Pathological and High Resolution CT Findings in Churg-Strauss Syndrome

doi:10.1016/S1001-9294(11)60012-6

Abstract

Abstract: Objective To investigate the Churg-Strauss syndrome (CSS) associated lung involvement, concentrating on clinical characteristics, pathological findings of lung involvements, response to treatment, and prognosis.
Methods We retrospectively analyzed the characters of the clinical manifestations, thin-section CT and pathological findings of CSS. The study involved 16 patients. Clinical data were obtained by chart review. All patients underwent transbronchial lung biopsy (TBLB). Six of them underwent surgical lung biopsy as well.
Results The patients included 7 men and 9 women, aged from 14 to 61 years (median, 47.5 years). Extrathoracic organs involved included nervous system (7/16) and skin (5/16). Respiratory symptoms included cough (12/16), exertional dyspnea (11/16), hemoptysis (4/16), and chest pain (3/16). CT findings included bilateral ground-glass opacities (12/16), bilateral patchy opacities (12/16), and centrilobular nodules (6/16). The pathological findings of TBLB demonstrated increased eosinophils (3/16), vasculitis (3/16), and interstitial pneumonia (16/16). The pathological findings of surgical lung biopsy of 6 cases showed necrotizing vasculitis in 4 cases, capillaries in 5, eosinophilic pneumonia in 3, granulomas in 2, and airway abnormalities in 3. All patients improved in symptoms after therapy during the study period (range, 3 to 51 months; median, 15 months).
Conclusions Asthma may be present in CSS patient when there is bronchial involvement. Ground-glass opacities and consolidation seen on high-resolution CT reflect the presence of eosinophilic pneumonia, vasculitis, and pulmonary alveolar hemorrhage. TBLB has significant limitations for the diagnosis of CSS. Early diagnosis and therapy can result in satisfactory prognosis.

Key words: vasculitis, asthma, Churg-Strauss syndrome, granulomatosis, hypereosinophilia

Funding:

Tthe Peking Union Medical College Foundation Institutional Review Boardrecords for research wereexcluded from this study.

Rui-e Feng, Wen-bing Xu, Ju-hong Shi, Artin Mahmoudi, Wen-bing Mu, Wen-jie Zheng, Yuan-jue Zhu, and Hong-rui Liu. Pathological and High Resolution CT Findings in Churg-Strauss Syndrome[J].Chinese Medical Sciences Journal, 2011, 26(1): 1-8.

