2,817 results match your criteria Churg-Strauss Syndrome Allergic Granulomatosis


Low-Dose Mepolizumab Effectiveness in Patients Suffering From Eosinophilic Granulomatosis With Polyangiitis.

Allergy Asthma Immunol Res 2020 Sep;12(5):885-893

Immunoallergology Unit, Careggi University Hospital, Florence, Italy.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a vasculitis characterized by multisystemic manifestations including asthma. Mepolizumab (300 mg/4 weeks) has recently been approved for EGPA. However, real-life data are scarce and report experiences with high doses of mepolizumab intravenously administered (750 mg/4 weeks). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4168/aair.2020.12.5.885DOI Listing
September 2020

Cochlear Implantation as a Treatment for Sudden Autoimmune Sensorineural Hearing Loss in a Patient Affected by Eosinophilic Granulomatosis with Polyangiitis: A Case Report and A Review of Literature.

Ann Otol Rhinol Laryngol 2020 Jul 2:3489420938827. Epub 2020 Jul 2.

Department of Otorhinolaryngology-Head and Neck Surgery, IRCCS Ospedale Policlinico San Martino, University of Genoa, Genoa, Italy.

Objective: To report presentation, diagnostic process, management and outcome of a case of autoimmune inner ear disease (AIED) related with Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA), treated with cochlear implantation, and review of relevant literature.

Case Presentation And Management: A retrospective case report of AIED associated with EGPA treated with cochlear implantation was described. A multi-step approach for diagnosis and confirmation of AIED and hearing rehabilitation was conducted, eventually leading to left cochlear implantation. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/0003489420938827DOI Listing

Update on vasculitis: overview and relevant dermatological aspects for the clinical and histopathological diagnosis - Part II.

An Bras Dermatol 2020 Jul - Aug;95(4):493-507. Epub 2020 May 24.

Department of Dermatology, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.

Vasculitis is a group of several clinical conditions in which the main histopathological finding is fibrinoid necrosis in the walls of blood vessels. This article assesses the main dermatological aspects relevant to the clinical and laboratory diagnosis of small- and medium-vessel cutaneous and systemic vasculitis syndromes. The most important aspects of treatment are also discussed. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.abd.2020.04.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7335877PMC
May 2020
0.866 Impact Factor

How to improve the histopathological diagnosis of systemic vasculitides in daily practice?

Authors:
Eva Honsová

Cesk Patol 2020 ;56(2):68-73

The most common group of systemic vasculitides in adulthood are anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). AAV represent autoimmune systemic vasculitides and include 3 clinical phenotypes: Granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis), Microscopic polyangiitis (MPA) and Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome). Histological features are similar to each other in all affected locations, and there are represented by necrotizing vascular inflammation of small and medium calibers, often venules, capillaries or arteriols, typically with fibrinoid vessel wall necrosis. Read More

View Article

Download full-text PDF

Source
January 2020

Pathophysiology of ANCA-associated vasculitis.

Cesk Patol 2020 ;56(2):65-67

ANCA positive vasculitis (AAV) is a serious autoimmune disease mainly affecting small vessels in various organ systems, accompanied by the presence of ANCA antibodies in serum. AAV represents a group of the most common systemic vasculitis in adulthood, and based on clinical manifestations this disease entity includes 3 phenotypes, namely: granulomatosis with polyangiitis (formerly Wegeners granulomatosis), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome). Similar to other autoimmune diseases, AAV develops in patients with a predisposing genetic background who have been exposed to causative environmental factors, such as infections. Read More

View Article

Download full-text PDF

Source
January 2020

[Mononeuritis Multiplex: A Diagnostic Challenge].

Praxis (Bern 1994) 2020 ;109(7):509-512

Medizinische Klinik, Zuger Kantonsspital, Baar.

