Eosinophilic Granulomatosis Research Articles

Factors Predictive of Eosinophilic Granulomatosis with Polyangiitis in Surgical Cases of Eosinophilic Chronic Rhinosinusitis

好酸球性多発血管炎性肉芽腫症 (EGPA) は気管支喘息, アレルギー性鼻炎や好酸球性副鼻腔炎が先行し, 好酸球増多を伴う血管炎を来す症候群であり, 発症機序は不明である. 当施設で好酸球性副鼻腔炎の術後に EGPA を発症した症例で, その予測因子を明らかにすることを目的に検討した. 2014年4月から2019年3月の5年間に好酸球性副鼻腔炎に手術を施行した症例を対象とした. EGPA の発症の有無で2群に分割し, 末梢血好酸球率, アレルギー疾患の合併, 鼻ポリープスコア, 副鼻腔 CT スコア, 鼻腔通気度検査, 嗅覚障害自覚症状, 嗅覚同定検査, 基準嗅力検査, 静脈性嗅覚検査の結果を比較検討した. 対象84例中3例に EGPA の発症を認めた. EGPA 群の末梢血好酸球率は平均20.6%で, 非 EGPA 群の9.2%よりも有意に高値であった. 術前と術後の CT スコアの差である CT 改善値は EGPA 群で低く, 術後の中鼻道ポリープのスコアも高値であった. また, EGPA 群は全例とも気管支喘息を合併しており, 好酸球性副鼻腔炎の重症群であった. 嗅覚検査では術前の嗅覚同定検査が EGPA 群で有意に正答数が多かった. 好酸球性副鼻腔炎において, 末梢血好酸球増多を認める症例, アレルギー疾患を合併して重症群に相当する症例や, 術後も CT や鼻ポリープの改善が乏しい症例では, 全身症状が出現して EGPA と診断される前の状態を見ている可能性があり, 注意を要する.

Association of IgG4-related disease and systemic rheumatic disorders

PurposeAutoimmune disorders can occur together especially in genetically predisposed individuals. We here aimed to assess the occurrence of IgG4-related disease (IgG4-RD) in association with other systemic immune-mediated conditions. MethodsWe retrospectively analyzed the clinical records of patients with IgG4-RD followed at the IgG4-RD Clinic of San Raffaele Hospital (Milan, Italy) for pre-existing or concomitant immune-mediated disorders. IgG4-RD was diagnosed based on histological findings and on the 2011 Comprehensive Diagnostic criteria. Associated immune-mediated disorders were diagnosed based on available classification and/or diagnostic criteria. ResultsTwo-hundred and thirty-four patients with a definitive diagnosis of IgG4-RD were included in this study. A pre-existing immune-mediated connective tissue disease was reported in 6/234 patients (3%): one case each of sarcoidosis, Takayasu arteritis (TA), eosinophilic granulomatosis with polyangitis (EGPA), and rheumatoid arthritis; and two cases of granulomatosis with polyangitis (GPA). Organs involved by IgG4-RD included the lungs, the pancreas, the peritoneum, lacrimal glands, meninges and orbits. Sarcoidosis, EGPA, and TA preceded the onset of IgG4-RD. GPA preceded IgG4-RD onset in one case and occurred simultaneously in the other case. Rheumatoid arthritis occurred together with IgG4-RD in one case. ConclusionOur observation suggests that “secondary” IgG4-RD can present in the context of pre-existing systemic immune-mediated disorders and complicate systemic autoimmune diseases as well as chronic granulomatous conditions. Further studies are needed to define whether this peculiar clinical scenario is associated with different genetic backgrounds, pathological bases, and long-term outcomes.

Churg Strauss Syndrome

Churg Strauss syndrome (CSS) also known as Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare condition characterized by asthma, high levels of eosinophils (a type of white blood cell that helps fight infection), and inflammation of small to medium sized blood vessels (vasculitis).1 The inflamed vessels can affect various organ systems including the lungs, gastrointestinal tract, skin, heart and nervous system. No known cause of disease is found but believe to be an auto immune disorder and genetic factors and allergies play vital role to trigger the disease. CSS has 3 stages, with its own clinical manifestations. It’s not necessary that all patients with CSS will go through all 3 stages and they also do not manifest in same order always. The first stage is an allergic stage presents with respiratory problems, second stage is eosinophilic stage characterized by intra organ infiltration of eosinophils and final stage is a vasculitic stage with swollen lypmph nodes, joint pain, necrotizing inflammation of small vessels. Morbidity rate is high and it also need long term monitoring.

