8,171 results match your criteria Wegener Granulomatosis


Rituximab as an effective and probably safe treatment for granulomatosis with polyangiitis (Wegener's Granulomatosis).

Int Immunopharmacol 2019 Feb 20;70:67-68. Epub 2019 Feb 20.

Rheumatology and Internal Medicine, Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran.

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http://dx.doi.org/10.1016/j.intimp.2019.01.029DOI Listing
February 2019

Anemia Combined Significantly Increased High-Sensitivity C Reactive Protein and Lung Lesions Lead to the Diagnosis of Granulomatosis with Polyangiitis Proven by Lung Biopsy and Anti-neutrophil Cytoplasmic Antibody Test.

Clin Lab 2019 Jan;65(1)

Background: To report an atypical case misdiagnosed as lung abscess over the past 2 months, but persistent anemia combined with significantly increased hs-CRP and lung lesions indicated systemic lesion, which led to the diagnosis of granulomatosis with polyangiitis proven by lung biopsy and anti-neutrophil cytoplasmic antibody test (ANCA).

Methods: The complete blood count, hs-CRP, and anti-neutrophil cytoplasmic antibody (ANCA) test were performed. The pathology consultation for the lung biopsy was arranged. Read More

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http://dx.doi.org/10.7754/Clin.Lab.2018.180733DOI Listing
January 2019

Granulomatosis With Polyangiitis Induced by Infection.

Kidney Int Rep 2019 Feb 26;4(2):341-345. Epub 2018 Sep 26.

Division of Nephrology and Hypertension, Department of Internal Medicine, St. Marianna University School of Medicine, Kanagawa, Japan.

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http://dx.doi.org/10.1016/j.ekir.2018.09.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6365307PMC
February 2019

IgG4-Related Disease and Eosinophilic Granulomatosis with Polyangiitis: Similarity or Coexistence?

Isr Med Assoc J 2019 Feb;21(2):122-123

Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel Hashomer, Israel.

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February 2019

Portal Vein Thrombosis and Thrombocytopenia in Eosinophilic Granulomatosis with Polyangiitis: A Paradox?

Eur J Case Rep Intern Med 2018 28;5(11):000971. Epub 2018 Nov 28.

Division of Rheumatology, Emek Medical Center, Itzhak Rabin Blvd., Afula, Israel.

A 36-year-old woman with eosinophilic granulomatosis with polyangiitis (EGPA) presented with necrotic skin lesions and pulmonary infiltrates. There was eosinophilic vasculitis on skin biopsy, and substantial tissue eosinophilia in her bone marrow. She had unexplained worsening thrombocytopenia, which prompted a thrombophilia work-up. Read More

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http://dx.doi.org/10.12890/2018_000971DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6346807PMC
November 2018
1 Read

Incidence and prevalence of granulomatosis with polyangiitis and microscopic polyangiitis in health management organization in Argentina: a 15-year study.

Clin Rheumatol 2019 Feb 11. Epub 2019 Feb 11.

Rheumatology Unit, Internal Medicine Services, Hospital Italiano de Buenos Aires, Perón 4190, 1414, Buenos Aires, Argentina.

Background/objective: Our objective was to estimate incidence and prevalence rates of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) using data from a university hospital-based health management organization (HIMCO) in Latin America.

Methods: Multiple methods for case finding were used to ensure complete ascertainment. GPA was diagnosed if fulfilling ACR 1990 criteria or a clinical diagnosis was made by an experienced rheumatologist. Read More

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http://dx.doi.org/10.1007/s10067-019-04463-yDOI Listing
February 2019
1 Read

Shedding light on vasculitis in Egypt: a multicenter retrospective cohort study of characteristics, management, and outcome.

Clin Rheumatol 2019 Feb 9. Epub 2019 Feb 9.

Rheumatology and Rehabilitation Department, Faculty of Medicine, Assiut University, Assiut, Egypt.

Objectives: The frequency of different vasculitides and their characteristics vary among different regions. The identification of geographic disparities of disease phenotypes helps the development of international criteria, allowing the classification of patients of different ethnicities. This study aimed to describe the frequency, characteristics, course, response to treatment, and outcome of the different adulthood vasculitides in Egypt. Read More

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http://dx.doi.org/10.1007/s10067-019-04441-4DOI Listing
February 2019
1 Read

Distinction between MPO-ANCA and PR3-ANCA-associated glomerulonephritis in Chinese patients: a retrospective single-center study.