Mononeuritis Multiplex: A Diagnostic Challenge Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) is a multifaceted disease. Due to the variability in vascular and organ involvement, EPGA can manifest itself very differently. We report a case of a 60-year-old patient with a known bronchial asthma, pansinusitis and newly blood eosinophilia with a rapid-onset mononeuritis multiplex. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1024/1661-8157/a003445DOI Listing
January 2020

Definite IgG4-related disease had no overlap with eosinophilic granulomatosis with polyangiitis in Korean patients: a pilot study in one centre.

Clin Rheumatol 2020 May 4. Epub 2020 May 4.

Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea.

Objectives: Serum IgG4 may often increases in allergic diseases. Eosinophilic granulomatosis with polyangiitis (EGPA) has an allergic phase. For this reason, it was questionable whether IgG4-RD and EGPA may share some clinical and laboratory features. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s10067-020-05104-5DOI Listing

Effects of surgery and topical medication on eosinophilic granulomatosis with polyangiitis with otitis media and sinusitis: a case report.

J Int Med Res 2020 Apr;48(4):300060520920049

Department of Otolaryngology, Peking University People's Hospital, Beijing, China.

Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is eosinophil-rich, necrotizing granulomatous inflammation often involving the respiratory tract. Furthermore, EGPA is necrotizing vasculitis that predominantly affects small to medium vessels and is associated with asthma and eosinophilia. Most patients with EGPA have sinusitis and some complain of hearing loss and refractory otitis media with effusion. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/0300060520920049DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7218976PMC

Retinal artery occlusion followed by contralateral amaurosis fugax in association with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).

Am J Ophthalmol Case Rep 2020 Jun 8;18:100683. Epub 2020 Apr 8.

Department of Ophthalmology, University of Colorado School of Medicine, Aurora, CO, USA.

Purpose: To describe two cases of retinal artery occlusion followed by contralateral amaurosis fugax associated with eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome).

Observations: Case 1 is a 57 year-old male who presented with transient vision loss in the right eye two weeks after a cilioretinal artery occlusion in the left eye. Evaluation eventually led to a diagnosis of EGPA. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ajoc.2020.100683DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7154996PMC

[A perimyocarditis revealing eosinophilic granulomatosis with polyangiitis].

Ann Cardiol Angeiol (Paris) 2020 May 4;69(3):148-150. Epub 2020 Apr 4.

Service de médecine interne, hôpital Habib Thameur, 8, rue Ali Ben Ayed, 1008 Tunis, Tunisie; Faculté de médecine de Tunis, université de Tunis El Manar, Tunis, Tunisie.

Churg-Strauss syndrome (CSS) or eosinophilic granulomatosis with polyangiitis is a systemic vasculitis characterized by necrotizing arteritis, eosinophilic infiltration and extravascular granuloma; that may involve several organs. Cardiac involvement is the first cause of death in this vasculitis. These include myocarditis, pericarditis, coronary heart disease, dysrhythmias, and rarely valvular involvement. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ancard.2019.09.014DOI Listing

Cutaneous manifestations of childhood Eosinophilic Granulomatosis with Polyangiitis (cEGPA): A case-based review.

Pediatr Dermatol 2020 Mar 24. Epub 2020 Mar 24.

Dermatology Inc., Indianapolis, Indiana.

Background/objectives: This study seeks to better define the clinical presentation and histopathology of cutaneous manifestations in childhood eosinophilic granulomatosis with polyangiitis (cEGPA).

Methods: Case reports were collected from Ovid Medline Database and PubMed using keyword identifiers from 1946 to 2017. Adult patients ≥ 18 years and cases not diagnosed with EGPA by the author were excluded. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/pde.14144DOI Listing

Syndrome of inappropriate antidiuretic hormone associated with eosinophilic granulomatosis and polyangiitis.

BMJ Case Rep 2020 Mar 24;13(3). Epub 2020 Mar 24.

Intensive Care and Neurointensive Care, Sparrow Hospital, Lansing, Michigan, USA.

