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https://www.readbyqxmd.com/read/28091914/altered-liver-proteoglycan-glycosaminoglycan-structure-as-a-manifestation-of-extracellular-matrix-remodeling-upon-bcg-induced-granulomatosis-in-mice
#1
L B Kim, V A Shkurupy, A N Putyatina
Experimental BCG-induced granulomatosis in mice was used to study changes in the dynamics of individual liver proteoglycan components reflecting phasic extracellular matrix remodeling, determined by the host-parasite interaction and associated with granuloma development. In the early BCG-granulomatosis period, the increase in individual proteoglycan components promotes granuloma formation, providing conditions for mycobacteria adhesion to host cells, migration of phagocytic cells from circulation, and cell-cell interaction leading to granuloma development and fibrosis...
January 14, 2017: Bulletin of Experimental Biology and Medicine
https://www.readbyqxmd.com/read/28089971/incidence-of-malignancy-prior-to-antineutrophil-cytoplasmic-antibody-associated-vasculitis-compared-to-the-general-population
#2
Emma E van Daalen, Chinar Rahmattulla, Ron Wolterbeek, Jan A Bruijn, Ingeborg M Bajema
OBJECTIVE: Previous studies have reported an increased malignancy risk preceding antineutrophil cytoplasmic antibody-associated vasculitis (AAV), suggesting common pathogenic pathways in these 2 entities. However, the study results were conflicting and often limited to patients with granulomatosis with polyangiitis (GPA). Here, we study the malignancy risk prior to AAV diagnosis [either GPA or microscopic polyangiitis (MPA)] to elaborate on the putative association between malignancy and AAV...
January 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28089164/low-median-nerve-palsy-as-initial-manifestation-of-churg-strauss-syndrome
#3
Young Hak Roh, Young Do Koh, Jung Ho Noh, Hyun Sik Gong, Goo Hyun Baek
Anterior interosseous nerve (AIN) syndrome is typically characterized by forearm pain and partial or complete dysfunction of the AIN-innervated muscles. Although the exact etiology and pathophysiology of the disorder remain unclear, AIN syndrome is increasingly thought to be an inflammatory condition of the nerve rather than a compressive neuropathy because the symptoms often resolve spontaneously following prolonged observation. However, peripheral neuropathy can be 1 of the first symptoms of systemic vasculitis that needs early systemic immunotherapy to prevent extensive nerve damage...
January 11, 2017: Journal of Hand Surgery
https://www.readbyqxmd.com/read/28087591/clinical-trials-in-antineutrophil-cytoplasmic-antibody-associated-vasculitis-what-we-have-learnt-so-far-and-what-we-still-have-to-learn
#4
Divi Cornec, Emilie Cornec-Le Gall, Ulrich Specks
The prognosis of the antineutrophil cytoplasmic antibody associated vasculitides (AAV), microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA), has been fundamentally improved over the last five decades by the use of glucocorticoids and immunosuppressants, turning them from consistently fatal diseases into chronic conditions. The long-term course is now largely determined by the frequency of disease flares and by accruing damage caused by disease activity and treatment-related comorbidities...
January 12, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28079854/time-course-of-serum-pro-inflammatory-cytokines-and-chemokines-levels-observed-in-granulomatosis-with-polyangiitis-a-case-report
#5
Yutaka Kozu, Yusuhiro Gon, Yuki Takano, Takao Noguchi, Shinichi Hayashi, Fumio Kumasawa, Kenji Mizumura, Shuichiro Maruoka, Shu Hashimoto
A 77-year-old man visited our hospital with chief complaints of difficulty in hearing, nasal discharge and fever. The patients was diagnosed with otitis media, and his fever continued at approximately 38°C despite the administration of clarithromycin. After that, dyspnea on exertion developed and chest X-ray examination indicated multiple infiltrative shadows. PR3-ANCA levels were high (238-fold of the normal levels), and granulomatosis with polyangiitis (GPA) was diagnosed on the basis of clinical symptoms, laboratory results, and pathological findings...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28079597/histologic-findings-of-severe-therapy-resistant-asthma-from-video-assisted-thoracoscopic-surgery-biopsies
#6
Humberto E Trejo Bittar, Daniel Doberer, Mitra Mehrad, Diane C Strollo, Joseph K Leader, Sally Wenzel, Samuel A Yousem
The histologic changes occurring in severe/therapy-resistant asthma (SA) as defined by the European Respiratory Society/American Thoracic Society guidelines, particularly at the level of the distal airways are unknown. This study describes the clinical, radiologic, and histologic characteristics of 29 SA patients who underwent video-assisted thoracoscopic surgery lung biopsy. Pathologic observations were correlated with clinical features, especially the presence of autoimmune disease (AID) (15/29, 51.7%). Ten biopsies (10/29, 34...