Clin Rheumatol 2019 Feb 8. Epub 2019 Feb 8.

Jinling Clinical Medical College of Nanjing Medical University, Nanjing, China.

Objectives: To retrospectively investigate the clinical and histological features and outcomes of ANCA-associated glomerulonephritis (AAGN) with different ANCA serotypes.

Method: A total of 467 AAGN patients were divided into MPO-AAGN (MPO) and PR3-AAGN (PR3) groups according to ANCA serotype. Clinical and histological features and renal outcomes were compared. Read More

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http://dx.doi.org/10.1007/s10067-019-04458-9DOI Listing
February 2019
2 Reads

Consideration concerning similarities and differences between ANCA-associated vasculitis and IgG-4-related diseases: case series and review of literature.

Immunol Res 2019 Feb 8. Epub 2019 Feb 8.

Department of Rheumatology and Allergy, Japanese Red Cross Society Narita Hospital, 90-1, Iida-cho, Narita-shi, Chiba, 286-8523, Japan.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and immunoglobulin G4-related diseases (IgG4-RD) are regarded as entirely different disease types with different etiological mechanisms. However, we experienced two cases that had clinical features of both AAV and IgG4-RD. The first case is an 81-year-old woman who showed periaortitis and retroperitoneal fibrosis and periarteritis with elevation of myeloperoxidase-anti-neutrophil cytoplasmic antibody and IgG4 levels. Read More

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http://dx.doi.org/10.1007/s12026-019-9070-7DOI Listing
February 2019
1 Read
3.098 Impact Factor

[Unexpected death due to eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)].

An Sist Sanit Navar 2019 Feb 5;0(0):67385. Epub 2019 Feb 5.

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Eosinophilic granulomatosis with polyangiitis (EGPA), or Churg-Strauss syndrome, is a rare disease characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring among patients with asthma and tissue eosinophilia. Clinically, it presents in various ways and progresses in three phases: prodromic (asthma and rhino-sinusitis), eosinophilic (peripheral eosinophilia and organ involvement), and vasculitic (clinical manifestations due to small vessel vasculitis). The differential diagnosis of EGPA principally includes eosinophilic and vasculitic disorders, early treatment is needed to avoid a fatal outcome. Read More

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http://dx.doi.org/10.23938/ASSN.0395DOI Listing
February 2019
1 Read

Differential Diagnosis of Churg-Strauss Syndrome with Tingling Sensation and Weakness of the Lower Extremities.

Anesth Pain Med 2018 Dec 28;8(6):e84179. Epub 2018 Nov 28.

Department of Internal Medicine, Division of Rheumatology, Jeju National University Hospital, Jeju, Korea.

Introduction: Churg-Strauss syndrome (CSS; or eosinophilic granulomatosis with polyangiitis) is an extremely rare autoimmune condition characterized by hypereosinophilia and vasculitis of small or medium-sized blood vessels, occurring in patients with asthma and allergic rhinitis.

Case Presentation: A 47-year-old man (height, 175.8 cm and weight, 74. Read More

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http://dx.doi.org/10.5812/aapm.84179DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6347737PMC
December 2018
1 Read

Development of CT-based methods for longitudinal analyses of paranasal sinus osteitis in granulomatosis with polyangiitis.

BMC Med Imaging 2019 Feb 4;19(1):13. Epub 2019 Feb 4.

Division of Radiology and Nuclear Medicine, Oslo University Hospital, PB 4950 Nydalen, Oslo, 0424, Norway.

Background: Even though progressive rhinosinusitis with osteitis is a major clinical problem in granulomatosis with polyangiitis (GPA), there are no studies on how GPA-related osteitis develops over time, and no quantitative methods for longitudinal assessment. Here, we aimed to identify simple and robust CT-based methods for capture and quantification of time-dependent changes in GPA-related paranasal sinus osteitis and compare performance of the methods under study in a largely unselected GPA cohort.

