scholarly journals AB0394 CLINICAL CHARACTERISTICS OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS: A SINGLE-CENTER RETROSPECTIVE ANALYSIS ON 52 CASES OF CHINESE PATIENTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1225.1-1225
Author(s):  
L. F. Chen ◽  
Y. Mo ◽  
Q. H. Li ◽  
D. H. Zheng ◽  
L. Dai
Keyword(s):  
Paranasal Sinuses ◽  
Clinical Characteristics ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Disease Process ◽  
Chinese Patients ◽  
Eosinophil Infiltration ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Eosinophilic Granulomatosis

Background:Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare and heterogeneous systemic vasculitis. Different patients or the same patient in different stages show different manifestations, which may lead to misdiagnosis and delay treatment.Objectives:To analyze the clinical characteristics in Chinese patients with EGPA.Methods:EGPA patients who fulfilled the 1990 ACR classification criteria were included between December 2003 and April 2020. The demographic and clinical characteristics were collected and analyzed retrospectively.Results:There were 52 EGPA patients recruited, 34 (65.4%) patients were males and the median age at onset was 47(38~55) years. The median duration from disease onset to diagnosis was 30(4~96) months. For initial symptoms, respiratory manifestations (61.5%) were the most common, including 42.3% patients beginning with asthma, followed by 21.2% with nose/paranasal sinuses manifestations. Respiratory medicine (53.8%) were the most common department at first visit, followed by rheumatology medicine (11.5%, Figure 1A). There were 44.2% EGPA patients definitely diagnosed at the department of rheumatology or after consultation by rheumatologists.During the whole disease process, the most common clinical manifestations were asthma (88.5%), then nose/ paranasal sinuses (84.6%), pulmonary (76.9%) and nervous system (61.5%) manifestations, followed by constitutional symptom (44.2%), heart (36.5%) and skin (23.1%) involvement. Only 9.6% patients had gastrointestinal tract involvement and 3.8% had renal involvement (Figure 1B).There were 46 (88.5%) patients showing the ratio of peripheral blood eosinophils >10% at diagnosis. Among the rest 6 patients, 3 had higher eosinophil ratio before diagnosis, while the other 3 patients had been treated with glucocorticoid before diagnosis, of whom 2 patients showed pathological eosinophil infiltration in lung or paranasal sinuses mucosa, respectively. There were 8(15.4%) patients with positive ANCA. Compared with EGPA patients with negative ANCA, they had lower incidence of asthma (62.5% vs. 93.2%), but higher incidence of constitutional symptoms (87.5% vs. 36.4%), arthralgia (50.0% vs. 6.8%) and renal involvement (25.0% vs. 0.0%), higher peripheral eosinophil count [2.06(0.80~4.51) ×109/L vs. 1.81(0.93~3.32) ×109/L], ESR [20(7~77) mm/h vs. 18(9~42) mm/h] and CRP [18.5(3.2~65.9) mg/L vs. 3.3(3.2~13.0) mg/L], higher Birmingham vasculitis activity score [17(10~22) vs. 13(9~15)] and vasculitis damage index [3(1~3) vs. 2(1~2), all P<0.05]. There were 21.2% EGPA patients showing poor prognostic factors according to five-factor score (FFS) and 34.6% patients according to the revised FFS.Conclusion:EGPA patients may have no asthma especially those with positive ANCA. Multi-disciplinary collaboration especially based on rheumatologists and pulmonologists should be emphasized for early identification and prompt treatment.Figure 1.Department at first visit (A) and clinical manifestations during the whole disease process of 52 Chinese EGPA patients(B).Disclosure of Interests:None declared

scholarly journals Peripheral Neuropathy in ANCA Vasculitis

2021 ◽  
Author(s):  
Mouna Snoussi ◽  
Faten Frikha ◽  
Zouhir Bahloul
Keyword(s):  
Peripheral Neuropathy ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Anca Vasculitis ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

