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CASE REPORT
Year : 2014  |  Volume : 28  |  Issue : 2  |  Page : 131-134

Churg-Strauss syndrome: Report of 3 cases


1 Department of Internal Medicine, Imam Khomeini Hospital, School of Medicine, Ardabil University of Medical Sciences, Ardabil, Iran
2 Department of Internal Medicine, Shohadaye Khalige Fars Hospital, Bushehr University of Medical Sciences, Bushehr, Iran

Date of Web Publication18-Sep-2014

Correspondence Address:
Dr. Nasrollah Maleki
Resident of Internal Medicine, Department of Internal Medicine, Imam Khomeini Hospital, School of Medicine, Ardabil University of Medical Sciences, Ardabil
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-4958.141114

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  Abstract 

Churg-Strauss syndrome (CSS) is a vasculitis of medium to small sized vessels. Diagnosis is mainly clinical with findings of asthma, eosinophilia, rhinosinusitis and signs of vasculitis in major organs. This is a rare syndrome of unknown etiology, affecting both genders and all age groups. Outcome and long-term survival are usually good with steroids alone or in combination with immunosuppressive agents. The syndrome has a low mortality rate compared with other systemic vasculitides. We report three patients with CSS from Ardabil, who presented with asthma, pansinusitis, peripheral eosinophilia, peripheral neuropathy, cutaneous eosinophilic vasculitis. The diagnosis of CSS was made by the diagnostic criteria of American College of Rheumatology (ACR). CSS is typically suspected in patients whose asthma is poorly controlled on moderate doses of inhaled glucocorticoids. Prolonged treatment of asthma with glucocorticoid therapy may partially or totally suppress the usual clinical signs of untreated CSS. The disease may therefore not become evident until glucocorticoids are reduced or stopped.

Keywords: Asthma, Churg-Strauss syndrome, eosinophilia, vasculitis


How to cite this article:
Azami A, Mirzaaghazadeh M, Maleki N, Tavosi Z. Churg-Strauss syndrome: Report of 3 cases. J Med Soc 2014;28:131-4

How to cite this URL:
Azami A, Mirzaaghazadeh M, Maleki N, Tavosi Z. Churg-Strauss syndrome: Report of 3 cases. J Med Soc [serial online] 2014 [cited 2020 May 29];28:131-4. Available from: http://www.jmedsoc.org/text.asp?2014/28/2/131/141114


  Introduction Top


The Churg-Strauss syndrome (CSS), also called allergic granulomatosis and angiitis, is a multisystem disorder characterized by allergic rhinitis, asthma, and prominent peripheral blood eosinophilia. [1],[2],[3],[4],[5],[6],[7],[8] CSS is classified as a vasculitis of the small and medium sized arteries, although the vasculitis is often not apparent in the initial phases of the disease. The most commonly involved organ is the lung, followed by the skin. CSS, however, can affect any organ system. The epidemiology of CSS remains unclear because of the uncertainties related to diagnosis. [9] As these often do not coexist temporally or spatially, in 1990 the American College of Rheumatology (ACR) proposed six criteria for the definition of CSS, at least four of them being needed for diagnosis:

  1. Asthma;
  2. Peripheral eosinophilia (>10% of total leukocyte count);
  3. Mononeuropathy or polyneuropathy;
  4. Paranasal sinus abnormality;
  5. Pulmonary infiltrates;
  6. Histological proof of vasculitis and extravascular eosinophils. [8],[10]


Sensitivity of these criteria for diagnosis was 85% and specificity was 99.7%. [10],[11]


  Case Reports Top


Here we report three patients with CSS from Ardabil, Iran.