Oral Corticosteroids for Patients with Eosinophilic Diseases: An Expert Panel View on Use, Overuse, and Strategies to Reduce Use

Severe asthma (SA), chronic rhinosinusitis with nasal polyps (CRSwNP), and eosinophilic granulomatosis with polyangiitis (EGPA) are three conditions driven by IL-5 and eosinophilic inflammation. As these conditions have high use of healthcare services, as well as lifestyle and psychological burdens, safe treatment to achieve optimal control is key. In all three conditions, as for many other eosinophilic diseases (ED), oral corticosteroids (OCS) are often used for both acute and maintenance treatment where disease activity is high. While, in general, OCS are very effective, their use is limited by a well-recognised high potential for adverse effects (AE). Moreover, cumulative exposure to OCS may not be acknowledged in many patients, especially for those predominantly treated in primary care, exposing patients to potentially damaging long-term OCS-related AEs. To discuss the use of OCS for these eosinophilic diseases, as well as to provide guidance on how to help limit their use, a board of European experts within each field was gathered. The experts completed questionnaires regarding treatment and referral pathways for patients with SA, CRSwNP, or EGPA; then, in an online meeting, discussed a number of issues in regard to OCS use. Here, the authors present the key recommendations from the expert advisory panel alongside some background to these conditions regarding treatment with OCS.

Acute coronary syndrome in antineutrophil cytoplasmic antibody-associated vasculitis: a Korean single-centre cohort study.

This study investigated the incidence and patterns of the acute coronary syndrome (ACS) after AAV diagnosis and searched for the predictors of ACS in a single-centre cohort of Korean patients diagnosed with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). A total of 262 patients with AAV were included in this study. ST-segment elevation myocardial infarction (STEMI), non-STEMI (NSTEMI), and unstable angina (UA) were defined as ACS in this study. Only ACS that occurred during or after AAV diagnosis was counted. The incidence of ACS in patients with AAV was 2.7% (7 patients), and the most common type of ACS was NSTEMI regardless of the affected site or the number of coronary arteries. Five patients with ACS were diagnosed with microscopic polyangiitis (MPA) and all of them had myeloperoxidase (MPO)-ANCA (or perinuclear [P]-ANCA), whereas the remaining two patients were diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA). Of the seven patients, 2 patients experienced ACS within the first year after AAV diagnosis, and 2 experienced ACS 5 years after AAV diagnosis. Among clinical variables, only the male sex was a predictor of ACS during the follow-up period in patients diagnosed with AAV. The incidence of ACS was 2.7%, and the most common type of ACS was NSTEMI in Korean patients with AAV.

Management of antineutrophil cytoplasmic antibody-associated vasculitis: a review of recent guidelines.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is an autoimmune rheumatic disease consisting of three discrete diagnoses of microscopic polyangiitis, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis. Among diseases treated in a rheumatology department, AAV has poor clinical outcomes, with high rates of mortality and progression to end-stage renal disease and frequent disease relapse. Due to the frequent negative patient outcomes, optimal therapeutic strategies are essential in the management of AAV. In the present review, four guidelines for management of AAV are summarized: British Society for Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guideline for the management of adults with AAV; European League Against Rheumatism (EULAR)/European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) recommendation for the management of AAV; 2021 American College of Rheumatology (ACR)/Vasculitis Foundation Guideline for the Management of AAV; Kidney Disease: Improving Global Outcome (KDIGO) 2021 Clinical Practice Guideline for the Management of Glomerular Diseases, which will aid in clinicians' medical decisions. Finally, the summary of the 2022 Update of the EULAR Recommendations on the Management of AAV, presented in the EULAR Congress 2022 is also introduced.