A 62-year-old man presented with classic signs and symptoms of eosinophilic granulomatosis and polyangiitis (EGPA, also known as Churg-Strauss syndrome)-mononeuritis multiplex, palpable purpura, hypereosinophilia, positive P-ANCA (perinuclear anti-neutrophil cytoplasmic antibodies) developed diffuse alveolar haemorrhage. The patient had longstanding mild hyponatraemia, but developed moderate and symptomatic hyponatraemia characteristic of the syndrome of inappropriate antidiuretic hormone. The patient's serum sodium returned to his baseline- mildly hyponatraemic, after initiation of treatment targeted towards EGPA. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1136/bcr-2019-231249DOI Listing

Symmetric polyneuropathy after viral symptomatology - not always Guillain-Barré Syndrome.

Acta Clin Belg 2020 Mar 13:1-8. Epub 2020 Mar 13.

Department of Nephrology, AZ St. Elisabeth Hospital, Zottegem, Belgium.

: Guillain-Barré Syndrome usually presents with ascending symmetric polyneuropathy, typically preceded by a viral infection. Despite the low incidence, physicians will often include Guillain-Barré Syndrome in their differential diagnosis. However, another underlying cause of polyneuropathy known as ANCA-associated vasculitis (AAV) is even more rare than Guillain-Barré Syndrome and therefore is usually overlooked. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1080/17843286.2020.1740858DOI Listing

Multiple ulcerations and perforation in the small intestine after steroid treatment in eosinophilic granulomatosis with polyangiitis: a case report and literature review.

Cardiovasc Pathol 2020 Jul - Aug;47:107193. Epub 2019 Dec 18.

Department of Cellular and Organ Pathology, Graduate School of Medicine, Akita University, Akita, Japan.

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome, is an uncommon disease with pathological features consisting of systemic necrotizing vasculitis, eosinophilic infiltration, and granulomatous or nongranulomatous extravascular eosinophilic inflammation. EGPA preferentially affects certain organ systems, including the airways, peripheral nerves, heart, kidney, and gastrointestinal tract. Although gastrointestinal involvement, such as ulcerations, is common in EGPA, gastrointestinal perforation is relatively uncommon and is associated with a poor prognosis. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.carpath.2019.107193DOI Listing

Updates in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis for the ENT surgeon.

Clin Otolaryngol 2020 May 20;45(3):316-326. Epub 2020 Mar 20.

West Suffolk Hospital, Bury St. Edmunds, Suffolk, UK.

ENT involvement is common in ANCA-associated vasculitis (AAV), particularly in GPA and EGPA. Early recognition and treatment is important for good outcomes, yet evidence suggests that UK ENT surgeons may not consistently recognise the early features of AAV, despite a similar incidence to vestibular schwannoma. AAV is a rapidly advancing field, with significant developments in the understanding of its pathogenesis, classification and treatment over the past decade. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/coa.13524DOI Listing

The continuous rediscovery and the benefit-risk ratio of thioguanine, a comprehensive review.

Expert Opin Drug Metab Toxicol 2020 Feb 24;16(2):111-123. Epub 2020 Feb 24.

Amsterdam UMC, Department of Gastroenterology and Hepatology, VU University Medical Center, AG&M Research Institute, Amsterdam, Netherlands.

: In the 1950s, thioguanine (TG), a thiopurine-derivative together with azathioprine (AZA) and mercaptopurine (MP), were developed for the treatment of childhood leukemia. Over the years, the use of TG was also explored for other, mainly immune-mediated and inflammatory, diseases such as in the field of dermatology and rheumatology (. psoriasis, systemic lupus erythematosus (SLE)) and gastroenterology and hepatology (. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1080/17425255.2020.1719996DOI Listing
February 2020

Clinical associations of renal involvement in ANCA-associated vasculitis.

Autoimmun Rev 2020 Apr 15;19(4):102495. Epub 2020 Feb 15.