Case 1

A 35-year-old woman suffered from bronchial asthma for five years and was followed up by a pulmonologist. She had been prescribed fluticasone/salmeterol Inhaler six months before. Dyspnea, fever, night sweating, malaise, headache, general weakness, cough, diarrhea, and purpura on the lower extremities occurred in the patient 10 days earlier. The patient was referred to our hospital because of the exacerbation of asthma. On physical examination, she was in respiratory distress, and had coarse crackles and generalized wheezing in the lung bases. The level of O 2 saturation upon admission was 82% and increased to 94% with administration of 8 liters of 40% oxygen via venturi mask. Heart sounds were normal, and no organomegaly was detected. Palpable, purpuric rashes were noted on both legs [Figure 1]. Her white blood cell count was 11,500 cell/mm 3 , with 50% neutrophil, 18% lymphocytes, 27% eosinophils and her hemoglobin was 11.2 g/dl. Laboratory findings also showed an elevation of erythrocyte sedimentation rate (45 mm/1h), and C- reactive protein (3+). Rheumatoid factor (RF), antinuclear antibody (ANA), and antineutrophil cytoplasmic antibodies (ANCA) were negative. Chest X-ray identified only few discrete pulmonary abnormalities, but thoracic computed tomography (CT) evidenced diffuse ground glass opacities associated with diffuse bronchial wall thickening [Figure 1]. Coronal CT scan of the paranasal sinuses showed mucosal changes consistent with sinusitis in both maxillary and ethmoid sinuses and bilateral soft tissue infiltration [Figure 2]. Cardiac echography was normal. He underwent electromyography (EMG) and nerve conduction velocity (NCV) studies that were normal. She could not tolerate flexible fiberoptic bronchoscopic examination. The skin biopsy specimen showed a leukocytoclastic vasculitis [Figure 2]. The diagnosis of CSS was made by the four diagnostic criteria of ACR (asthma, peripheral eosinophilia, paranasal sinusitis, histological proof of vasculitis and extravascular eosinophils). We prescribed methylprednisolone 500 mg/day for 3 days. Subsequently, oral prednisolone 50 mg/day was administered. All clinical symptoms and abnormalities in the laboratory tests resolved after these treatments. Glucocorticoid was thereafter tapered gradually without recurrence of CSS syndrome.
Figure 1: Palpable, purpuric rashes on both legs (a), Chest CT scan showing ground glass opacities associated with diffuse bronchial wall thickening (b)

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Figure 2: CT scan of the paranasal sinuses showed mucosal changes consistent with sinusitis (a), The skin biopsy specimen showing a leukocytoclastic vasculitis (H and E×400)

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Case 2

The patient was a 46-year-old woman with a medical history of bronchial asthma for eight years, who was admitted for fever, myalgia, sensory deficits in the lower extremities, diffuse musculoskeletal, productive cough, worsening of dyspnea in the past five months. Also, generalized skin rash without induration was described by patient few days before the hospital admission. Physical examination revealed inspiratory crackles and wheezing over both lungs. Standard laboratory tests revealed a leukocyte count 18,800 cell/mm 3 with 15.7% eosinophils, erythrocyte sedimentation rate was 87 mm/1h. Tests for RF, ANA, and ANCA were negative. Laboratory findings also showed an elevation of IgE. Chest CT scan showed multiple pulmonary nodule. CT scan of the paranasal sinuses revealed pansinusitis [Figure 3]. Cardiac echography was normal. The results of NCV studies revealed axonal sensorimotor neuropathy of the lower limbs. She eventually underwent bronchoscopy and transbronchial lung biopsy (TBLB). Tissue samples obtained by TBLB revealed extra-vascular granuloma and limited number of multinucleated giant cells [Figure 3]. Bronchoalveolar lavage (BAL) revealed neither mycobacteria nor cancerous cells. Considering the asthma, hyperoesinophilia, paranasal sinusitis, sensorimotor neuropathy and histological proof of vasculitis, diagnosis of CSS was established and the therapy with glucocorticoid and cyclophosphamide was started.
Figure 3: CT scan of the paranasal sinuses showing pansinusitis (a), The lung biopsy showing extra-vascular granuloma and limited number of multinucleated giant cells (b)