Methods: GPA patients (n = 121) with ≥3 paranasal CT scans obtained ≥12 months apart and control patients not having GPA or rhinosinusitis (n = 15) were analysed by: (i) Global osteitis scoring scale (GOSS), originally developed for chronic rhinosinusitis; (ii) Paranasal sinus volume by manual segmentation; (iii) Mean maxillary and sphenoid diameter normalised to landmark distances (i. Read More

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http://dx.doi.org/10.1186/s12880-019-0315-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6360792PMC
February 2019
2 Reads

Isolated choroid plexus involvement in a case of granulomatosis with polyangiitis negative for antineutrophil cytoplasmic antibodies (ANCA).

J Neurol Sci 2019 Jan 26;398:128-130. Epub 2019 Jan 26.

Division of Neurology, Department of Neurosciences, Santa Chiara Hospital, Trento, Italy.

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http://dx.doi.org/10.1016/j.jns.2019.01.045DOI Listing
January 2019
1 Read

Clinical significance of ocular manifestations in granulomatosis with polyangiitis: association with sinonasal involvement and damage.

Rheumatol Int 2019 Mar 31;39(3):489-495. Epub 2019 Jan 31.

Department of Ophthalmology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga No. 15, Col. Sección XVI, Tlalpan, CP 14000, Mexico City, CP, Mexico.

Ocular involvement is present in 50-60% of granulomatosis with polyangiitis (GPA) patients and can affect any part of the ocular globe. The present study describes ophthalmologic manifestations, association with systemic symptoms, disease activity and damage in GPA. A cross-sectional study was conducted including patients with GPA who underwent rheumatologic and ophthalmologic evaluation. Read More

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http://dx.doi.org/10.1007/s00296-019-04242-7DOI Listing
March 2019
3 Reads

IgG4:IgG RNA ratio differentiates active disease from remission in granulomatosis with polyangiitis: a new disease activity marker? A cross-sectional and longitudinal study.

Arthritis Res Ther 2019 Jan 31;21(1):43. Epub 2019 Jan 31.

Department of Rheumatology & Clinical Immunology and Amsterdam Rheumatology and Immunology Center (ARC), Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.

Objectives: An important limitation in granulomatosis with polyangiitis (GPA) is the lack of disease activity markers. Immunoglobulin G4-positive (IgG4) B cells and plasma cells are implicated in the pathogenesis of GPA. We hypothesized that the presence of these cells in peripheral blood could serve as disease activity parameter in GPA. Read More

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https://arthritis-research.biomedcentral.com/articles/10.118
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http://dx.doi.org/10.1186/s13075-018-1806-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6357433PMC
January 2019
3 Reads

Diagnostic algorithm for antineutrophil cytoplasmic antibody-associated systemic vasculitis.

Authors:
T V Beketova

Ter Arkh 2018 May;90(5):13-22

V.A. Nasonova Scientific Research Institute of Rheumatology, Moscow, Russia.

Antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) are rare autoimmune disorders and characterized by severe multiple organ lesions with a potential fatal outcome. AAV comprises granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). Early diagnosis and treatment can significantly improve the AAV prognosis, but there are can be difficult, largely due to the lack of criteria for the classification MPA, whose main difference is the absence of granulomatous inflammation. Read More

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http://dx.doi.org/10.26442/terarkh201890513-22DOI Listing
May 2018
1 Read

Pulmonary vasculitis: diagnosis and treatment.

Ter Arkh 2018 Apr;90(3):99-106

Pirogov Russian National Research Medical University, Moscow, Russia.

Pulmonary vasculitis is a group of rapidly progressing severe diseases characterized by vascular inflammation, destruction and necrosis of the pulmonary tissue. The pathological process in the lungs varies from diffuse alveolar hemorrhage to inflammation of the parenchyma, pleural effusion, thrombotic and thromboembolic complications. Depending on the size of the affected vessels, vasculites are divided into vasculites of large, medium and small vessels. Read More

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http://dx.doi.org/10.26442/terarkh201890399-106DOI Listing
April 2018
6 Reads

Imaging in small and medium vessel vasculitis.

Int J Rheum Dis 2019 Jan;22 Suppl 1:78-85

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

Vasculitis includes a group of disorders characterized by inflammation of the vessel wall and classified based on the diameter of the predominantly involved vessels. Granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis and Henoch-Schonlein purpura are the important entities in the small vessel vasculitis group, while polyarteritis nodosa and Kawasaki disease represent the medium vessel vasculitis group. The clinical manifestations may be non-specific and there may be considerable overlap with the other disorders. Read More

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http://dx.doi.org/10.1111/1756-185X.13390DOI Listing
January 2019
1 Read

Aortitis and pachymeningitis: an unusual combination in granulomatosis with polyangiitis (myeloperoxidase-associated vasculitis).