Norwich Medical School, University of East Anglia, Norwich, United Kingdom.

Objective: Renal involvement in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis is associated with significant morbidity and higher mortality rates. This study examined clinical manifestations associated with renal involvement in ANCA-associated vasculitis within a large, international cross-sectional cohort.

Methods: Univariate and multivariate analyses were performed to identify clinical factors associated with renal disease, which was defined as i) a serum-creatinine >30% above normal and a fall in creatinine-clearance >25%; or ii) haematuria attributable to active vasculitis. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.autrev.2020.102495DOI Listing

Review of montelukast use over the past 20 years and monitoring of its effects.

Clin Exp Pediatr 2020 Feb 5. Epub 2020 Feb 5.

Asthma and Allergy Center, Department of Pediatrics, Inje University Sanggye Paik Hospital, Seoul, Korea.

Montelukast is a representative leukotriene receptor antagonist that was launched 20 years ago in Korea. It is recommended as an alternative initial treatment to control asthma in children with mild persistent symptoms or as an add-on treatment to low-dose inhaled corticosteroids (ICS) in children that require additional treatment. However, in a real-world setting, many doctors and patients prefer using montelukast over ICS, even though the efficacy of montelukast is lower than that of ICS. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3345/cep.2019.00325DOI Listing
February 2020

Pharmacogenetic investigation of efficacy response to mepolizumab in eosinophilic granulomatosis with polyangiitis.

Rheumatol Int 2020 Aug 3;40(8):1301-1307. Epub 2020 Feb 3.

Genetics, GlaxoSmithKline, Upper Providence, PA, USA.

Treatment of patients with the rare disease eosinophilic granulomatosis with polyangiitis (EGPA) with mepolizumab, a monoclonal antibody to interleukin-5 (IL-5) that reduces blood eosinophil counts, as an add-on therapy to glucocorticoid treatment, results in more accrued weeks in remission, reductions in glucocorticoid use and reductions in relapse rate. However, treatment response varies across a continuum. Therefore, to investigate if large genetic effects could identify responders, the impact of genetic variants on efficacy in EGPA subjects taking mepolizumab and glucocorticoids was assessed in this post hoc study. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00296-020-04523-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7316687PMC
August 2020
1.627 Impact Factor

Recurrent bilateral eyelid and conjunctival granulomatosis in Churg-Strauss syndrome.

Med J Malaysia 2020 01;75(1):86-87

University Malaya, Faculty of Medicine, Department of Ophthalmology, Kuala Lumpur, Malaysia.

A 47-year-old woman with poorly controlled asthma and allergic rhinitis presented with recurrent episodes of bilateral upper eyelid swelling associated with forniceal conjunctival mass for the past 10 years. Routine blood investigations showed raised IgE levels and raised eosinophil counts. The diagnosis of Churg-Strauss syndrome (CSS) was made following biopsy of the conjunctival mass. Read More

View Article

Download full-text PDF

Source
January 2020

ANCA associated vasculitis.

Eur J Intern Med 2020 Apr 29;74:18-28. Epub 2020 Jan 29.

Autoimmune Diseases Unit, Internal Medicine Department, Centro Hospitalar de Trás-os-Montes e Alto Douro, Avenida da Noruega, 5000-508 Vila Real, Portugal; NEDAI/SPMI Autoimmune Diseases Group/Portuguese Society of Internal Medicine, Portugal. Electronic address:

Vasculitis is characterized by inflammation of the vascular wall. It reaches vessels of different sizes and locations, conditioning multisystem and complex manifestations that require a holistic approach. Antineutrophil cytoplasmic antibody-associated vasculitis has an annual incidence rate of 20 per million inhabitants. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ejim.2020.01.011DOI Listing

[SYSTEMIC REVIEW OF EOSINOPHILIC DERMATOSES PATIENTS TREATED WITH TNF-Α INHIBITORS AND USTEKINUMAB].

