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Pleural Effusion in Churg-Strauss Syndrome

1989; Elsevier BV; Volume: 95; Issue: 6 Linguagem: Inglês

10.1378/chest.95.6.1357

1931-3543

Serpil C. Erzurum, Gregory A. Underwood, Daniel L. Hamilos, James A. Waldron,

Eosinophilic Disorders and Syndromes

A 33-year-old man with a two-year history of asthma and sinusitis presented with wheezing, pleuritis, bilateral pleural effusions, and patchy basilar infiltrates on chest roentgenogram. Laboratory studies revealed peripheral blood eosinophilia, and pulmonary function studies showed an obstructive pattern which was bronchodilator responsive. Thoracocentesis yielded an acidotic exudative effusion with low glucose, low C3, eosinophilia, and a markedly increased rheumatoid factor. Open lung biopsy revealed extensive eosinophilic interstitial pneumonitis with necrotizing eosinophilic vasculitis. Although pleural effusions are present in 29 percent of Churg-Strauss patients, these effusions have not been well described. This report describes the pleural fluid findings in a case of Churg-Strauss syndrome. A 33-year-old man with a two-year history of asthma and sinusitis presented with wheezing, pleuritis, bilateral pleural effusions, and patchy basilar infiltrates on chest roentgenogram. Laboratory studies revealed peripheral blood eosinophilia, and pulmonary function studies showed an obstructive pattern which was bronchodilator responsive. Thoracocentesis yielded an acidotic exudative effusion with low glucose, low C3, eosinophilia, and a markedly increased rheumatoid factor. Open lung biopsy revealed extensive eosinophilic interstitial pneumonitis with necrotizing eosinophilic vasculitis. Although pleural effusions are present in 29 percent of Churg-Strauss patients, these effusions have not been well described. This report describes the pleural fluid findings in a case of Churg-Strauss syndrome. Churg-Strauss syndrome is a disorder of hypereosinophilia and systemic vasculitis in subjects with asthma and allergic rhinitis. Pleural effusions are commonly reported as a manifestation of this syndrome; however, the cellular and biochemical characteristics have not been well described.1Lanham JG Elkon KB Pusey GD Hughes GR. Systemic Vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome.Medicine. 1984; 63: 65-81Crossref PubMed Scopus (1086) Google Scholar, 2Crofton JW Livingstone JL Oswald NC Roberts ATM. Pulmonary eosinophilia.Thorax. 1952; 7: 1-35Crossref PubMed Scopus (138) Google Scholar, 3Harkavy J. Allergic vasculitis.J Allergy. 1943; 14: 507-528Abstract Full Text PDF Scopus (25) Google Scholar In this case of Churg-Strauss syndrome, the patients pleural effusion is fully described, and the differential diagnosis of acidotic eosinophilic pleural effusions is reviewed. A 33-year-old nonsmoking man presented with a two-year history of sinusitis and progressively worsening asthma. Two weeks before admission, he developed pleurisy, first on the right and then on the left side of his chest, and a chest roentgenogram showed bilateral pleural effusions. His peripheral WBC was 34,000 cells/cu mm with 66 percent eosinophils. A thoracocentesis on the right yielded cloudy fluid with the following findings: LDH, 2856 IU/L; protein, 4.2 g/dL; pH, 7.08; glucose, less than 10 mg/dL; amylase, less than 30 U/L; RBC, 600 cells/cu mm, and WBC 10,400 cells/cu mm with a 95 percent eosinophils. Cultures for bacteria and mycobacteria were negative, and cytology was negative for malignancy. The patient was referred to National Jewish Center for Immunology and Respiratory Medicine for further evaluation. Physical examination was unremarkable except for the lung exam, which revealed dullness at both bases and diffuse expiratory wheezing. The patient was afebrile. Blood studies revealed WBC 22,500 cells/cu mm with 62.8 percent eosinophils. Total eosinophil count was 14,130 cells/cu mm. Serum rheumatoid factor was 1:5120, serum antinuclear antibody was positive at 1:40, CH50 was 34 U/ml (normal 22-43), and C3, 113 mg/dl (normal 83 to 77). Immediate and delayed Aspergillus skin tests were negative. The PPD was negative. Examination