BMJ Case Rep 2019 Jan 28;12(1). Epub 2019 Jan 28.

Internal Medicine, Maricopa Integrated Health System, Phoenix, Arizona, USA.

Aortitis and pachymeningitis are uncommon manifestations of the antineutrophil cytoplasmic antibody-associated vasculitides, a group of systemic autoimmune diseases mainly affecting small vessels. We present a case of a 71-year-old woman with a remote history of idiopathic chronic granulomatous pachymeningitis complicated with aortic thickening due to autoimmune aortitis. Read More

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http://dx.doi.org/10.1136/bcr-2018-226795DOI Listing
January 2019
1 Read

Erythematous and Vascular Oral Mucosal Lesions: A Clinicopathologic Review of Red Entities.

Head Neck Pathol 2019 Jan 29. Epub 2019 Jan 29.

The Ohio State University, 305 W. 12 Ave, Columbus, OH, 43210, USA.

Erythematous lesions of the oral mucosa are common and can reflect a variety of conditions, ranging from benign reactive or immunologically-mediated disorders to malignant disease. Together with vascular abnormalities, which can vary from reddish to bluish-purple in color, the differential diagnosis for erythematous oral mucosal change is quite diverse. This review focuses on salient clinical features and histopathologic findings of selected conditions which clinically present as red or vascular-like oral mucosal alterations, including oral vascular malformations and neoplasms, pyogenic granuloma, localized juvenile spongiotic gingival hyperplasia, denture stomatitis, benign migratory glossitis (geographic tongue), orofacial granulomatosis, granulomatosis with polyangiitis (Wegener granulomatosis), megaloblastic anemia, and erythroplakia. Read More

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http://dx.doi.org/10.1007/s12105-019-01002-8DOI Listing
January 2019
2 Reads

Lumps and Bumps of the Gingiva: A Pathological Miscellany.

Head Neck Pathol 2019 Jan 29. Epub 2019 Jan 29.

Academic Unit of Oral and Maxillofacial Medicine and Pathology, School of Clinical Dentistry, University of Sheffield, Claremont Crescent, Sheffield, S10 2TA, UK.

Lesions of the gingivae are amongst the commonest lesions seen in patients and the vast majority are reactive hyperplasias, related to a number of chronic irritant stimuli. However, there are a number of entities that have a predilection for the gingivae, which are much less common in other parts of the oral cavity. The purpose of this paper is to discuss the clinical and histological differential diagnoses when presented with a lump on the gingivae, including the approach to diagnosis and diagnostic pitfalls. Read More

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http://dx.doi.org/10.1007/s12105-019-01000-wDOI Listing
January 2019
1 Read

A Case of Eosinophilic Granulomatosis with Polyangiitis Complicated with A IgG4 Related Disease Like Symptoms.

Case Reports Immunol 2018 4;2018:3763084. Epub 2018 Nov 4.

Department of Pulmonary Medicine, School of Medicine, Fukushima Medical University, Fukushima, Japan.

We report a case of eosinophilic granulomatosis with polyangiitis (EGPA) complicated with a IgG4 related disease like symptoms presenting as eyelid swellings. In the present case, the serum level of IgG4 and the ratio of IgG4 to IgG were generally increased by the disease course of EGPA. Considering the course of clinical symptoms, there is a possibility that orbital manifestations were one of the clinical features during the disease course of EGPA while the histological features of right eyelid tissue and other ocular manifestations were consistent with the diagnosis of IgG4 related disease. Read More

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http://dx.doi.org/10.1155/2018/3763084DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6332970PMC
November 2018
1 Read

Hemorrhagic conversion after alteplase administration in a patient with vasculitis and acute ischemic stroke.

Am J Health Syst Pharm 2019 Feb;76(4):211-213

Department of Pharmacy, Wesley Medical Center, Wichita, KS.

Purpose: A case of alteplase administration to a patient with vasculitis and acute ischemic stroke (AIS) is reported.