EGPA Phenotyping: Not Only ANCA, but Also Eosinophils

Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a small-vessel necrotizing vasculitis. The anti-neutrophil cytoplasmic antibodies' (ANCA) role in defining clinical EGPA phenotypes is well established. Although the role of eosinophils in disease pathogenesis has been clearly demonstrated, the value of blood eosinophil count (BEC) as a biomarker of disease phenotypes is currently uncertain. We retrospectively analyzed EGPA patients referred to our Immunology Clinic. Demographic, laboratory and clinical features were retrieved from clinical records, and a Logistic Regression was fitted to evaluate the predictive power of all baseline clinical and laboratory features to define EGPA phenotypes. 168 patients were recruited. BEC ≤ 1500 cells/mL was predictive of a clinical involvement characterized by asthma, chronic rhinosinusitis with nasal polyps (CRSwNP) and lung opacities (OR 0.18, 95% CI 0.07-0.43; respiratory-limited phenotype); BEC > 3500/mL was predictive of extrapulmonary organ involvement (OR 3.5, 95% CI 1.7-7.1; systemic phenotype). BEC was also predictive of peripheral nervous system (PNS) involvement, with a positive trend with increasing BEC (<1500/mL: OR 0.17, 95%CI, 0.06-0.47; >3500/mL: OR 2.8, 95% CI, 1.5-5.28). ANCA positivity was also predictive of extrapulmonary involvement (OR 4.7, 95% CI 1.9-11.99). according to BEC and irrespective of the ANCA status, two EGPA phenotypes could be identified, named systemic and respiratory-limited phenotypes, with different organ involvement and possibly different prognoses.

Development and internal validation of a model to predict long-term survival of ANCA associated vasculitis.

Risk stratification and prognosis prediction are critical for appropriate management of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). Herein, we aim to develop and internally validate a prediction model specifically for long-term survival of patients with AAV. We thoroughly reviewed the medical charts of patients with AAV admitted to Peking Union Medical College Hospital from January 1999 to July 2019. The Least Absolute Shrinkage and Selection Operator method and the COX proportional hazard regression was used to develop the prediction model. The Harrell's concordance index (C-index), calibration curves and Brier scores were calculated to evaluate the model performance. The model was internally validated by bootstrap resampling methods. A total of 653 patients were included in the study, including 303 patients with microscopic polyangiitis, 245 patients with granulomatosis with polyangiitis and 105 patients with eosinophilic granulomatosis with polyangiitis, respectively. During a median follow-up of 33 months (interquartile range 15-60 months), 120 deaths occurred. Age at admission, chest and cardiovascular involvement, serum creatinine grade, hemoglobin levels at baseline and AAV sub-types were selected as predictive parameters in the final model. The optimism-corrected C-index and integrated Brier score of our prediction model were 0.728 and 0.109. The calibration plots showed fine agreement between observed and predicted probability of all-cause death. The decision curve analysis (DCA) showed that in a wide range of threshold probabilities, our prediction model had higher net benefits compared with the revised five factor score (rFFSand) and the birmingham vasculitis activity score (BVAS) system. Our model performs well in predicting outcomes of AAV patients. Patients with moderate-to-high probability of death should be followed closely and personalized monitoring plan should be scheduled.

Inflammatory rheumatic diseases developed after COVID-19 vaccination: presentation of a case series and review of the literature.

