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

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

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

Objective: To analyze and summarize the diagnosis, treatment and prognosis of granulomatosis with polyangiitis (GPA) with nasal symptoms as the first clinical manifestation. Methods: The data of 18 patients of GPA with nasal mucosal symptoms as the first clinical manifestation from the Department of Otorhinolaryngology Head and Neck Surgery, Shandong Provincial Hospital Affiliated to Shandong First Medical University between 2005 and 2019 was collected, including 8 males and 10 females, aged from 5 to 68 years. Nasal endoscopy, imaging examination, laboratory examination, immunological and histopathological examination of nasal mucosa were completed. All patients were treated with glucocorticoid combined with cyclophosphamide and were followed up for 2 to 15 years. Descriptive statistical method was used for analysis. Results: All the 18 patients had the nasal mucosal symptoms as the first clinical manifestation, including nasal obstruction, running nose and epistaxis. Nasal endoscopy showed swelling, erosion, scab and bleeding of nasal mucosa, and 6 cases had nasal septal perforation. Nasal sinus CT scan showed high density shadow of sinus, as well as hyperostosis and osteosclerosis. CT imaging features of pulmonary showed nodular lesion or patchy infiltration in 12 patients and cavitation was found in 6 cases. Laboratory results showed that 13 cases were positive for anti-neutrophil cytoplasmic antibodies (ANCA), and 5 cases were negative. During follow-up period, thirteen patients were symptomatic controlled and survived; two patients died of disease progression; one patient gave up treatment and died; two patients were lost to follow-up. Conclusions: Nasal symptoms are the first clinical manifestation of GPA. Early diagnosis and early treatment with glucocorticoid combined with cyclophosphamide can effectively improve the survival rate.
Antibodies, Antineutrophil Cytoplasmic ; Cyclophosphamide ; Endoscopy ; Female ; Granulomatosis with Polyangiitis/diagnosis* ; Humans ; Male ; Paranasal Sinuses

Bilateral facial palsy, which is usually combined with other diseases, occurs infrequently. It may imply a life-threatening condition. Therefore, the differential diagnosis of bilateral facial palsy is important. However, the etiology is variable, which makes diagnosis challenging. We report a rare case of progressive bilateral facial palsy as a manifestation of granulomatosis with polyangiitis (GPA). A 40-year-old male with otitis media and right facial palsy was referred for electroneurography (ENoG), which showed a 7.7% ENoG. Left facial palsy occurred after 2 weeks, and multiple cavitary opacities were noted on chest images. GPA was diagnosed by lung biopsy. His symptoms deteriorated and mononeuropathy multiplex developed. The possibility of systemic disease, such as GPA, should be considered in patients presenting with bilateral facial palsy, the differential diagnosis of which is summarized in this report.
Adult ; Biopsy ; Diagnosis ; Diagnosis, Differential ; Facial Nerve Diseases ; Facial Paralysis* ; Granulomatosis with Polyangiitis* ; Humans ; Lung ; Male ; Mononeuropathies ; Otitis Media ; Thorax

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

PURPOSE: Delta neutrophil index (DNI) represents the immature granulocytes count associated with neutrophil-consumption. We investigated whether DNI might be associated with Birmingham vasculitis activity score (BVAS) at diagnosis and could predict relapse during the follow-up in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). MATERIALS AND METHODS: We reviewed the medical records of 97 patients having DNI results. Twenty patients had granulomatosis with polyangiitis (GPA), 58 had microscopic polyangiitis (MPA), and 19 had eosinophilic GPA (EGPA). We collected clinical and laboratory data including BVAS, five factor score (FFS), and DNI. The correlation coefficient and cumulative relapse free survival rate were obtained. The optimal cut-off of DNI was extrapolated by calculating the area under the receiver operator characteristic curve. RESULTS: DNI was significantly related to cross-sectional BVAS. Furthermore, among continuous variables, only DNI could reflect BVAS of GPA and MPA, but not EGPA. Severe AAV was defined as BVAS ≥20 (the highest quartile). At diagnosis, patients having DNI ≥0.65% had a significantly higher risk of severe GPA and MPA than those having not (relative risk 4.255) at diagnosis. During the follow-up, DNI ≥0.65% could predict the higher relapse rate. CONCLUSION: DNI could reflect BVAS at diagnosis and furthermore, DNI ≥0.65% could not only identify severe AAV at diagnosis, but also predict relapse during the follow-up in patients with GPA and MPA.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* ; Cytoplasm ; Diagnosis ; Eosinophils ; Follow-Up Studies ; Granulocytes ; Granulomatosis with Polyangiitis ; Humans ; Medical Records ; Microscopic Polyangiitis ; Neutrophils* ; Recurrence* ; Survival Rate ; Vasculitis*

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

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