Summary: A 38-year-old woman with a recent diagnosis of granulomatosis with polyangiitis (GPA) received alteplase for AIS and developed symptomatic hemorrhagic conversion. Published reports regarding the safety of thrombolytic therapy in patients with a high inflammatory burden are inconsistent. Read More

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http://dx.doi.org/10.1093/ajhp/zxy048DOI Listing
February 2019
1 Read

Central Nervous System Involvement in ANCA-Associated Vasculitis: What Neurologists Need to Know.

Front Neurol 2018 10;9:1166. Epub 2019 Jan 10.

Department of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China.

To provide a comprehensive review of the central nervous system (CNS) involvement in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), including the pathogenesis, clinical manifestations, ancillary investigations, differential diagnosis, and treatment. Particular emphasis is placed on the clinical spectrum and diagnostic testing of AAV. AAV is a pauci-immune small-vessel vasculitis characterized by neutrophil-mediated vasculitis and granulomatousis. Read More

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https://www.frontiersin.org/article/10.3389/fneur.2018.01166
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http://dx.doi.org/10.3389/fneur.2018.01166DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6335277PMC
January 2019
7 Reads

Eosinophilic granulomatosis with polyangiitis in a continuous flow left ventricular assist device patient: a case report and review of literature.

Cardiovasc Pathol 2018 Dec 28;39:70-73. Epub 2018 Dec 28.

Department of Pathology and Laboratory Medicine, The University of Texas Health Science Center at Houston, Houston, TX, USA.

Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome (CSS), is a rare autoimmune disease with an estimated incidence of approximately 0.11 to 2.66 new cases per 1 million people per year and an overall prevalence of 10. Read More

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http://dx.doi.org/10.1016/j.carpath.2018.12.004DOI Listing
December 2018
1 Read

[Modern management of otologic consequences of Wegener's granulomatosis - a case report and review of the literature].

Orv Hetil 2019 Jan;160(4):151-157

Klinikai Központ, Fül-Orr-Gégészeti és Fej-Nyaksebészeti Klinika, Pécsi Tudományegyetem, Általános Orvostudományi Kar Pécs, Munkácsy M. u. 2., 7621.

Wegener's granulomatosis - or, in other words, granulomatosis with polyangiitis - is an anti-neutrophil cytoplasmic antibody associated granuloma forming vasculitis, mainly affecting the respiratory tract and the renal system. Otologic manifestations of Wegener's disease can be otitis media with effusion or chronic silent mastoiditis with conductive hearing loss, but sensorineural hearing loss can also evolve. The diagnosis is based on the clinical appearance as well as the immunoserological and histopathological results. Read More

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http://dx.doi.org/10.1556/650.2018.31266DOI Listing
January 2019
1 Read

Granulomatosis with polyangiitis in Northeastern Brazil: study of 25 cases and review of the literature.

Adv Rheumatol 2018 Jun 28;58(1):10. Epub 2018 Jun 28.

Post-Graduate Program in Medical Sciences, University of Fortaleza (UNIFOR), Fonseca Lobo 560 apto. 1202, Aldeota, Fortaleza, Ceará, CEP 60175020, Brazil.

Background: Little has been published about the epidemiology of Granulomatosis with polyangiitis (GPA) in South America, especially in the intertropical zone, and no epidemiological data from Brazil are available. The purpose of the present study was to draw a clinical and demographic profile of GPA patients living in Northeastern Brazil based on laboratory, histological and imaging findings, and evaluate the frequency of organic involvement.

Methods: Clinical, epidemiological and treatment data of GPA patients were collected retrospectively and compared with the literature. Read More

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http://dx.doi.org/10.1186/s42358-018-0010-3DOI Listing
June 2018
2 Reads

Relapsing granulomatosis with polyangiitis provoked by trauma: A case report.

Medicine (Baltimore) 2019 Jan;98(3):e14189

Department of Rheumatology, The Second Affiliated Hospital, Dalian Medical University, Dalian 116023, China.

Rationale: The precise cause of granulomatosis with polyangiitis (GPA) remains unclear. Herein we present a case of refractory GPA occurring after trauma.

