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granulomatosis with polyangiitis

Mehdi Mahmudpour, Abdolhamid Esmaili, Mohammad Zaki Abbasi, Mehdi Khazaei, Khadijeh Ghasemi, Fatemeh Nili, Farshad Zohrabi
No abstract text is available yet for this article.
March 2018: Urology Case Reports
Riccardo Draghi, Giovanna Mantovani, Letterio Runza, Giorgio Carrabba, Nicola Fusco, Paolo Rampini, Antonella Costa, Marco Locatelli
The authors present an unusual case of a patient suffering from visual deficit due to pituitary granulomatosis with polyangiitis (GPA) associated with Rathke's cleft cyst (RCC). The patient was referred to our Neurosurgery Department presenting right eye amaurosis, third cranial nerve palsy, and left temporal hemianopsia. Magnetic resonance imaging documented a sellar or suprasellar lesion with solid and cystic components. The dura mater of the skull base was also strongly enhanced. The patient underwent surgery...
February 2018: Radiology Case Reports
Giuseppe Brescia, Claudia Zanotti, Daniela Parrino, Umberto Barion, Gino Marioni
PURPOSE: Endotyping chronic rhinosinusitis with nasal polyps (CRSwNP) poses a challenge for rhinologists nowadays. Phenotyping CRSwNP proved inappropriate as an approach to their classification because of their common clinical features. Endotyping, being based on the pathogenic mechanism, provides a precise picture more appropriate for use in clinical practice. Patients' treatment and follow-up can thus be tailored to cope with the degree of aggressiveness of a specific CRSwNP endotype...
March 7, 2018: American Journal of Otolaryngology
Wan Kee Kim, Joon Bum Kim
A 58-year-old man visited the emergency department with vague anterior chest discomfort. Based on computed tomographic findings, our initial suspicion was intramural hematoma (IMH) in the ascending aorta. After a comprehensive discussion with relevant departments, emergent operation was planned. Intra-pericardial space, however, was severely adhered suggesting chronic inflammation. Biopsies were performed, and additional surgical procedures were discontinued. The patient was finally diagnosed as having Granulomatosis with Polyangiitis...
March 9, 2018: Annals of Thoracic Surgery
Fiona A Pearce, Peter C Lanyon, Richard A Watts, Matthew J Grainge, Abhishek Abhishek, Richard B Hubbard
Objectives: We aimed to provide insights into the aetiology of granulomatosis with polyangiitis (GPA), by conducting a large case-control study using a general population-based, prospectively collected database of healthcare records. Methods: We compared all incident cases of GPA in the Clinical Practice Research Datalink 1990-2014, with up to 10 age-, sex- and general practice-matched controls. We identified potential risk factors, recorded numbers of cases and controls exposed to each, and calculated odds ratios (ORs) using conditional logistic regression...
February 26, 2018: Rheumatology
Marc R Matthews, Asia N Quan, Alexandra S Weir, Kevin N Foster, Daniel M Caruso
Introduction: Leaving the abdominal cavity open is a well-described and frequently utilized technique in the treatment of severe intra-abdominal sepsis. Irrigation through a negative pressure wound therapy device is a technique employed to assist in the closure of wounds as well as the reduction of bacterial contamination. Furthermore, hypochlorous acid has been found to be safe and effective in microorganismal elimination from extremity wounds. There is no literature regarding the infusion of hypochlorous solution into the abdominal cavity for intra-abdominal sepsis or mucopurulent abscesses or biofilm...
2018: Eplasty
Yushiro Endo, Tomohiro Koga, Midori Ishida, Yuya Fujita, Sosuke Tsuji, Ayuko Takatani, Toshimasa Shimizu, Remi Sumiyoshi, Takashi Igawa, Masataka Umeda, Shoichi Fukui, Ayako Nishino, Shin-Ya Kawashiri, Naoki Iwamoto, Kunihiro Ichinose, Mami Tamai, Hideki Nakamura, Tomoki Origuchi, Atsushi Kawakami
A 72-year-old Japanese woman diagnosed with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis was admitted to our hospital with hearing loss, temporal pain, and sudden blindness. We finally diagnosed recurrent granulomatosis with polyangiitis and initiated methyl-prednisolone pulse therapy (1,000 mg) followed by prednisolone (30 mg/day) and rituximab (RTX). After the third RTX administration, she developed bloody stools along with acute thrombocytopenia and low complement levels. We diagnosed rituximab-induced acute thrombocytopenia (RIAT), and her platelet counts spontaneously recovered...
