3.Churg-Strauss syndrome in children: a case evolving in endomyocardiopathy.
Shan LU ; Wen-Yan ZHOU ; Wei ZHOU ; Zhao-Ping LI ; Yan-Mei CHANG ; Ling WEI ; Xiao-Mei TONG
Chinese Journal of Contemporary Pediatrics 2008;10(5):625-628
A 13-year-old girl presented with a 1-month history of progressive exertional dyspnea (NYHA class IV) and exophthalmos for 6 months. She had a history of long-standing asthma and the presence of allergy. Hypereosinophilia and increased serum IgE levels (2472 IU/mL) were observed. Chest radiography and a high resolution CT scan documented a massive interstitial pulmonary infiltration. Echocardiography confirmed mild tricuspid regurgitation, apical obliteration of the right ventricle by fibrocalcific thickening of the endocardium and echogenic material suggestive of thrombosis. Churg-Strauss syndrome with cardiac involvement (endomyocardiopathy) was diagnosed. The patient received anticoagulation and corticosteroid therapy. In view of rapidly progressive severe endomyocardiopathy and stable hematology, the patient was referred for cardiac surgery. Histopathological examination of resected specimens confirmed laminated thrombus but without any trace of eosinophils embedded.
Adolescent
;
Churg-Strauss Syndrome
;
complications
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Endocardium
;
pathology
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Endomyocardial Fibrosis
;
etiology
;
Female
;
Humans
4.Churg-Strauss syndrome with perforating ulcers of the colon.
Young Bae KIM ; Seung Won CHOI ; In Seo PARK ; Jee Young HAN ; Yoon Seok HUR ; Young Chae CHU
Journal of Korean Medical Science 2000;15(5):585-588
We report a case of a 72-year-old woman with Churg-Strauss syndrome, who presented with intestinal perforation. She has had bronchial asthma with peripheral blood eosinophilia for 30 years. Gross findings of a resected colon showed multiple ulcers with perforation. Histologic findings demonstrated transmural inflammation infiltrated with large numbers of eosionophils, neutrophils and lymphoplasma cells, and characteristic extravascular granuloma in the subserosa. There were multifocally-distributed transmural vasculitis showing all stages of activity in medium and small-sized arteries and veins located in the submucosa, and proper muscle and subserosal layers of the colon, some of which revealed granulomatous inflammation. Histologic finding of liver showed chronic viral hepatitis B with mild inflammatory activity and macronodular cirrhosis. Immunohistochemical findings, acid fuschin orange G staining and electromicroscope found no evidence of hepatitis B virus infection contributing to the pathogenesis of this lesion.
Aged
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Case Report
;
Churg-Strauss Syndrome/virology
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Churg-Strauss Syndrome/pathology
;
Churg-Strauss Syndrome/complications*
;
Colon/virology
;
Colon/pathology*
;
Colonic Diseases/virology
;
Colonic Diseases/pathology
;
Colonic Diseases/etiology*
;
Female
;
Hepatitis B/pathology
;
Hepatitis B Antigens/analysis
;
Human
;
Immunohistochemistry
;
Intestinal Perforation/virology
;
Intestinal Perforation/pathology
;
Intestinal Perforation/etiology*
5.Churg-Strauss syndrome presenting with focal segmental glomerulosclerosis without proteinuria: response to unconventional therapy.
Pradipta GUHA ; Indranil THAKUR ; Arindam RAY ; Sanjoy Kumar CHATTERJEE
Singapore medical journal 2013;54(1):e13-5
Churg-Strauss syndrome (CSS), or allergic granulomatosis, is a rare disease manifested by tissue infiltration, hypereosinophilia and vasculitis. Renal involvement may be seen in up to 50% of cases. We report the case of a 25-year old man who presented with a history of fever for two months, tingling, numbness, and paraesthesia of the upper limbs and left lower limb, along with diarrhoea for one month and an inability to walk for the past seven days. Serial laboratory investigations helped to reach the final diagnosis of CSS with mononeuritis multiplex, and skin, pulmonary and gastrointestinal involvement with hypertension. This is due to renal involvement in the form of focal segmental glomerulosclerosis without any nephrotic range proteinuria, which is a very rare clinical entity. The patient's symptoms were relieved after the administration of an unconventional mode of therapy.
Adult
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Biopsy
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Churg-Strauss Syndrome
;
complications
;
diagnosis
;
Fever
;
Glomerulosclerosis, Focal Segmental
;
complications
;
diagnosis
;
Humans
;
Kidney
;
pathology
;
Kidney Diseases
;
complications
;
Male
;
Proteinuria
;
diagnosis
;
Radiography, Thoracic
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Skin
;
pathology
;
Treatment Outcome
6.Anterior Ischemic Optic Neuropathy in a Patient with Churg-Strauss Syndrome.
Ji Eun LEE ; Seung Uk LEE ; Soo Young KIM ; Tae Won JANG ; Sang Joon LEE
Korean Journal of Ophthalmology 2012;26(6):469-472
We describe a patient with Churg-Strauss syndrome who developed unilateral anterior ischemic optic neuropathy. A 54-year-old man with a history of bronchial asthma, allergic rhinitis, and sinusitis presented with sudden decreased visual acuity in his right eye that had begun 2 weeks previously. The visual acuity of his right eye was 20 / 50. Ophthalmoscopic examination revealed a diffusely swollen right optic disc and splinter hemorrhages at its margin. Goldmann perimetry showed central scotomas in the right eye and fluorescein angiography showed remarkable hyperfluorescence of the right optic nerve head. Marked peripheral eosinphilia, extravascular eosinophils in a bronchial biopsy specimen, and an increased sedimentation rate supported the diagnosis of Churg-Strauss syndrome. Therapy with methylprednisolone corrected the laboratory abnormalities, improved clinical features, and preserved vision, except for the right central visual field defect. Early recognition of this systemic disease by ophthalmologists may help in preventing severe ocular complications.
Biopsy
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Churg-Strauss Syndrome/*complications/diagnosis
;
Diagnosis, Differential
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Fluorescein Angiography
;
Fundus Oculi
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Humans
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Male
;
Middle Aged
;
Ophthalmoscopy
;
Optic Neuropathy, Ischemic/diagnosis/*etiology
;
Visual Acuity
;
Visual Field Tests
7.Churg-Strauss syndrome that presented with mediastinal lymphadenopathy and calculous cholecystitis.
Jung Yoon CHOI ; Ji Eun KIM ; In Young CHOI ; Ju Han LEE ; Je Hyeong KIM ; Chol SHIN ; Seung Heon LEE
The Korean Journal of Internal Medicine 2016;31(1):179-183
No abstract available.
Adult
;
Biopsy
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Cholecystitis/diagnostic imaging/*etiology/therapy
;
Churg-Strauss Syndrome/*complications/diagnosis/drug therapy
;
Diagnosis, Differential
;
Female
;
Gallstones/diagnostic imaging/*etiology/therapy
;
Glucocorticoids/therapeutic use
;
Humans
;
Lymphadenopathy/diagnostic imaging/*etiology/therapy
;
Magnetic Resonance Imaging
;
Mediastinum
;
Methylprednisolone/therapeutic use
;
Predictive Value of Tests
;
Tomography, X-Ray Computed
;
Treatment Outcome