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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 30  |  Issue : 2  |  Page : 105-108

Churg-Strauss syndrome: A rare cause of pleural effusion


1 Department of Respiratory Medicine, Institute of Respiratory Diseases, SMS Medical College, Jaipur, Rajasthan, India
2 Department of Pulmonary Medicine, Vallabhbhai Patel Chest Institute, University of Delhi, New Delhi, India
3 Department of Pulmonary Medicine, SDMH, Jaipur, Rajasthan, India

Date of Web Publication5-Dec-2016

Correspondence Address:
Aashish Kumar Singh
A-13B, Ram Marg, Vijay Path, Tilak Nagar, Jaipur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-6691.195270

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  Abstract 

Churg-Strauss syndrome (CSS) is a rare, small-vessel vasculitis associated with a prominent allergic component, asthma, and blood or tissue eosinophilia. Granulomas, eosinophils, and palisading histiocytes in extravascular tissues are hallmarks of this disorder. The presence of asthma or allergy as well as more than 10% of eosinophils in blood is 95% sensitive and 99% specific, respectively, in distinguishing CSS among a subgroup of patients with well-documented systemic vasculitis. We present a case of pleural effusion which was finally diagnosed as CSS. Considering its rarity, this case is reported.

Keywords: Asthma, Churg-Strauss syndrome, eosinophilia, pleural effusion, small-vessel vasculitis


How to cite this article:
Rajawat GS, Singh AK, Batra S, Gupta M L, Yadav G S, Koolwal S. Churg-Strauss syndrome: A rare cause of pleural effusion. Indian J Allergy Asthma Immunol 2016;30:105-8

How to cite this URL:
Rajawat GS, Singh AK, Batra S, Gupta M L, Yadav G S, Koolwal S. Churg-Strauss syndrome: A rare cause of pleural effusion. Indian J Allergy Asthma Immunol [serial online] 2016 [cited 2017 Apr 30];30:105-8. Available from: http://www.ijaai.in/text.asp?2016/30/2/105/195270


  Introduction Top


Churg-Strauss syndrome (CSS) is a type of vasculitis that commonly affects the lung. The Chapel Hill Consensus defines the disease as "eosinophil-rich and granulomatous inflammation involving the respiratory and necrotizing vasculitis affecting small to medium-sized vessels and associated with asthma and eosinophilia." [1] Three distinct phases of the disease have been described. The first is a prodromal allergic phase with asthma and may last for years. The second is an eosinophilic phase with prominent peripheral and tissue eosinophilia. This phase may also last a number of years and the manifestations may remit and recur over this time period. The third is vasculitic phase which consists of systemic vasculitis and may be life-threatening. These three phases are not seen in all patients and do not necessarily occur in this order; they may even concur. However, asthma usually predates vasculitic symptoms by a mean of 7 years. [2],[3],[4],[5]

Pleural effusion has been described as a manifestation of CSS with a frequency of 29%. [6]


  Case Report Top


A 30-year-old male presented to us with the chief complaints of dry cough since 3 months, chest pain, shortness of breath on exertion, and decreased appetite for the past 2 months. His general physical examination was within normal limits, except tachycardia and tachypnea. On auscultation, bilateral generalized wheeze was present.

His past history revealed that he was a known case of bronchial asthma for the past 10 years, for which he was taking inhaled bronchodilators. Two months back, he was admitted in another hospital with the chief complaints of fever, dry cough, chest pain, and shortness of breath on exertion, where he was treated for pneumonia. He showed symptomatic improvement, but had an attack of stroke in the hospital. After this episode, he was unable to communicate or write. Then, he was referred to the neurology department of our hospital. Magnetic resonance imaging of the brain was done and it showed early subacute infract in the left temporo-parietal and frontal lobe. Magnetic resonance angiogram of the brain and neck vessels was normal. He was diagnosed as a case of the left middle cerebral artery ischemia, stroke in young (recovering global aphasia) with atypical pneumonia. He was prescribed cerebroprotective and antiplatelets, and he showed improvement.

However, at the same time, the patient complained of the right-sided chest pain. Chest radiograph revealed right-sided pleural effusion with cardiomegaly [Figure 1]. Hence, contrast-enhanced computed tomography chest was advised, which showed right-sided pleural effusion with pericardial effusion [Figure 2]. After this, the patient was referred to our department for further management.
Figure 1: Chest X-ray posteroanterior view showing right-sided pleural effusion and cardiomegaly

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Figure 2: Computerized tomography scan of the chest showing right-sided pleural effusion and pericardial effusion

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After admission, the patient was thoroughly reviewed. His previous chest radiographs showed migrating infiltrates in bilateral lung parenchyma [Figure 3] and [Figure 4]. His previous blood report showed eosinophilia (eosinophils - 23%). At our hospital, repeat blood investigations were within normal limits except raised eosinophils (32%). Spirometry showed obstructive pattern with postbronchodilator reversibility. The patient was put on bronchodilators, antibiotics, and symptomatic treatment. Then, pleural biopsy was performed (after stopping anti-platelets for 4 days). Pleural biopsy report showed fibro-collagenous tissue, neutrophils, lymphocytes, and eosinophils [Figure 5]. The report was negative for malignancy, tuberculosis, and fungal infections. High blood eosinophil count and pleural tissue invasion of eosinophils pointed toward a case of eosinophilic disorder. Literature reveals that eosinophilic disorder associated with asthma is allergic bronchopulmonary aspergillosis (ABPA) and CSS. However, extrapulmonary involvement is more common in CSS as compared to ABPA. [7] Among all eosinophilic disorders, tissue invasion of eosinophils is seen only in CSS. Hence, the patient was evaluated as a case of CSS. His P-ANCA was negative, and computed tomography of paranasal sinuses showed sinonasal polyposis. Nerve conduction study showed bilateral peroneal motor affection. Two-dimensional echocardiography showed pericardial effusion with the right ventricular mass. Hence, the patient was diagnosed as a case of CSS as five out of six major criteria were fulfilled. [8] The patient was put on steroids and he improved [Figure 6].
Figure 3: Chest X-ray posteroanterior view showing the left lower zone consolidation

