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Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by necrotizing vasculitis of small-sized vessels with extravascular granulomas and eosinophilic infiltration. The case of a 48-year-old Japanese woman with EGPA, who presented concurrently with subarachnoid hemorrhage (SAH) and coronary vasculitis, is reported. She initially presented with bronchial asthma, and then 8 months later she developed various symptoms caused by systemic eosinophilic vasculitis and was admitted to our hospital. Three days after admission, she started oral corticosteroid therapy, and her 2009 Five-Factor Score (FFS) was 0. However, she developed an SAH, followed by coronary vasculitis 1 day later. With extensive treatment with a combination of betamethasone, cyclophosphamide, intravenous immunoglobulin, and rituximab, her systemic vasculitis improved dramatically. This seems to be the first case of EGPA with SAH and coronary vasculitis. In previous reports of EGPA with SAH, 4 of 11 cases developed SAH as an exacerbation of systemic vasculitis during remission induction therapy. The present patient also had SAH during remission induction therapy. However, the period between bronchial asthma and SAH was only 8 months. This is the shortest among case reports of EGPA with SAH. In addition, the present patient rapidly developed coronary vasculitis. These findings suggest that EGPA causes SAH and coronary vasculitis as early complications of systemic vasculitis. In EGPA, it is necessary to pay careful attention to rapid changes of disease activity, even when the FFS indicates a good prognosis. [ABSTRACT FROM AUTHOR]

Granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) are multisystem diseases of small blood vessels, collectively known as the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). This study explores the patient’s perspective on the use of glucocorticoids, which are still a mainstay of treatment in AAV. Patients with AAV from the UK, USA, and Canada were interviewed, using purposive sampling to include a range of disease manifestations and demographics. The project steering committee, including patient partners, designed the interview prompts and cues about AAV, its treatment, and impact on health-related quality of life. Interviews were transcribed and analysed to establish themes grounded in the data. A treatment-related code was used to focus analysis of salient themes related to glucocorticoid therapy. Fifty interviews were conducted. Individual themes related to therapy with glucocorticoids emerged from the data and were analysed. Three overarching themes emerged: (1) Glucocorticoids are effective at the time of diagnosis and during relapse, and withdrawal can potentiate a flare, (2) glucocorticoids are associated with salient emotional, physical, and social effects (depression, anxiety, irritation, weight gain and change in appearance, diabetes mellitus, effect on family and work); and (3) patient perceptions of balancing the risks and benefits of glucocorticoids. Patients identified the positive aspects of treatment with glucocorticoids; they are fast-acting and effective, but, they voiced concerns about adverse effects and the uncertainty of the dose-reduction process. These results may be informative in the development of novel glucocorticoid-sparing regimens. [ABSTRACT FROM AUTHOR]

Introduction. Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a systemic vasculitis of the small vessels that often associates asthma and blood/tissue eosinophilia. Case presentation. A 58-year-old woman was admitted to our hospital for progressive exertional dyspnea. She had been diagnosed with asthma three years earlier. Recent multiple thoracic computed tomography scans displayed non-fixed interstitial lung abnormalities, whereas the infectious workup (HIV, parasites) was negative. On admission, the clinical examination noted prolonged expiratory phase. Paraclinical tests revealed biological inflammatory syndrome, eosinophilia, polyclonal hypergammaglobulinemia, elevated total IgE level, negative anti-neutrophil cytoplasmic antibodies and proteinuria of nephritic pattern. The pulmonary evaluation reconfirmed the obstructive ventilatory dysfunction and interstitial lung abnormalities - interlobular septal thickening and diffuse ground-glass opacification. The histopathological examination of a transbronchial biopsy specimen identified leukocytoclastic necrotizing vasculitis and tissue eosinophilia. Provided the clinical and paraclinical setting, specifically the asthma, blood and extravascular eosinophilia, paranasal sinus abnormalities, non-fixed pulmonary infiltrates and the histopathologically confirmed necrotizing vasculitis, the diagnosis of EGPA seemed appropriate. Conclusion. In the absence of diagnostic criteria, EGPA diagnosis is often challenging. Although certain clinical and imaging features could assist the diagnosis, biopsy remains the diagnostic gold standard. In the setting of lung involvement, open lung biopsy is usually required for EGPA histopathological proof, but few cases diagnosed by transbronchial biopsy have been reported. This method often identifies discrete, nonspecific lesions or an incomplete spectrum of pathognomonic abnormalities but has the advantage of minimal invasivity that justifies its use as an alternative diagnostic technique. [ABSTRACT FROM AUTHOR]

