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https://www.readbyqxmd.com/read/28231569/biomarkers-in-autoimmune-salivary-gland-disorders-a-review
#1
Benedikt Hofauer, Klaus Thuermel, Constanze Gahleitner, Andreas Knopf
Salivary glands are frequent sites of manifestations of autoimmune disorders in the head and neck. Sjögren syndrome, sarcoidosis, granulomatosis with polyangiitis, and IgG4-related sialadenitis represent the most important autoimmune salivary gland disorders. Due to the lack of specific symptoms, diagnosis of these conditions remains a challenge. Diagnosis is usually based on classification criteria involving clinical tests, histopathological evaluation, and serological examinations. Depending on the disease, biomarkers are of different value and have to be interpreted carefully...
February 24, 2017: ORL; Journal for Oto-rhino-laryngology and its related Specialties
https://www.readbyqxmd.com/read/28229830/neutrophil-extracellular-traps-formation-in-patients-with-eosinophilic-granulomatosis-with-polyangiitis-association-with-eosinophilic-inflammation
#2
Joanna Natorska, Michał Ząbczyk, Jakub Siudut, Piotr Krawiec, Lucyna Mastalerz, Anetta Undas
OBJECTIVES: Eosinophilic granulomatosis with polyangiitis (EGPA) is associated with an inflammation and the presence of antineutrophil cytoplasmic antibodies (ANCA). Thus, we investigated the impact of ANCAs and eosinophilic inflammation on neutrophil activation and extracellular traps (NETs) formation. METHODS: We recruited 29 patients in the remission of EGPA (17 ANCA-negative and 12 ANCA-positive, including 7 p-ANCA-positive and 5 c-ANCA-positive patients). Healthy donors' neutrophils were stimulated with EGPA patients' serum...
February 9, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28229825/anca-associated-pauci-immune-glomerulonephritis-always-pauci-immune
#3
Valeria Scaglioni, Marina Scolnik, Luis J Catoggio, Silvia B Christiansen, Carlos F Varela, Gustavo Greloni, Guillermo J Rosa-Diez, Enrique R Soriano
OBJECTIVES: Anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (GN) is considered "pauci-immune" with absent or mild glomerular tuft staining for immunoglobulin (Ig) and/or complement. However, it is not unusual to see some immune deposits (ID) within glomeruli on immunofluorescence (IF). We determined to evaluate the prevalence and clinical significance of immune deposits in ANCA-associated GN. METHODS: We included all patients with ANCA associated vasculitis with renal biopsies between January 2002 and May 2014: granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis and renal limited vasculitis...
January 31, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28225490/clinical-characteristics-and-outcome-of-spanish-patients-with-anca-associated-vasculitides-impact-of-the-vasculitis-type-anca-specificity-and-treatment-on-mortality-and-morbidity
#4
Roser Solans-Laqué, Guadalupe Fraile, Monica Rodriguez-Carballeira, Luis Caminal, Maria J Castillo, Ferran Martínez-Valle, Luis Sáez, Juan J Rios, Xavier Solanich, Joaquim Oristrell, Francisco Pasquau, Eva Fonseca, Monica Zamora, Jose L Callejas, Begoña Frutos, Monica Abdilla, Patricia Fanlo, Isabel García-Sánchez, Miguel López-Dupla, Bernardo Sopeña, Almudena Pérez-Iglesias, Josep A Bosch
The aim of this study was to describe the clinical characteristics of ANCA-associated vasculitides (AAV) at presentation, in a wide cohort of Spanish patients, and to analyze the impact of the vasculitis type, ANCA specificity, prognostic factors, and treatments administered at diagnosis, in the outcome.A total of 450 patients diagnosed between January 1990 and January 2014 in 20 Hospitals from Spain were included. Altogether, 40.9% had granulomatosis with polyangiitis (GPA), 37.1% microscopic polyangiitis (MPA), and 22% eosinophilic granulomatosis with polyangiitis (EGPA)...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28220286/characteristics-of-hypertrophic-pachymeningitis-in-patients-with-granulomatosis-with-polyangiitis
#5
Hyun Ah Choi, Mi Ji Lee, Chin-Sang Chung
Hypertrophic pachymeningitis (HP) is an important neurologic complication of granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis). The aim of this study is to investigate the clinical features, radiological findings, and diagnostic pitfalls of GPA-related HP. A retrospective chart review was performed to screen patients diagnosed with GPA at Samsung Medical Center between 1997 and 2016. Neurologic manifestation, laboratory findings, neuroimaging data, and clinical course were evaluated in all patients...