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Figure 4: Chest X-ray posteroanterior view showing consolidation in the right lung upper, mid, and lower zone with the left upper zone involvement

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Figure 5: Eosinophilic infiltration of pleural tissue

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Figure 6: Chest X-ray posteroanterior view (after treatment) showing near-normal except blunting of the right costophrenic angle

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  Discussion Top


CSS also referred as eosinophilic granulomatosis with polyangiitis is a multisystem disorder characterized by chronic rhinosinusitis, asthma, and prominent peripheral blood eosinophilia. The disease bears the name after pathologists Churg and Strauss, in 1951, they identified 13 patients with disseminated necrotizing vasculitis occurring in patients with severe asthma and hypereosinophilia. [9] The annual incidence has been estimated at two to three cases per million. The erythrocyte sedimentation rate, C-reactive protein, and blood eosinophil count are elevated in more than 80% of the patients during the acute phase of vasculitis or exacerbations. The diagnosis of CSS can be made, even when histological features are less than definitive, provided the clinical and laboratory features are characteristic.

In 1990, the American College of Rheumatology [8] published diagnostic criteria for CSS, based on the assessments of the sensitivity and specificity of the diagnostic criteria used previously. The presence of at least four out of six of the following criteria yielded 85% sensitivity and 99.7% specificity in establishing the diagnosis:

  1. Asthma
  2. Peripheral eosinophilia >10%
  3. Mono or polyarthropathy
  4. Migratory or transient pulmonary infiltrates
  5. Paranasal sinus abnormality
  6. Extravascular eosinophils in a blood vessel on a biopsy specimen.


In our case, five out of six criteria were fulfilled, and the patient was in the second phase of disease since a tissue eosinophilia was demonstrable (pleural biopsy). Pleural effusion has been described as a manifestation of CSS, but an eosinophilic effusion has not been scrutinized as an important feature in the clinical progression of the disease. [10]

Patients in whom CSS goes untreated have a poor prognosis; up to 50% die within 3 months after the onset of vasculitis. As such, efforts at early recognition and treatment are important. Corticosteroid generally leads to dramatic clinical improvement, with disease stabilization or cure. [3],[11],[12] Prednisone 0.5-1.5 mg/kg/day is given for 6-12 weeks, aiming to eliminate constitutional symptoms and cardiac, renal, neurological, or other vasculitic manifestations. Treatment with cytotoxic immunosuppressive agents [13],[14] such as azathioprine, cyclophosphamide, high-dose methylprednisolone, or chlorambucil should be considered in patients whose condition fails to improve with steroids or who have severe systemic involvement or poor prognostic features, including cardiac or gastrointestinal involvement, renal insufficiency, or proteinuria >1 g/day.


  Conclusion Top


Although common differential diagnosis for pleural effusion is considered, background of bronchial asthma must alert both the clinicians and pathologists about the possibility of a CSS.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994;37:187-92.  Back to cited text no. 1
    
2.
Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore) 1999;78:26-37.  Back to cited text no. 2
    
3.
Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: A clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore) 1984;63:65-81.  Back to cited text no. 3
    
4.
Keogh KA, Specks U. Churg-Strauss syndrome: Clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med 2003;115:284-90.  Back to cited text no. 4
    
5.
Chumbley LC, Harrison EG Jr., DeRemee RA. Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases. Mayo Clin Proc 1977;52:477-84.  Back to cited text no. 5
    
6.
Loughlin JE, Cole JA, Rothman KJ, Johnson ES. Prevalence of serious eosinophilia and incidence of Churg-Strauss syndrome in a cohort of asthma patients. Ann Allergy Asthma Immunol 2002;88:319-25.  Back to cited text no. 6
    
7.
Allen JN, Davis WB. Eosinophilic lung diseases. Am J Respir Crit Care Med 1994;150(5 Pt 1):1423-38.  Back to cited text no. 7
    
8.
Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990;33:1094-100.  Back to cited text no. 8
    
9.
Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27:277-301.  Back to cited text no. 9
    
10.
Deepak NM, Sushma VB, Srilatha PS, Indira S, Girish S. Churg-Strauss syndrome presenting as an eosinophilic pleural effusion. Int J Sci Res 2013;2:510-2.  Back to cited text no. 10
    
11.
Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore) 1996;75:17-28.  Back to cited text no. 11
    
12.
Abu-Shakra M, Smythe H, Lewtas J, Badley E, Weber D, Keystone E. Outcome of polyarteritis nodosa and Churg-Strauss syndrome. An analysis of twenty-five patients. Arthritis Rheum 1994;37:1798-803.  Back to cited text no. 12
    
13.
Chow CC, Li EK, Lai FM. Allergic granulomatosis and angiitis (Churg-Strauss syndrome): Response to 'pulse' intravenous cyclophosphamide. Ann Rheum Dis 1989;48:605-8.  Back to cited text no. 13
    
14.
Guillevin L, Lhote F, Jarrousse B, Fain O. Treatment of polyarteritis nodosa and Churg-Strauss syndrome. A meta-analysis of 3 prospective controlled trials including 182 patients over 12 years. Ann Med Interne (Paris) 1992;143:405-16.  Back to cited text no. 14
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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