An increasing number of new on-set autoimmune-inflammatory rheumatic diseases (AIRD) after COVID-19 vaccination has begun to be reported in the literature. In this article, we present our patients with new-onset AIRD after vaccination for COVID-19 and review the literature on the subject. We investigated the clinical characteristics and laboratory parameters of previously described "newly developed AIRD in individuals recently vaccinated for COVID-19", in 22 cases vaccinated with one of the COVID-19 vaccines (BNT162b2 or CoronaVac) approved in our country. We collected 22 cases (14 female, 63.6%) that developed an AIRD after COVID-19 vaccination. Mean age was 53±14.4 (24-87) years. The interval between the last dose of vaccination and the development of the first complaint was 23.9±19.5 (4-90) days. CoronaVac was administered to four patients, and the BNT162b2 to 18 patients. AIRD-related symptoms developed in 12 patients after the first dose, in 8 patients after the second dose, and in two patients after the third dose. Twelve out of the 22 (54.5%) cases were diagnosed with rheumatoid arthritis, two with SLE, and the remaining eight patients each with leukocytoclastic vasculitis, Sjogren's syndrome, psoriatic arthritis, ankylosing spondylitis, systemic sclerosis, mixed connective tissue disease, eosinophilic granulomatosis with polyangiitis, and inflammatory myositis, respectively. Six patients had a history of documented antecedent COVID-19 infection. Autoimmune/inflammatory rheumatic diseases may develop after COVID-19 vaccinations. In the era of the COVID-19 pandemic, vaccination should be questioned carefully in newly diagnosed AIRD patients.

Risk of acute myocardial infarction, stroke, and venous thromboembolism among patients with anti-neutrophil cytoplasmic antibody-associated vasculitis in South Korea: A nationwide population-based study.

To investigate the incidence and risk of cerebro-cardiovascular comorbidities (stroke, acute myocardial infarction, venous thromboembolism, and pulmonary embolism) in anti-neutrophil cytoplasmic antibody-associated vasculitis using nationwide Korean population-based medical claims data. We identified 1905 patients with newly diagnosed anti-neutrophil cytoplasmic antibody-associated vasculitis during 2009-2019. Incidence rates and hazard ratios with 95% confidence intervals were calculated to estimate the risk of cerebro-cardiovascular comorbidities in these patients and compared to age- and sex-matched controls (1:10) using the Cox proportional hazards model. Most patients had microscopic polyangiitis (42.5%), followed by granulomatosis with polyangiitis (29.1%) and eosinophilic granulomatosis with polyangiitis (28.4%). The annual incidence rate of anti-neutrophil cytoplasmic antibody-associated vasculitis in 2019 was 0.55 per 100,000 person-years. Cerebro-cardiovascular comorbidities occurred in 12.6%. Stroke was most common (64.6%), followed by venous thromboembolism (34.6%), pulmonary embolism (18.3%), and acute myocardial infarction (5.4%). Korean patients with anti-neutrophil cytoplasmic antibody-associated vasculitis were at a significantly (2.3 times) higher overall risk for cerebro-cardiovascular comorbidities than the general population (adjusted hazard ratios, 4.5, 3.1, and 2.0 times higher for pulmonary embolism, venous thromboembolism, and stroke, respectively). These findings were similar for patients with each subtype of anti-neutrophil cytoplasmic antibody-associated vasculitis. This is the first nationwide population-based study to demonstrate a significant risk of cerebro-cardiovascular comorbidities as complications of anti-neutrophil cytoplasmic antibody-associated vasculitis in Korean patients. Knowing these risks may enable personalized patient care and improve overall survival.

Eosinophilic granulomatosis with polyangiitis presenting as recurrent myocardial infarction.

Eosinophilic granulomatosis with polyangiitis presenting as recurrent myocardial infarction.

Patient-reported outcomes in ANCA-associated vasculitis: a cross-sectional study to explore the interactions between patients' and physicians' perspectives.

To evaluate associations between the domains of the ANCA-associated vasculitis patient-reported outcome (AAV-PRO) instrument and clinical variables. Patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), or renal-limited vasculitis (RLV) were recruited from a tertiary care center in Mexico City. Demographic, clinical, serological, and treatment-related data were retrieved. Disease activity, damage, patient and physician global assessments (PtGA and PhGA) were evaluated. All patients completed the AAV-PRO questionnaire, male patients also completed the International Index of Erectile Function (IIEF-5) questionnaire. Seventy patients (44 women and 26 men) were included, with a median age of 53.5years (43-61), and a disease duration of 82months (34-135). Moderate correlations were identified between the PtGA and the AAV-PRO domains: social and emotional impact, treatment side effects, organ-specific symptoms, and physical function. The PhGA correlated with the PtGA and prednisone doses. Subanalyses of the AAV-PRO domains according to sex, age, and disease duration showed significant differences in the treatment side effects domain, with higher scores in women, in patients < 50years, and in patients with disease duration < 5years. The domain of concerns about the future showed a higher score in patients with disease duration < 5years. A total of 17/24 (70.8%) of men who completed the IIEF-5 questionnaire were classified as having some degree of erectile dysfunction. The domains of AAV-PRO correlated with other outcome measures, while differences were found between some of the domains according to sex, age, and disease duration.

