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https://www.readbyqxmd.com/read/28645447/colitis-as-a-form-of-presentation-of-eosinophilic-granulomatosis-with-polyangiitis
#1
Eugenia Ameneiros-Lago, Javier Pérez-Valcarcel, Francisco José Fernández-Fernández, Tamara Caínzos-Romero
No abstract text is available yet for this article.
June 20, 2017: Gastroenterología y Hepatología
https://www.readbyqxmd.com/read/28637076/-clinical-course-of-an-early-childhood-onset-granulomatosis-with-polyangiitis
#2
Marco Krasselt, Matthias Pierer, Kerstin Amann, Anette Bachmann, Tom H Lindner
History A 26-year-old male patient presented with an eight-week history of unspecific symptoms such as weight loss and fever. Besides, he also suffered from haemoptysis, cough, and arthralgia. Since the age of twelve years, the patient has been treated for Wegner's granulomatosis. At the age of 20 years he received a kidney transplant which failed only four years later. Investigations The relapse we clinically suspected was confirmed by CT scan showing bilateral pulmonary manifestations. Moreover, we found highly positive antibodies against proteinase 3...
June 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28631702/-the-frequency-of-ophthalmologic-manifestations-of-granulomatosis-with-polyangiitis-wegener-s-and-their-relationship-to-systemic-diseases
#3
D S Ismailova, P I Novikov, Ya O Grusha, Yu V Abramova, N M Bulanov, E A Makarov
AIM: To estimate the frequency of lesions in the organ of vision in granulomatosis with polyangiitis (GPA) (Wegener's) and to determine their relationship to systemic diseases. SUBJECTS AND METHODS: The retrospective study enrolled 218 patients followed up at the E.M. Tareyev Clinic of Nephrology, Internal and Occupational Diseases, with a diagnosis of GPA. The frequency and association of ophthalmic manifestations with systemic involvement were statistically analyzed using PASW Statistics 18...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28628344/rituximab-induction-and-maintenance-treatment-in-patients-with-scleritis-and-granulomatosis-with-polyangiitis-wegener-s
#4
Caiyun You, Lina Ma, Andres F Lasave, C Stephen Foster
AIMS: To evaluate the efficacy and safety of rituximab (RTX) induction and maintenance treatment for patients with scleritis and granulomatosis with polyangiitis (GPA), Wegener's. METHODS: Nine patients (12 eyes) with scleritis with GPA who did not respond to corticosteroids and more than one immunosuppressive agent who received ongoing maintenance RTX treatment were identified. Demographics and outcome measures were recorded. RESULTS: Median follow-up time of 30 months (range, 15 to 87 months)...
June 19, 2017: Ocular Immunology and Inflammation
https://www.readbyqxmd.com/read/28623488/vasculitis-for-the-internist-focus-on-anca-associated-vasculitis
#5
REVIEW
Benjamin Chaigne, Loïc Guillevin
Vasculitides comprise several diseases affecting vessels of different sizes, mainly arteries, but also capillaries and veins. Antineutrophil cytoplasm antibody-associated vasculitides (AAVs) belong to the systemic necrotizing small-vessel vasculitis group that comprises granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. Internists often see AAV patients, and although their outcomes are usually good, the disease can be responsible for morbidity and mortality...
June 16, 2017: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/28616269/elevated-serum-levels-of-immunoglobulin-a-correlate-with-the-possibility-of-readmission-in-patients-with-microscopic-polyangiitis
#6
Huijuan Wang, Chao Zhang, Zhaohui Tong, Xiaoning Bu
BACKGROUND: The evidence for the short-term prognosis of patients with microscopic polyangiitis (MPA) is weak, and the objective of this study was to analyze the clinical features of the disease and evaluate the risk factors for readmission in patients with MPA. METHODS: Fifty-seven patients with MPA were recruited into this study. The clinical data of these MPA patients were collected. Clinical manifestations, laboratory results, and imaging results were analyzed...
May 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28615072/chemokine-receptor-co-expression-reveals-aberrantly-distributed-th-effector-memory-cells-in-gpa-patients
#7
Lucas L Lintermans, Abraham Rutgers, Coen A Stegeman, Peter Heeringa, Wayel H Abdulahad
BACKGROUND: Persistent expansion of circulating CD4(+) effector memory T cells (TEM) in patients with granulomatosis with polyangiitis (GPA) suggests their fundamental role in disease pathogenesis. Recent studies have shown that distinct functional CD4(+) TEM cell subsets can be identified based on expression patterns of chemokine receptors. The current study aimed to determine different CD4(+) TEM cell subsets based on chemokine receptor expression in peripheral blood of GPA patients...
