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Although sporadic case reports have demonstrated successful management of eosinophilic granulomatosis with polyangiitis (EGPA) by anti-IL-5 therapy, larger-scale monocentric studies for the efficacy of mepolizumab (MEP), an IL-5 monoclonal antibody, are still lacking in Taiwan. Hospitalized EGPA patients aged at least 18 years were enrolled from November 1998 to October 2023, and analyzed for demographic, clinical, laboratory, medication and outcome data, focusing on the efficacy and safety of biologics use, particularly induction therapy with MEP. Twenty-seven EGPA patients aged 10–70 years (43 ± 15) at disease diagnosis were recruited with 21 under combined corticosteroids/cyclophosphamide induction therapy. Seventeen patients received biologics with 13 under MEP therapy. Ten patients aged 19–71 years (48 ± 15) completed 12-month induction therapy with a 100 mg quadri-weekly subcutaneous injection regimen indicated for active or relapse disease. There were reduced BVAS with complete remission in 6 and partial remission in 4 patients, lower CRP levels, decreased eosinophil counts with an inhibition of 92∼96 %, and tapered prednisolone dosages from 5 to 25 (13.0 ± 6.3) to 0–10 (3.3 ± 3.1) mg/day. Only one patient had an adverse event of injection site reactions. Nine patients received the same regimen for annual maintenance therapy. All had a persistent clinical remission. In these patients, 13–56 injections (41 ± 15) were prescribed with a follow-up period of 12∼52 months (38 ± 14). In this retrospective study, induction therapy with a 12-month 100 mg MEP quadri-weekly subcutaneous injection regimen demonstrates the efficacy and safety for active and relapsing EGPA patients. [ABSTRACT FROM AUTHOR]

Cytomegalovirus (CMV) infection is a common and typically benign disease in immunocompetent individuals. However, immunocompromised patients are at a greater risk of reactivation, leading to more severe outcomes. Patients with rheumatic diseases have a particularly high risk of opportunistic infections due to both the inherent immunosuppressive state conveyed by the disease itself and the use of immunosuppressors, although varying in the type of drug, dosage and time of exposure. Limited data are available regarding prophylactic or preemptive treatment of CMV infection in patients with rheumatic diseases. In this article the authors present two cases of rheumatic conditions complicated by CMV infection. The first case describes a patient with eosinophilic granulomatosis with polyangiitis, previously treated with glucocorticoids and cyclophosphamide, who developed CMV colitis with bowel perforation. The second case involves a woman with systemic lupus erythematosus who was diagnosed with CMV meningitis. Both cases reinforce the importance of establishing guidelines for surveillance and prophylaxis of CMV infection in these patients. [ABSTRACT FROM AUTHOR]

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare vasculitis of small and medium sized blood vessels. Case Description: Thirteen-year-old male, with history of rhinitis and asthma, who presented to the emergency department with one week of asthenia, arthralgias and myalgias and two days of fever. A diffuse petechial rash, palpable purpura and polyarthritis were detected on examination. Leukocytosis (34 990/μL) with eosinophilia (66%) and elevated C-reactive protein were identified. The patient was admitted and ceftriaxone and doxycycline were started. The clinical status deteriorated in the following days. The patient developed myopericarditis, bilateral pulmonary infiltrates and pleural effusion, requiring mechanical ventilation and aminergic support. Non-clonal eosinophils were detected on the bone marrow aspiration and the skin biopsy showed leukocytoclastic vasculitis with eosinophils. Antineutrophil cytoplasmic antibodies and genetic analysis for hypereosinophilic syndrome mutations were negative. After treatment with methylprednisolone for three days a fast clinical, laboratory and radiological improvement occurred. The patient started azathioprine and reduced steroids progressively. No relapses occurred since diagnosis five years ago. Discussion: Clinical suspicion and early treatment of EGPA are crucial to improve prognosis. [ABSTRACT FROM AUTHOR]

This review aims to describe the epidemiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of eosinophilic granulomatosis with polyangiitis (EGPA). Eosinophilic granulomatosis with polyangiitis is a small to medium vessel necrotizing vasculitis, typically classified with granulomatosis with polyangiitis (GPA) and microscopic polyangitis (MPA) as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). However, less than 50% of patients with EGPA have a positive ANCA test. Among all the vasculitides, asthma and eosinophilia are unique features of EGPA. Eosinophilic granulomatosis with polyangiitis is very rare and the diagnosis may be missed as the disease evolves over time. Polyneuropathies are common and may be severe, requiring aggressive immunosuppressive therapy. Heart involvement is the most common cause of death in EGPA. Biopsy of involved tissue supports a clinically suspected diagnosis but is not always feasible. Treatment of EGPA is primarily dictated by the severity of disease and prognostic factors. More severe disease frequently requires the use of aggressive therapy such as cyclophosphamide. Once treatment is initiated, patients can achieve good control of symptoms; unfortunately, disease relapses are common and prolonged treatment with corticosteroids is often necessary for asthma management. A better understanding of the disease heterogeneity is needed for the development of better therapies. [ABSTRACT FROM AUTHOR]

Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) compromise a rare group of necrotizing small to medium vessel vasculitides that constitute three distinct disorders: granulomatosis with polyangiitis (GPA) (formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) (formerly known as Churg-Strauss syndrome). AAV is characterized by the usual presence of circulating autoantibodies to the neutrophil proteins leukocyte proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA). These antibodies can activate neutrophils and the complement system resulting in vessel wall inflammation and damage. The clinical presentation of AAV varies from non-severe (non-life threatening) to severe often with potentially life-threatening multi-organ involvement. Early recognition and diagnosis are crucial. In the past two decades, advances in understanding the pathophysiology of AAV have led to development of new treatments and resulted in significant improvement in general outcomes and survival rates. This narrative review will focus on GPA and MPA. We will highlight clinical manifestations, diagnosis, disease monitoring, and treatment strategies in patients with AAV. [ABSTRACT FROM AUTHOR]