Harefuah 2020 Jan;159(1):34-37

Department of Dermatology, Emek Medical Center, Afula, Israel.

Aims: This study aims to critically review the pros and cons of biological drugs as treatments and triggers of eosinophilic dermatoses.

Background: Eosinophilic dermatoses syndromes are rare diseases with a prominent eosinophilic infiltration mechanism. These syndromes have several known treatments with limited success. Read More

View Article

Download full-text PDF

Source
January 2020

Asthma control in eosinophilic granulomatosis with polyangiitis treated with rituximab.

Clin Rheumatol 2020 May 2;39(5):1581-1590. Epub 2020 Jan 2.

Division of Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First St SW, Rochester, MN, 55905, USA.

Objectives: Rituximab (RTX) treatment is used for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, but its benefits in eosinophilic granulomatosis with polyangiitis (EGPA) are unclear. Our aim was to characterize asthma control and glucocorticoid (GC) sparing after RTX treatment.

Methods: A retrospective, computer-assisted search was performed to identify patients with EGPA and GC-dependent asthma diagnosed between 2000 and 2017 who received RTX for remission induction. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s10067-019-04891-wDOI Listing

The color of skin: purple diseases of the skin, nails, and mucosa.

Clin Dermatol 2019 Sep - Oct;37(5):528-547. Epub 2019 Jul 17.

The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York, USA. Electronic address:

The color purple can be seen in several types of eruptions including inflammatory dermatoses like lichen planus, infectious dermatoses like ecthyma gangrenosum, neoplasms like Kaposi sarcoma, and vasculitis and vasculopathy. The current review focuses on the clinical appearance, pathophysiology, and treatment of several vasculitides and vasculopathies including capillaritis, cutaneous small-vessel vasculitis, immunoglobulin A (IgA) vasculitis, cryoglobulinemia, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, polyarteritis nodosum, warfarin-induced skin necrosis, heparin-induced thrombocytopenia, purpura fulminans, antiphospholipid antibody syndrome, calciphylaxis, levamisole-induced vasculopathy, and thrombotic thrombocytopenic purpura. Dermatologists play a central role in treating patients with cutaneous vasculitis and vasculopathy and may have the opportunity to facilitate identification of systemic disease by diagnosing cutaneous vasculitis and vasculopathy. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.clindermatol.2019.07.016DOI Listing

No evident association of nasal carriage of Staphylococcus aureus or its small-colony variants with cotrimoxazole use or ANCA-associated vasculitis relapses.

Rheumatology (Oxford) 2020 01;59(1):77-83

Department of Internal Medicine, Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, APHP, Université Paris-Descartes, Paris Cedex 14, France.

Objective: To identify the role of Staphylococcus aureus (SA) or its intracellular small-colony variant phenotype (SCV) with co-trimoxazole (CTX) or ANCA-associated vasculitis (AAV) activity.

Methods: All consecutive AAV patients with granulomatosis with polyangiitis (GPA), eosinophilic GPA or microscopic polyangiitis, followed at the French National Vasculitis Referral Center (09/2012-05/2013), and hospitalized non-AAV controls, exclusively for SA/SCV carriage comparisons, were enrolled. All had bilateral anterior nasal swab cultures. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/rheumatology/kez236DOI Listing
January 2020

Chronic rhinosinusitis in eosinophilic granulomatosis with polyangiitis: clinical presentation and antineutrophil cytoplasmic antibodies.

Int Forum Allergy Rhinol 2020 02 2;10(2):217-222. Epub 2019 Dec 2.

Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, MN.

Background: In this study we aim to describe presenting characteristics and identify prognostic factors for disease resolution in patients with chronic rhinosinusitis (CRS) in the setting of eosinophilic granulomatosis with polyangiitis (EGPA).

Methods: Patients evaluated at a tertiary care center with diagnoses of EGPA and CRS were identified. Descriptive statistics were obtained. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/alr.22503DOI Listing
February 2020
2.371 Impact Factor

The Reply.