An insidious onset of Eosinophilic granulomatosis with polyangiitis

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare small-vessel vasculitis, often associated with MPO p-ANCA (myeloperoxidase–perinuclear–anti-neutrophil cytoplasmic antibody) positivity. It is often difficult to diagnose.

Coronary thromboembolism in a young patient with eosinophilic granulomatosis with polyangiitis.

aDepartment of Radiology bDepartment of Cardiology, University of Pamukkale, Denizli, Turkey Received 20 November 2022 Accepted 19 January 2023. Supplemental Digital Content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's website, www.coronary-artery.com. Correspondence to Furkan Ufuk, MD, Department of Radiology, University of Pamukkale, 20100 Denizli, Turkey, Tel: +90 554 511 50 88; fax: +90 272 512 51 78; e-mail: [email protected]

Systemic vasculitis diagnosed during the post-partum period: case report and review of the literature

IntroductionThe vasculitis diagnosed specifically in the post-partum period are less well known. We report here such a case followed by a descriptive review of the literature.Case reportA 25 year-old French nurse reported abrupt-onset musculoskeletal pain 15 days after delivery of her first infant. Her first pregnancy was uneventful. The physical examination yielded only bilateral conjunctivitis and purpuric eruption of lower limbs, and complementary investigations evidenced pulmonary renal syndrome in connection with the diagnosis of Granulomatosis with Polyangiitis.MethodsWe screened previous articles in Medline database using keywords (i) “post-partum” or “puerperium” (ii)”peripartum” (iii) “pregnancy” associated with “vasculitis”. Full texts were obtained from case reports or cases series whose title or abstract included keywords of interest (or synonyms). These references were secondarily excluded if the diagnosis of vasculitis was not confirmed or made before or more than 6 months after delivery and if vasculitis occurred only in the new born or affected only the cerebral vasculature or the retina.ResultsFifty-six cases of vasculitis diagnosed in the post-partum period were included, 50 (89.3%) were secondary to an immunological process and 44 corresponded to primary vasculitis, 4 were secondary to Systemic Lupus Erythematosus, 1 to cryoglobulinaemia and 1 to cryoglobulinaemia associated with inflammatory bowel disease. The main primary vasculitis diagnosed were Takayasu Arteritis (n = 10), Eosinophilic granulomatosis with polyangiitis (n = 9), Granulomatosis with Polyangiitis (n = 7), Periarteritis Nodosa (n = 5) and Behcet’s disease (n = 4). The first symptom occurred before delivery in 26 (59.1%) and maternal death occurred in 4 (9.1%) out of the 44 primary vasculitis cases.ConclusionThe vasculitis diagnosed in the post-partum period were mainly primary vasculitis among patients in whom the diagnosis had not been made during pregnancy. In less than half of cases and as described in ours, there were no reported symptoms before delivery. Therefore, the physicians should pay attention to symptoms and keep a high degree of suspicion for vasculitis before as well as after delivery.

Predictive potential of albumin-alkaline phosphatase ratio for cerebrovascular accident in patients with antineutrophil cytoplasmic antibody-associated vasculitis.