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare autoimmune disease that can affect multiple organ systems in the body. A majority of patients with EGPA present with asthma-like symptoms and may be misdiagnosed with refractory asthma. It is necessary to distinguish EGPA from asthma and provide a theoretical basis for effective future prevention and treatment. Objective: This study aimed to compare the clinical features of EGPA and the clinical features of allergic asthma in an effort to make an early diagnosis possible. Methods: We reviewed the basic information, test results, pre-onset conditions, and prognosis of 44 adult patients with EGPA who were admitted to our hospital between January 2013 and June 2021, and conducted a 1:1 matched case-control study to compare patients with EGPA and patients with allergic asthma. Results: The 44 patients with EGPA were older than those with allergic asthma, but more than half of the patients with EGPA had been diagnosed with bronchial asthma, with a history of 10 months to 40 years, and had previously used inhalers or systemic steroids. The proportion of male-to-female cases was ~1:1, with seven antineutrophil cytoplasmic antibodies (ANCA) positive cases (15.9%), 20 limited EGPA cases (45.45%), and 24 systemic EGPA cases (54.55%). Although the peripheral blood eosinophil count and percentage were lower in the male patients than in the female patients, male patients with higher five-factor scores might indicate worse prognosis. The fractional exhaled nitric oxide (FeNO) level, eosinophil percentage and count, and total immunoglobulin E (IgE) level were higher in the EGPA group than in the allergic asthma group. Unlike in allergic asthma, the FeNO level is not correlated with the blood eosinophil count or percentage in EGPA. Seven patients received cardiac emission computed tomography (ECT) tests, with abnormalities suggested in six patients. Results of an electrocardiogram, color-Doppler echocardiography, myocardial enzyme level, and troponin level suggested no obvious abnormality. Conclusion: The proportion of patients with EGPA who tested positive for ANCA is not high, and patients with high eosinophil counts should be alert to the possibility of having EGPA. For patients with infiltration of eosinophils into the airway, a diagnosis should not be based on peripheral blood eosinophil counts. It is recommended that the FeNO level and pulmonary function should also be monitored for patients who present with symptoms in other body systems. The sensitivity of cardiac ECT tests is higher than routine tests, so timely screening by cardiac ECT is recommended for all patients with EGPA. [ABSTRACT FROM AUTHOR]

We present the case of a 61-year-old man who reported diplopia due to a right abducens nerve palsy. The patient complained of fever every night (37.5° C), paresthesia of the second and third hand fingers, and he showed an increased C-reactive protein, high erythrocyte sedimentation rate, and high eosinophilia. He had a history of allergic asthma, chronic rhinosinusitis, and surgically treated nasal polyps. His past medical history and labs led us to identify the eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome. EGPA is a potentially life-threatening condition, and a proper diagnosis was critical to managing this patient's abducens nerve palsy. [ABSTRACT FROM AUTHOR]

Background: Eosinophils have a double-edged role in the human body, being essential in important physiologic functions but whose presence is conspicuous in a variety of diseases characterized by a T2 inflammation phenotype. Eosinophils are exquisitely sensitive to corticosteroids, and the latter have, until recently, represented the cornerstone of treatment of eosinophilic diseases. However, most patients remain dependent on oral corticosteroids, with a notable adverse effect burden and experience a chronic relapsing disease that leads to high morbidity and mortality. Treatment prospects have changed with the advent of biologic drugs that target the eosinotropic cytokine interleukin (IL) 5 or its receptor. The success of the latter drugs in severe eosinophilic asthma has paved the way for their use in other, rarer, eosinophilic lung diseases. Recently, mepolizumab, a humanized monoclonal anti-body that works against IL-5, was approved for the add-on treatment of relapsing-remitting or refractory eosinophilic granulomatosis with polyangiitis (EGPA) in patients ages ( 6 years. Benralizumab, a humanized antibody that binds to the a portion of the IL-5 receptor, is also being tested for its efficacy in EGPA in two clinical trials, after a growing number of case reports and case series supported its use as a steroid-sparing agent in the treatment of EGPA. Methods: In this review, we summarized the scientific literature evaluating the efficacy of benralizumab treatment in patients afflicted with rare primary eosinophilic lung diseases. Results: The literature we found, largely case reports, reported that the use of benralizumab in EGPA, chronic eosinophilic pneumonia (CEP) and allergic bronchopulmonary aspergillosis (ABPA) often led to a depletion of eosinophils, less exacerba)tions and a decreased systemic corticosteroid burden. No adverse effects were reported. Conclusion: Benralizumab has a prospective role in the treatment of rare eosinophilic lung diseases, which needs to be fur)ther elucidated in randomized controlled trials. [ABSTRACT FROM AUTHOR]

The antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides are a group of small-vessel vasculitides that results in inflammation of the small blood vessels. It has a diverse range of clinical manifestations that commonly involve not only the upperairways, lungs, and kidneys but also the eyes, skin, joints, nerves, and, potentially, many other sites. This diversity is why ANCA-associated vasculitides can have such a wide scope of clinical presentations and disease severity. Entities in this group of diseases include granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly known as Churg--Strauss syndrome). Skin involvement isacommonclinical feature in all three forms of ANCA-associated vasculitis, with various manifestations. [ABSTRACT FROM AUTHOR]