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Case 3

A 45-year-old man had a history of bronchial asthma for 10 years. He attended our hospital because of fever, cough, malaise, headache, general weakness and worsening of dyspnea in the past four months. Physical examination revealed wheezing and rhonchi over both lungs. On admission, laboratory analyses revealed increased erythrocyte sedimentation rate (105 mm/1h), leukocyte count 13,500 cell/mm 3 with 38% eosinophils, and positive perinuclear-ANCA (p-ANCA). IgE increased to 1960 IU/ml (normal <173). Chest CT scan showed bilateral patchy ground glass opacities, predominantly in the basal zones. He underwent bronchoscopy and TBLB. Histopathological examination showed leukocytoclastic vasculitis, granuloma formation, and extravascular eosinophilic infiltration. Considering the asthma, hyperoesinophilia, pulmonary infiltrations and histological proof of vasculitis, diagnosis of CSS was established and the patient was treated with glucocorticoid.


  Discussion Top


Churg-Strauss Syndrome (CSS) is a small vessel vasculitis, which was first described in 1951. [1] CSS is a rare disease with an annual incidence ranging between 0.5 and 6.8 per million inhabitants, with mean age at onset around 50 years. [4] A higher number of female than of male patients has been reported in some studies, in contrast to the predominance of male patients reported by other authors. [1],[5] HLA-DRB4 is known to be a genetic risk factor for the development of CSS and increases the likelihood of development of vasculitic manifestations of the disease. [12]

The clinical features of the CSS typically develop in several sequential phases, although these phases are not always clearly distinguishable [4],[7]:

  1. Prodromal phase - The prodromal phase occurs among individuals in the second and third decades of life and is characterized by atopic disease, allergic rhinitis, and asthma.
  2. Eosinophilic phase - Features of the eosinophilic phase include peripheral blood eosinophilia and eosinophilic infiltration of multiple organs, especially the lung and gastrointestinal tract. Almost 40 percent of patients with CSS present with pulmonary opacities, asthma, and peripheral eosinophilia prior to the development of a systemic vasculitis (polyangiitis). [7]
  3. Vasculitic phase - In the third and fourth decades of life, a life-threatening systemic vasculitis of the medium and small vessels frequently occurs, and is often associated with vascular and extravascular granulomatosis. [6] The vasculitic phase may be heralded by nonspecific constitutional symptoms and signs, especially fever, weight loss, malaise, and lassitude.


Asthma is the cardinal clinical feature of CSS and is present in more than 95 percent of patients. Asthma usually precedes the vasculitic phase by approximately 8 to 10 years. [6],[13] CSS is typically suspected in patients whose asthma is poorly controlled on moderate doses of inhaled glucocorticoids. Prolonged treatment of asthma with glucocorticoid therapy may partially or totally suppress the usual clinical signs of untreated CSS. The disease may therefore not become evident until glucocorticoids are reduced or stopped. [14],[15] Other pulmonary findings are reported in 50 to 70 percent and include pulmonary opacities with eosinophilia, pleural effusion (often eosinophilic), nodules that are rarely cavitary, and alveolar hemorrhage. In one series, venous thromboembolic disease was noted in 8 percent. [3] Allergic rhinitis is a common finding in CSS. [16] In a series of 29 patients with CSS, nasal polyposis was detected in 60 percent. [17]

Skin involvement is one of the most common features of the vasculitic phase of CSS. Half to two-thirds of patients with CSS have skin lesions, which usually appear as tender subcutaneous nodules on the extensor surfaces of the arm, particularly the elbows, hands, and legs. [3],[9]

