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Tatiana V Beketova, Mikhail Y Volkov, Evgeniy A Naryshkin, Tatiana M Novoselova, Evgeniy L Nasonov
Recent advances in pharmacology have greatly expanded the drug repertoire for treatment of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystemic disorder, a type of the ANCA-associated vasculitis. Important features of this disease are eosinophilia and anti-myeloperoxidase ANCA presence in around 30-70% of patients. Primary therapy of EGPA includes steroids and cytotoxic drugs, e.g., cyclophosphamide, azathioprine, or methotrexate...
March 21, 2018: Clinical Rheumatology
D D K Abeyaratne, C Liyanapathirana, C L Fonseka, P W M C S B Wijekoon
Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is an antineutrophil cytoplasmic antibody- (ANCA-) associated small vessel vasculitis with multisystem involvement. It is characterized with asthma, eosinophilia, and renal and peripheral nervous system involvement. However, EGPA presenting with bullous skin eruption is an uncommon dermatological manifestation. We report a rare case of EGPA overlapped with mixed essential cryoglobulinemia presenting with a bullous skin eruption...
2018: Case Reports in Medicine
Radosław Jeleniewicz, Dorota Suszek, Maria Majdan
OBJECTIVE: Introduction: Musculoskeletal symptoms are not characteristic for granulomatosis with polyangiitis (GPA) patients. However, they could be present in two-thirds of patients at the onset of the disease and may provoke diagnostic difficulties. The aim: To assess retrospectively musculoskeletal manifestations in a group of GPA patients. PATIENTS AND METHODS: Material and methods: The analyzed group consisted of 44 patients with GPA (27M, 17F) aged 46.7 years (16-75) and treated at the Department of Rheumatology and Connective Tissue Diseases in Lublin between 2010-2016...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Juyoung Yoo, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
We investigated the development rate and time, risk factors, predictors, and aetiologies of hospitalised infection in Korean patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We retrospectively reviewed the medical records of 154 patients with AAV. Hospitalised infection was considered only when patients were admitted for serious infection related to AAV or AAV treatment. The gap-time was defined as the period from diagnosis to the first hospitalised infection or to the last visit for uninfected patients...
March 20, 2018: Clinical Rheumatology
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
Alessandro Antônio Costa Pereira, Christian Barros Ferreira, João Adolfo Costa Hanemann, Livia Maris Ribeiro Paranaiba, Patricia Peres Iucif Pereira, Carla Isabelly Rodrigues-Fernandes, Celeste Sánchez-Romero, Oslei Paes de Almeida, Felipe Paiva Fonseca
Lymphomatoid granulomatosis (LYG) is a rare B-cell lymphoproliferative disorder driven by Esptein-Barr virus (EBV) that most commonly affects the lungs, although extra pulmonary sites like the central nervous system, skin, liver and kidney can also be involved. It is microscopically characterized by an angiocentric and angiodestructive growth pattern, predominantly composed by small T-cells, although a smaller population of atypical large B-cells is considered the true neoplastic component. Oral cavity involvement of LYG has rarely been described and the diagnosis of this neoplasm is very difficult...
March 14, 2018: Head and Neck Pathology
Ann Marie Szymanski, Michael J Ombrello
The intersection of granulomatosis and autoinflammatory disease is a rare occurrence that can be generally subdivided into purely granulomatous phenotypes and disease spectra that are inclusive of granulomatous features. NOD2 (nucleotide binding oligomerization domain containing 2)-related disease, which includes Blau syndrome and early onset sarcoidosis, is the prototypic example of granulomatous inflammation in the context of monogenic autoinflammation. Granulomatous inflammation has also been observed in two related autoinflammatory diseases caused by mutations in PLCG2 (phospholipase C gamma 2)...
March 12, 2018: International Immunology
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
Stefan D Dojcinov, Falko Fend, Leticia Quintanilla-Martinez
The contribution of Epstein-Barr virus (EBV) to the development of specific types of benign lymphoproliferations and malignant lymphomas has been extensively studied since the discovery of the virus over the last 50 years. The importance and better understanding of the EBV-associated lymphoproliferative disorders (LPD) of B, T or natural killer (NK) cell type has resulted in the recognition of new entities like EBV+ mucocutaneous ulcer or the addition of chronic active EBV (CAEBV) infection in the revised 2016 World Health Organization (WHO) lymphoma classification...
March 7, 2018: Pathogens
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
Mohamed Amine Bani, Alia Zehani, Ines Chelly, Aida Khadhar, Slim Haouet, Nidhameddine Kchir
No abstract text is available yet for this article.
June 2017: La Tunisie Médicale
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
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