Churg–Strauss syndrome diagnosed after lobar atelectasis: Case report

1. Introduction 

Churg–Strauss syndrome was first described in 1951 as a pathological syndrome of allergic granulomatosis and angiitis. It was defined during the 1994 International Consensus Conference as an eosinophil-rich and granulomatous inflammation involving the respiratory tract with necrotizing vasculitis affecting small-to-medium-sized vessels and associated with asthma and eosinophilia.1, 2 Here we reported a rare radiological manifestation of Churg–Strauss syndrome.

2. Case report 

A 24-year-old women presented to the emergency department of our hospital with a 2-week history of fever and cough. On chest radiographs, a nonhomogeneous density in the right upper zone of the lung was seen (Fig. 1). The diagnosis was pneumonia, and the patient was prescribed levofloxacin (500mg/day) for 14 days. Follow-up examination at 2 weeks revealed an oxygen saturation level which was 95% while breathing room air. Results of a physical examination were remarkable for bilateral diffuse rhonchi, and diminished breath sounds on the anterior surface of the right chest wall with dullness to percussion were evident. She had a moveable soft mass (9cm) on her left tibia. Findings on chest radiograph showed no improvement. The patient was hospitalized to determine the etiology of the atelectasis, and a differential diagnosis of tuberculosis, unresolved pneumonia, vasculitis, allergic bronchopulmonary aspergillosis, hypersensitivity pneumonitis, and foreign body aspiration was given.

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Fig. 1 PA-right sided chest radiography.

Asthma had been diagnosed in the patient 5 years earlier owing to occasional wheezing, and inhaled budesonide (160μg) and formoterol (4.5μg) were prescribed; oral montelukast (10mg tablet) was added to the regimen last year. On pulmonary function testing, the patient had reversible airflow obstruction. She also had been treated for nasal polyposis with nasal polypectomy 6 months earlier. Her history was notable for anteroseptal myocardial infarction and a cerebrovascular accident in 2003. Cranial magnetic resonance imaging (MRI) at that time demonstrated an acute/subacute infarct at the superior right cerebellar hemisphere and medial temporal lobe. She had no history of other lung diseases, tobacco use, recent travel, change in her home environment, or a new pet.

On admission, her white blood cell count was 12,400 per cubic millimeter with 70% polymorphonuclear lymphocytes and 12% eosinophils. The erythrocyte sedimentation rate was 38mm/hour and C-reactive protein was 56mg/dL. Thoracic computed tomography (CT) scan revealed total atelectasis in the right upper and middle lobes, consolidation in the atelectatic regions, narrowing in the bronchi of the right upper and middle lobes, several small mediastinal and bilateral hilar lymph nodes, and compensatory hyperaeration of the right lower lobe and the left upper lobe; there was no evidence of pulmonary thromboembolism (Fig. 2)

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Fig. 2 Thorax CT: Total atelectasis in right upper and medium lobes, consolidation in atelectatic regions, bronchial narrowing in right upper and medium lobes' bronchi, several small mediastinal and bilateral hilar lymph nodes and compensatory hyperaeration of right lower lobe and left upper lobe; no evidence of pulmonary thromboembolism.

Total eosinophil count and IgE were elevated. Results of a urine analysis were negative for blood and protein. An echocardiogram revealed normal systolic and diastolic functions and no intraventricular thrombi. Tests for antineutrophil cytoplasmic antibodies (ANCA), antinuclear antibody (ANA), rheumatoid factor (RF), anti-double-stranded DNA (anti ds-DNA), anti-Ro/SSA, anti-La/SSB, Scl 70, and indirect Coombs test results were negative. Anti-cardiolipin IgG and IgM levels were normal. Results of serologic tests for aspergillus-specific antibodies were negative. Cranial MRI and diffusion MRI revealed defective changes in the bilateral maxillary sinus medial walls and the medial concha secondary to surgery, diffuse mucosal thickening and nasal polyposis in the paranasal sinuses, cystic encephalomalacia in the superior right cerebellar hemisphere, and minimal gliosis around the cystic encephalomalacia (Fig. 3).

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Fig. 3 Cranial MRI: Defective changes in bilateral maxillary sinus medial wall and medium concha secondary to surgery, diffuse mucosal thickness and nasal polyposis in paranasal sinuses.

