OBJECTIVES: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis, which often starts with respiratory symptoms such as asthma, and it is difficult to make early clinical diagnosis.This study aims to improve the therapeutic level of EGPA with lung involvement via analyzing the clinical characteristics, diagnosis, and treatment . METHODS: We retrospectively analyzed the clinical data of 13 EGPA patients with lung involvement who were diagnosed from February 1, 2014 to July 31, 2021 in the Second Xiangya Hospital, Central South University. RESULTS: The ratio of male to female in 13 patients was 7꞉6. The patients were diagnosed at median age 52 (46-68) years old and 6 had been diagnosed as "bronchial asthma". Pulmonary clinical manifestations mainly included cough, expectoration, wheezing, and shortness of breath; while extra-pulmonary manifestations mainly included rash and subcutaneous mass, fever, limb numbness, muscle and joint pain, abdominal pain, etc. Peripheral blood tests of all patients showed that 11 patients had eosinophils ≥10%, 10 had elevated inflammatory indicators, and 3 were anti-neutrophil cytoplasmic antibody (ANCA) positive. The major lung imaging features were patches or strips of increased density, multiple nodules, bronchiectasis, bronchial wall thickening, exudation, mediastinal lymph nodes, and so on. Eight patients had sinusitis and 9 with abnormal electromyography. Extravascular eosinophil infiltration was found in 9 patients. Six patients with lung biopsy showed eosinophil, lymphocyte, and plasma cell infiltration, 3 patients were involved in small blood vessels, and 1 had granuloma. Pulmonary function tests were performed in 7 patients, 5 of them showed different degrees of pulmonary ventilation dysfunction, and 4 of them had diffusion dysfunction. Almost all patients respond well to glucocorticoid and immunosuppressant. CONCLUSIONS EGPA is rare in clinical, often involving multiple systems with great harm and may combine with asthmatic manifestations. Pulmonary involvement is relatively common. However, due to insufficient recognition of this disease and huge heterogeneity of pulmonary imaging manifestations, misdiagnosis and missed diagnosis are easy to occur. Relevant laboratory, imaging, and biopsy examination should be performed as early as possible with comprehensive consideration of extrapulmonary involvement. Early identification has great significance to improve the diagnosis rate and prognosis of diseases.
Humans ; Male ; Female ; Middle Aged ; Aged ; Churg-Strauss Syndrome/pathology* ; Granulomatosis with Polyangiitis/pathology* ; Retrospective Studies ; Lung/pathology* ; Asthma

Eosinophilic granulomatosis with polyangiitis(EGPA),also known as Churg-Strauss syndrome,is a clinically rare small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) and the hypereosinophilic syndromes (HESs),characterized by asthma,disseminated necrotizing vasculitis,extravascular granulomas,peripheral eosinophilia,and tissue eosinophilia. This article reviews the pathology,imaging,and clinical features of EGPA.
Antibodies, Antineutrophil Cytoplasmic ; Asthma ; Churg-Strauss Syndrome ; diagnosis ; pathology ; Eosinophilia ; Granulomatosis with Polyangiitis ; diagnosis ; pathology ; Humans

Eosinophilic myocarditis is a disease characterized by eosinophilic infiltration of the myocardium, consisting of acute necrotic stage, thrombotic stage, and fibrotic stage. Although T1 mapping has been increasingly used in various cardiac pathologies, there has been no report of T1 mapping in eosinophilic myocarditis. We report a case of 75-year-old female with eosinophilic myocarditis, whose cardiac magnetic resonance imaging included native T1 mapping, in which apical thrombi were distinctly seen as areas with decreased T1 values, next to areas of inflammation seen as increased T1 value in subendocardium.
Aged ; Churg-Strauss Syndrome* ; Eosinophils* ; Female ; Humans ; Inflammation ; Magnetic Resonance Imaging ; Myocarditis* ; Myocardium ; Necrosis ; Pathology ; Thrombosis