February 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28079316/clinical-characteristics-and-treatment-response-of-peripheral-neuropathy-in-the-presence-of-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome-experience-at-a-single-tertiary-center
#7
Hye Jin Cho, Sehyo Yune, Jin Myoung Seok, Eun Bin Cho, Ju Hong Min, Yeon Lim Seo, Byung Jae Lee, Byoung Joon Kim, Dong Chull Choi
BACKGROUND AND PURPOSE: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic small-vessel vasculitis accompanied by asthma, eosinophilia, and eosinophilic inflammation of various tissues including the peripheral nerves. This study investigated the clinical course and long-term outcomes of peripheral neuropathy in patients with EGPA. METHODS: Seventy-one patients with physician-diagnosed EGPA were identified at Samsung Medical Center between January 1995 and April 2014...
January 2017: Journal of Clinical Neurology
https://www.readbyqxmd.com/read/28076879/igm-antibodies-to-proteinase-3-in-granulomatosis-with-polyangiitis-and-microscopic-polyangiitis
#8
J M Clain, A M Hummel, J H Stone, F C Fervenza, G S Hoffman, C G M Kallenberg, C A Langford, W J McCune, P A Merkel, P A Monach, P Seo, R F Spiera, E W St Clair, S R Ytterberg, And U Specks
Antineutrophil cytoplasmic antibodies (ANCA) appear to play an important role in the pathogenesis of ANCA-associated vasculitis (AAV). Yet, ANCA alone are not sufficient to generate disease, and some evidence suggests that infectious triggers may serve as inciting events for AAV disease activity. Antibodies of the IgM isotype often serve as markers of recent infection, and IgM ANCA have previously been identified in patients with AAV, though the frequency and clinical relevance of IgM ANCA is not well established...
January 11, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28072629/the-key-clues-to-reach-the-diagnosis-of-loeffler-endomyocardial-fibrosis-associated-with-eosinophilic-granulomatosis-with-polyangiitis
#9
Enrico Ammirati, Domenico Sirico, Linda Brevetti, Laura Scudiero, Diana Artioli, Patrizia Pedrotti, Maria Frigerio
Loeffler endomyocardial fibrosis is a rare restrictive cardiomyopathy due to chronic eosinophilic exposure (Loeffler endomyocarditis). Loeffler endomyocarditis is associated with different eosinophilic disorders, including eosinophilic granulomatosis with polyangiitis (EGPA). These images recapitulate all typical findings that can be observed in patients presenting with heart failure attributable to this specific cardiomyopathy associated with EGPA, a systemic medical condition that can be easily detectable just putting together different diagnostic elements...
January 7, 2017: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/28072564/clinical-factors-associated-with-the-diagnosis-of-granulomatosis-with-polyangiitis
#10
Erdem Eren, Toygar Kalkan, Seçil Arslanoğlu, Mustafa Özmen, Kazım Önal, Emine Figen Tarhan, Servet Akar
Objective To determine the predictive value of nasal endoscopic findings and symptoms in the diagnosis of granulomatosis with polyangiitis (GPA). Study Design A cross-sectional study. Setting A tertiary university hospital. Subjects and Methods A total of 116 adults were enrolled in the study: 19 patients with GPA, 29 patients with other rheumatic diseases, and 68 healthy volunteers. All patients were examined with a flexible endoscope, and nasal endoscopic images were recorded and evaluated blindly. The medical history of each patient was taken by a physician blinded to the patient's diagnosis...