References 35

1.	Guillevin L, Dunogué B. Definition for eosinophilia in Churg-Strauss syndrome. Presse Med 2011;40:224-5.
2.	Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. The proposal of an international consensus conference. Arthritis Rheum 1994; 37:187-92.
3.	Lanham JG, Elkon KB, Pusey CD, et al. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine 1984; 63:65-81.
4.	Masi AT, Hunder GG, Lie JT, 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.
5.	Churg A, Brallas M, Cronin SR, et al. Formes frustes of Churg-Strauss syndrome. Chest 1995; 108:320-3.
6.	Reid AJ, Harrison BDW, Watts RA, et al. Churg Strauss syndrome in a district hospital. Q J Med 1998; 91:219-29.
7.	Buschman DL, Waldron JA Jr, King TE Jr. Churg-Strauss pulmonary vasculitis: high-resolution computed tomography scanning and pathologic findings. Am Rev Respir Dis 1990; 142:458-61.
8.	Casta?er E, Alguersuari A, Gallardo X, et al. When to suspect pulmonary vasculitis: radiologic and clinical clues. Radiographics 2010; 30:33-53.
9.	Kallenberg CG. Pathophysiology of ANCA-associated small vessel vasculitis. Curr Rheumatol Rep 2010; 12:399-405.
10	Keogh KA, Specks U. Churg-Strauss syndrome. Clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med 2003; 115: 284-90.
11	Guillevin L, Cohen P, Gayraud M, et al. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine 1999; 78:26-37.
12	Sablé-Fourtassou R, Cohen P, Mahr A, Pagnoux C, Mouthon L, Jayne D, Blockmans D, Cordier JF, Delaval P, Puechal X, Lauque D, Viallard JF, Zoulim A, Guillevin L; French Vasculitis Study Group. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med 2005; 143:632-8.
13	Sevinc A, Hasanoglu HC, Gokirmak M, et al. Allergic granulomatosis and angiitis in the absence of asthma and blood eosinophilia: a rare presentation of limited Churg- Strauss syndrome. Rheumatol Int 2004; 24:301-4.
14	Malik TQ, Youmbissi TJ, Gacha R, et al. Atypical presentation of Churg-Strauss syndrome: another “forme fruste” of the disease? Am J Med Sci 2002; 324:276-8.
15	Morimatsu Y, Kinoshita M, Koga T, et al. A case of allergic granulomatosis and angiitis without symptoms of asthma. Nihon Kokyuki Gakkai Zasshi 2003; 41:655-9.
16	Jessurun J, Azevedo M, Saldana M. Allergic angiitis and granulomatosis (Churg-Strauss syndrome): report of a case with massive thymic involvement in a nonasthmatic patient. Hum Pathol 1986; 17:637-9.
17	Chen KR, Ohata Y, Sakurai M, et al. Churg-Strauss syndrome: report of a case without preexisting asthma. J Dermatol 1992; 19:40-7.
18	Yamashita Y, Yorioka N, Taniguchi Y, et al. Nonasthmatic case of Churg-Strauss syndrome with rapidly progressive glomerulonephritis. Intern Med 1998; 37:561-3.
19	Schnabel A, Holl-Ulrich K, Dalhoff K, et al. Efficacy of transbronchial biopsy in pulmonary vaculitides. Eur Respir J 1997; 10:2738-43.
20	Silva CI, Müller NL, Fujimoto K, et al. Churg-Strauss syndrome: high resolution CT and pathologic findings. J Thorac Imaging 2005; 20:74-80.
21	Conron M, Beynon HL. Churg-Strauss syndrome. Am J Clin Pathol 2000; 114:767-72.
22	Churg A. Recent advances in the diagnosis of Churg- Strauss syndrome. Mod Pathol 2001; 14:1284-93.
23	Keogh KA, Specks U. Churg-Strauss syndrome. Semin Respir Crit Care Med 2006; 27:148-57.
24	Furuiye M, Yoshimura N, Kobayashi A, et al. Churg- Strauss syndrome versus chronic eosinophilic pneumonia on high-resolution computed tomographic findings. J Co- mput Assist Tomogr 2010; 34:19-22.
25	Sinico RA, Di Toma L, Maggiore U, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum 2005; 52:2926-35.
26	Green RJ, Ruoss SJ, Kraft SA, et al. Pulmonary capillaritis and alveolar hemorrhage. Chest 1996; 110:1305-16.
27	Schnabel A, Reuter M, Csernok E, et al. Subclinical alveolar bleeding in pulmonary vasculitides: correlation with indices of disease activity. Eur Respir J 1999; 14:118-24.
28	Clutterbuck EJ, Pusey CD. Severe alveolar haemorrhage in Churg-Strauss syndrome. Eur J Respir Dis 1987; 71:158- 63.
29	Lai RS, Lin SL, Lai NS, et al. Churg-Strauss syndrome presenting with pulmonary capillaritis and diffuse alveolar hemorrhage. Scand J Rheumatol 1998; 27:230-2.
30	Worthy SA, Müller NL, Hansell DM, et al. Churg-Strauss syndrome: the spectrum of pulmonary CT findings in 17 patients. AJR Am J Roentgenol 1998; 170:297-300.
31	Choi YH, Im JG, Han BK, et al. Thoracic manifestation of Churg–Strauss syndrome: radiologic and clinical findings. Chest 2000; 117:117-24.
32	Johkoh T, Müller NL, Akira M, et al. Eosinophilic lung diseases: diagnostic accuracy of thin-section CT in 111 patients. Radiology 2000; 216:773-80.
33	Guillevin L, Pagnoux C. When should immunosuppressants be prescribed to treat systemic vasculitides? Intern Med 2003; 42:313-7.
34	Watts RA, Scott DG. Classification and epidemiology of the vasculitides. Baillieres Clin Rheumatol 1997; 11:191-217.
35	Borao-Cengotita-Bengoa M, Corral-Gudino L, Del Pino- Montes J, et al. Long-term follow-up of microscopic poly- angiitis, 17-year experience at a single center. Eur J Intern Med 2010; 21:542-7.

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