The antineutrophil cytoplasm autoantibody (ANCA)-associated vasculitides (AAV), although rare in childhood, can have devastating effects on affected organs, especially the kidney. In this review we present an update on the pathogenesis and treatment of ANCA vasculitis, with a particular emphasis on the role of the alternative pathway of complement. The rationale and evidence for the current treatment strategies are summarized. Targeting the activation of neutrophils by the anaphylatoxin C5a may serve as an additional therapeutic strategy, however the results of clinical studies are awaited. [ABSTRACT FROM AUTHOR]

Background: 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. [ABSTRACT FROM AUTHOR]

Background: In 1951 Churg and Strauss first described the clinical condition now known as eosinophilic granulomatosis with polyangiitis (EGPA), characterized by asthma, nasal polyposis, rhinosinusitis, hypereosinophilia with organ infiltration, and necrotizing vasculitis. It is classified as an antineutrophil cytoplasmic antibody (ANCA) associated vasculitis, but ANCA negativity is common and more frequently encountered in EGPA with myocardial involvement. Long-term survival has substantially improved with corticosteroid treatment but myocardial involvement is still the leading cause of death in EGPA.Case Presentation: A 53-year old man with a history of asthma and nasal polyposis presented with acute chest pain and elevated troponin; a percutaneous coronary intervention was performed. The left ventricle was described as hypertrophic. After 20 days the myocardium had markedly increased in thickness of both the right and left ventricle. Evaluation revealed hypereosinophilia in the blood and nasal mucosal tissue, which confirmed the diagnosis of EGPA. He presented with signs of active vasculitis including weight loss, tiredness, intracerebral hemorrhage, and increasing serum creatinine. After 6 days of corticosteroid treatment, the myocardium returned to its initial thickness.Conclusion: Rapid and marked thickening of the myocardium is not frequently reported but may occur in EGPA. Myocardial thickening in EGPA can be quickly reversed by corticosteroids, and is most likely caused by edema. [ABSTRACT FROM AUTHOR]

We investigated the all-inclusive echocardiographic features in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) within 3 months before and after diagnosis. We reviewed the medical records of 89 AAV patients taking echocardiography and 35 age- and gender-matched controls. We collected clinical and laboratory data and echocardiographic results. We compared the variables between patients with AAV of each variant and controls and among those with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). The mean age and period from diagnosis to echocardiography were 54.5 years (30 men) and 1.4 months. The mean age of controls was 51.6 years. AAV patients exhibited lower mean left ventricle ejection fraction (LVEF) (64.0 vs. 69.1%, P = 0.002) and higher mean E/E′ ratio (11.7 vs. 8.8, P = 0.001) and right ventricle systolic pressure (RVSP) (30.2 vs. 23.2 mm Hg, P < 0.001) than the controls. Each variant of AAV presented a different pattern of echocardiographic features in comparison with controls. MPA patients exhibited systolic and diastolic dysfunctions and pulmonary arterial hypertension more often than controls. Meanwhile, GPA patients exhibited only diastolic dysfunction, and EGPA patients had systolic dysfunction and pulmonary arterial hypertension more frequently than controls. No meaningful differences in echocardiographic features appeared among AAV variants. AAV patients exhibited reduced systolic function and advanced diastolic dysfunctions and pulmonary arterial hypertension near the time of diagnosis compared with controls. Each variant of AAV presented a different pattern in echocardiographic features. [ABSTRACT FROM AUTHOR]

