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

PURPOSE: We aimed to analyze the frequency of eosinophilia-associated diseases and to search for possible markers that may be useful for their differential diagnosis. METHODS: We retrospectively reviewed the medical records of 148 patients with peripheral blood eosinophil count of more than 500/µL who visited the Allergy Department of Chonnam National University Hospital for the first time from January to December 2016. Blood eosinophilia was categorized as mild (<1,500/µL), moderate (1,500–5,000/µL), and severe (>5,000/µL). RESULTS: Blood eosinophilia was mostly caused by allergic diseases (41.9%), parasitic infestation (23.6%), and drug allergy (19.6%). Eosinophil count was higher in patients with parasitic infestation (P<0.01), drug allergy (P<0.01), hypereosinophilic syndrome (HES, P<0.001), or eosinophilic granulomatosis with polyangiitis (EGPA, P<0.001) than in those with allergic diseases. The eosinophilic cationic protein level was higher in patients with HES than in those with allergic diseases (P<0.05) and parasitic infestation (P<0.05). The total IgE level was lower in patients with HES than in those with parasitic infestation (P<0.05) and EGPA (P<0.05). The vitamin B12 level was higher in patients with HES than in those with parasitic infestation (P<0.05). There was no statistically significant difference in tryptase levels between the groups. The most common cause of mild eosinophilia was allergic diseases (59.8%), followed by parasitic infestation (22.7%) and drug allergy (13.4%). The common causes of moderate eosinophilia were drug allergy (37.8%), parasitic infestation (29.7%), and allergic diseases (10.8%). The common causes of severe eosinophilia were EGPA (28.6%), HES (21.4%), parasitic infestation (14.3%), and drug allergy (14.3%). CONCLUSION: Common causes of blood eosinophilia in patients who visit the allergy department are allergic diseases, parasitic infestation, and drug allergy. Several markers, including eosinophil count, total IgE, and vitamin B12, may be useful for the differential diagnosis of eosinophilia-associated diseases.
Diagnosis, Differential ; Drug Hypersensitivity ; Eosinophilia ; Eosinophils ; Granulomatosis with Polyangiitis ; Humans ; Hypereosinophilic Syndrome ; Hypersensitivity ; Immunoglobulin E ; Jeollanam-do ; Medical Records ; Parasitic Diseases ; Retrospective Studies ; Tryptases ; Vitamin B 12

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis that commonly affects the peripheral nervous system. EGPA rarely presents with acute polyneuropathy resembling Guillain-Barré syndrome (GBS). A 51-year-old female patient with a history of asthma suddenly developed bilateral lower extremityparesthesia that progressed to asymmetric ascending paralysis within 10 days of onset. Nerve conduction study results were compatible with acute motor sensory axonal neuropathy, consistent with a GBS subtype. A clinical and neurophysiological diagnosis of GBS was made, and high-dose intravenous immunoglobulins were administered. However, the patient's painful motor weakness persisted. Furthermore, she had newly developed skin lesions on her back, face, and arms. Her blood test revealed marked eosinophilia (>60%). In addition, antineutrophil cytoplasmic antibodies were reported positive. A Water's view radiographic image showed bilateral maxillary sinusitis. Considering the history of asthma, we suspected EGPA-associated polyneuropathy and started steroid treatment. The patient's strength and eosinophilia improved rapidly and dramatically. EGPA can mimic GBS and should be differentiated because of different treatment strategies. Early diagnosis and prompt treatment help achieve a good outcome.
Antibodies, Antineutrophil Cytoplasmic ; Arm ; Asthma ; Axons ; Diagnosis ; Early Diagnosis ; Eosinophilia ; Eosinophils ; Female ; Granulomatosis with Polyangiitis ; Guillain-Barre Syndrome ; Hematologic Tests ; Humans ; Immunoglobulins, Intravenous ; Maxillary Sinus ; Maxillary Sinusitis ; Middle Aged ; Neural Conduction ; Paralysis ; Peripheral Nervous System ; Polyneuropathies ; Skin ; Systemic Vasculitis

PURPOSE: We investigated whether red blood cell distribution width (RDW) predicts vasculitis activity based on Birmingham vasculitis activity score (BVAS) or BVAS for granulomatosis with polyangiitis (GPA) at diagnosis and poor prognosis during follow-up in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). MATERIALS AND METHODS: We reviewed the medical records of 150 patients with AAV. We defined severe GPA as BVAS for GPA ≥7 (the highest quartile). Correlation and standardised correlation coefficients were analysed by linear regression tests. The differences between groups were evaluated by Mann-Whitney test. Relative risk (RR) was assessed by chi square test and Cox hazards model. RESULTS: RDW was correlated only with the vasculitis activity of GPA among patients with AAV. An increase in RDW was associated with the absence of ear nose throat (ENT) manifestation, but not proteinase 3-ANCA. Significant differences were noted in cumulative refractory free survival according to RDW ≥15.4% (p=0.007) and the absence of ENT manifestation (p=0.036). Multivariate Cox hazards analysis identified RDW ≥15.4% as the only significant predictor of refractory disease in GPA (RR 17.573). CONCLUSION: RDW predicts vasculitis activity in GPA, and RDW ≥15.4% at diagnosis may increase the risk of severe GPA at diagnosis and predict refractory diseases during follow-up.
Aged ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/*blood/diagnosis ; *Erythrocyte Indices ; Female ; Follow-Up Studies ; Granulomatosis with Polyangiitis/*blood/diagnosis ; Humans ; Linear Models ; Middle Aged ; Multivariate Analysis ; Prognosis