A 57-year-old man was admitted to our hospital due to repeated chest pain. Coronary spastic angina was diagnosed by emergent coronary angiography. His chest attack was not suppressed with vasodilator therapy; however, it finally improved after administration of 20 mg prednisolone. His symptoms were controlled and elevation of the eosinophil count was normalized, even after tapering the dosage. His episodes of asthma, hypereosinophilia, mononeuropathy, and pulmonary infiltrate led to a diagnosis of eosinophilic granulomatosis with polyangiitis. < Learning objective: Coronary spastic angina (CSA) is rarely observed as cardiac involvement in patients with eosinophilic granulomatosis with polyangiitis (EGPA). Although previous reports have suggested that coronary vasospasm is resistant to standard therapy with vasodilator drugs, no appropriate treatment protocol has been established. This is the first case of CSA with EGPA successfully treated with a low-dose corticosteroid.> [ABSTRACT FROM AUTHOR]

Eosinophilic granulomatosis with polyangiitis (EGPA) is one form of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Identical to what has been called Churg-Strauss syndrome, EGPA exhibits both allergic and vasculitis features. EGPA was first described as a syndrome consisting of asthma, fever, eosinophilia, and organ involvement including heart failure, neuropathy, and kidney damage, by Churg and Strauss in 1951. On the basis of the 2012 Chapel Hill Consensus Conferences Nomenclature of Vasculitis, EGPA comprises three typical allergic components, including asthma, peripheral eosinophilia, and eosinophil-rich granuloma of the respiratory tracts. EGPA has three clinical and histological stages. The first is an allergic stage composed of asthma and sinusitis, and the second is an eosinophilic stage characterised by peripheral hypereosinophilia and intra-organ infiltration of eosinophils. The last is a vasculitic stage, including necrotising inflammation of small vessels and end-organ damage. In this review, we describe the classification criteria for EGPA and recommendations for the evaluation and management of EGPA with conventional and newly suggested drugs for EGPA. Also, we discuss a variety of clinical aspects such as predictive values for prognosis and associations with other Th2-mediated diseases and hepatitis B virus. [ABSTRACT FROM AUTHOR]

Background: An example of aggressive eosinophilic polyposis can be found in eosinophilic granulomatosis with polyangiitis (EGPA). Intercellular adhesion molecule-1 (iCAM-1) and vascular cell adhesion molecule-1 (vCAM-1) play a part in mediating the recruitment and adhesion of leukocytes to the vessel wall, and their blood-to-tissue migration under inflammatory conditions. Objective: This prospective study compared 3 groups—patients with a definite diagnosis EGPA, non-EGPA patients with phenotypic features suggestive of EGPA, and patients with non-eosinophilic nasal polyposis (controls)—in terms of nasal tissue histology, iCAM-1 and vCAM-1 expression, and blood inflammatory cells. Methods: A total of 58 adults underwent sinus surgery (13 patients with EGPA, 23 suspected of having EGPA, and 22 controls). Results: Mean tissue eosinophil counts were significantly higher in EGPA patients and suspected cases of EGPA than in controls. Although iCAM-1 and vCAM-1 were diffusely expressed in sinonasal tissues, they did not differently stain EGPA, eosinophilic-type and non-eosinophilic polyposis. Blood basophil and eosinophil levels were high in both the EGPA and the suspected EGPA groups. Intergroup differences were found for eosinophils but not for basophils. Conclusions: We do not have yet blood or tissue markers able to differentiate the early phase of EGPA from chronic rhinosinusitis with nasal polyps. Further investigations are mandatory considering EGPA patients at their initial diagnosis and before any treatment, in terms of nasal histology and blood inflammatory cells, to identify markers characterizing sinonasal mucosa inflammation and useful for an early diagnosis of EGPA. [ABSTRACT FROM AUTHOR]

In a recent phase III trial (NCT02020889) 53% of mepolizumab-treated versus 19% of placebo-treated patients with eosinophilic granulomatosis with polyangiitis (EGPA) achieved protocol-defined remission. We sought to investigate post hoc the clinical benefit of mepolizumab in patients with EGPA using a comprehensive definition of benefit encompassing remission, oral glucocorticoid (OGC) dose reduction, and EGPA relapses. The randomized, placebo-controlled, double-blind, parallel-group trial recruited patients with relapsing/refractory EGPA receiving stable OGCs (prednisolone/prednisone, ≥7.5–50 mg/d) for 4 or more weeks. Patients received 300 mg of subcutaneous mepolizumab or placebo every 4 weeks for 52 weeks. Clinical benefit was defined post hoc as follows: remission at any time (2 definitions used), 50% or greater OGC dose reduction during weeks 48 to 52, or no EGPA relapses. The 2 remission definitions were Birmingham Vasculitis Activity Score of 0 plus OGC dose of 4 mg/d or less (remission 1/clinical benefit 1) or 7.5 mg/d or less (remission 2/clinical benefit 2). Clinical benefit was assessed in all patients and among subgroups with a baseline blood eosinophil count of less than 150 cells/μL, baseline OGC dosage of greater than 20 mg/d, or weight of greater than 85 kg. With mepolizumab versus placebo, 78% versus 32% of patients experienced clinical benefit 1, and 87% versus 53% of patients experienced clinical benefit 2 (both P <.001). Significantly more patients experienced clinical benefit 1 with mepolizumab versus placebo in the blood eosinophil count less than 150 cells/μL subgroup (72% vs 43%, P =.033) and weight greater than 85 kg subgroup (68% vs 23%, P =.005); in the OGC greater than 20 mg/d subgroup, results were not significant but favored mepolizumab (60% vs 36%, P =.395). When a comprehensive definition of clinical benefit was applied to data from a randomized controlled trial, 78% to 87% of patients with EGPA experienced benefit with mepolizumab. [ABSTRACT FROM AUTHOR]

