Churg–Strauss syndrome: Case series

Alfaro, Tiago M.; Duarte, Cátia; Monteiro, Rita; Simão, Adélia; Calretas, S.; Costa, Nascimento

doi:10.1016/j.rppnen.2012.01.005

Cited by 10 publications

(5 citation statements)

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“…Renal involvement (most commonly focal and segmental necrotizing glomerulonephritis) and gastrointestinal system involvement (stomachache caused by eosinophilic gastroenteritis and vasculitis) are also reported. Pancreatitis, gastrointestinal perforation, and hemorrhage indicate underlying vasculitis and are accepted as the indicator of a poor diagnosis [25,26,[33][34][35]. In our study, paranasal sinus involvement was present in 3 patients, nasal polyposes in 1 case, skin involvement in 3 cases, cardiac involvement in 2 cases, and neurological involvement in 1 case.…”

Section: Discussionmentioning

confidence: 54%

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Eosinophilic Lung Disease: Accompanied with 12 Cases

et al. 2015

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OBJECTIVES:Eosinophilic lung diseases are a rare group of heterogeneous diseases characterized by the increase of the eosinophil ratio in airways and lung parenchyma. In our clinic, patients diagnosed with eosinophilic lung disease were evaluated with their clinical features and prognoses. MATERIAL AND METHODS:In our clinic, 12 cases that were diagnosed and followed up for eosinophilic lung disease [eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) (n=4), chronic eosinophilic pneumonia (CEP) (n=7), and simple pulmonary eosinophilia (Löffler's syndrome) (n=1)] were retrospectively evaluated. RESULTS:Of the 12 cases, 8 were females, and the average age was 43 (28-72) years. All cases were undergoing bronchodilator therapy with asthma diagnosis (2 months-40 years). Additionally, 4 of the cases had sinusitis, and 1 had allergic rhinitis. The most common complaints of the patients were difficulty in breathing and coughing, and the duration of complaints was a median of 2 months. Peripheral eosinophilia and total IgE elevation were present during the admission of all cases; additionally, leucocyte elevation was recorded in 10 of them, anemia in 4 of them, and thrombocytosis in 4 of them. Moreover, 43% of the recorded DLCO values were lower than normal. Of the 10 cases that underwent bronchoalveolar lavage (BAL), the eosinophil ratio was above 25% in 7 subjects. Of the 8 cases that underwent transbronchial biopsy, eosinophil-involving infiltration was detected in 6 subjects. Additional findings in cases diagnosed with EGPA were nasal polyposis (n=1), sinusitis (n=2), polyneuropathy (n=1), cardiac involvement (n=2), and skin involvement in biopsy (n=1). Spontaneous recovery was observed in the patient diagnosed with simple pulmonary eosinophilia during the follow-up that was performed based on the history and laboratory and BAL results of the patient. Prednisolone treatment was started for all cases, except for simple pulmonary eosinophilia, and their controls were performed. Relapse was observed in eight cases (EGPA: 4, CEP: 4); during the relapse treatment of one case diagnosed with EGPA, exitus occurred. One case rejected treatment despite the presence of peripheral eosinophilia, and the other cases are being followed-up without medication. CONCLUSION:Given that the clinical pictures in pulmonary eosinophilia syndromes are on a wide spectrum, a specific diagnosis is important. Progression may differ in each patient, and a close follow-up is necessary during and after the treatment. 24.12.2014 KEYWORDS:

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Section: Discussionmentioning

confidence: 54%

“…Atopic asthma and rhinitis that can be difficult to control is dominant, and it can last for many years. This stage is followed by the eosinophilic stage that involves the eosinophilia infiltration of the lung and various organs, after which the vasculitic stage ensues, observed with severe systemic vasculitis [25][26][27][28].…”

Section: Discussionmentioning

confidence: 99%

Eosinophilic Lung Disease: Accompanied with 12 Cases

et al. 2015

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“…Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis, was first described in 1951 by Churg and Strauss as a rare multisystemic disorder with necrotizing vasculitis of the small vessels, associated with extravascular eosinophilic granulomas that occur exclusively in patients with asthma and peripheral eosinophilia (25). Churg-Strauss syndrome affects both sexes equally and can manifest at any age (average age, 50 years), with an incidence of one to seven cases per million per year.…”

Section: Churg-strauss Syndromementioning

confidence: 99%

Cardiopulmonary and Gastrointestinal Manifestations of Eosinophil- associated Diseases and Idiopathic Hypereosinophilic Syndromes: Multimodality Imaging Approach