A lower-extremity MRI revealed a 9×3,5×2cm solid mass on the anterior surface of the left tibia. An excisional biopsy of the mass was performed demonstrating a lipoma. The patient had no electromyographic evidence of neuropathy. A bronchoscopy revealed that the bronchi in the right upper and middle lobes were concentrically narrowed; the medial and lateral segments of the middle lobe were not seen (Fig. 4). Samples from a bronchoalveolar lavage were negative for acid-fast bacilli, and bacteriological cultures were sterile. The bronchoalveolar lavage fluid contained 78% eosinophils, and results of a mucosal biopsy showed diffuse eosinophilic infiltrates in the interstitium and thickening in the basal membrane (Fig. 5). The patient's presentation and results of the bronchoscopy were consistent with Churg–Strauss syndrome. Montelukast was discontinued, and treatment with methyl prednisolone (80mg/day) was initiated, and the dose has been tapering. One-month control thoracic CT revealed fibrotic sequela in the middle lobe of the right lung and improvement of the atelectasis (Fig. 6).

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Fig. 4 Fiberoptic Bronchoscopy: Concentrically narrowed bronchi in the right upper and middle lobes; medial and lateral segments of middle lobe not seen.

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Fig. 5 Bronchial mucosal biopsy: Diffuse eosinophilic infiltrates in interstitium and thickness in basal membrane.

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Fig. 6 First month control thorax CT: Fibrotic sequel changes in middle lobe of right lung, and atelectasis improved.

3. Discussion 

The patient's symptoms, the findings on physical examination, and eosinophilia were consistent with presence of asthma. However, the atelectasis and elevated diaphragm evident on radiography, as well as the elevated sedimentation rates were not consistent with asthma. Another possible diagnosis might have been allergic bronchopulmonary aspergillosis, but the results of a serologic test for aspergillus-specific antibodies were negative, and a thoracic CT scan did not reveal central bronchiectasis or interstitial changes. Tuberculosis also was excluded after examining the Ziehl–Nielson staining of the bronchoalveolar fluid. Hypereosinophilic syndrome and Churg–Strauss syndrome have many common features including neuropathy, rashes, and eosinophilia; but asthma and elevated IgE levels, common to Churg–Strauss syndrome, are rare in the hypereosinophilic syndrome and were ruled out in our patient.3 Our patient had nasal polyposis, which can accompany asthma and Churg–Strauss syndrome.

The diagnosis of Churg–Strauss syndrome can be made based on the presence of 4 or more of the following: asthma, peripheral eosinophilia greater than 10%, peripheral neuropathy, transient or migratory pulmonary opacities, paranasal sinus abnormality, and extravascular eosinophils on biopsy.4 Our patient had nasal polyposis, asthma, peripheral eosinophilia, pulmonary consolidation in the atelectatic regions, and diffuse eosinophilic infiltrates in the bronchial biopsy sample; diagnosed as Churg–Strauss syndrome.

The term ANCA-associated vasculitis has been applied to small vessel vasculitis, such as Wegener's granulomatosis, microscopic polyangiitis, and Churg–Strauss syndrome. The frequency of ANCA in patients with Churg–Strauss syndrome is 50–78%.5 ANCA negativity, as in our patient, did not exclude the diagnosis.

Previous reports have suggested that the leukotriene receptor antagonists used to treat asthma may cause Churg–Strauss syndrome.6, 7 Introducing these agents allows tapering of systemic glucocorticoids and unmasking of a vasculitic syndrome in any patient receiving a medication that suppresses asthmatic airway obstruction.8 This is supported in our case because our patient did not use montelukast between the onset of asthma and vasculitis (montelukast had been initiated in 2006, and her cerebrovascular accident and myocardial infarction took place in 2001).

Radiologic pulmonary manifestations vary in Churg–Strauss syndrome. The most common CT findings are areas of ground-glass attenuation or consolidation in either a patchy or a predominantly peripheral distribution. Less common abnormalities include small centrilobular nodules, mediastinal lymphadenopathy, pericardial effusion, and atelectasis.9 Previously, Silva and coworkers reviewed the high-resolution CT and pathological findings in Churg–Strauss syndrome of 7 patients.10 Only one patient had atelectasis and in that patient, eosinophilic infiltration of airway walls was present as in our patient; this might help to explain the lobar atelectasis. Physicians should consider Churg–Strauss syndrome in young patients presenting with asthma, nasal polyposis, cerebrovascular accident, coronary artery disease, and lobar atelectasis.

Conflict of interest Statement 

None of the authors have a conflict of interest to declare in relation to this work.