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*

PURPOSE: We investigated whether C-reactive protein (CRP) to serum albumin ratio (CAR) could be an independent predictor of all-cause mortality in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). MATERIALS AND METHODS: We retrospectively reviewed the medical records of 170 patients with AAV. We collected clinical and laboratory data. We also examined AAV-related and traditional risk factors of all-cause mortality. To assess the hazard ratios of variables, we performed univariable and multivariable Cox hazard model analyses. RESULTS: The mean age was 55.0 years and 53 patients (31.2%) were male among 170 patients with AAV (88 microscopic polyangiitis, 43 granulomatosis with polyangiitis, and 39 eosinophilic granulomatosis with polyangiitis). ANCA was detected in 129 patients (75.9%). The initial mean CRP and serum albumin were 41.1 (mg/L) and 3.6 (g/dL), and the mean CAR at diagnosis was 14.8. The most common risk factor of mortality was hypertension (42.4%), followed by chronic kidney disease ≥stage 3 (25.9%). Fourteen patients (8.2%) died during the mean follow-up of 56.7 months. In both multivariable Cox hazard model analyses, CAR at diagnosis was identified as an independent predictor of all-cause of mortality comparable to diabetes mellitus (DM). Moreover, patients with CAR ≥10.35 and having DM exhibited a higher frequency of all-cause mortality than those without. CONCLUSION: CAR at diagnosis can be an independent predictor of all-cause mortality, comparable to DM, the conventional risk factor of mortality.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* ; Antibodies, Antineutrophil Cytoplasmic ; C-Reactive Protein* ; Diabetes Mellitus ; Diagnosis ; Eosinophils ; Follow-Up Studies ; Granulomatosis with Polyangiitis ; Humans ; Hypertension ; Male ; Medical Records ; Microscopic Polyangiitis ; Mortality* ; Proportional Hazards Models ; Renal Insufficiency, Chronic ; Retrospective Studies ; Risk Factors ; Serum Albumin* ; Vasculitis

Eosinophilic granulomatosis with polyangiitis (EGPA) is an immune related systemic disease that is caused by vasculitis affecting multiple organ systems. It is characterized by asthma, fever, eosinophilia, cardiac problems, renal injury, and peripheral neuropathy. In this report, we describe a patient with EGPA with concurrent cerebral infarction and acute polyneuropathy mimicking a Guillain-Barre syndrome (GBS). A 46-year-old man presented with rapidly progressing gait disturbance, muscular weakness, and tingling sensation in all four limbs. A nerve conduction study revealed sensorimotor polyneuropathy in all four limbs, and a test of the cerebrospinal fluid showed an albumin-cytologic dissociation. In addition, brain magnetic resonance imaging (MRI) using fluid-attenuated inversion recovery and diffusion weighted MRI revealed high signal intensity lesions with gadolinium enhancement on T1-weighted MRI in the right caudate nucleus. After performing laboratory tests, paranasal sinus computed tomography, and a nasal smear, the patient was diagnosed with EGPA and treated with high dose glucocorticoid and oral cyclophosphamide. In conclusion, our findings indicate that a diagnosis of EGPA should be considered when a patient presents with rapidly progressing polyneuropathy mimicking a GBS along with unusual systemic symptoms or brain lesions.
Asthma ; Brain ; Caudate Nucleus ; Cerebral Infarction ; Cerebrospinal Fluid ; Churg-Strauss Syndrome ; Cyclophosphamide ; Diagnosis ; Diffusion Magnetic Resonance Imaging ; Eosinophilia ; Eosinophils* ; Extremities ; Fever ; Gadolinium ; Gait ; Granulomatosis with Polyangiitis* ; Guillain-Barre Syndrome ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Muscle Weakness ; Neural Conduction ; Peripheral Nervous System Diseases ; Polyneuropathies* ; Sensation ; Vasculitis ; Vasculitis, Central Nervous System*

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

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

OBJECTIVE: A substantial portion of granulomatosis with polyangiitis (GPA) patients present with localized disease limited to the upper respiratory tract, however; disease spectrum and prognosis of these patients are unclear. The aim of this study is to describe the clinical characteristics and outcome of patients with localized GPA. METHODS: This was a retrospective descriptive case series of patients with a biopsy proven localized GPA presenting to a single tertiary rheumatology service between January 1995 and September 2015. RESULTS: A total of 5 patients, median age 56 years (range 48 to 59 years) at diagnosis and 80% female, were identified. The median follow-up period was 42 months (range 15 to 62 months). Diagnosis was delayed with median time to diagnosis of 12 months (range 3 to 36 months), and patients underwent 1-3 ear, nose, and throat surgeries during the period of diagnostic delay. Sinusitis was the most frequent symptom in all patients, followed by otomastoiditis with cranial nerve palsies (n=2) and orbital mass (n=1). Antineutrophil cytoplasmic antibody (ANCA) was positive initially in 2/5 patients (40%). Two patients with otomastoiditis and cranial nerve palsies progressed to systemic disease with ANCA positive conversion. These two cases along with a case with orbital mass were refractory to standard treatment of cyclophosphamide with glucocorticoids requiring rituximab treatment. CONCLUSION: Patients with localized GPA may progress to systemic disease over the disease course, and may have aggressive disease refractory to standard treatment. Close monitoring for systemic symptoms and repeated ANCA testing is required in patients with localized GPA.
Antibodies, Antineutrophil Cytoplasmic ; Biopsy ; Cranial Nerve Diseases ; Cyclophosphamide ; Diagnosis ; Ear ; Female ; Follow-Up Studies ; Glucocorticoids ; Granulomatosis with Polyangiitis* ; Humans ; Nose ; Orbit ; Pharynx ; Prognosis ; Respiratory System ; Retrospective Studies ; Rheumatology ; Rituximab ; Sinusitis