of stool for ova and parasites was negative, and filaria and paragonia serologies were negative. Pulmonary function tests revealed airflow limitation which improved after inhaled bronchodilator therapy. The chest x-ray film revealed bilateral pleural effusions with patchy basilar infiltrates (Fig 1). Computed tomography of the chest revealed an interstitial process in the right upper lung, right lower lung, and left lower lung, as well as bilateral effusions. A left thoracocentesis with Cope needle pleural biopsy revealed the following: clear yellow fluid; pH, 7.28; LDH, 1714 IU/L; protein, 6.5 g/dL, glucose, 6 mg/dL; cholesterol, 98 mg/dL; ANA, negative; rheumatoid factor, 1:10240; C3, 25.5 mg/dL; WBCs, 4290 cells/µl; eosinophils, 2,394 cells/cu mm; and RBCs, 5250 cells/cu mm. Pleural fluid cytology was negative for malignant cells. Pleural biopsy showed chronic pleuritis with eosinophilic infiltration. Bronchoalveolar lavage yielded 1.28 × 106Campbell GD Webb WR. Eosinophilic pleural effusion.Am Rev Respir Dis. 1964; 90: 194-201PubMed Google Scholar WBC/ml with 89 percent eosinophils, 3 percent lymphocytes, and 7 percent macrophages. Open lung biospy of the right lower lung revealed patchy interstitial and intraalveolar inflammatory infiltrates rich in eosinophils, with prominent cuffs of inflammatory cells about small pulmonary vessels (Fig 2). Subpleural and interlobular connective tissue was heavily involved with an eosinophil-rich inflammatory infiltrate, and dilated lymphatic channels were present in these structures (Fig 3). There was hypertrophy of bronchiolar musculature, bronchial basement membrane thickening, and reduplication of bronchiolar mucosa with goblet cell hyperplasia. Occasional arteries contained segmental or circumferential transmural inflammatory infiltrates with associated necrosis of a component of the vessel wall; mural macrophage giant cells were often present in these lesions (Fig 4). These histologic findings confirmed the clinical diagnosis of Churg-Strauss syndrome. Prednisone, 60 mg/day was started, and within two weeks, the asthma symptoms, eosinophilia, and roentgenographic abnormalities resolved. The patient has been followed for ten months on tapering doses of prednisone without recurrence of his symptoms.Figure 3Highly cellular eosinophil-rich inflammation of subpleural and interlobular connective tissue is accompanied by lymphatic lumenal dilatation (arrows) (hematoxylin-eosin, original magnification × 10).View Large Image Figure ViewerDownload (PPT)Figure 4Necrotizing vasculitis is evident in this artery by a segmental transmural inflammatory infiltrate (arrowhead) containing a mural macrophage giant cell (hematoxylin-eosin, original magnification ×25).View Large Image Figure ViewerDownload (PPT) Although pleural effusions are present in 29 percent of Churg-Strauss patients, these effusions tend to be small and manifest only as occasional pleurisy.1Lanham JG Elkon KB Pusey GD Hughes GR. Systemic Vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome.Medicine. 1984; 63: 65-81Crossref PubMed Scopus (1086) Google Scholar There are only two reported cases in which pleural fluid has been examined in Churg-Strauss syndrome, and neither of these reports comment on pleural fluid pH or chemistries.2Crofton JW Livingstone JL Oswald NC Roberts ATM. Pulmonary eosinophilia.Thorax. 1952; 7: 1-35Crossref PubMed Scopus (138) Google Scholar, 3Harkavy J. Allergic vasculitis.J Allergy. 1943; 14: 507-528Abstract Full Text PDF Scopus (25) Google Scholar In this case of Churg-Strauss syndrome, two separate thoracocenteses show these effusions to be acidotic exudates with marked eosinophilia and markedly low glucose. The differential diagnosis of acidotic pleural effusions with low glucose is limited to esophageal rupture, infection, malignancy, and rheumatoid effusions.4Campbell GD Ferrington E. Rheumatoid pleuritis with effusion.Dis Chest. 1968; 53: 521-527Abstract Full Text Full Text PDF PubMed Scopus (17) Google Scholar In this case, these causes were excluded by the results of pleural and open lung biopsies and by negative cultures of pleural fluid, pleura, and lung tissue. Rheumatoid effusions may rarely present before manifestations of joint disease, and rheumatoid arthritis patients may have peripheral eosinophilia.4Campbell GD Ferrington E. Rheumatoid pleuritis with effusion.Dis Chest. 