The following topics will be included in this issue: Positive ANCA assay and diagnosis and treatment of ANCA-associated vasculitis Large vessel vasculitis Takayasu's arteritis EPGA vasculitis Behcet's disease Polyarteritis nodosa vasculitis Leukocytoclastic cutaneous vasculitis Declaration of interest The contents of the paper and the opinions expressed within are those of the authors, and it was the decision of the authors to submit the manuscript for publication. Lastly, among vasculidities associated with either systemic disease or probably etiology include lupus vasculitis, rheumatoid vasculitis, sarcoid vasculitis and hepatitis C virus-associated vasculitis, hepatitis B virus-associated vasculitis, syphilis-associated vasculitis, drug-associated immune complex vasculitis, drug-associated ANCA-positive vasculitis, and cancer-associated vasculitis; many of which may be touched upon in various subsections of this issue. Newer nomenclature for other vasculidities include variable vessel vasculitis, single-organ vasculitis, vasculitis associated with systemic disease, and vasculitis associated with probable etiology. [Extracted from the article]

Eosinophilic granulomatosis with polyangiitis is a rare multisystemic disorder, characterized by necrotizing vasculitis affecting small to medium-sized vessels, associated with asthma and eosinophilia. Cardiac involvement is the most important predictor of mortality and it seems to be more frequent in anti-neutrophil cytoplasmic antibodies-negative patients. Cardiomyo - pathy and congestive heart failure can occur but a signif icant proportion of patients are asymptomatic. We present a case of this condition in a 65-year-old wo man with a past medical history of rhinosinusitis and recent episodes of asthma, that developed palpable purpura, sensory deficiency and excruciating pain mainly in the lower limbs. A significant hypereosinophilia and elevated troponin level were found, although she had not cardiac symptomatology. Cardiovascular magnetic re sonance revealed late gadolinium enhancement and a severe reduction of the left ventricular ejection fraction. Mononeuritis multiplex was documented and diagnosis was confirmed by biopsy. Complementary cardiac investigation is mandatory in any patient with suspicion of eosinophilic granulomatosis with polyangii - tis. Early detection and the appropriate treatment are crucial due to the possible life-threatening manifestations. [ABSTRACT FROM AUTHOR]

Introduction Eosinophilic mastitis caused by eosinophil infiltration of the mammary gland is very rare. To date, no report has been published on treating patients with this disorder using anti-allergic drugs. Steroids are commonly used in these cases, but have greater burden. Presentation of case A 33-year-old woman presented to the author’s clinic with a tumor and pain in the upper inner quadrant of the left breast. She underwent core needle biopsy (CNB) and was diagnosed with eosinophilic mastitis based on histopathological analysis. The serum eosinophil count at the time of biopsy increased to 1560/μL. She was administered 100 mg of suplatast tosilate (brand name: IPD capsule 100), an anti-allergic drug, 3 times daily after each meal. Thereafter, the patient’s symptoms improved and her serum eosinophil count returned to normal after 4 months. To date, the patient has been recurrence-free for 3 years since the first presentation. Discussion Organ damage induced by eosinophil infiltration was limited to the mammary gland and improved with anti-allergic drug administration. Conclusion This report presents the successful treatment of an isolated case of eosinophilic mastitis solely using anti-allergic drugs. [ABSTRACT FROM AUTHOR]

Oral ulcerations are an extremely rare manifestation of Churg-Strauss Syndrome (CSS), which have not yet been documented as a potential prodromal sign of this multisystemic autoimmune vasculitis which may also involve the kidneys. The authors present a very rare case report of widespread oral ulcerations which preceded the onset of CSS with crescentic glomerulonephritis and persisted throughout the course of the disease. Oral manifestations included atypical clusters of oral aphthae and other lingual and mucosal ulcerations. The case demonstrates the disease spectrum and review of current understanding of this disease. The dentist may be the first health care professional to see patients with symptoms and findings of this condition. Definitive diagnosis is challenging owing to the subtle onset of the disease and variable clinical and laboratory findings. Clinicians should be informed about the possibility of oral manifestations of CSS to facilitate prompt disease recognition and to provide continued oral health care to these medically complex patients. Early diagnosis and treatment is the most important factor in the management of this potentially fatal disease as well as to promote quicker remission. [ABSTRACT FROM AUTHOR]

Life-threatening arrhythmias are often found in heart diseases, but they are rare as clinical symptoms of Churg-Strauss syndrome. We report a case of a 66-year-old woman with symptomatic monomorphic ventricular tachycardia as the first sign of Churg-Strauss syndrome. Cardiac manifestations were the main clinical symptoms of the disease, and changes in other organs were weakly expressed. Furthermore, increased serum IgG4 level was revealed. It was the reason for the differential diagnosis with IgG4-related diseases. Echocardiography, cardiac magnetic resonance imaging, and histopathological analysis of biopsies had an important role in diagnosis. < Learning objective: Ventricular arrhythmias are rare clinical symptoms of Churg-Strauss syndrome. This case is interesting because cardiac manifestations were the main clinical symptom of the disease, and changes in other organs were weakly expressed. Echocardiography, cardiac magnetic resonance imaging, and histopathological analysis of biopsies had an important role in diagnosis. Increased serum IgG4 level was the reason for the differential diagnosis with IgG4-related diseases. Churg-Strauss syndrome should be considered as part of the differential diagnosis in patients with noncoronary ventricular arrhythmias.> [ABSTRACT FROM AUTHOR]