This study investigated the predictive potential of the albumin-to-alkaline phosphatase ratio (AAPR) for cerebrovascular accident (CVA) occurrence in patients with antineutrophil cytoplasmic antibody-associated vasculitis. This study included 239 AAV patients and reviewed their medical records retrospectively. AAPR was calculated using the following formula: AAPR = serum albumin (g/dL)/serum alkaline phosphatase (IU/L). CVA was defined only as cerebral infarction after AAV diagnosis in this study. In patients with CVA and those without CVA, the follow-up duration based on CVA was defined as the period from AAV diagnosis to CVA occurrence and to the last visit day, respectively. The median age of 239 AAV patients (130 microscopic polyangiitis, 64 granulomatosis with polyangiitis, and 45 eosinophilic granulomatosis with polyangiitis) was 59.0years and 32.6% were men. The median serum albumin and alkaline phosphatase levels, and AAPR were 3.7g/dL, 70.5 IU/L and 0.051, respectively. Nineteen patients had CVA during the median follow-up duration of 34.8months. Using the receiver operator characteristic curve analysis, the optimal cut-off of AAPR for CVA occurrence was obtained as ≤ 0.035. AAV patients with AAPR ≤ 0.035 showed a significantly higher risk of CVA occurrence after AAV diagnosis than those with AAPR >0.035 (relative risk 5.000, p < 0.001). In the multivariable Cox analysis, only AAPR ≤ 0.035 was independently associated with CVA occurrence among AAV patients (hazard ratio 3.195, 95% confidence interval 1.014, 10.062). This study demonstrated the predictive potential of AAPR calculated at AAV diagnosis for CVA occurrence after AAV diagnosis among AAV patients.

EGPA/Hypereosinophilic Overlap Syndrome in two 16-year-old patients

Eosinophilic Granulomatosis with Polyangiitis (EGPA) and Hypereosinophilic syndrome are two eosinophilic diseases that can result in severe systemic manifestations. Generally, they are rare in children.

Patient Experience of Physician-Patient Communication and Delayed or Misdiagnosis In Patients With Eosinophil-Driven Diseases

Eosinophil-driven diseases, such as severe eosinophilic asthma (SEA), eosinophilic granulomatosis with polyangiitis (EGPA), and hypereosinophilic syndromes (HES), are characterized by persistently high eosinophil numbers that cause damage to tissues and organs. This study looked at social media use by patients and caregivers to assess discussions of delayed or misdiagnosis with SEA, EGPA, and HES.

Subendocardial Ischemia Caused by Acute Severe Aortic Regurgitation Due to Aortic Root Dissection: A Case Report and Literature Review.

Electrocardiogram (ECG) findings showing ST-segment depression in a wide range of leads and ST-segment elevation in aVR are found in patients with acute coronary syndrome with multivessel coronary lesions and left main trunk lesions. A 64-year-old man with a history of eosinophilic granulomatosis presented with chest pain and dyspnea. Although an ECG showed the above findings, he was diagnosed with acute severe aortic regurgitation (AR) complicating aortic root dissection and successfully underwent urgent Bentall operation. These ECG findings indicated that acute severe AR caused subendocardial ischemia.

Successful treatment with rituximab and plasmapheresis of renal involvement of eosinophilic granulomatosis with polyangiitis

BackgroundEosinophilic granulomatosis with polyangiitis (EGPA) is a rare disorder characterized by asthma, eosinophilia, and systemic vasculitis. Renal involvement is not regarded as a prominent feature and the treatment is still under study.Case presentationA 68-year-old woman was admitted to our hospital because of fever, renal dysfunction, eosinophilia, and the presence of MPO-ANCA. Based on the renal pathological examination which showed extravascular eosinophilic-predominant inflammation and crescentic glomerulonephritis, EGPA was diagnosed. Considering the acute kidney injury, prominent eosinophilia, and strongly positive anti-MPO antibodies, pulse steroid therapy was administered, followed by intravenous rituximab. Plasmapheresis was also provided (9 sessions). The eosinophil count was normalized, and renal dysfunction was reversed. The patient no longer requires dialysis.ConclusionsRenal involvement of EGPA is rare, and consensus on its treatment is still lacking, because of a lack of large-scale randomized controlled trials. We treated our patient as a case with high severity. For patients with severe disease, the addition of cyclophosphamide to glucocorticoid therapy is commonly used. However, rituximab and plasmapheresis combined with systemic glucocorticoid therapy were found to be beneficial because the renal function and other clinical conditions were almost fully recovered. Thus, our treatment is highly effective against renal involvement of eosinophilic granulomatosis with polyangiitis.