive agents such as azathioprine, cyclophosphamide, high-dose methylprednisolone, or chlorambucil may prove effective and should be considered in patients whose condition fails to improve with steroid treatment or who have severe systemic involvement. Poor prognostic features, including cardiac or GI involvement, renal insufficiency. Other drugs used are Intravenous immunoglobulin which may be beneficial for reducing symptoms and organ involvement. [12] The immunoregulatory cytokine interferon-α (IFN-α) has led to improved pulmonary function tests, reduction in corticosteroid dose, and decreased WBC count and may be considered as another alternative treatment in persons with refractory disease. [13] Plasma exchange may also be a successful adjunct treatment in some patient.

Long-term overall remission can be achieved in approximately 81 to 92% of patients; relapses, if they occur, are maximum in the first year.


  Conclusion Top


Our patient fulfilled the criteria of the Churg Strauss Syndrome and presently is responding well to treatment with corticosteroids with regular follow up.

 
  References Top

1.Bhupinder Mann. Eosinophilic lung disease. Clin Med Circ Respir Pulm Med 2008;2:99-108.  Back to cited text no. 1
    
2.Jeong YJ, Kim KI, Seo IJ, Lee CH, Lee KN, Kim KN, et al. Eosinophilic lung diseases: A clinical radiologic, and pathologic overview. Radiographics 2007;27:617-37.  Back to cited text no. 2
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3.Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27:277-301.  Back to cited text no. 3
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4.Gross WL, Reinhold -Keller E; Churg Stauss Syndrome. Orphanet encyclopedia. 2002.http://www.orpha.net/data/patho/GB/uk-CSS.pdf.  Back to cited text no. 4
    
5.Matsui K, Nishijima K. A case of montelukast -Induced Churg -Strauss syndrome associated with liver dysfunction. Case Report Hepatol 2011;2011:412524.  Back to cited text no. 5
    
6.Michael AB, Murphy D. Montelukast- associated Churg Straus Syndrome. Age Aging 2003;32:551-2.  Back to cited text no. 6
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7.Crofton and Douglas's respiratory Diseases. 2. Anthony Seaton, Douglas Seaton, A. Gordon Leitch. 5 th ed. Pulmonary Eosinophilias. ???;. p. 1028-9.  Back to cited text no. 7
    
8.Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: A clinical approach to the Churg-Strauss syndrome. Medicine 1984;63:65-81.  Back to cited text no. 8
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9.Goeken JA. Antineutrophil cytoplasmic antibody- A useful serological marker for vasculitis. J Clin Immunol 1991;11:161-73.  Back to cited text no. 9
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10.Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Churg Struss syndrome. Arthritis Rheum 1990;33:1094-100.  Back to cited text no. 10
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11.Churg A. Recent advances in the diagnosis of Churg-Strauss syndrome. Mod Pathol 2001;14:1284-93.  Back to cited text no. 11
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12.Tatsis E, Schnabel A, Gross WL. Interferon-alpha treatment of four patients with Churg Strauss syndrome. Ann Intern Med 1998;129:370-4.  Back to cited text no. 12
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13.Tsurikisawa N, Taniguchi M, Saito H, Himeno H, Ishibashi A, Suzuki S, et al. Treatment of Churg Strauss syndrome with high dose intravenous immunoglobulin. Ann Allergy Asthma Immunol 2004;92:80-7.  Back to cited text no. 13
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    Figures

  [Figure 1], [Figure 2]



 

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