Churg–Strauss syndrome is a necrotizing vasculitis of small vessels characterized by upper and lower airway disease followed by peripheral eosinophilia and multiple organ involvement. Herein we present the case of a 45-year-old female patient with Churg–Strauss syndrome and myopericardial disease who improved upon cyclophosphamide treatment. Apart from discussing the characteristics of myopericardial disease in eosinophilic syndromes, we highlight the crucial role of cardiac imaging in the prompt recognition and management of such patients. < Learning objective: Churg–Strauss syndrome is a vasculitic disorder characterized by massive hypereosinophilia and multi-organ disease. Myocardial involvement may manifest as myopericarditis, valvular dysfunction, myocardial infarction, or left ventricular thrombi or alternatively may only cause subclinical changes difficult to diagnose with conventional echocardiography. Cardiac magnetic resonance imaging is the gold-standard for the detection of active inflammation and fibrosis that may characterize myocardial disease. This is of major importance since timely diagnosis may enable the establishment of appropriate treatments and arrhythmia screening.> [ABSTRACT FROM AUTHOR]

We describe a patient with severe multiple mononeuropathy associated with hypereosinophilia, asthma and pulmonary non cavitating micronodules. Sural nerve biopsy revealed marked perineural thickening and microfasciculation with inflammatory infiltrates in the perinerium and in the epinerium. The patient markedly improved with steroid therapy. Our final diagnosis was Churg-Strauss related multiple mononeuropathy. Thus, we report a case of Churg-Strauss related multiple mononeuropathy with uncommon pathological findings on sural nerve and we underline the importance of clinical evaluation for this diagnosis. [ABSTRACT FROM AUTHOR]

Nasal polyps occur in 1-4% of the population, usually occurring in the setting of an underlying local or systemic disease. The most common associated condition is chronic rhinosinusitis (CRS). A high prevalence of nasal polyps is also seen in allergic fungal rhinosinusitis, aspirin-exacerbated respiratory disease, Churg-Strauss syndrome, and cystic fibrosis. In the setting of CRS, nasal polyps are not likely to be cured by either medical or surgical therapy; however, control is generally attainable. The best medical evidence supports the use of intranasal corticosteroids for maintenance therapy and short courses of oral corticosteroids for exacerbations. The evidence for short- and long-term antibiotics is much less robust. For patients with symptomatic nasal polyposis nonresponsive to medical therapies, functional endoscopic sinus surgery provides an adjunctive therapeutic option. [ABSTRACT FROM AUTHOR]

Objective: To determine the frequencies and types of ophthalmologic manifestations in patients with systemic necrotizing vasculitides (SNV), including polyarteritis nodosa (PAN) and ANCA-associated vasculitides (granulomatosis with polyangiitis (Wegener''s, GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA); Churg–Strauss syndrome (CSS)) and review the literature on eye involvement in these diseases. Methods: This retrospective analysis was conducted on the ophthalmologic manifestations of SNV patients entered into the French Vasculitis Study Group database between July 1955 and August 2008. Results: Among the 1286 identified patients, 214 (16.6%) had ophthalmologic manifestations at diagnosis, significantly more often in GPA (117/343, 34.1%) than in EGPA (30/270, 11.1%; P = 0.0001), PAN (42/393, 10.7%; P = 0.0001) or MPA (25/280, 8.9%; P = 0.0001). The 3 most common recorded ophthalmologic manifestations were conjunctivitis (89, (7%)), episcleritis (56, (4%)), and/or blurred vision (44, (3%)), mainly caused by retinal vasculitis in 5, oculomotor nerve palsy in 4, uveitis in 4 and/or optic neuropathy in 3. Orbital inflammatory tumor, another common feature was rather specific to GPA (23/349, 6.6% (P = 0.0001)) compared to other SNV. The literature on ophthalmologic manifestations of SNV is limited to case reports except for GPA, in which the eye involvement frequency ranged from 29% to 57%. Conclusions: Eye manifestations were more common in GPA than MPA, PAN and EGPA, but can be sight-threatening in any SNV. Given the heterogeneity of ophthalmologic involvement in SNV, close collaboration between the ophthalmologists and internists is critical. [Copyright &y& Elsevier]

Copyright of Revue du Rhumatisme Monographies is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Revista FAMECOS - Mídia, Cultura e Tecnologia is the property of EDIPUCRS - Editora Universitaria da PUCRS and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Anti-neutrophil cytoplasmic antibody associated vasculitis is part of a multi-systemic idiopathic, small vessel pouci-immune vasculitis. Given the heterogeneous spectrum of the disease, and the need to update therapeutic protocols, the aim of this review was to evaluate clinical-diagnostic approaches. We examined statistical data available in the literature, in particular the 2010 review of St. Hamour et al. Management of Anca-associated Vasculitis, published in Therapeutics and Clinical Risk Management. Acute immunosuppressive therapy and long-term maintenance, with the use of prednisolone, have significantly changed the prognosis of this disease, particularly compared with the 1970s before the introductions of steroids and cyclophosphamide. New drugs such as rituximab, monoclonal antibodies and other modulating immune system molecules are entering clinical use, and experience will confirm whether or not therapeutic guidelines are appropriate. The current diagnostic tools, ranging from laboratory and autoimmune tests, chest X-ray, broncho-alveolar lavage to capillaroscopy, allow prompt diagnosis and early treatment through a first phase of induction-remission, and a second phase of maintenance. There are, however, recurrent and refractory forms of the disease that require long-term immunosuppression and further research into this is merited. These issues have continued to drive the search for safer and more effective modulation of the immune system using targeted immunotherapy. However, the treatment limitations of incomplete efficacy, infection, and cumulative toxicity persist. Modifications to traditional treatment protocols by the use of azathioprine or methotrexate rather than cyclophosphamide, and the introduction of newer agents, such as rituximab, have meant that outcomes have been maintained while toxicity has been reduced. [ABSTRACT FROM AUTHOR]