Abstract: Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by eosinophilic vasculitis. Patients rarely present without asthma. Cases developing subarachnoid hemorrhage from central nervous system vasculitis are rarely reported. We report a 48‐year‐old woman with rapidly evolving and progressive multi‐system eosinophilic vasculitis in the absence of asthma. Tissue eosinophilia was apparent in a breast lump biopsy. Prior otitis media and prominent lymphoid tissue in the postnasal spaces hinted at otolaryngological disease. She had rapid disease progression with mononeuritis multiplex and eventually succumbed to complications of intracranial hemorrhage secondary to central nervous system vasculitis. This case demonstrates the diagnostic dilemma and treatment considerations in EGPA without asthma. It also raises the question if a reliable biomarker can aid diagnosis in atypical presentations of disease. [ABSTRACT FROM AUTHOR]

Skin lesions are frequent manifestations of underlying systemic conditions, including systemic autoimmune vasculitis. In particular, anti-neutrophil cytoplasmic antibodies (ANCA) are associated with distinct forms of vasculitis characterized by inflammatory cell infiltration of the walls of small and medium-sized vessels leading to vascular destruction and tissue necrosis. ANCA-associated vasculitis is rare and systemic diseases, which can be classified based on different distribution of vascular inflammation and presence or absence of granulomatosis and asthma. Despite their diversities, ANCA-associated vasculitis, namely microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis, can all display a broad variety of cutaneous manifestations, which can appear during the course of the disease or even as first sign at the time of onset. Different skin manifestations might coexist in the same patient and occur in different occasions during the course of the vasculitis. Thus, a deep knowledge of the spectrum of skin lesions as part of ANCA-associated vasculitis is mandatory for a correct diagnostic process, whenever cutaneous vasculitis is suspected. Due to this broad variety of manifestations, the diagnosis of skin involvement in ANCA-associated vasculitis is very challenging and it must be supported by a detailed medical history, accurate physical examination, specific histopathological analysis of skin biopsy and the presence of ANCA serology. In this review, we focus on the cutaneous manifestations that can develop in the context of ANCA-associated vasculitis, detailing the clinical features, the histopathological aspects as well as the direct immunofluorescence studies for each of the three conditions. Moreover, we acknowledged the differential diagnoses that must be ruled out in the diagnostic process and the main therapeutic approaches available for treatment of ANCA-associated vasculitis. [ABSTRACT FROM AUTHOR]

Background: Eosinophilic myocarditis (EM) is an acute life-threatening inflammatory disease of the heart. Neither large case series nor clinical trials on this specific myocarditis have been reported.Objectives: Based on a systematic revision of all published histologically proven cases, this study aimed to describe the clinical presentation, treatment, and outcome of EM.Methods: The study screened 443 manuscripts in MEDLINE and EMBASE on cases of EM published until June 2017. The authors identified 264 patients and included in the main analysis 179 patients admitted to hospital with histologically proven EM.Results: Median age was 41 years (interquartile range: 27 to 53 years) with similar prevalence in both sexes; pediatric cases (≤16 years of age) accounted for 10.1%. The main symptom at presentation was dyspnea (59.4%), with peripheral eosinophilia observed in 75.9%. Median left ventricular ejection fraction at presentation was 35% (interquartile range: 25% to 50%). The disorders most frequently associated with EM were hypersensitivity and eosinophilic granulomatosis with polyangiitis, which accounted for 34.1% and 12.8% of cases, respectively, whereas idiopathic or undefined forms accounted for 35.7% of cases. Steroids were administered in 77.7% of patients. A temporary mechanical circulatory support (n = 30) was instituted in 16.8% of patients. In-hospital death was 22.3% (n = 40), with the highest occurrence in the hypersensitivity form (36.1%; p = 0.026).Conclusions: EM has a poor prognosis during the acute phase, despite a publication bias that could have led to an overestimation of mortality. Associated conditions are identified in approximately 65% of cases. Specific trials and multicenter registries are needed to provide evidence-based treatments to improve in-hospital outcome. [ABSTRACT FROM AUTHOR]

