8,652 results match your criteria Wegener Granulomatosis


Updates in the treatment of Granulomatosis with Polyangiitis and Microscopic Polyangiitis.

Authors:
S Garner N Khalidi

Presse Med 2020 Jul 4:104038. Epub 2020 Jul 4.

McMaster University, Hamilton, Ontario, Canada.

There have been great advances in the management of ANCA associated vasculitis over the past decades. We have gone from an era where the disease was almost universally fatal to trying to prevent long term side effects of treatment regimens. With the ability to use pulse cyclophosphamide or rituximab as alternates to oral cyclophosphamide for induction of remission, side effects of therapy have been greatly reduced. Read More

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http://dx.doi.org/10.1016/j.lpm.2020.104038DOI Listing

Nasal reconstructive surgery for vasculitis affecting the nose: our two-centre international experience.

Eur Arch Otorhinolaryngol 2020 Jul 4. Epub 2020 Jul 4.

Royal National ENT and Eastman Dental Hospitals, 47-49 Huntley Street, London, WC1E 6DG, UK.

Purpose: To recommend an international multidisciplinary medical and surgical algorithm of treatment in nasal vasculitis, which will create a more streamlined approach.

Methods: A two-centre, international retrospective analysis of granulomatosis with polyangiitis (GPA) and levamisole-associated vasculitis (LAV) cases presenting between 2005 and 2019 was carried out. Demographic data, and surgical and medical treatment were recorded. Read More

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http://dx.doi.org/10.1007/s00405-020-06180-8DOI Listing

Rituximab maintenance therapy for patients with antineutrophil cytoplasmic antibody-associated vasculitis in Japan.

Mod Rheumatol 2020 Jul 2:1-25. Epub 2020 Jul 2.

Department of Rheumatology, Faculty of Medicine, Saga University, Saga, Japan.

We examined the efficacy and safety of rituximab (RTX) maintenance therapy for patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Japan. We conducted a retrospective study using a multi-center cohort database of vasculitis patients. All maintenance treatment courses were divided into three groups: a RTX group, a group treated with other immunosuppressant drugs (IS) and a group receiving glucocorticoid monotherapy (GC). Read More

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http://dx.doi.org/10.1080/14397595.2020.1790778DOI Listing

Granulomatosis with polyangiitis: a life-threatening cause of granulomatous mastitis.

ANZ J Surg 2020 Jul 2. Epub 2020 Jul 2.

Anatomical Pathology Department, Waikato District Health Board, Hamilton, New Zealand.

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http://dx.doi.org/10.1111/ans.16106DOI Listing

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

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

Granulomatosis with polyangiitis and pregnancy: Anti-neutrophil cytoplasmic antibody, placental inflammation, chorangiosis and pre-eclampsia.

J Obstet Gynaecol Res 2020 Jul 1. Epub 2020 Jul 1.

Division of Perinatology, Department of Obstetrics and Gynecology, Hacettepe University, Ankara, Turkey.

Granulomatosis with polyangiitis (GPA) is a rare necrotizing autoimmune disease involving small vessel vasculitis. Pregnancies with GPA have increased rates of obstetric complications including pre-eclampsia. Differential diagnosis of GPA flares up and pre-eclampsia may be difficult and necessitates careful clinical practice. Read More

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http://dx.doi.org/10.1111/jog.14356DOI Listing

Facial necrotic ulcerative lesions in an adolescent female with granulomatosis with polyangiitis (GPA).

Clin Rheumatol 2020 Jul 1. Epub 2020 Jul 1.

Department of Pediatrics, Attikon General Hospital, National and Kapodistrian University of Athens, 1 Rimini Str., 124 62, Athens, Greece.

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http://dx.doi.org/10.1007/s10067-020-05264-4DOI Listing

IgG4-related Paravertebral Mass and Peri-aortitis.

Intern Med 2020 Jun 30. Epub 2020 Jun 30.

Department of General Medicine, National Defense Medical College, Japan.

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http://dx.doi.org/10.2169/internalmedicine.4770-20DOI Listing

Sweet Syndrome in Eosinophilic Granulomatosis with Polyangiitis.

