Adult ; Female ; Granulomatosis with Polyangiitis ; pathology ; Humans ; Pituitary Diseases ; pathology

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

Aged, 80 and over ; Conjunctivitis ; diagnosis ; etiology ; pathology ; Granulomatosis with Polyangiitis ; complications ; diagnosis ; pathology ; Humans ; Male

Encephalocele ; pathology ; Granulomatosis with Polyangiitis ; pathology ; Histiocytosis, Sinus ; pathology ; Humans ; Otorhinolaryngologic Diseases ; pathology ; Pathology ; trends ; Precancerous Conditions ; pathology ; Tuberculosis, Laryngeal ; pathology

Aged ; B-Lymphocytes ; pathology ; Granulomatosis with Polyangiitis ; diagnosis ; Humans ; Lymphocytosis ; diagnosis ; Male

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

Salivary gland involvement is a rare presenting clinical feature of Wegener's granulomatosis (WG). Early recognition and identification of any unusual presentations of WG may enable the early commencement of appropriate treatment. We report a case in which the initial manifestation of the disease was parotid gland swelling, and discuss the management of the patient. WG should be considered in the differential diagnosis when salivary gland enlargement occurs with other otolaryngological symptoms.
Adult ; Biopsy ; Diagnosis, Differential ; Female ; Granulomatosis with Polyangiitis ; diagnosis ; Humans ; Parotid Gland ; diagnostic imaging ; pathology ; Tomography, X-Ray Computed

Adolescent ; Anti-Glomerular Basement Membrane Disease ; pathology ; Diagnosis, Differential ; Granulomatosis with Polyangiitis ; pathology ; Humans ; Kidney ; pathology ; Lung ; pathology ; Lupus Erythematosus, Systemic ; pathology ; Male

A case is presented of Wegener's granulomatosis limited to the testis and epididymis, simultaneously, in a 69-year-old man. Orchiectomy was carried out through an inguinal incision under the presumptive diagnosis of a right testicular tumor. A hard, irregular mass occupied the upper testicle and a portion of the epididymal head was visualized. Histopathologic examination of the specimen showed granulomatous inflammation of the testis and epididymis with prominent angiocentric granulomata in the walls of arteries, veins and foci of fibrinoid necrosis, surrounded by palisading inflammatory cells with a few giant cells. The diagnosis of limited Wegener's granulomatosis was considered, although antineutrophil cytoplasmic antibody (c-ANCA) test was negative 2 weeks after orchiectomy. The patient showed an excellent response after local complete excision. He remains free of disease 18 months after orchiectomy.
Aged ; Antibodies, Antineutrophil Cytoplasmic ; analysis ; Epididymis ; Genital Diseases, Male ; pathology ; surgery ; Granulomatosis with Polyangiitis ; pathology ; surgery ; Humans ; Male ; Orchiectomy ; Testicular Diseases ; pathology ; surgery

Adrenal Cortex Hormones ; therapeutic use ; Antiviral Agents ; therapeutic use ; Diagnosis, Differential ; Granulomatosis with Polyangiitis ; diagnosis ; Humans ; Lung Neoplasms ; diagnostic imaging ; drug therapy ; pathology ; Lymphomatoid Granulomatosis ; diagnostic imaging ; drug therapy ; pathology ; Prognosis ; Radiography ; Sarcoidosis ; diagnosis ; Tuberculosis, Pulmonary ; diagnosis