This Special Issue of Autoimmunity Reviews constitutes summaries of presentations at the 20th International Meeting on Immunopathology and Orphan Diseases, held in Torino, Italy, 25–28th January 2017. As such, these presentations represent the state of the art on the pathophysiology of autoimmune diseases as well as the most recent insights into the management of these pathologic conditions. The latter includes both the optimal use of established drugs and approaches as well as novel knowledge on the means and consequences of targeted blocking of molecules or cellular mechanisms. The 2nd Turin Congress on systemic idiopathic vasculitis concluded the works of the International Meeting on Immune Pathology and Orphan Diseases. This Satellite Congress was mainly addressed to the management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitis: advances on induction therapy and maintenance treatment. Guidelines and recommendations were critically discussed, reviewing available evidence and providing experts' insights. New intensive therapeutic approaches had been also reported. [ABSTRACT FROM AUTHOR]

Loeffler’s endocarditis is cardiac damage mediated by hypereosinophilia with a poor prognosis. A 45-year-old woman with Loeffler’s endocarditis caused by eosinophilic granulomatosis with polyangiitis had hypereosinophilia (blood eosinophil count 32.4 × 10 9 /L) and left ventricular systolic and diastolic dysfunction (left ventricular ejection fraction 45%, e’ 5.1 cm/s). Echocardiography showed a left ventricular apical thrombus (32 × 30 mm). The cardiac magnetic resonance imaging scan showed a subacute thrombus (an iso-intensity area on T1-weighted images and a high-intensity area on T2-weighted images). We administered prednisolone and anticoagulant immediately. The eosinophil count rapidly decreased. After 8 weeks, the thrombus gradually decreased in size (28 × 17 mm) and cardiac function improved (left ventricular ejection fraction 58%, e’ 8 cm/s). On the follow-up cardiac magnetic resonance imaging scan, the thrombus gradually became organized (the high-intensity area on T2-weighted images became iso-intense). The subendocardial late gadolinium enhancement showed fibrosis only in a small part of the anterior left ventricular wall. Early steroids and optimal anticoagulant therapy may improve cardiac function and prognosis. Additionally, cardiac magnetic resonance imaging may be an important modality for diagnosis and follow-up. < Learning objective: In patients with Loeffler’s endocarditis, early steroid treatment inhibits progression to the fibrotic stage and improves cardiac function. Anticoagulants decrease the size of the thrombus and also lead to improvement in left ventricular function. Cardiac magnetic resonance imaging can detect the phase of Loeffler’s endocarditis by identifying fibrosis and characteristics of the thrombus. Therefore, cardiac magnetic resonance imaging may be an important modality for diagnosis and follow-up.> [ABSTRACT FROM AUTHOR]

Eosinophils are multifunctional granulocytes that contribute to initiation and modulation of inflammation. Their role in asthma and parasitic infections has long been recognized. Growing evidence now reveals a role for eosinophils in autoimmune diseases. In this review, we summarize the function of eosinophils in inflammatory bowel diseases, neuromyelitis optica, bullous pemphigoid, autoimmune myocarditis, primary biliary cirrhosis, eosinophilic granulomatosis with polyangiitis, and other autoimmune diseases. Clinical studies, eosinophil-targeted therapies, and experimental models have contributed to our understanding of the regulation and function of eosinophils in these diseases. By examining the role of eosinophils in autoimmune diseases of different organs, we can identify common pathogenic mechanisms. These include degranulation of cytotoxic granule proteins, induction of antibody-dependent cell-mediated cytotoxicity, release of proteases degrading extracellular matrix, immune modulation through cytokines, antigen presentation, and prothrombotic functions. The association of eosinophilic diseases with autoimmune diseases is also examined, showing a possible increase in autoimmune diseases in patients with eosinophilic esophagitis, hypereosinophilic syndrome, and non-allergic asthma. Finally, we summarize key future research needs.