Am J Med 2019 11;132(11):e812

Department of Dermatology, Stanford University School of Medicine, Redwood City, Calif; Department of Pathology, Stanford University School of Medicine, Stanford, Calif. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.amjmed.2019.07.027DOI Listing
November 2019

Occam's Razor and Its Ethical Implications in Eosinophilic Granulomatosis With Polyangiitis.

Authors:
Oscar M P Jolobe

Am J Med 2019 11;132(11):e811

Manchester Medical Society, United Kingdom. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.amjmed.2019.07.004DOI Listing
November 2019

Eosinophilic granulomatosis with polyangiitis: the multifaceted spectrum of clinical manifestations at different stages of the disease.

Expert Rev Clin Immunol 2020 Jan 17;16(1):51-61. Epub 2020 Jan 17.

INSERM UMR1227, Lymphocytes B et Autoimmunité, Université de Bretagne Occidentale, CHU de Brest, Brest, France.

: Eosinophilic granulomatosis with polyangiitis (EGPA) usually occurs in patients with late-onset asthma and sustained peripheral blood eosinophilia and classically presents with a clinical multifaceted spectrum of manifestations, which may vary at the different stages of the natural history of the disease.: We reviewed EGPA clinical presentation, focusing on clinical manifestations at three different phases of the disease: 1/before the development of overt vasculitis, 2/at vasculitis diagnosis and 3/during the long-term follow-up. An update on current classification criteria and recent therapeutic advances has been provided as well. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1080/1744666X.2019.1697678DOI Listing
January 2020

Idiopathic Orbital Pseudotumor Preceding Systemic Inflammatory Disease in Children.

J Pediatr Ophthalmol Strabismus 2019 Nov;56(6):373-377

Purpose: To describe four pediatric cases in which isolated orbital pseudotumor preceded the development of a systemic inflammatory disease by months to years.

Methods: The medical records of all patients with the clinical diagnosis of orbital pseudotumor seen at the Ocular Oncology Service of Wills Eye Hospital and Northern Virginia Ophthalmology Associates from 2010 to 2015 were reviewed retrospectively, and those associated with systemic inflammatory disease were selected for further study. Data were retrospectively collected from medical record review regarding patient demographics and clinical features, time to development of systemic inflammatory disease, and medical management. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3928/01913913-20190923-02DOI Listing
November 2019

Multiple cerebral infarction diagnosed as Eosinophilic Granulomatosis with Polyangiitis by autopsy.

BMC Neurol 2019 Nov 15;19(1):288. Epub 2019 Nov 15.

Department of Neurology, Juntendo University Urayasu Hospital, 2-1-1 Tomioka, Urayasu, Chiba, 279-0021, Japan.

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis of unknown cause involving the brain and accompanied by prominent eosinophilia. Intracardiac thrombosis is a major cardiac complication of EGPA that may cause thromboembolism.

Case Presentation: A 53-year-old man presenting with abulia (consciousness disturbance) and left upper limb paralysis was admitted to our hospital. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12883-019-1515-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6857209PMC
November 2019

Allergic bronchopulmonary aspergillosis.

Allergy Asthma Proc 2019 11;40(6):421-424

Allergic bronchopulmonary aspergillosis (ABPA) occurs in patients with asthma or cystic fibrosis, and results in pulmonary infiltrates, tenacious mucus plugs that harbor hyphae of Aspergillus fumigatus, elevations of total serum immunoglobulin E concentration and peripheral blood and sputum eosinophilia. Bronchiectasis is an irreversible complication of ABPA. The key to early diagnosis is to consider ABPA in anyone with asthma or cystic fibrosis and with a positive skin test result for , and/or recurrent infiltrates on radiographs. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.2500/aap.2019.40.4262DOI Listing
November 2019
1 Read

A Case of Cutaneous Arteritis Presenting as Infiltrated Erythema in Eosinophilic Granulomatosis With Polyangiitis: Features of the Unique Morphological Evolution of Arteritis as a Diagnostic Clue.