Antineutrophil cytoplasmic antibodies (ANCA)-associated diseases are necrotizing systemic vasculitides that affect small blood vessels (arterioles, capillaries and venules). This entity represents three main systemic vasculitides: granulomatosis with polyangiitis (GPA; formerly Wegener’s granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss’ syndrome). Their clinical manifestations are polymorphous, being the most frequent respiratory, oto-laryngo-pharyngeal and renal involvement. Peripheral neuropathy (PN) is reported in almost 50% of the patients. The aim of this chapter is to discuss the prevalence, clinical presentation, treatment and prognosis of PN in ANCA-associated vasculitis.

scholarly journals Addison’s disease presenting with perimyocarditis

2018 ◽  
Vol 31 (1) ◽  
pp. 101-105
Author(s):  
Elisa Baranski Lamback ◽  
Grazia Morandi ◽  
Eleni Rapti ◽  
Georgi Christov ◽  
Paul A. Brogan ◽  
...  
Keyword(s):  
Systemic Inflammation ◽  
Granulomatosis With Polyangiitis ◽  
Clinical Manifestations ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Polyglandular Autoimmune Syndrome ◽  
Case Presentation ◽  
Prompt Treatment ◽  
Autoimmune Syndrome ◽  
Life Saving ◽  
Eosinophilic Granulomatosis

AbstractBackground:Polyglandular autoimmune syndrome (PGA) and eosinophilic granulomatosis with polyangiitis (EGPA) do not seem to represent a coincidental association.Case presentation:A case of a 15-year-old boy is reported who presented with severe systemic inflammation, perimyocarditis and cardiogenic shock, in whom EGPA was initially suspected and later diagnosed with autoimmune adrenalitis with PGA.Conclusions:The severity of the systemic inflammation and perimyocarditis suggests a more widespread autoimmune-mediated process. Autoimmune adrenal insufficiency should be considered in all cases of pericarditis and perimyocarditis, especially when the severity of clinical manifestations exceeds the expected for the severity of the cardiac findings, as timely identification and prompt treatment may be life-saving.

Comparison of patient self-reported data to physician-reported data in eosinophilic granulomatosis with polyangiitis (Preprint)

2021 ◽  
Author(s):  
Irena Doubelt ◽  
Jason M. Springer ◽  
Tanaz A. Kermani ◽  
Antoine G. Sreih ◽  
Cristina Burroughs ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Clinical Manifestations ◽  
The United States ◽  
Research Network ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
American College Of Rheumatology ◽  
Organ Systems ◽  
Patient Reported ◽  
Reported Data ◽  
Eosinophilic Granulomatosis

BACKGROUND Patient-based registries can help advance research in rare diseases such as eosinophilic granulomatosis with polyangiitis (EGPA), a complex, multi-organ form of anti-cytoplasm neutrophil antibody (ANCA)-associated vasculitis. OBJECTIVE To compare patient-reported vs. physician-reported manifestations, treatments, and outcomes for patients with EGPA. METHODS Comparative analysis of patients ≥18 years with EGPA in Canada or the United States from two separate cohorts: i) The Vasculitis Patient-Powered Research Network (VPPRN), a self-enrolled, secure portal with patient-entered data updated quarterly (2013-2019), vs. ii) The Vasculitis Clinical Research Consortium (VCRC) observational studies, a physician-entered database (2003-2019) of patients who fulfilled the 1990 American College of Rheumatology classification criteria for EGPA. Studied parameters included demographics, clinical manifestations, ANCA status, treatments, and relapses. RESULTS Data from 195 patients with a validated diagnosis of EGPA in the VPPRN and 354 patients enrolled in the VCRC were analyzed. Compared to the VCRC cohort, the patients in the VPPRN cohort were more commonly female (69.2% vs. 59.0% in the VCRC cohort; P =.02), younger at diagnosis (47.3 vs. 50.0 years; P =.03), reported similar frequencies of asthma (96.2% vs 92.9% in VCRC; P =.13), cardiac manifestations (28.8% vs 21.2%; P =.06), but less frequent lung manifestations other than asthma, and more frequent disease manifestations in all other organ systems. ANCA positivity was 48.9% in the VPPRN patients vs. 38.9% (P=.05) in the VCRC cohort. Relapsing disease after study enrollment was reported in 32.3% patients in the VPPRN compared 35.7% of patients in the VCRC. Most therapies (glucocorticoids, cyclophosphamide, mepolizumab) were used at similar frequencies in both groups, except for rituximab with VPPRN patients reporting more use than VCRC cohort (24.1% vs. 10.5%; P =<.001). CONCLUSIONS Patients with EGPA generally report having more manifestations of disease than physicians report for patients with EGPA. These differences imply the need to reconsider how patient- and physician-reported data are collected for the study of EGPA, and reevaluate disease specific definitions. CLINICALTRIAL ClinicalTrials.gov: (1) VCRC Longitudinal Study (LS) NCT00315380 https://clinicaltrials.gov/ct2/show/NCT00315380 and (2) One-Time DNA (OT) study NCT01241305 https://clinicaltrials.gov/ct2/show/NCT01241305