Mononeuritis multiplex is a common manifestation of many illnesses which includes Hansen’s disease and certain types of systemic vasculitis. The Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis (AAV) is a group of rare diseases which show typical characteristic inflammatory cell infiltration and blood vessel wall necrosis. AAV syndromes include Granulomatosis with Polyangiitis (GPA), Microscopic Polyangiitis (MPA) and Eosinophilic Granulomatosis with Polyangiitis (EGPA). We describe a patient who presented with mononeuritis multiplex and had features of overlap between EGPA and MPA. The patient was treated with standard regimen of steroids and pulsed cyclophosphamide and she achieved excellent clinical remission.

Eosinophilic granulomatosis with polyangiitis (EGPA) characterized by the coexistence of asthma, eosinophilia and characteristic histopathologic changes, is one of the primary systemic small vessel inflammation of unknown etiology. Cardiac involvement in the course of EGPA occur in 17–92% of patients affecting poor prognosis and acting major cause of mortality in this group of patients. EGPA should always be considered in the differential diagnosis of acute heart failure, especially in young patients with no risk factors for coronary heart disease, and in patients with the presence of asthma, allergic rhinitis or nasal polyps. A rare complication of cardiac involvement is the thrombus formation in the left ventricular apex requiring anticoagulation. We report a case of 49 year old man with cardiac involvement that mimic acute coronary syndrome and led to the diagnosis of EGPA.

Background Although peripheral nervous system involvement is common in eosinophilic granulomatosis with polyangiitis (EGPA), central nervous system (CNS) manifestations are poorly described. This study aimed to describe CNS involvement in EGPA. Patients and methods This retrospective, observational, multicenter study included patients with EGPA and CNS involvement affecting cranial nerves, brain and/or spinal cord. We also undertook a systematic literature review. Results We analyzed 26 personal cases and 62 previously reported cases. At EGPA diagnosis, asthma was noted in 97%, eosinophilia in 98%, peripheral neuropathy in 55% and cardiac involvement in 41%. 38/71 (54%) were ANCA-positive, with a perinuclear-labeling pattern and/or anti-MPO specificity. CNS was involved in 86% at EGPA diagnosis, preceded EGPA in 2%, and occurred during follow-up in 12% after a median of 24 months. Main neurological manifestations were ischemic cerebrovascular lesions in 46 (52%), intracerebral hemorrhage and/or subarachnoid hemorrhage in 21 (24%), loss of visual acuity in 28 (33%) (15 with optic neuritis, 9 with central retinal artery occlusion, 4 with cortical blindness), and cranial nerves palsies in 18 (21%), with 25 patients having ≥ 1 of these clinical CNS manifestations. Among the 81 patients with assessable neurological responses, 43% had complete responses without sequelae, 43% had partial responses with long-term sequelae and 14% refractory disease. After a mean follow-up of 36 months, 11 patients died including 5 from intracerebral hemorrhages. Conclusion EGPA-related CNS manifestations form 4 distinct neurological pictures: ischemic lesions, intracerebral hemorrhages, cranial nerve palsies and loss of visual acuity. Such manifestation should prompt practitioners to consider EGPA in such conditions. Long-term neurological sequelae were common, and intracerebral hemorrhages had the worst prognostic impact. [ABSTRACT FROM AUTHOR]

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), is a small vessel vasculitis associated with eosinophilia and asthma. Clinical manifestations commonly seen in patients presenting with EGPA range from upper airway and lung involvement to neurological, cardiac, cutaneous, and renal manifestations. Treatment for severe presentations includes steroids, cyclophosphamide, plasmapheresis, and recently, rituximab. Rituximab is associated with a good response in the treatment of vasculitis, but a variable response for the control of allergic symptoms. Here, we report a 16-year-old female patient with severe EGPA (gastrointestinal and cutaneous vasculitis, rhinitis and asthma) refractory to conventional treatment. She was treated with rituximab, which enabled rapid control of the vasculitis component of the disease, but there was no response to rhinitis and asthma. Additionally, she developed severe bronchospasm during rituximab infusion. Sequential rituximab and omalizumab were initiated, leading to remission of all manifestations of vasculitis, rhinitis, and asthma, in addition to bronchospasm related to rituximab infusion. [ABSTRACT FROM AUTHOR]