In addition to breast cancer and other conditions limited to the breast, mammograms sometimes reveal signs of systemic disease. These signs include breast arterial calcifications, axillary lymphadenopathy, dilated veins in the breast, breast edema and skin thickening, and masses not associated with cancer or a benign breast condition. This article discusses the indicators of systemic disease seen on mammograms and some of the many diseases they may signify. Several case studies are presented. [ABSTRACT FROM AUTHOR]

Abstract: Churg–Strauss syndrome is an uncommon multisystem disorder characterized by asthma, eosinophilia and vasculitis. We report on a 12-year-old boy with asthma and deterioration of his general condition, who was eventually diagnosed with an ANCA-negative Churg–Strauss syndrome. The propositus included, 50 cases of childhood Churg–Strauss syndrome have been reported. The patient characteristics and clinical characteristics of these children are summarized. The respiratory tract is most frequently involved with pulmonary infiltrates, asthma and sinusitis. Early recognition of childhood Churg–Strauss syndrome is important as delayed diagnosis can lead to severe organ involvement, and possible fatal outcome. [Copyright &y& Elsevier]

Asthma is often triggered by allergic and nonallergic factors in atopic individuals and readily responds to anti-inflammatory and bronchodilator therapy. The differential diagnosis for poorly responsive disease includes severe persistent asthma with associated allergic rhinitis, cardiac disorders such as left ventricular failure or mitral stenosis, vocal cord dysfunction, gastroesophageal reflux disease, recurrent aspiration, chronic obstructive pulmonary disease, emphysema, alpha-1-antitrypsin deficiency, sarcoidosis, hypersensitivity pneumonitis, bronchiectasis, allergic bronchopulmonary aspergillosis, airway neoplasm, and Churg-Strauss vasculitis. A careful history and physical in conjunction with appropriate screening of laboratory information will usually direct the clinician to the correct diagnosis. [ABSTRACT FROM AUTHOR]

Children with rheumatic disorders may have a wide variety of clinical features ranging from fever or a simple arthritis to complex multisystem autoimmune diseases. Information about the prevalence of neurological manifestations in children with rheumatologic disorders is limited. This review describes the neurologic complications of childhood Rheumatic disease either solely or combined with symptoms of other organs involvement, as a primary manifestation or as a part of other symptoms, additionally. [ABSTRACT FROM AUTHOR]

Background: Eosinophils are blood cells that are often found in high numbers in the tissues of allergic conditions and helminthic parasite infections. The pathophysiologic roles that eosinophils may serve in other human “eosinophil-associated” diseases remain obscure. Objective: National Institutes of Health (NIH) Institutes and the Office of Disease Prevention assembled an international taskforce of clinical and basic scientists with the charge to propose and prioritize unmet research needs in eosinophil-associated diseases. Methods: The taskforce used an organ system approach to identify the different and common themes of eosinophil cell involvement in these diseases. In early 2012, a draft document was circulated for review. The document was amended and the prioritizations were set at a NIH-organized workshop in June 2012. Results: The taskforce identified significant research needs. These needs cross disease entities but some are disease specific. There are substantial shortcomings to the various preclinical animal models, as well as significant gaps in our epidemiologic, pathophysiologic, diagnostic, prognostic, and therapeutic knowledge. The taskforce recognized that recent efforts by patient advocacy groups have played instrumental roles in improving the identification and characterization of these disorders. However, communications among the eosinophil-interested communities, for example, governmental funding and regulatory agencies, and industry and clinician scientists need to be more comprehensive. Conclusions: Significant efforts are required to address our knowledge gaps to improve the outcomes of eosinophil-associated diseases. NIH Institutes, other federal agencies, lay organizations, and the pharmaceutical industry should consider the taskforce''s recommendations in their future research activities. [Copyright &y& Elsevier]

Hypereosinophilic syndromes (HESs) are a diverse group of conditions characterized by clinical manifestations attributable to eosinophilia and eosinophilic infiltration of tissues. HESs are chronic disorders with significant morbidity and mortality. Although the availability of targeted chemotherapeutic agents, including imatinib, has improved quality of life and survival in some patients with HESs, additional agents with increased efficacy and decreased toxicity are sorely needed. The purpose of this review is to provide an overview of eosinophil biology with an emphasis on potential targets of pharmacotherapy and to provide a summary of potential eosinophil-targeting agents, including those in development, in clinical trials, or approved for other disorders. [Copyright &y& Elsevier]

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The article describes the case of Churg-Strauss syndrome in a patient who developed bilateral central retinal artery occlusion simultaneously. Ophthalmoscopic and angiographic analysis showed a central retinal artery occlusion with choroidal circulatory disturbances in both patient's eyes. Medical conditions, including bronchial asthma and hypereosinophilia, thus fulfilled the American College of Rheumatology criteria for Churg-Strauss syndrome. Visual acuity in the right eye did not improve.