J Rheumatol 2020 Jul;47(7):1031-1032

Division of Arthritis & Rheumatic Diseases, Oregon Health & Science University, Portland, Oregon, USA.

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http://dx.doi.org/10.3899/jrheum.190909DOI Listing

The Proximal Airway Is a Reservoir for Adaptive Immunologic Memory in Idiopathic Subglottic Stenosis.

Laryngoscope 2020 Jun 30. Epub 2020 Jun 30.

Department of Medicine, Division of Infectious Disease, Vanderbilt University Medical Center, Nashville, Tennessee, U.S.A.

Objectives/hypothesis: Characterization of the localized adaptive immune response in the airway scar of patients with idiopathic subglottic stenosis (iSGS).

Study Design: Basic Science.

Methods: Utilizing 36 patients with subglottic stenosis (25 idiopathic subglottic stenosis [iSGS], 10 iatrogenic post-intubation stenosis [iLTS], and one granulomatosis with polyangiitis [GPA]) we applied immunohistochemical and immunologic techniques coupled with RNA sequencing. Read More

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http://dx.doi.org/10.1002/lary.28840DOI Listing

"Headache and dural enhancement: two case studies of different treatable pathologies".

World Neurosurg 2020 Jun 25. Epub 2020 Jun 25.

Department of Biomedical and Neuromotor Sciences, University of Bologna, Italy; IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy.

Background: Hypertrophic pachymeningitis and spontaneous intracranial hypotension are different treatable diseases which should promptly be recognized and treated to prevent neurological sequelae. Headache and dural enhancement are the main features of both diseases, thus differentiating between these two conditions can be difficult.

Cases Description: We present two cases with headache and dural enhancement, in which the differential diagnosis was challenging at presentation because, in both cases, clear positional pain modification was not reported. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.06.126DOI Listing

Response to: 'Eosinophilic granulomatosis with polyangiitis can manifest lacrimal and salivary glands swelling by granulomatous inflammation: a potential mimicker of IgG4-related disease' by Akiyama .

Ann Rheum Dis 2020 Jun 26. Epub 2020 Jun 26.

Ludwig Boltzmann Institute of Osteology at Hanusch Hospital of Österreichische Gesundheitskasse (ÖGK) and Research Funds of the Austrian Workers Compensation Board (AUVA),1st Medical Department, Hanusch Hospital, Wien, Austria.

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http://dx.doi.org/10.1136/annrheumdis-2020-218174DOI Listing

Eosinophilic granulomatosis with polyangiitis can manifest lacrimal and salivary glands swelling by granulomatous inflammation: a potential mimicker of IgG4-related disease.

Ann Rheum Dis 2020 Jun 26. Epub 2020 Jun 26.

Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan.

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http://dx.doi.org/10.1136/annrheumdis-2020-218124DOI Listing

International Consensus on ANCA Testing in Eosinophilic Granulomatosis with Polyangiitis.

Am J Respir Crit Care Med 2020 Jun 25. Epub 2020 Jun 25.

University of Alberta, Medicine, Edmonton, Alberta, Canada.

An international consensus on anti-neutrophil cytoplasm antibodies (ANCA) testing in eosinophilic granulomatosis with polyangiitis (EGPA) is presented. ANCA, specific for myeloperoxidase (MPO), can be detected in 30-35% of EGPA patients. MPO-ANCA should be tested with antigen-specific immunoassays in any patient with eosinophilic asthma and clinical features suggesting EGPA, including constitutional symptoms, purpura, polyneuropathy, unexplained heart, gastrointestinal or kidney disease, and/or pulmonary infiltrates or hemorrhage. Read More

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http://dx.doi.org/10.1164/rccm.202005-1628SODOI Listing

Serum Mannose-Binding Lectin Levels Are Correlated with the Disease Activity of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Single-Center Study.

Tohoku J Exp Med 2020 06;251(2):117-123

Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine.