Patient Concerns: A 43-year-old man suffered fractures of the left orbital in an accident, and then received surgical repair. Read More

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http://dx.doi.org/10.1097/MD.0000000000014189DOI Listing
January 2019
2 Reads

Serum cytokine and chemokine levels in patients with eosinophilic granulomatosis with polyangiitis, hypereosinophilic syndrome, or eosinophilic asthma.

Clin Exp Rheumatol 2019 Jan 14. Epub 2019 Jan 14.

Division of Rheumatology and Department of Biostatistics, Epidemiology, and Informatics, University of Pennsylvania, Philadelphia, PA, USA.

Objectives: The pathogenesis of eosinophilic granulomatosis with polyangiitis (EGPA) remains poorly understood, and may overlap with eosinophilic asthma and primary hypereosinophilic syndrome (HES). The aim of this study was to analyse a panel of serum cytokines and chemokines as markers of disease activity in patients with these conditions.

Methods: The levels of 54 cytokines and chemokines were measured in the sera of 40 patients with active EGPA, 10 of these patients during inactive disease, 6 patients with HES, 8 with asthma, and 10 healthy controls. Read More

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January 2019
3 Reads

Circulating CD24hiCD38hi regulatory B cells correlate inversely with the ThEM17 cell frequency in granulomatosis with polyangiitis patients.

Rheumatology (Oxford) 2019 Jan 10. Epub 2019 Jan 10.

Department of Rheumatology and Clinical Immunology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.

Objectives: To investigate whether there is a direct relation between expanded proportions of Th17 effector memory (ThEM17) cells and regulatory B cells (Bregs) in peripheral blood of granulomatosis with polyangiitis (GPA) patients.

Methods: Frequencies of Bregs and ThEM17 cells, as well as ThEM1 cells, were determined by flow cytometry in blood samples from 42 GPA patients in remission and 18 matched healthy controls (HCs). The Breg frequency was defined as CD24hiCD38hiCD19+ cells. Read More

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https://academic.oup.com/rheumatology/advance-article/doi/10
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http://dx.doi.org/10.1093/rheumatology/key412DOI Listing
January 2019
6 Reads

Hypertrophic pachymeningitis as an important neurological complication of granulomatosis with polyangiitis.

Reumatologia 2018 23;56(6):399-405. Epub 2018 Dec 23.

Clinic of Internal Medicine, Connective Tissue Diseases, and Geriatrics, Medical University of Gdańsk, Poland.

Headache is a common symptom in patients with granulomatosis with polyangiitis (GPA) mainly due to chronic sinusitis or orbital disease. Meningeal involvement may thus remain unrecognized for a long time. This can lead to a significant delay in accurate diagnosis, serious local damage of the central nervous system and high relapse rates. Read More

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http://dx.doi.org/10.5114/reum.2018.80719DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6330683PMC
December 2018
1 Read

Silent acute myocarditis in eosinophilic granulomatosis with polyangiitis.

Acta Reumatol Port 2018 Oct-Dec;43(4):309-313

São João Hospital Centre.

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. Cardiomyopathy and congestive heart failure can occur but a significant proportion of patients are asymptomatic. Read More

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January 2019
2 Reads

A Perianal Mass Associated With Eosinophilic Granulomatosis With Polyangiitis: Challenge.

Am J Dermatopathol 2019 Jan 11. Epub 2019 Jan 11.

Department of Pathology, Cork University Hospital, Cork, Ireland.

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http://dx.doi.org/10.1097/DAD.0000000000001338DOI Listing
January 2019
2 Reads

[Differential diagnosis of hypereosinophilia].

Z Rheumatol 2019 Jan 11. Epub 2019 Jan 11.

Vaskulitiszentrum-Süd, Klinik für Innere Medizin, Rheumatologie und Immunologie, Medius Kliniken, Akademisches Lehrkrankenhaus, Universität Tübingen, Kirchheim u. Teck, Deutschland.

Eosinophilia is defined as an elevated absolute number of eosinophilic leukocytes in peripheral blood or tissue. Its absolute number also defines the grade of eosinophilia. The main causes are allergic (including drug side effects) and infectious triggers but malignant and autoimmune diseases can also result in eosinophilia. Read More

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http://dx.doi.org/10.1007/s00393-018-0587-2DOI Listing
January 2019
1 Read

Pregnancy in a patient with eosinophilic granulomatosis with polyangiitis.