Churg-Strauss syndrome (CSS), which is characterized by systemic small-vessel vasculitis of unknown etiology, is associated with a history of asthma. Although reports of CSS occurring in children are limited, effective treatment of pediatric patients with severe CSS remains challenging. A 10-year-old Japanese boy with a 6-month history of asthma treated with a leukotriene modifier, pranlukast, developed high fever, pleural infiltration, and pericarditis that were associated with marked hypereosinophilia (10,350 eosinophils/ µl). Owing to his persistent high fever, mononeuritis multiplex, and severe abdominal pain that was refractory to prednisolone, his general condition progressively deteriorated thereafter. Although intravenous high-dose immunoglobulin administration was transiently effective for mononeuritis multiplex, the recurrent high fever and severe abdominal pain remained refractory. An endoscopic study revealed ulcerative lesions of the total colon. In this context, we treated the patient with an aggressive multidrug immunosuppressive regimen consisting of a high-dose methylprednisolone pulse plus short-course intravenous cyclophosphamide pulse therapy, followed by oral tacrolimus combined with prednisolone. After the rescue multidrug treatment, his severe clinical signs dramatically subsided within a short time, and the concomitantly administered prednisolone was successfully tapered without flare. At present, 12 months after the presentation, he is free from CSS signs or therapy-related toxicity except for an occasional mild asthma attack. Although further close observation should be needed to draw a long-term outcome in this patient, we believe that aggressive multidrug immunosuppressive treatment should be considered as an alternative rescue treatment in selected patients with severe CSS, even with pediatric-onset disease, that is refractory to prednisolone. [ABSTRACT FROM AUTHOR]

Copyright of Archivos de Bronconeumología (English Edition) is the property of Sociedad Espanola de Neumologia y Cirugia Toracica (SEPAR) and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Because of advances in our understanding of the hypereosinophilic syndrome (HES) and the availability of novel therapeutic agents, the original criteria defining these disorders are becoming increasingly problematic. Here, we discuss shortcomings with the current definition of HES and recent developments in the classification of these disorders. Despite significant progress in our understanding of the pathogenesis of some forms of HES, the current state of knowledge is still insufficient to formulate a new comprehensive etiologic definition of HESs. Nevertheless, we suggest a new working definition that overcomes some of the most obvious limitations with the original definition. [Copyright &y& Elsevier]

Eosinophil-associated disease is a term used to encompass a range of disorders from hypereosinophilic syndrome to asthma. Despite the longstanding belief that eosinophils can be primary contributors to disease pathophysiology, it is only in recent years that direct and selective reduction or elimination of eosinophils can be achieved in animals or human subjects. These developments have been made possible in mice through clever targeting of eosinophil production. Antibodies and other agents that target soluble eosinophil-related molecules, such as IL-5, or cell-surface structures, such as CCR3, have also proved useful in reducing blood and tissue eosinophil counts. In human subjects the only eosinophil-selective agents tested in clinical trials thus far are neutralizing antibodies to IL-5, with promising but mixed results. At the very least, such forms of pharmacologic hypothesis testing of the role of eosinophils in certain airway, gastrointestinal, and hematologic diseases has finally provided us with new insights into disease pathogenesis. At its optimistic best, these and other targeted agents might someday become available for those afflicted with eosinophil-associated disorders. This review summarizes what has been learned in vivo in both preclinical and clinical studies of eosinophil-directed therapies, with an emphasis on recent advances. [Copyright &y& Elsevier]

Background: Treatments for Churg-Strauss syndrome (CSS), a rare eosinophilic vasculitis characterized by asthma, sinusitis, peripheral eosinophilia, pulmonary infiltrates, and tissue infiltration, are limited by toxicity or poor efficacy. Levels of IL-5, a cytokine regulating eosinophils, can be increased in patients with CSS. Mepolizumab, a humanized monoclonal anti–IL-5 antibody, decreases steroid requirements in patients with non-CSS hypereosinophilic syndromes. Objective: The purpose of this study was to assess whether mepolizumab would safely allow corticosteroid tapering in patients with steroid-dependent CSS while decreasing serum markers of disease activity. Methods: This open-label pilot study treated 7 patients with 4 monthly doses of mepolizumab to assess whether it safely decreased CSS disease activity and permitted tapering of systemic corticosteroids. Results: Mepolizumab was safe and well tolerated in patients with CSS. Mepolizumab reduced eosinophil counts and allowed for safe corticosteroid reduction in all 7 subjects. On cessation of mepolizumab, CSS manifestations recurred, necessitating corticosteroid bursts. Conclusion: Mepolizumab is a safe and well-tolerated therapy in patients with CSS, offering clinical benefit by enabling corticosteroid tapering while maintaining clinical stability. [Copyright &y& Elsevier]

Copyright of Medicina (Buenos Aires) is the property of Medicina (Buenos Aires) and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract: The term vasculitis refers to inflammation in the blood vessel walls. The vasculitic conditions which affect children are a varied group of diseases many of which carry a potentially significant morbidity and mortality. This review describes the classification, diagnosis and management of the main primary systemic paediatric vasculitides. The conditions described include Henoch-Schönlein purpura, Kawasaki disease, Takayasu''s arteritis, polyarteritis nodosa, and the ANCA associated vasculitic (AAV) conditions Wegener''s granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome. [Copyright &y& Elsevier]

Abstract: A 24-year-old women presented with unresolved right upper lobe consolidation. Her medical history was notable for asthma, coronary artery disease, cerebrovascular accident, and nasal polyposis. Thoracic computed tomography scan revealed total atelectasis in the right upper and middle lobes, and consolidation in the atelectatic regions. A bronchial mucosal biopsy revealed diffuse eosinophilic infiltrates in the interstitium. Clinical presentation and pathological results of bronchoscopy were consistent with Churg–Strauss syndrome. In Churg–Strauss syndrome, radiologic pulmonary manifestations vary and atelectasis is very rare. In this case, mechanism of the lobar atelectasis might be explained by eosinophilic infiltration of airway walls resulting in airway narrowing. [Copyright &y& Elsevier]