Mannose-binding lectin (MBL) is a soluble pattern-recognition molecule, which plays a crucial role in the innate immune system and the activation of lectin complement pathway. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is an autoimmune disease affecting the small vasculatures and is characterized by the alteration of innate and adaptive immunity and complement activation. In this study, we investigated whether serum MBL is associated with disease activity of AAV, which was measured by ELISA. Read More

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http://dx.doi.org/10.1620/tjem.251.117DOI Listing

Rituximab as therapy to induce remission after relapse in ANCA-associated vasculitis.

Ann Rheum Dis 2020 Jun 24. Epub 2020 Jun 24.

University of Cambridge, Cambridge, UK.

Objectives: Evaluation of rituximab and glucocorticoids as therapy to induce remission after relapse in ANCA-associated vasculitis (AAV) in a prospective observational cohort of patients enrolled into the induction phase of the RITAZAREM trial.

Methods: Patients relapsing with granulomatosis with polyangiitis or microscopic polyangiitis were prospectively enrolled and received remission-induction therapy with rituximab (4×375 mg/m) and a higher or lower dose glucocorticoid regimen, depending on physician choice: reducing from either 1 mg/kg/day or 0.5 mg/kg/day to 10 mg/day by 4 months. Read More

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http://dx.doi.org/10.1136/annrheumdis-2019-216863DOI Listing

Posterior reversible encephalopathy syndrome and digital gangrene in a patient with granulomatosis with polyangiitis - a rare case report.

Acta Reumatol Port 2020 Jan-Mar;45(1):65-68

Hospital de Santa Maria, Centro Hospitalar Universitário de Lisboa Norte, Centro Académico de Medicina de Lisboa.

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Cavernous sinus lesions.

Clin Imaging 2020 Jun 18;68:71-89. Epub 2020 Jun 18.

NYU Langone Health, Department of Radiology, New York, NY, United States of America. Electronic address:

The cavernous sinus is a complex structure susceptible to a wide variety of vascular, neoplastic and inflammatory pathologies. Vascular pathologies include ICA aneurysms, carotid-cavernous fistulas, cavernous sinus thrombosis, and cavernous hemangioma. Neoplasms that involve the cavernous sinus include pituitary adenoma, meningioma, schwannoma, lymphoma, perineural tumor spread, metastases, and direct tumor invasion. Read More

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http://dx.doi.org/10.1016/j.clinimag.2020.06.029DOI Listing

Reduction of Myeloperoxidase-Antineutrophil Cytoplasmic Antibody by Mepolizumab in Eosinophilic Granulomatosis With Polyangiitis.

J Clin Rheumatol 2020 Jun 18. Epub 2020 Jun 18.

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

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http://dx.doi.org/10.1097/RHU.0000000000001453DOI Listing

Demographic and clinical characteristics of patients with ANCA-positive vasculitis in a Colombian University Hospital over a 12-year period: 2005-2017.

Rheumatol Int 2020 Aug 20;40(8):1283-1290. Epub 2020 Jun 20.

Department of Internal Medicine, School of Medicine, Pontificia Universidad Javeriana, Bogotá, Colombia.

Vasculitides associated with anti-neutrophil cytoplasmic antibodies are heterogeneous, systemic, low prevalence and high morbidity and mortality entities. They include granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. In Latin America, there are few descriptive registries of these patients. Read More

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http://dx.doi.org/10.1007/s00296-020-04631-3DOI Listing

Granulomatosis with polyangiitis (wegener's).

Authors:
Xavier Puéchal

Joint Bone Spine 2020 Jun 17. Epub 2020 Jun 17.

Centre de Référence des Maladies Systémiques Auto-immunes rares, Université Paris Descartes, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, 27, rue du faubourg Saint Jacques, 75014, Paris, France; Institut Cochin, INSERM U1016, CNRS UMR 8104, Paris, France. Electronic address:

Granulomatosis with polyangiitis (GPA) (Wegener) is a necrotizing vasculitis combining inflammation of the vascular wall and peri- and extravascular granulomatosis. Clinically, GPA is characterized in its full form by ENT signs, lung, and kidney involvement. One of the key features of GPA is the presence of ANCAs - cytoplasmic in approximately 90% of systemic forms and in 50% of localized forms - directed against proteinase 3 in most cases. Read More

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http://dx.doi.org/10.1016/j.jbspin.2020.06.005DOI Listing

Eosinophilic Granulomatosis With Polyangiitis: An Unusual Case of Pediatric Subglottic Stenosis.