Cardiac involvement is one of the more serious manifestations of CSS, accounting for approximately one-half of deaths attributable to CSS. [18],[19] Clinical manifestations include clinical signs of heart failure or pericarditis and cardiac rhythm abnormalities. [18] Patients with cardiac involvement typically have a shorter duration of CSS related symptoms than those without. Cardiac involvement is more frequent in patients with higher eosinophil counts at the time of diagnosis. [18] In a series of 383 patients with CSS, 16 percent had a cardiomyopathy and 15 percent had pericarditis. [20] In a separate series of 22 patients with evidence of cardiac involvement, cardiac abnormalities included an abnormal electrocardiogram (ECG) in all patients; valvular insufficiency, peri-cardial effusion, and heart failure were noted in 73.50, and 41 percent, respectively. [18] Endomyocardial involvement was found in 12 patients based on cardiac magnetic resonance imaging (MRI) findings of mural thrombus and a positive endomyocardial biopsy. [18] The majority of patients improved with treatment, although 2 of the patients with endomyocardial disease died of heart failure. Patients with cardiac involvement were less likely to have a positive ANCA and more likely to have higher peripheral blood eosinophil counts than other CSS patients. [20]

A peripheral neuropathy, usually mononeuritis multiplex, is seen in up to 75 percent of patients with CSS. [3],[6],[21],[22],[23] The frequency of renal involvement varies among studies. [24],[25] In the largest series of 116 patients with CSS, renal involvement was found in 31 patients (27 percent). [25] One-half had rapidly progressive or acute renal insufficiency (plasma creatinine concentration >1.4 mg/dL, while the others had isolated proteinuria or microscopic hematuria. Sixteen patients underwent renal biopsy, which demonstrated necrotizing glomerulonephritis in 11 patients. A positive test for ANCA was found in all patients with glomerulonephritis, compared with 26 percent of patients without renal involvement. Myalgias, migratory polyarthralgias, and frank arthritis are less common, but may affect 40 to 50 percent of patients in the vasculitic phase of the disorder. [6] Eosinophilic lymphadenopathy has been noted in 30 to 40 percent of patients. [15]

Peripheral blood eosinophilia (usually 5000 to 9000 eosinophils/mm 3 ) is the most characteristic finding, although levels over 1500 cells/mm 3 (or greater than 10 percent of the total leukocyte count) should prompt suspicion for CSS. [7],[8],[25] ANCA are found in 40 to 60 percent of patients with CSS. [13],[21],[26],[27],[28],[29] Surgical lung biopsy, although not always available, is the "gold standard" for the diagnosis of CSS. [30] In contrast, transbronchial lung biopsy is generally not helpful. Diagnostic criteria specified by the ACR are most commonly used for diagnosis. A minimum of four criteria are required for a confident diagnosis of CSS.

The majority of patients with CSS achieve a remission with glucocorticoid therapy alone. [31] In a study of 72 patients without poor-prognosis factors (ie, no cardiac, renal or central nervous system involvement) followed for five years or longer, 93 percent achieved a remission with glucocorticoid therapy alone. [31] Cyclophosphamide is typically used in combination with glucocorticoids for patients with severe, multi-organ disease. [19],[32]


  Conclusions Top


Churg-Strauss Syndrome (CSS) can be difficult to diagnose because of its variable presentation. The clinicians should suspect a CSS in patients with asthma/atopy, who develop significant constitutional symptoms, increasing cough, dyspnea, pulmonary infiltrates in the absence of infection, in patients with significant gastrointestinal disease or cardiac disease, patients with skin abnormalities, peripheral eosinophilia, increased serum IgE and positive ANCA. Despite long-standing pulmonary symptoms and laboratory findings of eosinophilia, the appearance of skin changes raised suspicion of possible CSS.

 
  References Top

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32.Gayraud M, Guillevin L, Cohen P, Lhote F, Cacoub P, Deblois P, et al. Treatment of good-prognosis polyarteritis nodosa and Churg-Strauss syndrome: Comparison of steroids and oral or pulse cyclophosphamide in 25 patients. French Cooperative Study Group for Vasculitides. Br J Rheumatol 1997;36:1290-7.  Back to cited text no. 32
    


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