February 20, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28217942/hypertrophic-pachymeningitis-is-a-characteristic-manifestation-of-granulomatosis-with-polyangiitis-a-retrospective-study-of-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis
#6
Yasuhiro Shimojima, Dai Kishida, Akiyo Hineno, Masahide Yazaki, Yoshiki Sekijima, Shu-Ichi Ikeda
AIM: To elucidate the characteristics of patients with hypertrophic pachymeningitis (HP) in a population with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: We retrospectively investigated the clinical records of 39 patients who were diagnosed with AAV. To determine the characteristics of HP in AAV, the epidemiological and clinical data from patients with HP were statistically compared with those from patients without HP. RESULTS: Of 39 patients with AAV, seven (17...
February 20, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28208913/eosinophilic-granulomatosis-with-polyangiitis-egpa-and-hepatitis-b-infection-a-rare-association
#7
Nidhi Kaeley, Narula Kamakshi
Eosinophilic Granulomatosis with Polyangiitis (EGPA) or Churg-Strauss syndrome is a rare systemic illness which is characterized by necrotizing inflammation of small and medium sized vessels. The prominent features include asthma, eosinophilia, transient pulmonary infiltration and systemic vasculitis. Various triggering factors have been reported as putative aetiological agents of Churg-Strauss syndrome. They include infections, allergic desensitization, vaccinations like hepatitis B and influenza vaccination, cocaine abuse, drugs like leukotriene receptor antagonists and exposure to pigeons...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28207017/healing-of-leg-ulcers-associated-with-granulomatosis-with-polyangiitis-wegener-granulomatosis-after-rituximab-therapy
#8
Scott Kindle, Joseph Fanciullo
No abstract text is available yet for this article.
January 2017: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/28196580/anti-neutrophil-cytoplasmic-antibody-associated-vasculitis-with-renal-involvement-analysis-of-89-cases
#9
Fernando Caravaca-Fontán, Estefanía Yerovi, María Delgado-Yagu E, Cristina Galeano, Saúl Pampa-Saico, Maria Teresa Tenorio, Fernando Liaño
INTRODUCTION: The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis with renal involvement are associated with high morbi-mortality. In this study we analyse if the prognosis of these diseases have improved in recent years, and which factors influence the outcomes. MATERIAL AND METHODS: Retrospective single-centre observational study, which included all patients diagnosed with microscopic polyangiitis and granulomatosis with polyangiitis with renal involvement in the last 25 years...
January 6, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28184292/rituximab-therapy-in-pemphigus-and-other-autoantibody-mediated-diseases
#10
REVIEW
Nina A Ran, Aimee S Payne
Rituximab, a monoclonal antibody targeting the B cell marker CD20, was initially approved in 1997 by the United States Food and Drug Administration (FDA) for the treatment of non-Hodgkin lymphoma. Since that time, rituximab has been FDA-approved for rheumatoid arthritis and vasculitides, such as granulomatosis with polyangiitis and microscopic polyangiitis. Additionally, rituximab has been used off-label in the treatment of numerous other autoimmune diseases, with notable success in pemphigus, an autoantibody-mediated skin blistering disease...