Aim We investigated what variables at diagnosis might be associated with the relapse of eosinophilic granulomatosis with polyangiitis ( EGPA) and whether five factor score ( FFS) at diagnosis might predict it. Methods We reviewed the medical records of 30 patients with EGPA having results of myeloperoxidase ( MPO)-antineutrophil cytoplasmic antibodies ( ANCA) and proteinase 3 ( PR3)- ANCA and having achieved remission during the first therapeutic regimen administration within follow-up duration for at least more than 2 years. Clinical manifestations, the presence of relapse and items for FFS at diagnosis were collected and compared among groups. Results The mean age of patients (18 women) was 50.3 years and the mean follow-up duration was 2175 days. EGPA patients having MPO- ANCA had shown higher frequency of skin involvement and proteinuria over 1 g/day than those having PR3- ANCA and no ANCA. EGPA patients with relapse had more frequent baseline MPO- ANCA and kidney involvement,including proteinuria over 1 g/day and renal insufficiency and higher FFS at diagnosis than those without. However, only FFS showed the predictive significance for relapse (Exp(B) 2.624, P = 0.014) on Cox Hazard model analysis. When the cutoff of FFS for the relapse was set as 1, relapse was identified more frequently in patients with FFS ≥ 1 than those with FFS <1 (68.8% vs. 7.1%, P = 0.001; relative risk 28.6). Conclusion In this study, FFS at diagnosis was associated with relapse and FFS ≥ 1 was the independent predictor of relapse during follow-up for more than 2 years. [ABSTRACT FROM AUTHOR]

Hypereosinophilia-associated syndrome is a rare group of systemic diseases without certain underlying causes. Hypereosinophilic syndrome ( HES) and eosinophilic granulomatosis with polyangiitis ( EGPA), formerly known as Churg-Strauss syndrome ( CSS), are initial considerations, when underlying causes remains unexplained despite of complete evaluation of hypereosinophilia. In this study, we report two rare cases, one case of HES with Loeffler endocarditis, and the other one of EGPA with restrictive cardiomyopathy mimicking myocardial infarction, to further address differential chief cardiac manifestations between HES and EGPA. Key roles of echocardiography played in detection of cardiac involvements, diagnosis, and prognosis prediction are also highlighted. [ABSTRACT FROM AUTHOR]

Résumé Après les rhumatismes inflammatoires chroniques, la granulomatose éosinophilique avec polyangéite (GEPA, Churg-Strauss) illustre parfaitement le bouleversement qui est en train de s’opérer avec l’avènement des biomédicaments dans la prise en charge des vascularites nécrosantes associées aux ANCA. Pour améliorer la prise en charge des patients, ces médicaments biologiques devront montrer qu’ils permettent des rémissions prolongées, une épargne cortisonique, une réduction de la toxicité médicamenteuse et des séquelles cumulatives, une amélioration de la qualité de vie des patients avec un impact médico-économique acceptable. L’utilisation du rituximab dans le traitement de la GEPA reste actuellement non validée mais de larges essais thérapeutiques sont en cours en France, en traitement d’induction mais aussi de maintien de la rémission. Ils sont rendus possibles grâce à l’émergence de définition consensuelle de critères d’évaluation de cette vascularite et la structuration de réseaux d’investigateurs comme celui du Groupe français d’étude des vascularites (GFEV). L’interleukine-5 est une autre cible thérapeutique prometteuse au cours de la GEPA, dont le blocage fait l’objet d’un essai thérapeutique international avec le mépolizumab en traitement d’entretien, après son autorisation de mise sur le marché dans l’asthme éosinophilique. Les résultats préliminaires de cette étude sont encourageants. D’autres essais thérapeutiques testeront de nouvelles cibles dans cette maladie rare, comme l’interleukine-4 et -13. Gageons que cette nouvelle ère des médicaments biologiques au cours de la GEPA permette de voir émerger de nouvelles thérapeutiques qui amélioreront la qualité de vie des patients. After the inflammatory rheumatic diseases, eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss) perfectly illustrates the breakthrough of the advent of biologics in the management of ANCA-associated vasculitides. To improve the management of patients, biologics will have to demonstrate that they allow sustained remission, steroid sparing, and reduction of side effects and accrual damage, improvement of the quality of life of patients with an acceptable medico-economic ratio. Anti-CD20 rituximab treatment of EGPA remains at present not validated but large randomized trials are ongoing in France, both as induction and maintenance therapy. They are made possible thanks to the emergence of consensual definition of assessment criteria for this vasculitis and the structuring of investigators’ networks such as the French Vasculitis Study Group (FVSG). Interleukine-5 is another promising therapeutic target in EGPA, the blocking of which is the objective of an international pivotal mepolizumab trial, after its marketing authorization in eosinophilic asthma. Preliminary results are encouraging as maintenance therapy. Further trials will evaluate other targets in this rare disease, such as interleukin-4 and -13. Let us hope that this new era of biological response modifiers for EGPA allow the emergence of new therapies, which will improve patients’ quality of life. [ABSTRACT FROM AUTHOR]