January 1, 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/28069788/proteinase-3-antineutrophil-cytoplasmic-antibody-positive-ulcerative-colitis-presenting-with-abducens-neuropathy
#11
Yuki Kirito, Daisuke Yamamoto, Tsuyoshi Uchiyama
A 72-year-old man with ulcerative colitis (UC) presented with complete left abducens nerve palsy. Although MRI showed no significant changes, cerebrospinal fluid analysis revealed pleocytosis and elevated protein and interleukin (IL)-6 levels. His serum proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) level was also elevated to 31.1 U/mL, but granulomatosis with polyangiitis was not observed. On the basis of the diagnosis of autoimmune cranial neuropathy, he was treated with steroid therapy. While tapering steroid therapy, his serum PR3-ANCA levels; cerebrospinal fluid findings, including IL-6 levels; and symptoms improved...
January 9, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28067125/value-of-non-identified-anca-non-pr3-non-mpo-in-the-diagnosis-of-granulomatosis-with-polyangiitis-wegener-s-granulomatosis
#12
Mélanie Givaudan, Frédéric Vandergheynst, Patrick Stordeur, Annick Ocmant, Christian Melot, Valérie Gangji, Muhammad S Soyfoo
OBJECTIVE: Determine the frequency of granulomatosis with polyangiitis (GPA) associated with non-identified ANCA (non-MPO, non-PR3 ANCA) and secondarily compare their clinic with GPA associated with MPO-positive or PR3-positive ANCA. METHODS: In a monocentric retrospective observational study, clinical data of 398 patients with non-identified ANCA (titer of ANCA at least 1/80 by immunofluorescence on ethanol fixed PMN) was gathered over a period of 6 years. GPA patients from this population were compared with GPA patients with identified ANCA on the basis of clinical, biological, immunological and histological features...
January 9, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28065613/anca-negative-churg-strauss-syndrome-presenting-as-acute-multiple-cerebral-infarcts-a-case-report
#13
Klearchos Psychogios, Ilias Evmorfiadis, Spyros Dragomanovits, Athanasios Stavridis, Konstantinos Takis, Loukas Kaklamanis, Stathis Pantelis
Eosinophilic granulomatosis with polyangiitis (EGPA, previously named Churg-Strauss syndrome) is a form of necrotizing vasculitis occurring in patients with asthma and eosinophilia. Ischemic stroke is a relatively rare complication of the disease. We report a case of a 63-year-old woman with multiple embolic infarcts, hypereosinophilia (for >7 years), and skin rash. Elevated cardiac enzymes and cardiac magnetic resonance imaging were consistent with endomyocarditis. The simultaneous presence of history of asthma, sinusitis, hypereosinophilia, and vasculitis led to the diagnosis of EGPA...
January 5, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28062909/paediatric-anti-neutrophil-cytoplasmic-antibody-anca-associated-vasculitis-an-update-on-renal-management
#14
REVIEW
Lucy A Plumb, Louise Oni, Stephen D Marks, Kjell Tullus
The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of disorders characterized by necrotizing inflammation of the small to medium vessels in association with autoantibodies against the cytoplasmic region of the neutrophil. Included in this definition are granulomatosis with polyangiitis (GPA, formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome). AAV are chronic, often relapsing diseases that can be organ or life threatening...
January 6, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28056474/-granulomatosis-with-polyangiitis
#15
Anja Kerstein, Konstanze Holl-Ulrich, Antje Müller, Gabriela Riemekasten, Peter Lamprecht
Granulomatosis with polyangiitis (GPA) is a potentially life-threatening, rare disease. The etiology is unknown. GPA is histomorphologically characterized by extravascular necrotizing granulomatous inflammation and a systemic necrotizing vasculitis of small to medium-sized vessels. Clinically, a pulmonary-renal syndrome with pulmonary infiltrates, alveolar hemorrhage and a rapidly progressive glomerulonephritis is seen in about 80% of the cases with generalized disease. GPA is associated with proteinase 3-specific anti-neutrophil cytoplasmic autoantibodies (PR3-ANCA)...