June 14, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28611282/the-4-plus-2-rituximab-protocol-makes-maintenance-treatment-unneeded-in-patients-with-refractory-anca-associated-vasculitis-a-10-year-observation-study
#8
Dario Roccatello, Savino Sciascia, Daniela Rossi, Mirella Alpa, Carla Naretto, Massimo Radin, Roberta Fenoglio, Simone Baldovino, Elisa Menegatti
BACKGROUND: ANCA associated vasculitides (AAV) often present with a chronic relapsing course. Relapse leads to increased immunosuppressive exposure and consequent toxicity. While two randomized controlled trials have shown rituximab (RTX) to be the most effective induction treatment in patients with relapsing disease, the optimal treatment duration and RTX dose remain debated. Whether to administer a maintenance dose to every patient, at a fixed time interval or on the basis of B cell count and ANCA titre or only when disease manifestations do occur is still debated as well...
May 23, 2017: Oncotarget
https://www.readbyqxmd.com/read/28608042/-granulomatous-reaction-pattern-of-the-skin-interstitial-granulomatous-dermatitis-lymphoma-vasculitis
#9
REVIEW
C Rose, K Holl-Ulrich
A particular diagnostic situation in the classification of a granulomatous dermatitis results when no circumscribed granulomas but instead a diffuse interstitial histiocytic inflammatory infiltrate, a granulomatous vasculitis or a neoplastic lymphocytic infiltrate is found. Interstitial granulomatous dermatitis was originally described in patients with arthritis. Later, it was recognized that there are also associations with other usually immunological diseases. Differentiating between interstitial granulomatous dermatitis and the interstitial form of granuloma annulare, early morphea and variants of borreliosis or scleromyxedema as well as interstitial granulomatous drug reaction can be very difficult...
June 12, 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28599871/a-case-report-of-granulomatosis-with-polyangiitis-with-granulomas-in-bone-marrow
#10
Isabel de la Cámara Fernández, Fernando Lozano Morillo, Luis Morillas López, Mario Martínez López
No abstract text is available yet for this article.
June 6, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28598870/molecularly-targeted-agents-in-oculoplastic-surgery
#11
Richard C Allen
PURPOSE OF REVIEW: Significant advances have been made in oncology and rheumatology with the introduction of molecularly targeted agents (MTAs). MTAs consist of monoclonal antibodies and small molecule inhibitors. The purpose of this manuscript is to review the recent applications of MTAs to orbital, lacrimal, and eyelid disease. RECENT FINDINGS: The use of monoclonal antibodies has been described in the treatment of orbital vascular lesions, lymphoma, and squamous cell carcinoma...
June 8, 2017: Current Opinion in Ophthalmology
https://www.readbyqxmd.com/read/28598778/uncommon-presentation-of-granulomatosis-with-polyangiitis-in-association-with-subclinical-coeliac-disease
#12
Paraskevi V Voulgari, Fotini Apostolou, Evangelia Dounousi, George Liapis, Moises Elisaf, Alexandros A Drosos
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June 9, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28592713/pulmonary-limited-granulomatosis-with-polyangiitis-coexisting-with-mixed-connective-tissue-disease
#13
Masaru Ando, Akihiko Goto, Mari Yamasue, Yuko Usagawa, Hiroaki Oka, Takehiko Shigenaga, Jun-Ichi Kadota
Granulomatosis with polyangiitis (GPA) is a systemic disease characterized by necrotizing, granulomatous vasculitis of the upper and lower respiratory tracts and glomerulonephritis, and is classified as a classical or limited form. The classical form of GPA demonstrates the involvement of the upper respiratory tract, sinuses, lungs and kidneys, whereas the limited form is characterized by the lack of the renal involvement with female predominance. On the other hand, mixed connective tissue disease (MCTD) shows the clinical and laboratorial features of systemic lupus erythematosus, systemic sclerosis and polymyositis, along with high titers of anti-ribonucleoprotein antibodies and is characterized by good response to corticosteroid therapy and favorable prognosis...
2017: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28591497/-anca-associated-vasculitides-at-mexico-city-s-metropolitan-eastern-area
#14
Hernando Cervera-Castillo, Francisco Blanco-Favela, Yolanda Floriberta Silva-López, Guadalupe Eugenia Paredez-Rivera, Verónica Torres-Caballero
BACKGROUND: The anti-neutrophil cytoplasmic antibody-associated (ANCA) vasculitides includes granulomatosis with polyangiitis (Wegener's) (GPW), Chrug-Strauss syndrome (CSS) and microscopic polyangiitis (MPA). Since it has low incidence in our field, there are a few published papers. The aim of this study was to report the clinical characteristics, activity and damage of these vasculitides in Mexico City's metropolitan eastern area. METHODS: We studied 28 cases with ANCA-associated vasculitis in the aforementioned area...