Laryngoscope 2020 Jun 20. Epub 2020 Jun 20.

Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, U.S.A.

Systemic disease is an uncommon cause of subglottic stenosis (SGS). We report a case of severe SGS due to underlying eosinophilic granulomatosis with polyangiitis (EGPA) in a child presenting with isolated stridor. EGPA is a rare systemic vasculitis with very limited cases reported in the pediatric population. Read More

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http://dx.doi.org/10.1002/lary.28848DOI Listing

Cardiogenic shock in autoimmune rheumatologic diseases: an insight on etiologies, management, and treatment outcomes.

Heart Fail Rev 2020 Jun 20. Epub 2020 Jun 20.

Heart and Vascular Institute, West Virginian University, 1 Medical Center Dr., Morgantown, WV, 26505, USA.

Autoimmune rheumatological disorders are known to have an increased risk for cardiovascular diseases including coronary artery disease (CAD), myocarditis, pericarditis, valvulopathy, and in consequence cardiogenic shock. Data on cardiogenic shock in rheumatological diseases are scarce; however, several reports have highlighted this specific entity. We sought to review the available literature and highlight major outcomes and the management approaches in each disease. Read More

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http://dx.doi.org/10.1007/s10741-020-09990-4DOI Listing

COVID-19 and granulomatosis with polyangiitis (GPA): a diagnostic challenge.

Rheumatology (Oxford) 2020 Jun 17. Epub 2020 Jun 17.

Department of Radiology, Jahrom University of Medical Science, Jahrom, Iran.

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http://dx.doi.org/10.1093/rheumatology/keaa326DOI Listing

Trends in the Inpatient Burden of Coronary Artery Disease in Granulomatosis with Polyangiitis: A Study of Large National Dataset.

J Rheumatol 2020 Jun 15. Epub 2020 Jun 15.

National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD; Department of Medicine, Weill Cornell Medicine, New York, NY; Department of Medicine, Mount Sinai Morningside and Mount Sinai West, Icahn School of Medicine at Mount Sinai, New York, NY; Section of Cardiology, Department of Medicine, Baylor College of Medicine, Houston, TX; Department of Medicine, Mount Sinai Morningside and Mount Sinai West, Icahn School of Medicine at Mount Sinai, New York, NY; Department of Medicine, St. Vincent's Medical Center, Bridgeport, CT; Department of Medicine, Mount Sinai Morningside and Mount Sinai West, Icahn School of Medicine at Mount Sinai, New York, NY; Division of General Internal Medicine, Toronto General Hospital Research Institute, University of Toronto, Toronto, Ontario, Canada; Department of Healthcare Policy and Research, Weill Cornell Medicine, New York, NY; Department of Rheumatology, Hospital for Special Surgery, Weill Cornell Medicine, New York, NY. Corresponding author: Bella Mehta, 500 East 70th Street, New York, NY, 10021. Phone: 212-606-1987. Email:

Objective: Cardiovascular diseases are serious comorbidities in patients with granulomatosis with polyangiitis (GPA). In a sample of patients hospitalized for GPA, we sought to examine trends in the burden of coronary artery disease (CAD) and its two serious manifestations, acute myocardial infraction (AMI) and heart failure (HF).

Methods: We used the National Inpatient Sample to conduct a retrospective cross-sectional analysis. Read More

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http://dx.doi.org/10.3899/jrheum.200374DOI Listing
June 2020
3.187 Impact Factor

Favorable efficacy of rituximab in ANCA-associated vasculitis patients with excessive B cell differentiation.

Arthritis Res Ther 2020 06 15;22(1):141. Epub 2020 Jun 15.

School of Medicine, University of Occupational & Environmental Health, 1-1 Iseigaoka, Yahata-nishi, Kitakyushu, Fukuoka, 807-8555, Japan.