1968; 53: 521-527Abstract Full Text Full Text PDF PubMed Scopus (17) Google Scholar, 5Portner LMM Grade WA. Rheumatoid lung disease with cavitary nodules, pneumothorax, and eosinophilia.N Engl J Med. 1966; 275: 697-699Crossref PubMed Scopus (41) Google Scholar Also, there are case reports of eosinophilic pleural effusions in rheumatoid disease.4Campbell GD Ferrington E. Rheumatoid pleuritis with effusion.Dis Chest. 1968; 53: 521-527Abstract Full Text Full Text PDF PubMed Scopus (17) Google Scholar, 5Portner LMM Grade WA. Rheumatoid lung disease with cavitary nodules, pneumothorax, and eosinophilia.N Engl J Med. 1966; 275: 697-699Crossref PubMed Scopus (41) Google Scholar, 6Campbell GD Webb WR. Eosinophilic pleural effusion.Am Rev Respir Dis. 1964; 90: 194-201PubMed Google Scholar, 7Adelman M Albelda SM Gottlieb J Haponik EF. Diagnostic utility of pleural fluid eosinophilia.Am J Med. 1984; 77: 915-920Abstract Full Text PDF PubMed Scopus (131) Google Scholar However, these patients had between 2 and 31 percent eosinophils in their pleural fluid. In addition, all of these patients had rheumatoid skin nodules and most had joint disease and rheumatoid pleural nodules.4Campbell GD Ferrington E. Rheumatoid pleuritis with effusion.Dis Chest. 1968; 53: 521-527Abstract Full Text Full Text PDF PubMed Scopus (17) Google Scholar, 5Portner LMM Grade WA. Rheumatoid lung disease with cavitary nodules, pneumothorax, and eosinophilia.N Engl J Med. 1966; 275: 697-699Crossref PubMed Scopus (41) Google Scholar, 6Campbell GD Webb WR. Eosinophilic pleural effusion.Am Rev Respir Dis. 1964; 90: 194-201PubMed Google Scholar, 7Adelman M Albelda SM Gottlieb J Haponik EF. Diagnostic utility of pleural fluid eosinophilia.Am J Med. 1984; 77: 915-920Abstract Full Text PDF PubMed Scopus (131) Google Scholar The patient we describe lacked any joint, skin, pleural, or lung parenchymal evidence of rheumatoid arthritis. The increased rheumatoid factor in our patient is nonspecific and has been described in 52 percent of patients with Churg-Strauss syndrome.1Lanham JG Elkon KB Pusey GD Hughes GR. Systemic Vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome.Medicine. 1984; 63: 65-81Crossref PubMed Scopus (1086) Google Scholar There have been several reviews of eosinophilic pleural effusions.6Campbell GD Webb WR. Eosinophilic pleural effusion.Am Rev Respir Dis. 1964; 90: 194-201PubMed Google Scholar, 7Adelman M Albelda SM Gottlieb J Haponik EF. Diagnostic utility of pleural fluid eosinophilia.Am J Med. 1984; 77: 915-920Abstract Full Text PDF PubMed Scopus (131) Google Scholar, 8Kokkola K Valta R. Aetiology and findings in eosinophilic pleural effusion.Scand J Respir Dis. 1974; 89: 159-165Google Scholar, 9Vladutiu AO. Pleural effusion. Futura Publishing Co, Mount Kisco NY1986Google Scholar, 10Pbppius H Kokkola K. Diagnosis and differential diagnosis in tuberculous pleurisy.Scand J Respir Dis. 1968; 63: 105-110Google Scholar, 11Bower G. Eosinophilic pleural effusions: a condition with multiple causes.Am Rev Respir Dis. 1967; 95: 746-751PubMed Google Scholar Most reviews stress that eosinophilic effusions are rarely associated with malignancy and usually indicate a “benign” course. Conspicuously absent from all lists is Churg-Strauss syndrome, despite the common occurrence of effusions with this disease.7Adelman M Albelda SM Gottlieb J Haponik EF. Diagnostic utility of pleural fluid eosinophilia.Am J Med. 1984; 77: 915-920Abstract Full Text PDF PubMed Scopus (131) Google Scholar, 9Vladutiu AO. Pleural effusion. Futura Publishing Co, Mount Kisco NY1986Google Scholar This case describes the cellular and biochemical characteristics of the pleural effusions in a case of Churg-Strauss syndrome. The differential diagnosis of acidotic exudative pleural effusions with low glucose should include Churg-Strauss syndrome. The authors thank Dr. Talmadge E. King for the bronchoalveolar lavage analysis and for assistance with this paper.