Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multi-system disease that presents significant diagnostic challenges due to its complexity and low incidence (White and Dubey, 2023). It affects males and females equally, though males may exhibit more active disease at diagnosis and often require more aggressive treatment (Liu et al., 2023). The hallmark features of EGPA include delayed-onset asthma, eosinophilia in tissues and blood, and vasculitis affecting small to medium-sized arteries (White and Dubey, 2023). EGPA falls under the category of antineutrophil cytoplasmic antibody (ANCA)‍-associated vasculitis (AAV), whereas only about half of EGPA patients test positive for ANCA (Khoury et al., 2023).
Humans ; Male ; Hemorrhage/etiology* ; Granulomatosis with Polyangiitis/complications* ; Heart Arrest/etiology* ; Early Diagnosis ; Eosinophilia/diagnosis* ; Kidney Diseases/etiology* ; Churg-Strauss Syndrome/complications* ; Middle Aged

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

Wegener's granulomatosis (WG) is a multisystem disease characterized by necrotizing granulomatous inflammation and vasculitis. WG classically involves the upper airway, lung and kidneys. Otolaryngologic manifestations are often the presenting symptoms in WG, Subglottic stenosis is a less common, but a life-threatening manifestation of WG, This paper studies about the laryngeal and tracheal stenosis diagnosis and surgical treatment of Wegener's granulomatosis in the past 20 years.
Granulomatosis with Polyangiitis ; complications ; physiopathology ; Humans ; Laryngostenosis ; etiology ; physiopathology ; Larynx ; physiopathology ; Tracheal Stenosis ; etiology ; physiopathology

OBJECTIVE: To raise awareness of granulomatosis with polyangiitis by summarizing its nasal mani- festations and treatment experience. METHOD: Retrospective studies were done to the nasal clinical manifestations and treatment processes of 21 GPA patients in this study. All were treated by the combined treatment of glucocorticoid, cyclophosphamide as well as tripterygium wilfordii, and 4 of them who had much heavier nasal symptoms were treated by endoscopic sinus operation at the same time. RESULT: Eighteen cases were effective treated by medical treatments besides 3 were died of all 21 cases. The nasal symptoms of those 4 patients were obviously improved, and still had effective drainage of sinus after operation with 8-22 months follow up, although the sinus ostiums were reduced comparing to themselves intraoperation at different degree. CONCLUSION GPA is always been ignored which will lead to delay treatment due to the lack of specificity of its clinical manifestations. So, enough attention is the key point of avoiding misdiagnosis as well as providing timely treatment for these patients.
Combined Modality Therapy ; Diagnostic Errors ; Drainage ; Endoscopy ; Granulomatosis with Polyangiitis ; complications ; diagnosis ; therapy ; Humans ; Nose ; Paranasal Sinuses ; Retrospective Studies

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

Female ; Granulomatosis with Polyangiitis ; complications ; diagnosis ; Humans ; Otitis Media ; diagnosis ; etiology ; Young Adult

Facial Paralysis ; complications ; Granulomatosis with Polyangiitis ; complications ; Humans ; Male ; Young Adult

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

OBJECTIVETo evaluate the clinical features, pathologic diagnosis and laboratory test of otologic manifestation in Wegener's granulomatosis (WG).

METHODSFourteen eases of WG with ear involvement were reviewed. The clinical course, method of treatment and outcomes in all cases were evaluated. Diagnosis of WG was made when the patients had clinical findings, clear histologic findings and a positive titer of antineutrophil cytoplasmic autoantibodies (ANCA). RESULTS In all 14 cases, the most frequent finding was chronic otitis media Sensorineural hearing loss was present in 1 case and facial nerve paralysis was present in 2 cases separately. There were 5 patients whose otologic manifestations were the primary involvement of WG. Fourteen cases had positive histologic diagnosis of WC while 13 cases were confirmed positive for ANCA. All patients were treated with glucocorticoids, immunosuppressive drugs, and finally got marked improvement.

CONCLUSIONSWG should he included in the differential diagnosis in cases of atypical inflammatory states of the ear, facial nerve paralysis and sensorineural hearing loss. Early diagnosis was made basing on the histologic findings and a positive titer of ANCA. Appropriate treatment were important to improve the otologic manifestations of WG.

Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Facial Paralysis ; etiology ; Female ; Granulomatosis with Polyangiitis ; complications ; Hearing Loss, Sensorineural ; etiology ; Humans ; Male ; Middle Aged ; Otitis Media ; etiology ; Retrospective Studies ; Young Adult

Adolescent ; Antibodies, Antineutrophil Cytoplasmic ; analysis ; Child ; Granulomatosis with Polyangiitis ; complications ; Humans ; Lung Diseases ; etiology ; Male