Am J Dermatopathol 2019 Nov;41(11):832-834

Department of Dermatology, Nippon Kokan Hospital, Kanagawa, Japan.

Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) is a rare systemic vasculitis affecting small- and medium-sized vasculature, associated with asthma and eosinophilia. Different levels of vasculitis in cutaneous lesions have been observed, including dermal small vessel vasculitis and subcutaneous muscular vessel vasculitis. Although the EGPA-associated small vessel vasculitis described as leukocytoclastic vasculitis can be often found in the documented literature, the features of subcutaneous muscular vessel vasculitis in EGPA-associated cutaneous lesions have been rarely demonstrated clinically and histopathologically in English literature. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/DAD.0000000000001451DOI Listing
November 2019
2 Reads

Heart disease in a mutant mouse model of spontaneous eosinophilic myocarditis maps to three loci.

BMC Genomics 2019 Oct 11;20(1):727. Epub 2019 Oct 11.

Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.

Background: Heart disease (HD) is the major cause of morbidity and mortality in patients with hypereosinophilic diseases. Due to a lack of adequate animal models, our understanding of the pathophysiology of eosinophil-mediated diseases with heart complications is limited. We have discovered a mouse mutant, now maintained on an A/J inbred background, that spontaneously develops hypereosinophilia in multiple organs. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12864-019-6108-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6788080PMC
October 2019
1 Read

Recent advances in eosinophil-related diseases.

Authors:
Hiroshi Nakajima

Allergol Int 2019 Oct;68(4):401-402

Department of Allergy and Clinical Immunology, Chiba University, Chiba, Japan. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.alit.2019.09.001DOI Listing
October 2019

Do ANCA-associated vasculitides and IgG4-related disease really overlap or not?

Int J Rheum Dis 2019 Oct 30;22(10):1926-1932. Epub 2019 Sep 30.

Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and immunoglobulin G4-related disease (IgG4-RD) have some common features. The co-occurrence/concurrence of AAV and IgG4-RD was recently published by the collaborative European Vasculitis Study Group. First, we aimed to investigate ANCA positivity of our IgG4-RD cohort. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/1756-185X.13693DOI Listing
October 2019
2 Reads

Granulomatosis With Polyangiitis: A 17 Year Experience From A Tertiary Care Hospital In Pakistan.

J Ayub Med Coll Abbottabad 2019 Jul-Sep;31(3):478-479

Ziauddin University, Karachi, Pakistan.

Granulomatosis with Polyangitis (GPA) is an uncommon immunologically mediated necrotizing vasculitis affecting the small and medium sized systemic blood vessels. We previously reported our experience with this condition and herein, we document our study findings and compare them to the clinical and radiological findings of various studies from around the world. By doing so we hope to further create awareness of this condition afflicting not only our part of the population but is part of a larger global phenomenon. Read More

View Article

Download full-text PDF

Source
December 2019
2 Reads

Extracavitary primary effusion lymphoma in a post-transplantation patient.

Br J Haematol 2019 12 13;187(5):555. Epub 2019 Sep 13.

Pathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, Terni, Italy.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/bjh.16196DOI Listing
December 2019

Neurological Manifestations Do not Affect Cumulative Survival in Indian Patients with Antineutrophil Cytoplasmic Antibody Associated Vasculitis.

Neurol India 2019 Jul-Aug;67(4):1043-1047

Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Background: Neurological manifestations are an important cause of morbidity in antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). It is not clear whether or not they are indicative of a severe disease course with multiple organ involvement and shortened survival.