A 44-year-old woman had a 15-year history of asthma and had recently been treated for sinusitis and unidentified limb eruption. She had presented at a nearby clinic with inspiratory chest pain one week before hospitalization, and was diagnosed as having eosinophilic pneumonia based on peripheral blood eosinophilia and ground glass opacities in the right lung field, without pericardial effusion, as detected by chest computed tomography. She additionally presented with a feeling of chest tightness, and extensive pericardial effusion appeared within a week. She developed heart failure on admission, and we performed pericardiocentesis. We gave a clinical diagnosis of acute probable myopericarditis as the cause of pericardial effusion based on pleuritic chest pain, pericardial effusion, and elevation of cardiac enzymes, as well as eosinophilic granulomatosis with polyangiitis (EGPA) based on eosinophilia, her history of sinusitis, asthma, and migratory pulmonary opacities. We initiated oral prednisone 25 mg daily and pericardial effusion disappeared. In patients with EGPA, cardiac involvement is more serious than the involvement of other organs, and is associated with a poor prognosis. In this report we describe a rare case of EGPA complicated with progressive pericardial effusion and discuss the importance of the early diagnosis and treatment of EGPA. < Learning objective: Eosinophilic granulomatosis with polyangiitis (EGPA), or Churg Strauss syndrome, is a multisystem disorder. It is important for patients with EGPA complicated with cardiac involvement to be diagnosed and treated early because cardiac involvement may lead to a fatal outcome.> [ABSTRACT FROM AUTHOR]

Eosinophils are multifunctional granulocytes that contribute to initiation and modulation of inflammation. Their role in asthma and parasitic infections has long been recognized. Growing evidence now reveals a role for eosinophils in autoimmune diseases. In this review, we summarize the function of eosinophils in inflammatory bowel diseases, neuromyelitis optica, bullous pemphigoid, autoimmune myocarditis, primary biliary cirrhosis, eosinophilic granulomatosis with polyangiitis, and other autoimmune diseases. Clinical studies, eosinophil-targeted therapies, and experimental models have contributed to our understanding of the regulation and function of eosinophils in these diseases. By examining the role of eosinophils in autoimmune diseases of different organs, we can identify common pathogenic mechanisms. These include degranulation of cytotoxic granule proteins, induction of antibody-dependent cell-mediated cytotoxicity, release of proteases degrading extracellular matrix, immune modulation through cytokines, antigen presentation, and prothrombotic functions. The association of eosinophilic diseases with autoimmune diseases is also examined, showing a possible increase in autoimmune diseases in patients with eosinophilic esophagitis, hypereosinophilic syndrome, and non-allergic asthma. Finally, we summarize key future research needs. [ABSTRACT FROM AUTHOR]