J Obstet Gynaecol 2019 Jan 11:1-2. Epub 2019 Jan 11.

a Medical School 3rd Department of Obstetrics and Gynecology, Attikon Hospital , National and Kapodistrian University of Athens , Athens , Greece.

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http://dx.doi.org/10.1080/01443615.2018.1536879DOI Listing
January 2019
3 Reads

Face Transplantation for Granulomatosis With Polyangiitis (Wegener Granulomatosis): Technical Considerations, Immunological Aspects, and 3-Year Posttransplant Outcome.

Ann Plast Surg 2019 Mar;82(3):320-329

Department of Orthopedics, University of Illinois, Chicago, IL.

Background: We report new data for a rare face transplant performed 3 years ago. Granulomatosis with polyangiitis (GPA) (Wegener) is a severe autoimmune necrotizing vasculitis and parenchymal inflammatory disease that can affect any organ including those of the craniofacial region. Skin involvement manifests as malignant pyoderma. Read More

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http://dx.doi.org/10.1097/SAP.0000000000001735DOI Listing
March 2019
8 Reads

[Vasculitis mimics].

Z Rheumatol 2019 Feb;78(1):24-30

Internistisch-rheumatologische Praxis, Jürgensallee 44, 22609, Hamburg, Deutschland.

Vasculitis mimics need to be differentiated from primary and secondary vasculitides as described in the Chapel Hill nomenclature. The clinical symptomatology resembles that of vasculitis of small and medium, rarely also large vessels and hence imitates the classical vasculitic disorders. Pathogenetically, the causes are partly genetic mutations, embolization syndromes, infections and substance abuse. Read More

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http://link.springer.com/10.1007/s00393-018-0581-8
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http://dx.doi.org/10.1007/s00393-018-0581-8DOI Listing
February 2019
7 Reads

[Current treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)].

Authors:
F Moosig J Holle

Z Rheumatol 2019 Jan 9. Epub 2019 Jan 9.

Rheumazentrum Schleswig-Holstein Mitte, Kuhberg 5a-7, 24534, Neumünster, Deutschland.

For the treatment of eosinophilic granulomatosis with polyangiitis (EGPA) much less data are available when compared to the other anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). At the same time EGPA also differs in many aspects from AAVs. Treatment is guided by the German and international guidelines. Read More

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http://link.springer.com/10.1007/s00393-018-0580-9
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http://dx.doi.org/10.1007/s00393-018-0580-9DOI Listing
January 2019
6 Reads

Clinical characteristics of patients with granulomatosis with polyangiitis and microscopic polyangiitis in ENT practice: a comparative analysis.

Acta Otorhinolaryngol Ital 2018 Dec;38(6):517-527

Department and Clinic of Otolaryngology, Head and Neck Surgery, Wrocław Medical University Hospital, Borowska, Poland.

ENT manifestations are commonly observed in patients with small vessel vasculitis (SVV). The main aim of this study was to analyse and present the clinicopathological characteristics of individuals with SVV emphasising otorhinolaryngological symptoms. This study evaluated 64 patients, 41 with granulomatosis with polyangiitis (GPA) and 23 with microscopic polyangiitis (MPA). Read More

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http://dx.doi.org/10.14639/0392-100X-1776DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6325653PMC
December 2018
2 Reads

Molecular targeted therapies for microscopic polyangiitis and granulomatosis with polyangiitis.

Korean J Intern Med 2019 Jan 9. Epub 2019 Jan 9.

Department of Rheumatology, Ome Municipal General Hospital, Tokyo, Japan.

Clinical trials and observational studies have established cyclophosphamide (CY) or rituximab plus glucocorticoid (GC) as standard remission induction therapies in patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). However, because these regimens are associated with serious adverse drug reactions, the development of drugs with novel mechanisms of actions are needed. Progress in basic and clinical research has identified novel candidate targeting molecules, including B-cell activating factor (BAF), C5a receptor, and interleukin-6. Read More

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http://kjim.org/journal/view.php?doi=10.3904/kjim.2018.366
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http://dx.doi.org/10.3904/kjim.2018.366DOI Listing
January 2019
13 Reads

[A renal pseudotumor].

Rev Med Interne 2018 Dec 31. Epub 2018 Dec 31.