Objectives: B cell depletion by rituximab (RTX) is an effective treatment for anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). However, peripheral B cell phenotypes and the selection criteria for RTX therapy in AAV remain unclear.

Methods: Phenotypic characterization of circulating B cells was performed by 8-color flow cytometric analysis in 54 newly diagnosed AAV patients (20 granulomatosis with polyangiitis and 34 microscopic polyangiitis). Read More

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http://dx.doi.org/10.1186/s13075-020-02215-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7294638PMC

[MPO-ANCA-associated hypertrophic pachymeningitis with monoclonal gammopathy of undetermined significance: a case report].

Rinsho Shinkeigaku 2020 Jun 13. Epub 2020 Jun 13.

Department of Neurology, National Hospital Organization Sendai Medical Center.

A 66-year-old woman presented with dysesthesia over the right side of her face, hypoglossal nerve dysfunction, dysphagia, and dysgeusia of the right side. A MRI scan of the brain revealed cerebral dural thickening on the right side of the skull base, and histopathological examination revealed granulomatous inflammation of the dura. Based on paranasal sinusitis, bronchodilatation, laboratory tests showing weakly positive MPO-ANCA, intact renal function, and the patient's favorable response to steroids, we diagnosed the patient with limited granulomatosis with polyangiitis (GPA). Read More

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http://dx.doi.org/10.5692/clinicalneurol.60.cn-001411DOI Listing

Bowman's capsule rupture on renal biopsy improves the outcome prediction of ANCA-associated glomerulonephritis classifications.

Ann Rheum Dis 2020 Jun 12. Epub 2020 Jun 12.

U.O.C. Nefrologia, Fondazione Policlinico Universitario A. Gemelli IRCCS, Roma, Italia.

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http://dx.doi.org/10.1136/annrheumdis-2020-217979DOI Listing

A Dishevelled Man with Inflammatory Bowel Disease and Signs of Infection.

Gastroenterology 2020 Jun 5. Epub 2020 Jun 5.

I. Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

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http://dx.doi.org/10.1053/j.gastro.2020.05.075DOI Listing

Management of Primary Small-Vessel Vasculitis.

Skin Therapy Lett 2020 05;25(3):5-8

Center for Clinical Studies, Houston, TX, USA.

Small-vessel vasculitides (SVV) are a group of disorders that occur due to primarily systemic inflammation or as sequelae of an infection, malignancy, or other rheumatic disease. Arising in any organ including the skin, the clinical features of SVV encompass a variety of manifestations. A comprehensive diagnostic assessment should be performed as management protocols widely differ. Read More

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Strawberry gingivitis - First sign of Wegener's granulomatosis.

J Oral Maxillofac Pathol 2020 Jan-Apr;24(1):172-175. Epub 2020 May 8.

Department of Periodontology and Implantology, Government Dental College and Hospital, Aurangabad, Maharashtra, India.

Wegener's Granulomatosis (WG) is an immunologically mediated rare multisystem disease characterized by necrotizing granulomatous inflammation affecting the upper and lower respiratory tracts, disseminated vasculitis and glomerulonephritis. WG oral lesions are reported to occur in 6%-13% of patients; they were an initial presenting feature in only 2% of cases. A case of strawberry gingivitis as the first sign of WG presented with erythematous and painful localized gingival enlargement affecting the maxillary anterior region of jaw in a 17-year-old boy is reported here. Read More

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http://dx.doi.org/10.4103/jomfp.JOMFP_3_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7269271PMC

Benralizumab as a glucocorticoid-sparing treatment option for severe asthma in eosinophilic granulomatosis with polyangiitis.

J Allergy Clin Immunol Pract 2020 Jun 4. Epub 2020 Jun 4.

Rheumatology Unit, Department of Medicine DIMED, University of Padova, Padova, Italy. Electronic address:

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http://dx.doi.org/10.1016/j.jaip.2020.05.033DOI Listing

Risk factors and prevention of Pneumocystis jirovecii pneumonia in patients with autoimmune and inflammatory diseases.