Katre¹,

Sunnapwar²,

Restrepo³

et al. 2016

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Eosinophil-associated diseases (EADs) are a diverse group of disorders characterized by an increase in circulating or tissue eosinophils. Cardiopulmonary and gastrointestinal system involvement can be due to primary EAD with no known cause or can be secondary to known systemic disease. The cardiopulmonary spectrum of EADs comprises simple pulmonary eosinophilia, acute eosinophilic pneumonia, chronic eosinophilic pneumonia, Churg-Strauss syndrome, allergic bronchopulmonary aspergillosis, bronchocentric granulomatosis, parasitic infections, and idiopathic hypereosinophilic syndrome. Eosinophilic gastrointestinal disorders include eosinophilic esophagitis, eosinophilic gastroenteritis, and eosinophilic colitis. Diagnosis is often challenging and requires a combination of clinical and imaging features along with laboratory findings. The absolute eosinophil count in peripheral blood and the percentage of eosinophils in bronchoalveolar lavage fluid are crucial in evaluation of various eosinophilic lung diseases. Although chest radiography is the initial imaging modality used in suspected cases of pulmonary EAD, multidetector computed tomography may demonstrate more characteristic pulmonary patterns, nodules, and subtle parenchymal abnormalities. Barium esophagography is used to assess mucosal abnormalities and the length and diameter of esophageal strictures. Magnetic resonance imaging is superior in providing valuable information in select patients, especially in evaluation of cardiac and gastrointestinal system involvement. Many patients require a multimodality imaging approach to enable diagnosis, guide treatment, and assess treatment response. Knowledge of the clinical features and imaging findings of the spectrum of EADs involving the lungs, heart, and gastrointestinal tract permits optimal patient care.

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“…Churg-Strauss sindromas (CSS) -retas, sisteminis nekrozinis vaskulitas, pažeidžiantis smulkias ir vidutines įvairių organų (plaučių, periferinių nervų, odos, širdies, inkstų, virškinimo trakto) kraujagysles ir pasireiškiantis sunkia astma, kraujo ir audinių eozinofilija bei įvairių organų pažeidimo simptomais [1,2]. Vienas pagrindinių CSS bruožų -ryšys su alerginėmis ligomis: astma, sinusitu ir rinitu [3].…”

Section: įVadasunclassified

Churg-Strauss sindromas

Krivickaitė¹,

Orlovskytė²,

Chomičienė³

et al. 2013

Sveikatos Mokslai

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BIOMEDICINA / BIOMEDICINERaktažodžiai: Churg-Strauss sindromas (CSS), vaskulitas, eozinofilai, ANCA asocijuotas vaskulitas, Churg-Strauss granulioma. SantraukaChurg-Strauss sindromas (CSS) -tai retas, nežinomos etiologijos, sisteminis nekrozinis smulkiųjų ir vidutinių kraujagyslių vaskulitas. Pirmą kartą aprašytas J. Churg ir L. Strauss 1951 m. Šiuo metu klasifikuojamas kaip ANCA asocijuotas vaskulitas. CSS specifiški požymiai, padedantys atskirti šį sindromą nuo kitų smulkiųjų kraujagyslių vaskulitų: astma, prasidedanti suaugusiųjų amžiuje, ekstravaskulinės nekrotinės granuliomos, kraujo ir audinių eozinofilija. Literatūros duomenimis, odos pakitimai nustatomi daugiau nei pusei pacientų ir, kai kuriais atvejais, yra pirmieji šios ligos požymiai. Odos ir kitų organų pažeidimų vertinimas bei atlikta odos biopsija gali padėti anksti diagnozuoti CSS ir skirti etiopatogenetinį gydymą. Šiame straipsnyje pristatome CSS klinikinį atvejį bei literatūros apžvalgą. ĮvadasChurg-Strauss sindromas (CSS) -retas, sisteminis nekrozinis vaskulitas, pažeidžiantis smulkias ir vidutines įvairių organų (plaučių, periferinių nervų, odos, širdies, inkstų, virškinimo trakto) kraujagysles ir pasireiškiantis sunkia astma, kraujo ir audinių eozinofilija bei įvairių organų pažeidimo simptomais [1,2]. Vienas pagrindinių CSS bruožų -ryšys su alerginėmis ligomis: astma, sinusitu ir rinitu [3]. 30-40 proc. ligos atvejų nustatomi antineutrofiliniai citoplazminiai antikūnai (ANCA), todėl CSS vaskulitų klasifikacijoje priskiriamas ANCA asocijuotiems vaskulitams. Šiai grupei taip pat priklauso mikroskopinis poliangitas (MPA) bei granuliomatozė su poliangitu (Wegenerio GPA) [3][4][5]. Odos pakitimai nustatomi 40-81 proc. CSS

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Churg–Strauss syndrome: Case series

Cited by 10 publications

References 50 publications

Eosinophilic Lung Disease: Accompanied with 12 Cases

Eosinophilic Lung Disease: Accompanied with 12 Cases

Cardiopulmonary and Gastrointestinal Manifestations of Eosinophil- associated Diseases and Idiopathic Hypereosinophilic Syndromes: Multimodality Imaging Approach

Churg-Strauss sindromas

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