Summary Eosinophilic granulomatosis with polyangiitis (EGPA), or Churg-Strauss Syndrome (CSS) is a rare granulomatous necrotizing vasculitic disease characterized by the presence of asthma, sinusitis, and hypereosinophilia. We describe a patient who was initially diagnosed with tuberculous lymphadenitis and later diagnosed with EGPA.
Granulomatosis with Polyangiitis ; Tuberculosis, Cutaneous ; Tuberculosis, Lymph Node

ABSTRACT Granulomatosis with polyangiitis (GPA) is a rare multisystem disease. Although GPA is rare, it commonly presents in a localised stage where its manifestation involves the upper or lower respiratory tract before progressing to a generalised stage. Therefore, most patients with GPA will visit an oral surgeon or an otolaryngologist to seek treatment. However, the diagnosis of GPA is often delayed as GPA is not frequently considered as a differential diagnosis in common oral and facial diseases. The lack of gold standard investigation for the diagnosis of GPA makes management of this case, a diagnostic conundrum. We herein report a patient who was diagnosed with bilateral acute otitis media and left mastoiditis complicated with facial nerve palsy, and later developed tongue ulceration one month after his initial presentation. The ear, facial and oral symptoms represent a diagnostic red herring to a full-blown generalised stage of GPA.
Facial Paralysis ; Oral Ulcer ; Granulomatosis with Polyangiitis

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

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

Wegener' granulomatosis is an autoimmune diseases, often involving the lung and kidney, has a high mortality rate in nontreatment patients. The low incidence and nonspecific features, often lead to misdiagnosis and delayed treatment. This paper reported the diagnosis and treatment of a 55-year-old female patient with primary Wegener' granuloma of the lung diagnosed by percutaneous lung biopsy of pulmonary nodules, and reviews the relevant literature.
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Female ; Granulomatosis with Polyangiitis/drug therapy* ; Humans ; Lung Neoplasms ; Middle Aged ; Pneumonia

No abstract available.
Granulomatosis with Polyangiitis ; Vasculitis

PURPOSE: The present study investigated chest computed tomography (CT) patterns and lung histological features, as well as the consistency between radiological and histological features among patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic GPA (EGPA). MATERIALS AND METHODS: The medical records of 74 antineutrophil cytoplasmic antibody-associated vasculitis (AAV) patients with radiological lung parenchymal lesions were reviewed along with the histological results for 28 of them. Chest CT patterns were divided according 12 items mostly suggested by radiologists and histological features were divided according to necrotising granuloma, necrotising vasculitis, eosinophilic infiltration, and hemosiderin laden macrophages as defined by a pathologist. RESULTS: The mean age was 57.1 years (22 men). The most common clinical manifestation other than lung manifestation was renal manifestation (62.2%), and the most common chest CT pattern was lung involvement of vasculitis (35.1%). In MPA patients, the major histological features were hemosiderin-laden macrophages in the alveolar space and vasculitis. In GPA patients, the major histological features were necrotizing vasculitis and necrotizing granuloma, while in EGPA patients, the major histological feature was only necrotising vasculitis. The consistency rate in GPA patients was the highest (100%), followed by that in MPA patients (66.7%) and EGPA patients (50.0%). CONCLUSION: When lung involvement of AAV is suspected on chest CT, lung biopsy should be recommended for the proper classification of AAV, due to the discordance rate between radiological and histological findings in MPA and EGPA patients, but not GPA patients.
Biopsy ; Classification ; Cytoplasm ; Eosinophils ; Granuloma ; Granulomatosis with Polyangiitis ; Hemosiderin ; Humans ; Lung ; Macrophages ; Medical Records ; Microscopic Polyangiitis ; Thorax ; Tomography, X-Ray Computed ; Vasculitis

No abstract available.
Eosinophils* ; Gallbladder* ; Granulomatosis with Polyangiitis*

Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a group of systemic necrotising vasculitides, which often involve small vessels, and which lead to few or no immune deposits in affected organs. According to clinical manifestations and pathological features, AAV is classified into three variants: microscopic polyangiitis, granulomatosis with polyangiitis (GPA), and eosinophilic GPA. The American College of Rheumatology 1990 criteria contributed to the classification of AAV, although currently the algorithm suggested by the European Medicines Agency in 2007 and the Chapel Hill Consensus Conference Nomenclature of Vasculitides proposed in 2012 have encouraged physicians to classify AAV patients properly. So far, there have been noticeable advancements in studies on the pathophysiology of AAV and the classification criteria for AAV in Western countries. However, studies analysing clinical features of Korean patients with AAV have only been conducted and reported since 2000. One year-, 5 year-, and 10 year-cumulative patient survival rates are reported as 96.1, 94.8, and 92.8%. Furthermore, initial vasculitis activity, prognostic factor score, age and specific organ-involvement have been found to be associated with either all-cause mortality or poor disease course. The rate of serious infection is 28.6%, and 1 year-, 5 year- and 10 year-cumulative hospitalised infection free survival rates range from 85.1% to 72.7%. The overall standardised incidence ratio of cancer in AAV patients was deemed 1.43 compared to the general Korean population.
Antibodies, Antineutrophil Cytoplasmic ; Classification ; Consensus ; Cytoplasm* ; Eosinophils ; Granulomatosis with Polyangiitis ; Humans ; Incidence ; Korea* ; Microscopic Polyangiitis ; Mortality ; Rheumatology ; Survival Rate ; Vasculitis*

No abstract available.
Churg-Strauss Syndrome ; Eosinophils* ; Erythema* ; Granulomatosis with Polyangiitis* ; Humans ; Hypereosinophilic Syndrome