Chest 2020 Jun 2. Epub 2020 Jun 2.

AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Department of Internal Medicine and Clinical Immunology, F-75013, Paris, France; Centre national de références Maladies Autoimmunes systémiques rares et Centre national de références Maladies Autoinflammatoires et amylose inflammatoire; Sorbonne Universités, UPMC Université Paris 6, INSERM, UMR S 959, Immunology-Immunopathology- Immunotherapy (I3); F-75005, Paris, France. Electronic address:

Patients with autoimmune and/or inflammatory diseases (AID) are prone to serious infectious complications such as Pneumocystis Jirovecii pneumonia (PJP). In non-HIV patients, the prognosis is poorer and diagnosis tests are of lower sensitivity. Given the low incidence of PJP in AID, with the exception of granulomatosis with polyangiitis, and the non-negligible side effects of chemoprophylaxis, routine prescription of primary prophylaxis is still debated. Read More

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http://dx.doi.org/10.1016/j.chest.2020.05.558DOI Listing

Biologic therapy in severe and refractory peripheral ulcerative keratitis (PUK). Multicenter study of 34 patients.

Semin Arthritis Rheum 2020 May 15;50(4):608-615. Epub 2020 May 15.

Rheumatology, Ophthalmology and Internal medicine, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain. Electronic address:

Purpose: We assessed the efficacy and safety of biologic therapy in severe and refractory Peripheral Ulcerative Keratitis (PUK).

Design: Open-label multicenter study of biologic-treated patients with severe PUK refractory to conventional immunosuppressive drugs.

Subjects: We studied 34 patients (44 affected eyes) (24 women/10 men; mean age, 55. Read More

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http://dx.doi.org/10.1016/j.semarthrit.2020.03.023DOI Listing

Eosinophilic granulomatosis with polyangiitis mimicking coronavirus disease 2019: a case report.

J Eur Acad Dermatol Venereol 2020 Jun 4. Epub 2020 Jun 4.

Departmant of Pulmonology, Iwata City Hospital, Iwata, Japan.

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http://dx.doi.org/10.1111/jdv.16705DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7300604PMC

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

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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

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January 2020

Abdominal Pain, an Atypical Presenting Symptom of Granulomatosis with Polyangiitis.

Cureus 2020 Apr 27;12(4):e7864. Epub 2020 Apr 27.

Internal Medicine, Allegheny General Hospital, Pittsburgh, USA.

Granulomatosis with polyangiitis (GPA) formerly known as Wegener's granulomatosis, is an anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). It is an uncommon disease with an estimated prevalence of 3 in 100,000 individuals with an equal distribution in both sexes. It is characterized by necrotizing granulomatous vasculitis that primarily affects the upper and lower respiratory tracts and the kidneys. Read More

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http://dx.doi.org/10.7759/cureus.7864DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7255084PMC

Long-Term Rituximab Use to Maintain Remission of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Randomized Trial.

Ann Intern Med 2020 Jun 2. Epub 2020 Jun 2.

Cochin Hospital, Paris Descartes University, Paris, France (P.C., C.V., X.P., B.T., L.M., L.G.).

Background: Biannual rituximab infusions over 18 months effectively maintain remission after a "standard" remission induction regimen for patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV).

Objective: To evaluate the efficacy of prolonged rituximab therapy in preventing AAV relapses in patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) who have achieved complete remission after completing an 18-month maintenance regimen.

Design: Randomized controlled trial. Read More

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http://dx.doi.org/10.7326/M19-3827DOI Listing
June 2020
17.810 Impact Factor

An unusual neurological manifestation of granulomatosis with polyangiitis: A case report and literature review.

Clin Case Rep 2020 May 29;8(5):862-866. Epub 2020 Feb 29.

Baton Rouge General Internal Medicine Residency Program Baton Rouge General Medical Center Baton Rouge LA USA.