Churg-Strauss syndrome (CSS) is a rare disease characterized by asthma, peripheral eosinophilia and vasculitis. A quarter of CSS patients with clinical remission have experienced a relapse. We report here a case of lung abscess in a patient with relapsed CSS. A 46-year-old man who achieved clinical remission of CSS was confirmed for relapse by the presence of peripheral eosinophillia, pulmonary lesions and perivascular infiltrate of eosinophils in colon pathology. After administration of systemic glucocorticoid and one cycle of cyclophosphamide pulse therapy, he complained of dyspnea, sputum and chest pain. There were necrotic masses containing internal air-fluid levels in the right upper and the left lower lobes of the lung on chest radiography. Percutaneous needle aspiration culture specimens revealed the presence of K. pneumoniae. The patient was successfully treated with a 5-week course of antibiotics.
Anti-Bacterial Agents ; Asthma ; Chest Pain ; Churg-Strauss Syndrome* ; Colon ; Cyclophosphamide ; Dyspnea ; Eosinophilia ; Eosinophils ; Humans ; Lung Abscess* ; Lung* ; Middle Aged ; Needles ; Pathology ; Pneumonia ; Radiography ; Rare Diseases ; Recurrence ; Sputum ; Thorax ; Vasculitis

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 ; Biopsy ; Churg-Strauss Syndrome ; complications ; diagnosis ; Fever ; Glomerulosclerosis, Focal Segmental ; complications ; diagnosis ; Humans ; Kidney ; pathology ; Kidney Diseases ; complications ; Male ; Proteinuria ; diagnosis ; Radiography, Thoracic ; Skin ; pathology ; Treatment Outcome

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis. It is characterized by peripheral eosinophilia, asthma, neuropathy, skin manifestation, and less frequently gastrointestinal tract symptoms. Here we report a case of CSS which is initially manifested as acute acalculous cholecystitis. A 67-year-old asthmatic woman visited Emergency Room because of acute abdominal pain. Computed tomography showed acalculous cholecystitis and laparoscopic cholecystectomy was done. Pathology of gallbladder showed marked eosinophilic infiltration with necrotizing vasculitis and granuloma. On the basis of asthma, sinusitis, lung infiltration, combined peripheral eosinophilia and neuropathy, the patient was diagnosed as CSS. To our knowledge, this is the first case of pathologically proven eosinophilic cholecystitis in Korea.
Abdominal Pain ; Acalculous Cholecystitis* ; Aged ; Asthma ; Cholecystectomy, Laparoscopic ; Cholecystitis ; Churg-Strauss Syndrome* ; Emergencies ; Eosinophilia ; Eosinophils ; Female ; Gallbladder ; Gastrointestinal Tract ; Granuloma ; Humans ; Korea ; Lung ; Pathology ; Sinusitis ; Skin Manifestations ; Systemic Vasculitis ; Vasculitis

Adult ; Anti-Inflammatory Agents ; therapeutic use ; Churg-Strauss Syndrome ; drug therapy ; pathology ; Follow-Up Studies ; Humans ; Male ; Methylprednisolone ; therapeutic use

OBJECTIVETo investigate the Churg-Strauss syndrome (CSS) associated lung involvement, concentrating on clinical characteristics, pathological findings of lung involvements, response to treatment, and prognosis.

METHODSWe 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.

RESULTSThe 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).

CONCLUSIONSAsthma 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.

Adolescent ; Adult ; Asthma ; physiopathology ; Biopsy ; Churg-Strauss Syndrome ; diagnosis ; diagnostic imaging ; drug therapy ; pathology ; Cyclophosphamide ; Female ; Humans ; Immunosuppressive Agents ; therapeutic use ; Lung ; diagnostic imaging ; pathology ; physiopathology ; surgery ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Tomography, X-Ray Computed ; methods ; Treatment Outcome ; Young Adult

Child ; Churg-Strauss Syndrome ; pathology ; Female ; Humans

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 ; Endocardium ; pathology ; Endomyocardial Fibrosis ; etiology ; Female ; Humans