March 9, 2018: Internal Medicine
Supaporn Suwanchote, Muanpetch Rachayon, Pongsawat Rodsaward, Jongkonnee Wongpiyabovorn, Tawatchai Deekajorndech, Helen L Wright, Steven W Edwards, Michael W Beresford, Pawinee Rerknimitr, Direkrit Chiewchengchol
Anti-neutrophil cytoplasmic antibodies (ANCA) are a group of autoantibodies that cause systemic vascular inflammation by binding to target antigens of neutrophils. These autoantibodies can be found in serum from patients with systemic small-vessel vasculitis and they are considered as a biomarker for ANCA-associated vasculitis (AAV). A conventional screening test to detect ANCA in the serum is indirect immunofluorescence study, and subsequently confirmed by enzyme-linked immunosorbent assay. A positive staining of ANCA can be classified into three main categories based on the staining patterns: cytoplasmic, perinuclear, and atypical...
March 10, 2018: Clinical Rheumatology
H Marco, J Draibe, J Villacorta, L F Quintana, N Martin, R Garcia-Osuna, C Cabre, M A Martín-Gómez, A Balius, A Saurina, M Picazo, I Gich-Saladich, M Navarro-Díaz, M Praga, T Cavero, J Ballarin, M M Díaz-Encarnación
The classification of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) remains controversial. The main objective of this study was to define the respective values of ANCA serotype-based classification, clinicopathological classification, and histopathological classification in predicting patient and renal outcomes in a Spanish cohort of patients with ANCA with specificity for myeloperoxidase, MPO-ANCA, versus ANCA with specificity for proteinase 3, PR3-ANCA. Two hundred and forty-five patients with ANCA-AAV and biopsy-proven renal involvement diagnosed between 2000 and 2104 were recruited in 12 nephrology services...
March 9, 2018: Clinical Rheumatology
Khalid Gafoor, Shalin Patel, Francis Girvin, Nishant Gupta, David Naidich, Stephen Machnicki, Kevin K Brown, Atul Mehta, Bryan Husta, Jay Ryu, George Sarosi, Tomas Franquet, Johny Verschakelen, Takeshi Johkoh, William Travis, Suhail Raoof
Cavities are occasionally encountered on thoracic imaging. Their differential diagnosis is large and includes, among others, various infections, autoimmune conditions and primary and metastatic malignancies. We offer an algorithmic approach to their evaluation by initially excluding mimics of cavities then broadly classifying them according to the duration of clinical symptoms and radiological abnormalities.. An acute or subacute process (<12 weeks) suggests common bacterial and uncommon nocardial and fungal causes of pulmonary abscesses, necrotizing pneumonias, and septic emboli...
March 5, 2018: Chest
Hidetoshi Takedatsu, Keiichi Mitsuyama, Shuhei Fukunaga, Shinichiro Yoshioka, Ryosuke Yamauchi, Atsushi Mori, Hiroshi Yamasaki, Kotaro Kuwaki, Hideto Sakisaka, Shotaro Sakisaka, Takuji Torimura
BACKGROUND AND AIM: Proteinase 3 antineutrophil cytoplasmic antibodies (PR3-ANCAs) are well-known serological markers for granulomatosis with polyangiitis, but their role as serological markers for inflammatory bowel disease (IBD) remains uncertain. The present study aimed to evaluate the diagnostic and clinical role of PR3-ANCAs as markers for IBD. METHODS: Using a new methodology with chemiluminescence enzyme immunoassay, serum PR3-ANCA titres were assessed in 102 patients with ulcerative colitis (UC), 67 patients with Crohn's disease (CD), 44 controls with other intestinal diseases and 66 healthy controls...
March 7, 2018: Journal of Gastroenterology and Hepatology
Joseph R Berger, Vineeta Malik, Stuart Lacey, Paul Brunetta, Patricia B Lehane
This report assesses the observed risk of PML in patients treated with the anti-CD20 monoclonal antibody rituximab in the regulatory authority-approved autoimmune indications rheumatoid arthritis (RA), granulomatosis with polyangiitis (GPA), and microscopic polyangiitis (MPA). This was a cumulative analysis of confirmed PML cases in patients receiving rituximab for RA or GPA/MPA from both spontaneous reports and clinical trial sources, as captured in the manufacturer global company safety and clinical databases...