Service de médecine interne et immunologie clinique, CHU Dijon-Bourgogne, 2, boulevard Mal-de-Lattre-de-Tassigny, 21039 Dijon cedex, France.

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http://dx.doi.org/10.1016/j.revmed.2018.12.006DOI Listing
December 2018
1 Read

Refractory diffuse alveolar hemorrhage caused by eosinophilic granulomatosis with polyangiitis in the absence of elevated biomarkers treated successfully by rituximab and mepolizumab: A case report.

Respir Med Case Rep 2019 15;26:112-114. Epub 2018 Dec 15.

Department of Pulmonology, Kameda Medical Center, Kamogawa, Chiba, Japan.

Here we report on a 61-year-old man with refractory eosinophilic granulomatosis with polyangiitis (EGPA) who presented with dyspnea. Despite treatment with glucocorticoids, intravenous cyclophosphamide, and plasma exchange, his symptoms worsened despite his eosinophil count and myeloperoxidase antineutrophil cytoplasmic antibody titer trending downwards. EGPA with diffuse alveolar hemorrhage was diagnosed on analysis of bronchoalveolar lavage fluid. Read More

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http://dx.doi.org/10.1016/j.rmcr.2018.12.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6302118PMC
December 2018
1 Read

Clinical features of hypertrophic pachymeningitis in a center survey.

Neurol Sci 2018 Dec 26. Epub 2018 Dec 26.

Neurology Department, Hospital de Santo António, Centro Hospitalar do Porto, Porto, Portugal.

Background: Hypertrophic pachymeningitis (HP) is characterized by cranial and/or spinal thickening of the dura mater with or without associated inflammation. Neuroimaging studies reveal dura mater thickening and focal or diffuse contrast enhancement. It is described in association with trauma, infections, tumors, autoimmune/inflammatory diseases, and cerebrospinal fluid hypotension syndrome, with some cases remaining idiopathic. Read More

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http://link.springer.com/10.1007/s10072-018-3689-3
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http://dx.doi.org/10.1007/s10072-018-3689-3DOI Listing
December 2018
9 Reads

Main Oral Manifestations in Immune-Mediated and Inflammatory Rheumatic Diseases.

J Clin Med 2018 Dec 25;8(1). Epub 2018 Dec 25.

Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Internal Medicine, IRCCS Fondazione Ca' Granda, Ospedale Maggiore Policlinico, 20122 Milano, Italy.

Oral manifestations are frequent in patients with rheumatic diseases. The aim of this review is to offer readers practical advice concerning the onset, diagnosis and treatment of the main oral manifestations encountered in rheumatological and dental clinics. Signs and symptoms such as oral hyposalivation, xerostomia, temporomandibular joint disorders, periodontal disease, and dysphagia may be the first expression of a number of rheumatic diseases. Read More

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http://www.mdpi.com/2077-0383/8/1/21
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http://dx.doi.org/10.3390/jcm8010021DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6351952PMC
December 2018
8 Reads

Evaluation of clinical benefit from treatment with mepolizumab for patients with eosinophilic granulomatosis with polyangiitis.

J Allergy Clin Immunol 2018 Dec 19. Epub 2018 Dec 19.

Department of Medicine, National Jewish Health, Denver, Colo. Electronic address:

Background: 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.

Objective: 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.

Methods: The randomized, placebo-controlled, double-blind, parallel-group trial recruited patients with relapsing/refractory EGPA receiving stable OGCs (prednisolone/prednisone, ≥7. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00916749183278
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http://dx.doi.org/10.1016/j.jaci.2018.11.041DOI Listing
December 2018
7 Reads

Case 39-2018: An 18-Year-Old Man with Diplopia and Proptosis of the Left Eye.

N Engl J Med 2018 12;379(25):2452-2461

From the Departments of Ophthalmology (D.R.L., M.K.Y.) and Radiology (K.L.R.), Massachusetts Eye and Ear, the Departments of Medicine (J.H.S.) and Pathology (A.M.S.), Massachusetts General Hospital, and the Departments of Ophthalmology (D.R.L., M.K.Y.), Radiology (K.L.R.), Medicine (J.H.S.), and Pathology (A.M.S.), Harvard Medical School - all in Boston.

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http://dx.doi.org/10.1056/NEJMcpc1807503DOI Listing
December 2018
2 Reads