Introduction: Churg-Strauss syndrome (CSS) is a systemic granulomatous vasculitis rarely described in children, particularly associated with neurological involvement, exceptionally chorea. To our knowledge there are only 35 children and adolescent patients with CSS described in the literature. During a 25-year period 5283 patients were followed up at the Pediatric Rheumatology Unit of our University Hospital and only one (0.02%) presented CSS. Case report: A 7-year-old boy suffered from severe asthma, eosinophilia, history of allergy, recurrent non-fixed pulmonary infiltrates, several nodular lesions in both lungs and maxillary sinusitis. Transthoracic biopsy of the right lung revealed necrotizing extravascular eosinophilic infiltrates and the diagnosis of CSS was established. During the follow-up he had persistent vasculitis skin lesions and hemichorea. Despite the treatment with immunosuppressive drugs and intravenous immunoglobulin, he died because of pulmonary abscess and sepsis. Discussion: A rare case of CSS with chorea was reported, reinforcing the possibility of this disease in children with asthma, allergic rhinitis, hypereosinophilia and cutaneous vasculitis. [ABSTRACT FROM AUTHOR]

Objective: To describe the clinical characteristics of children with Churg–Strauss syndrome (CSS) in comparison with adult patients. Materials and methods: A systematic literature analysis was performed in the Medline database up to November 2007 and in rheumatology and pulmonology meeting scientific abstracts 2003-2007. Articles with reported childhood CSS cases were retrieved; clinical data were recorded. Descriptive statistical analyses and a comparison with 2 published adult CSS cohorts were performed. Results: Thirty-three cases of childhood CSS were identified. The mean age was 12 years and the male-to-female ratio was 0.74. All patients had significant eosinophilia and asthma. Histological evidence of eosinophilia and/or vasculitis was present in virtually all patients. Antineutrophil cytoplasmic antibodies were found in 25% of children with CSS. Initial treatment was corticosteroid monotherapy in 76% of childhood CSS patients, while 24% received additional immunosuppressive therapy. Another 18% required further immunosuppression at follow-up due to frequent relapses. Six deaths (18%), all related to the underlying disease, occurred after a mean disease duration of 14 months. As compared with adult CSS patients, children had a predominance of cardiopulmonary disease manifestations, a lower rate of peripheral nerve involvement, and higher mortality. Conclusions: Many aspects of CSS are similar in childhood and adult patients. However, pulmonary and cardiac involvement is predominant in pediatric CSS and mortality is substantial. [Copyright &y& Elsevier]

Copyright of Brazilian Journal of Pulmonology / Jornal Brasileiro de Pneumologia is the property of Sociedade Brasileira de Pneumologia e Tisiologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Chronic eosinophilic pneumonia (CEP) with asthma precedes the onset of Churg-Strauss syndrome (CSS) in half of all patients with CSS. It is not known what determines whether patients with CEP after asthma will have CSS. Objective: We examined whether activation of regulatory T cells in patients with CEP inhibits CSS development and is otherwise involved in the mechanism of CSS disease. Methods: In patients with CSS (n = 38), CEP with asthma (n = 20), and general adult asthma (n = 108), we examined the number of CD4+CD25+ T cells in peripheral blood, as well as levels of expression of the cytokines IL-2, IL-5, IL-10, and TGF-β by CD4+CD25+ T cells, CD4+CD25− T cells, or both. Results: At disease onset, patients with CSS, unlike patients with CEP, had significantly fewer CD4+CD25+ T cells than patients with any step of asthma. CD4+CD25+ T cells producing IL-10 were rarely detected in patients with CSS at disease onset or relapse, whereas the numbers of IL-10–producing T cells in patients with CEP were high at disease onset. There were fewer CD4+CD25− T cells producing IL-2 in patients with CSS before treatment than in patients with CEP at disease onset. The proportions of CD4+CD25+ T cells producing IL-10 and CD4+CD25− T cells producing IL-2 in patients with CSS increased at remission. Conclusions: Maintenance of the numbers of regulatory T cells in patients with CEP with asthma might inhibit CSS development through the action of cytokines, such as IL-10 and IL-2, produced by CD4+CD25+ or CD4+CD25− T cells. This might be part of a mechanism that influences progression and prognosis in these diseases. [Copyright &y& Elsevier]

Background: Churg–Strauss syndrome (CSS) is a necrotizing systemic vasculitis with extravascular granulomas and eosinophilic infiltrates of small vessels. CSS is usually revealed by nonspecific signs of necrotizing vasculitis in a context of late-onset asthma and blood eosinophilia. It is considered a systemic vasculitis with the highest prevalence of cardiac involvement and can lead to rapid-onset heart failure due to specific cardiomyopathy. Pericardial effusion may also occur during CSS and is usually well tolerated. Objective: The objective of these case reports was to indicate that CSS may present as tamponade, with or without other visceral involvement. Methods: Among CSS patients treated during the past 10 years at 2 French university hospitals, we have identified and described 2 cases revealed by tamponade with pericardial biopsy-proven granulomatous vasculitis. We have also reviewed the international medical literature in PubMed on cardiac involvement in CSS. Results: The first case report describes a 66-year-old man who had an isolated cardiac tamponade with both inflammatory syndrome and eosinophilia. Long-term remission was obtained with corticosteroids. The second case report describes a 46-year-old woman whose CSS presented with tamponade and associated central nervous system and myocardial involvement. Remission was obtained with corticosteroids and cyclophosphamide. In both cases, CSS was assessed by histological analysis of a pericardial sample. Conclusions: CSS may present as isolated cardiac tamponade. Whereas pericarditis with myocardial injury warrants immunosuppressive therapy, isolated pericarditis without other visceral involvement of poor prognosis only requires corticosteroid therapy. [Copyright &y& Elsevier]