Ischemic stroke is an incredibly rare manifestation of granulomatosis with polyangiitis. It is important for the clinician to be aware of this unusual complication so that efforts can be made to reduce the risk of this event. Read More

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http://dx.doi.org/10.1002/ccr3.2765DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7250989PMC

High incidence of venous thromboembolism but not of coronary artery disease in granulomatosis with polyangiitis in first years after diagnosis.

Sarcoidosis Vasc Diffuse Lung Dis 2019 1;36(3):202-208. Epub 2019 May 1.

Medical University of Warsaw, Poland.

Objectives: Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is one of antineutrophil cytoplasmic autoantibody (ANCA) - associated vasculitis. In patients with GPA an increased incidence of venous thromboembolism (VTE), mainly during active disease, has been described. The aim of the present study was to assess the incidence of VTE and its relation with classic risk factors for atherosclerosis, presence of coronary artery disease (CAD), echocardiographic parameters and laboratory findings in GPA patients. Read More

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http://dx.doi.org/10.36141/svdld.v36i3.8088DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7247083PMC

Long-Term Safety of Rituximab in Granulomatosis with Polyangiitis or Microscopic Polyangiitis.

Arthritis Care Res (Hoboken) 2020 May 31. Epub 2020 May 31.

F. Hoffmann-La Roche Ltd, Basel, Switzerland.

Objective: A Phase IV, open-label, prospective study was conducted to characterize the long-term safety of rituximab in a 4-year observational registry of adult patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) within the United States.

Methods: Patients initiating treatment with rituximab were evaluated every 6 months for up to 4 years. Outcomes were the incidence of serious adverse events (SAEs), infusion-related reactions (IRRs), and SAEs of specific interest, including serious infections, serious cardiac events, serious vascular events, and malignancies. Read More

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http://dx.doi.org/10.1002/acr.24332DOI Listing

Treatment of Granulomatosis with Polyangiitis and Microscopic Polyangiitis: Should Type of ANCA Guide the Treatment?

Clin J Am Soc Nephrol 2020 May 29. Epub 2020 May 29.

Department of Nephrology, First Faculty of Medicine and General University Hospital, Charles University, Prague, Czech Republic.

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http://dx.doi.org/10.2215/CJN.15861219DOI Listing

Clinical Characteristics of an Internet-Based Cohort of Patients with a Self-Reported Diagnosis of Granulomatosis with Polyangiitis or Microscopic Polyangiitis: Observational Study.

J Med Internet Res 2020 May 14. Epub 2020 May 14.

Division of Rheumatology, Department of Medicine, University of Pennsylvania, Philadelphia, US.

Background: The conduct of clinical trials in rare diseases utilizing the traditional centers of excellence approach remains challenging. Patient-based registries have been shown to be both feasible and valid in several other diseases.

Objective: This report outlines the clinical characteristics of a large internet registry cohort of participants with a self-reported diagnosis of granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA). Read More

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http://dx.doi.org/10.2196/17231DOI Listing

[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

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

Three Patterns of Cutaneous Involvement in Granulomatosis with Polyangiitis.

J Adv Res 2020 Jul 7;24:311-315. Epub 2020 May 7.

National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, AP-HP, Paris Descartes University, Paris, France.

Background: Skin involvement in granulomatosis with polyangiitis (GPA) is common and can appear as an initial presentation of the disease or more commonly through its course.

Case Presentation: We report a case of a 24-year-old male patient, previously diagnosed as having GPA, admitted with fever, hemoptysis, generalized hemorrhagic blisters associated with arthralgia, fatigue, myalgia, nasal crusting, and vertigo. Three weeks prior to admission, he developed erythematous papules on both elbows, and purpuric papules on both lower limbs. Read More

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http://dx.doi.org/10.1016/j.jare.2020.05.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7235938PMC

Benralizumab as a Potential Adjunctive Therapy in Eosinophilic Granulomatosis With Polyangiitis.

J Clin Rheumatol 2020 May 21. Epub 2020 May 21.

Department of Internal Medicine, Loma Linda University Medical Center, Loma Linda, CA Department of Rheumatology, Loma Linda University Medical Center, Loma Linda, CA.

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http://dx.doi.org/10.1097/RHU.0000000000001419DOI Listing