January 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28056294/-the-understanding-of-epstein-barr-virus-associated-lymphoproliferative-disorder
#16
X G Zhou, Y L Zhang, J L Xie, Y H Huang, Y Y Zheng, W S Li, H Chen, F Liu, H X Pan, P Wei, Z Wang, Y C Hu, K Y Yang, H L Xiao, M J Wu, W H Yin, K Y Mei, G Chen, X C Yan, G Meng, G Xu, J Li, S F Tian, J Zhu, Y Q Song, W J Zhang
In recent years, there are increasing articles concerning Epstein-Barr virus associated lymphoproliferative disorder (EBV+ LPD), and the name of EBV+ LPD is used widely. However, the meaning of EBV+ LPD used is not the same, which triggered confusion of the understanding and obstacles of the communication. In order to solve this problem. Literature was reviewed with combination of our cases to clarify the concept of EBV+ LPD and to expound our understanding about it. In general, it is currently accepted that EBV+ LPD refers to a spectrum of lymphoid tissue diseases with EBV infection, including hyperplasia, borderline lesions, and neoplastic diseases...
December 8, 2016: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28045322/orofacial-granulomatosis-as-early-manifestation-of-crohn-s-disease-report-of-a-case-in-a-paediatric-patient
#17
A Sarra, N G Nikitakis, A Daskalopoulos, G Chouliaras, A Sklavounou-Andrikopoulou, K Athanasaki
BACKGROUND: Orofacial granulomatosis (OFG) is a controversial entity mainly characterised by recurrent or permanent soft tissue swelling of sudden onset in the orofacial area with a histologic appearance of granulomatous inflammation. Differential diagnosis includes local diseases and systemic conditions, such as Crohn's disease (CD). A case of OFG in a paediatric patient is reported here, focusing on the clinical features, diagnostic procedures, treatment and long-term outcome. CASE REPORT: A 7 year-old boy presented with persistent and prominent lip swelling and painful oral mucosa lesions of six months duration...
December 2016: European Journal of Paediatric Dentistry: Official Journal of European Academy of Paediatric Dentistry
https://www.readbyqxmd.com/read/28043730/web-like-endobronchial-membranous-stenosis-a-rare-complication-in-granulomatosis-with-polyangiitis
#18
Miguel Angel Ariza-Prota, Manuel Vaquero-Cacho, Antonio Bango Álvarez
No abstract text is available yet for this article.
December 30, 2016: Archivos de Bronconeumología
https://www.readbyqxmd.com/read/28040323/environmental-factor-and-inflammation-driven-alteration-of-the-total-peripheral-t-cell-compartment-in-granulomatosis-with-polyangiitis
#19
Anja Kerstein, Silke Schüler, Otávio Cabral-Marques, Juliane Fazio, Robert Häsler, Antje Müller, Silke Pitann, Frank Moosig, Sebastian Klapa, Christian Haas, Dieter Kabelitz, Gabriela Riemekasten, Steffen Wolters, Peter Lamprecht
Autoimmune diseases are initiated by a combination of predisposing genetic and environmental factors resulting in self-perpetuating chronic inflammation and tissue damage. Autoantibody production and an imbalance of effector and regulatory T-cells are hallmarks of autoimmune dysregulation. While expansion of circulating effector memory T-cells is linked to disease pathogenesis and progression, the causes driving alterations of the peripheral T-cell compartment have remained poorly understood so far. In granulomatosis with polyangiitis (GPA), a prototypical autoimmune disorder of unknown aetiology, we performed for the first time a combined approach using phenotyping, transcriptome and functional analyses of T-cell populations to evaluate triggers of memory T-cell expansion...
December 28, 2016: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28039414/eosinophilic-granulomatosis-with-polyangiitis-presenting-with-a-vesicovaginal-fistula
#20
Bamidele Famokunwa, Jeremy Ockrim, Jessica J Manson
No abstract text is available yet for this article.
December 30, 2016: Rheumatology
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