July 2017: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/28588807/eosinophilic-angiitis-presenting-with-a-true-fusiform-temporal-artery-aneurysm
#15
Megan MacDiarmid, Dmitri Nepogodiev, Alok Tiwari, Martin Duddy, Martyn Carey, Paresh Jobanputra
Temporal artery aneurysm is a rare cause of temporal artery swelling in the absence of preceding trauma. Vasculitis other than giant cell arteritis, such as eosinophilic granulomatosis with polyangiitis, should be considered in such cases and a careful assessment of other medium-sized arteries undertaken.
June 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28588684/successful-treatment-of-granulomatosis-with-polyangiitis-with-hydropneumothorax-using-corticosteroids-and-immunosuppressant
#16
Xu-Hua Shi, Yong-Feng Zhang, Yue-Wu Lu
Pneumothorax and pleural effusion is a rare and serious complication of granulomatosis with polyangiitis (GPA). The present study reported a case with a history of sinusitis for 20 years, dry cough for three years and exacerbated purulent nasal discharge and recurrent skin ulcers for two years. The patient experienced sudden difficulty in breathing two months prior to presentation. Lung computed tomography (CT) showed multiple bilateral lung nodules and cavitary nodules as well as right hydropneumothorax. Paranasal sinus CT showed soft tissue infiltration...
June 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28587410/a-case-report-of-the-orbit-ocular-association-and-the-lung-in-granulomatosis-with-polyangiitis-a-diagnostic-challenge
#17
Cheng-Wei Lu, Xiu-Fen Liu, Ying Luan, Cheng-Bo Lu, Dan-Dan Zhou, Li-Min Guo, Ya-Bin Sun, Sheng-Nan Chen, Yun-Long Wu, Ji-Long Hao
Granulomatosis with polyangiitis (GPA) is a systemic form of vasculitis that involves small to medium sized vessels and is associated with high morbidity and mortality. GPA presents a continuous and difficult clinical diagnostic concern, due to the rarity of the disease and the diversity of the manifestations. This case report discusses the unusual symptoms presented by a particular patient, discusses these manifestations and explains how the final diagnosis was identified as GPA. A 40-year old Chinese woman was initially referred to the present institution for a progressive worsening pain, redness and gradual decrease in visual acuity in the eyes over the past 7-year period...
June 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28578472/cutaneous-manifestations-of-anca-associated-small-vessels-vasculitis
#18
REVIEW
Marzano Angelo Valerio, Raimondo Maria Gabriella, Berti Emilio, Meroni Pier Luigi, Ingegnoli Francesca
Skin lesions are frequent manifestations of underlying systemic conditions, including systemic autoimmune vasculitis. In particular, anti-neutrophil cytoplasmic antibodies (ANCA) are associated with distinct forms of vasculitis characterized by inflammatory cell infiltration of the walls of small and medium-sized vessels leading to vascular destruction and tissue necrosis. ANCA-associated vasculitis is rare and systemic diseases, which can be classified based on different distribution of vascular inflammation and presence or absence of granulomatosis and asthma...
June 3, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28577200/anca-associated-vasculitis-presenting-as-severe-pulmonary-hypertension-and-right-heart-failure
#19
Rakesh Kumar Pilania, Sumeet R Dhawan, Joseph L Mathew, Surjit Singh, K S Sodhi, Meenu Singh
Microscopic polyangiitis (MPA) is a small vessel multisystemic disorder characterised by necrotising small vessel vasculitis without any immune deposits. Kidney and lung are the predominant organs affected in MPA. Skin, gastrointestinal and neurological findings are also described. Isolated pulmonary manifestations are rare. The authors describe a two-year girl who presented with right heart failure and was subsequently diagnosed as ANCA-associated vasculitis. This case report is intended to sensitise pediatricians to consider systemic vasculitis with pulmonary hemorrhage in children with pulmonary hypertension even in the absence of severe pallor...
June 2, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28575531/myeloperoxidase-hla-class-ii-complexes-recognized-by-autoantibodies-in-microscopic-polyangiitis
#20
Ryosuke Hiwa, Koichiro Ohmura, Noriko Arase, Hui Jin, Kouyuki Hirayasu, Masako Kohyama, Tadahiro Suenaga, Fumiji Saito, Chikashi Terao, Tatsuya Atsumi, Hirotsugu Iwatani, Tsuneyo Mimori, Hisashi Arase
OBJECTIVE: Autoantibodies against myeloperoxidase (MPO) that are expressed in neutrophils play an important role in the pathogenesis of microscopic polyangiitis (MPA). We recently found that misfolded cellular proteins are transported to the cell surface by HLA class II molecules and are targeted by autoantibodies in rheumatoid arthritis and antiphospholipid syndrome, suggesting that HLA class II molecules play an important role in autoantibody recognition. Here, we demonstrate that MPO is expressed on the cell surface by associating with HLA class II molecules...
June 2, 2017: Arthritis & Rheumatology
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