scholarly journals Programmed cell death-1 blockade in kidney carcinoma may induce eosinophilic granulomatosis with polyangiitis: a case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Masanori Harada ◽  
Hyogo Naoi ◽  
Kazuyo Yasuda ◽  
Yutaro Ito ◽  
Namio Kagoo ◽  
...  
Keyword(s):  
Cell Death ◽  
Programmed Cell Death ◽  
Bronchial Asthma ◽  
Nerve Conduction ◽  
Granulomatosis With Polyangiitis ◽  
Allergic Inflammation ◽  
Eosinophil Infiltration ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Kidney Carcinoma ◽  
Eosinophilic Granulomatosis

Abstract Background Immune checkpoint inhibitors have potential applications in treating various cancers but are associated with immune-related adverse events, such as inflammation, in a wide range of organs; however, allergic inflammation caused by these agents has not been extensively studied. Case presentation A 65-year-old man was diagnosed with a kidney neuroendocrine carcinoma. Three months after kidney resection surgery, the tumor cells had metastasized to his liver and lymph nodes. Subsequently, the patient started chemotherapy; however, regardless of treatment, the tumor grew, and the patient experienced a series of adverse effects, such as taste disorder, anorexia, and general fatigue. Finally, he was administered a programmed cell death (PD)-1 inhibitor, nivolumab (biweekly, toal 200 mg/body), which was effective against kidney carcinoma. However, the patient had a bronchial asthma attack at 22 cycles of nivolumab treatment and chest computed tomography (CT) revealed an abnormal bilateral shadow after 37 cycles of nivolumab treatment. Bronchoscopy findings revealed eosinophil infiltration in the lungs along with severe alveolar hemorrhage. Paranasal sinus CT scanning indicated sinusitis and nerve conduction analysis indicated a decrease in his right ulnar nerve conduction velocity. Based on these findings, the patient was diagnosed with eosinophilic granulomatosis with polyangiitis; he was treated with prednisolone, which alleviated his bronchial asthma. To restart nivolumab treatment, the dose of prednisolone was gradually tapered, and the patient was administered a monthly dose of mepolizumab and biweekly dose of nivolumab. To date, there have been no bronchial attacks or CT scan abnormalities upon follow up. Conclusions We present a rare case in which a patient with cancer was diagnosed with eosinophilic granulomatosis with polyangiitis following treatment with a PD-1 inhibitor. Blockade of PD-1 and the programmed cell death ligand (PD-L) 1/PD-1 and PD-L2/PD-1 signaling cascade may cause allergic inflammation. Further studies are needed to identify the specific mechanisms underlying allergic inflammation after PD-1 blockade.

Sign in / Sign up

Export Citation Format

Share Document