2017: F1000Research
https://www.readbyqxmd.com/read/28179705/the-forgotten-disease-lemierres-syndrome
#11
Ahmed Farhan, Yawar Ali Shah, Bushra Tariq Ali, Uzma Mumtaz, Umar Farooq
Lemierre's syndrome refers to septic thrombosis of deep veins of the neck, is a rare and often life threatening complication following upper respiratory tract infections. We present here a case of Lemierre's syndrome in a previously healthy 30 years old female who had a febrile illness for two weeks with associated dysphagia, hoarse voice and right sided neck swelling. She was investigated for retropharyngeal and parapharyngeal abscess, granulomatosis with polyangiitis, tuberculosis and thyroiditis but finally concluded as Lemierre's syndrome based on the findings of thrombosis of the deep neck veins following respiratory tract infection, septic pulmonary emboli and clinical recovery with antibiotics and supportive care...
December 2016: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28169190/galactosylation-and-sialylation-levels-of-igg-predict-relapse-in-patients-with-pr3-anca-associated-vasculitis
#12
Michael J Kemna, Rosina Plomp, Pieter van Paassen, Carolien A M Koeleman, Bas C Jansen, Jan G M C Damoiseaux, Jan Willem Cohen Tervaert, Manfred Wuhrer
OBJECTIVE: The objective of our study is to investigate the Fc glycosylation profiles of both antigen-specific IgG targeted against proteinase 3 (PR3-ANCA) and total IgG as prognostic markers of relapse in patients with Granulomatosis with Polyangiitis (GPA). METHODS: Seventy-five patients with GPA and a PR3-ANCA rise during follow-up were included, of whom 43 patients relapsed within a median period of 8 (2-16) months. The N-glycan at Asn297 of affinity-purified and denatured total IgG and PR3-ANCA was determined by mass spectrometry of glycopeptides in samples obtained at the time of the PR3-ANCA rise and at the time of the relapse or time-matched during remission...
January 28, 2017: EBioMedicine
https://www.readbyqxmd.com/read/28163812/acute-urinary-retention-secondary-to-urethral-involvement-of-granulomatosis-with-polyangiitis
#13
Patrick T Anderson, Stephanie Gottheil, Manal Gabril, Lillian Barra, Nicholas Power
Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis) is a systemic necrotizing vasculitis of small- and medium-sized blood vessels, primarily affecting the upper and lower respiratory tracts, as well as the kidneys. Urogenital manifestations of GPA are exceedingly rare and usually respond well to systemic immunosuppressive therapy. Here, we present a case of a 36-year-old female presenting with acute urinary obstruction secondary to urethral GPA involvement in the immediate postpartum period...
January 2017: Canadian Urological Association Journal, Journal de L'Association des Urologues du Canada
https://www.readbyqxmd.com/read/28157661/eosinophilic-endomyocardial-disease
#14
Victor Y Liu, Raymundo A Quintana-Quezada, L Maximilian Buja
Eosinophilic granulomatosis with polyangiitis is the rarest of the antineutrophil cytoplasmic antibody-associated vasculitides. We describe the case of a 58-year-old woman with a history of asthma found to have a left ventricular apical thrombus. Cardiac catheterization with endomyocardial biopsy was consistent with eosinophilic endomyocardial disease. The disease affects several organ systems and shares many features with more common conditions, making it a difficult diagnosis.
January 23, 2017: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://www.readbyqxmd.com/read/28155222/skewing-towards-treg-and-th2-responses-is-a-characteristic-feature-of-sustained-remission-in-anca-positive-granulomatosis-with-polyangiitis
#15
Wojciech Szczeklik, Bogdan Jakieła, Katarzyna Wawrzycka-Adamczyk, Marek Sanak, Magdalena Hubalewska-Mazgaj, Agnieszka Padjas, Marcin Surmiak, Katarzyna Szczeklik, Jan Sznajd, Jacek Musiał
The objective of our study was to evaluate the T-helper (Th) and regulatory T (Treg) cell profile in ANCA-positive granulomatosis with polyangiitis (GPA) and its relation to disease activity. In a prospective study, we studied two groups of GPA patients: (1) disease flare (active-GPA, BVAS>6, n = 19), (2) sustained remission (≥ 1-year prior enrollment, inactive-GPA, BVAS = 0, n = 18). 24 age-sex matched healthy subjects served as controls. Active-GPA patients were followed for 6 months and reevaluated during remission (early remission; n = 13)...