March 5, 2018: Journal of Neurovirology
James C Wang, Brittany A Leader, Ryan A Crane, Bernadette L Koch, Matthew M Smith, Stacey L Ishman
Granulomatosis with polyangiitis (GPA, previously known as Wegener's granulomatosis) is an autoimmune systemic small-vessel vasculitis, associated with the presence of anti-neurophil cytoplasmic antibodies with a cytoplasmic staining pattern (c-ANCA). It is characterized by necrotizing granulomas, usually affecting the airways and kidneys. GPA should be considered when patients do not improve despite adequate treatment of otologic symptoms, when patients have unspecific symptoms suggesting systemic disease (e...
April 2018: International Journal of Pediatric Otorhinolaryngology
P Bielefeld, D Saadoun, E Héron, S Abad, H Devilliers, C Deschasse, S Trad, D Sène, G Kaplanski, P Sève
Scleritis is an inflammatory disease of the sclera; outer tunic of the eye on which the oculomotor muscles are inserted. It can be associated with a systemic disease up to one time out of 3. These associated diseases are mainly rheumatoid arthritis, vasculitis, including granulomatosis with polyangiitis in the first line and spondyloarthropathies. Before mentioning such an etiology, it is necessary to eliminate an infectious cause, mainly herpetic, which is regularly underestimated. The classification of scleritis is clinical...
February 26, 2018: La Revue de Médecine Interne
Edward C Kuan, Kevin A Peng, Lyndon O Gonzalez, Joel A Sercarz
We report a rare case of squamous cell carcinoma (SCC) of the nasal cavity arising in a patient with granulomatosis with polyangiitis (GPA). The patient was a 35-year-old man who had been diagnosed 15 years earlier with GPA and treated medically for sinonasal, pulmonary, and renal involvement. He presented to us with left-sided orbital and cheek pain and nasal obstruction. Endoscopy detected a friable, exophytic mass that involved the left lateral nasal wall and septum. Biopsy analysis identified the mass as an SCC...
January 2018: Ear, Nose, & Throat Journal
Vineeta Shobha, Saba Fathima, Ravi Prakash
Granulomatosis with polyangiitis (GPA) previously known as Wegener's granulomatosis is one of the forms of idiopathic systemic vasculitis. There is very scanty data available on GPA in Asian and Indian population. We studied data of 60 patients from southern India, diagnosed with GPA to describe the physical characteristics, the treatment, and outcome. Patients who fulfilled any two of the four criteria proposed by the American College of Rheumatology, and those with clinical features of GPA with ANCA positivity and histopathological confirmation, were included in the study...
February 28, 2018: Clinical and Experimental Medicine
Yasuhiro Nakano, Yoshia Miyawaki, Ken-Ei Sada, Yuriko Yamamura, Yuzuki Kano, Keigo Hayashi, Haruki Watanabe, Yoshinori Matsumoto, Tomoko Kawabata, Jun Wada
No abstract text is available yet for this article.
February 27, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
Amanda M Kong, Gilwan Kim, Margaret Michalska, Jennie H Best
INTRODUCTION: Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are two related forms of systemic vasculitis. Patients with these conditions often experience relapses affecting various body systems. Here we describe rates of relapse and review healthcare costs resulting from relapse among patients with GPA/MPA. METHODS: Two groups of patients with GPA and MPA were selected from the MarketScan claims databases between 2011 and 2013 based on diagnosis codes...
February 22, 2018: Rheumatology and Therapy
Therese Woodring, Ronnie Abraham, Stephanie Frisch
Cutaneous leukocytoclastic vasculitis (LCV) can occur as skin-limited disease or as part a systemic vasculitis. Appropriate workup includes the evaluation of antineutrophil cytoplasmic antibodies (ANCAs), with a positive titer raising concern for the associated primary vasculitides including microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), or eosinophilic granulomatosis with polyangiitis (EGPA). In the absence of systemic findings, however, a drug etiology must also be considered. Tumor necrosis factor (TNF) inhibitors, propylthiouracil, levamisole-adulterated cocaine, hydralazine, and minocycline have been previously documented to induce ANCA-positive vasculitis (APV), which may present with conspicuously high ANCA titers...
August 15, 2017: Dermatology Online Journal
Mathew X Joseph, Lauren E Jenkins, Michael E Wechsler, Loretta S Davis
No abstract text is available yet for this article.
February 21, 2018: JAMA Dermatology
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