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

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

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

OBJECTIVETo study the prevalence of IgG4-positive plasma cells in inflammatory disease of nasal cavity and paranasal sinuses and its association with IgG4-related sclerosing disease (IgG4-SD).

METHODSThe expression of IgG4 and IgG in plasma cells of 103 cases diagnosed as inflammatory disease of nasal cavity and paranasal sinuses with dense lymphoplasmacytic infiltrate was studied by immunohistochemistry (EnVision) and quantitatively analyzed by medical image analysis system.

RESULTSImmunohistochemical study showed marked infiltration by IgG4-positive plasma cells (>50 per high-power field) in 28 cases, moderate infiltration (30 to 50 per high-power field) in 23 cases, mild (10 to 29 per high-power field) in 30 cases and negative (<10 per high-power field) in 22 cases (P < 0.05). Twenty-two cases studied fulfilled the diagnostic criteria of IgG4-SD (>50 IgG4-positive plasma cells per high-power field and IgG4-to-IgG ratio > 40%), including 3 cases of chronic sinusitis (3/20), 3 cases of nasal polyps (3/18), 3 cases of inflammatory pseudotumor (3/17), 4 cases of fungal sinusitis (4/20), 1 case of rhinoscleroma (1/12), 7 cases of Wegener's granulomatosis (7/11) and 1 case of Rosai-Dorfman disease (1/2).

CONCLUSIONInflammatory disease of nasal cavity and paranasal sinuses fulfilling the diagnostic criteria IgG4-SD is not uncommon. Definitive diagnosis of IgG4-SD requires correlation with other clinical and laboratory findings. Some cases of unexplained inflammatory disease of nasal cavity and paranasal sinus may represent a member of the IgG4-SD spectrum. IgG4 carries diagnostic value in differential diagnosis of inflammatory disease occurring in nasal cavity and paranasal sinuses.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Diagnosis, Differential ; Female ; Granuloma, Plasma Cell ; metabolism ; pathology ; Granulomatosis with Polyangiitis ; metabolism ; pathology ; Histiocytosis, Sinus ; metabolism ; pathology ; Humans ; Immunoglobulin G ; metabolism ; Immunohistochemistry ; Male ; Middle Aged ; Nasal Cavity ; immunology ; pathology ; Nasal Polyps ; metabolism ; pathology ; Nose Diseases ; immunology ; pathology ; Paranasal Sinus Diseases ; immunology ; pathology ; Paranasal Sinuses ; immunology ; pathology ; Plasma Cells ; immunology ; Rhinoscleroma ; metabolism ; pathology ; Sinusitis ; metabolism ; pathology ; Young Adult

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 ; Adult ; Aspergillosis ; complications ; microbiology ; Aspergillus ; isolation & purification ; Candida albicans ; isolation & purification ; Candidiasis ; complications ; microbiology ; Female ; Follow-Up Studies ; Granulomatosis with Polyangiitis ; complications ; microbiology ; pathology ; Humans ; Male ; Middle Aged ; Mucor ; isolation & purification ; Mucormycosis ; complications ; microbiology ; Nocardia ; isolation & purification ; Nocardia Infections ; complications ; microbiology ; Retrospective Studies ; Young Adult

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

OBJECTIVETo improve the diagnostic accuracy of Wegener's granulomatosis (WG) with pulmonary involvement by analyzing the clinical data and chest radiographic features.

METHODSThe clinical data and chest radiographic features of 43 WG cases with pulmonary involvement were retrospectively analyzed.

RESULTSClinically, the patients frequently presented with multi-system involvement, with the incidence of pulmonary involvement of 72.8%. The patients with WG presented with such symptoms in the respiratory system as coughing, expectoration, hemoptysis, and dyspnea. The radiographic manifestations varied among the cases. Nodules and cavitations in the lungs were the signs most frequently found, and patchy infiltration and bronchial narrowing were also observed, which often led to misdiagnosis of WG as other pulmonary diseases.

CONCLUSIONWG often has multi-organ involvement, and the lung is one of the most commonly involved organs. The clinical symptoms and radiographic manifestations of WG have no specificity, and the disease can be easily misdiagnosed. Biopsy and ANCA assay can be important means for WG diagnosis.

Adolescent ; Adult ; Aged ; Antibodies, Antineutrophil Cytoplasmic ; blood ; Biopsy, Needle ; Diagnostic Errors ; Female ; Granulomatosis with Polyangiitis ; complications ; diagnosis ; diagnostic imaging ; Humans ; Lung ; diagnostic imaging ; pathology ; Lung Diseases ; diagnostic imaging ; etiology ; pathology ; Male ; Middle Aged ; Radiography ; Retrospective Studies ; Young Adult

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