February 2, 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/28152445/heart-failure-not-responsive-to-standard-immunosuppressive-therapy-is-successfully-treated-with-high-dose-intravenous-immunoglobulin-therapy-in-a-patient-with-eosinophilic-granulomatosis-with-polyangiitis-egpa
#16
Antonio Pecoraro, Ludovica Crescenzi, Laura Carucci, Arturo Genovese, Giuseppe Spadaro
Glucocorticoids and immunosuppressive drugs represent the first-line treatment of eosinophilic granulomatosis with polyangiitis (EGPA, former Churg-Strauss syndrome), even though the combined therapy is not successful in achieving the disease remission in some patients with neurological or cardiac involvement. We describe a case of an EGPA male patient with impaired left ventricular function not responsive to glucocorticoid and immunosuppressive therapy. We observed that high-dose (2g/kg/4weeks) intravenous immunoglobulin (IVIG) therapy significantly improved cardiac function, which was deteriorated after reducing IVIG dose at 0...
January 30, 2017: International Immunopharmacology
https://www.readbyqxmd.com/read/28148583/anca-associated-vasculitis
#17
Max Yates, Richard Watts
The vasculitides are a heterogeneous group of conditions typified by their ability to cause vessel inflammation with or without necrosis. They present with a wide variety of signs and symptoms and, if left untreated, carry a significant burden of mortality and morbidity. The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are three separate conditions - granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA; previously known as Churg-Strauss syndrome)...
February 2017: Clinical Medicine: Journal of the Royal College of Physicians of London
https://www.readbyqxmd.com/read/28146463/a-rare-case-of-complex-cardiac-involvement-in-granulomatosis-with-polyangiitis
#18
Rafał Gałąska, Dorota Kulawiak-Gałąska, Zenobia Czuszyńska, Anna Masiak, Zbigniew Zdrojewski, Marcin Gruchała
No abstract text is available yet for this article.
January 31, 2017: Polskie Archiwum Medycyny Wewnętrznej
https://www.readbyqxmd.com/read/28134084/pneumocystis-jiroveci-pneumonia-in-rheumatic-disease-a-20-year-single-centre-experience
#19
Christopher A Mecoli, Deanna Saylor, Allan C Gelber, Lisa Christopher-Stine
OBJECTIVES: Pneumocystis jiroveci pneumonia (PJP) is an opportunistic infection with high mortality among patients with underlying rheumatologic conditions. Given the paucity of prospective data to guide treatment, clinical guidelines to initiate PJP prophylaxis are based on expert opinion and identify patients on ≥20 mg daily prednisone for ≥4 weeks duration for treatment. Herein we describe the PJP experience in rheumatic disease over a 20-year period at a single academic medical centre to investigate this 20 mg threshold and risk associated with lymphocyte counts, co-existing lung disease and immunosuppressive medications...
January 27, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28134074/chest-and-renal-involvements-birmingham-vascular-activity-score-more-than-13-5-and-five-factor-score-1996-more-than-1-at-diagnosis-are-significant-predictors-of-relapse-of-microscopic-polyangiitis
#20
Yoon-Jeong Oh, Sung Soo Ahn, Eun Seong Park, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
OBJECTIVES: We investigated whether specified organ involvements, antineutrophil cytoplasmic antibody (ANCA) positivity, Birmingham vasculitis activity score (BVAS) and five factor scores (FFS) at diagnosis could predict relapse of microscopic polyangiitis (MPA). METHODS: We reviewed the medical records of 90 patients with MPA. We collected clinical and prognostic data, (MPO)-ANCA and proteinase 3 (PR3)-ANCA, BVAS and FFS at diagnosis, and we compared them between the two groups...
January 19, 2017: Clinical and Experimental Rheumatology
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