Aims And Objectives: To characterize the neurological manifestations of AAV and analyze their relationship with other organ system and cumulative survival. Read More

View Article

Download full-text PDF

Source
http://www.neurologyindia.com/text.asp?2019/67/4/1043/266234
Publisher Site
http://dx.doi.org/10.4103/0028-3886.266234DOI Listing
March 2020
2 Reads
1.084 Impact Factor

Eosinophilic cholangitis with eosinophilic granulomatosis with polyangiitis: A case report and review of the literature.

Allergol Int 2020 Jan 31;69(1):154-156. Epub 2019 Aug 31.

Department of Allergy and Rheumatology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.alit.2019.08.002DOI Listing
January 2020
1 Read

[Pulmonary Infiltrate - Not Always Due to Bacterial Infection].

Praxis (Bern 1994) 2019 Sep;108(11):733-736

Kantonsspital Münsterlingen, Abteilung Pneumologie, Münsterlingen.

Pulmonary Infiltrate - Not Always Due to Bacterial Infection A 65-year-old female patient with a history of controlled asthma was diagnosed with community-acquired pneumonia (CAP). After two weeks of treatment on co-amoxicillin, she failed to respond and was referred for further investigations. Clinical symptoms and laboratory results were suggestive for eosinophilic granulomatosis with polyangiitis (EGPA). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1024/1661-8157/a003282DOI Listing
September 2019
4 Reads

Oral Corticosteroid Use for the Treatment of Chronic Eosinophilic Disease: A Patient's and His Physician's Experience.

Adv Ther 2019 10 31;36(10):2558-2566. Epub 2019 Aug 31.

Department of Internal Medicine, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium.

This article, coauthored by a patient with eosinophilic granulomatosis with polyangiitis (EGPA) initially presenting as severe eosinophilic asthma and his physician-specialist, discusses the use and management of oral corticosteroid (OCS) treatment. It also considers the importance of early diagnosis of a rare disease and patient education. The patient describes his journey from progressive worsening of asthma and eventual diagnosis of EGPA to long-term OCS treatment and then participation in a clinical trial for this rare disease, involving the introduction of targeted biologic therapy with OCS tapering. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s12325-019-01070-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6822817PMC
October 2019
1 Read

A case of repetitive myocardial infarction with unobstructed coronaries due to Churg-Strauss syndrome.

Eur Heart J Case Rep 2019 Jun;3(2)

Department of Cardiology, Lister Hospital, East and North Hertfordshire NHS Trust, Stevenage, UK.

Background: Myocardial infarction is most commonly caused by thrombosis occurring on a background of coronary atherosclerosis, resulting in reduced coronary flow. Less often, myocardial infarction can occur in the absence of coronary disease. The pathomechanism of myocardial infarction in such patients is heterogeneous and more challenging to diagnose and treat. Read More

View Article

Download full-text PDF

Source
https://academic.oup.com/ehjcr/article/doi/10.1093/ehjcr/ytz
Publisher Site
http://dx.doi.org/10.1093/ehjcr/ytz041DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6601219PMC
June 2019
6 Reads

Eosinophilic granulomatosis with polyangiitis in a 10-year-old girl.

CMAJ 2019 08;191(33):E919

Department of Dermatology and Venereology, Sichuan Academy of Medical Sciences and Sichuan Provincial People's Hospital, Chengdu, China.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1503/cmaj.190247DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6699945PMC
August 2019
2 Reads

Rapid Disappearance of Intraventricular Mobile Structures with Steroids in Eosinophilic Granulomatosis with Polyangiitis.

J Stroke Cerebrovasc Dis 2019 Nov 14;28(11):104326. Epub 2019 Aug 14.

Department of Neurology, Kashiwa Hospital, The Jikei University School of Medicine, Chiba, Japan.

Endomyocarditis in Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare complication, commonly involving an apical mass compatible with a thrombus. However, no previous report has discussed mobile structures detected by echocardiography in a patient with EGPA. A 53-year-old man with asthma presented with low-grade fever, consciousness disturbance, and vomiting. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2019.104326DOI Listing
November 2019
4 Reads