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granulomatosis with polyangiitis

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https://www.readbyqxmd.com/read/29328034/characteristics-of-articular-syndrome-in-systemic-vasculitis
#1
O Syniachenko, O Khaniukov, Ye Yehudina, H Taktashov, K Gashynova
The purpose of the study - investigation the separate joint lesion in systemic vasculitis, their X-ray sonographic characteristics, the correlation of the articular syndrome severity with extra-articular manifestations of the diseases, as well as aspects of the arthritis pathogenesis in this category of patients. The study included 525 patients in the ratio of the examined with Henoch-Schonlen purpura, microscopic polyangiitis, cryoglobulinemic vasculitis, polyarteritis nodosa, Takayasu's arteritis, Wegener's granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis Churg-Strauss as a 7:4:3:1:1:1:1...
December 2017: Georgian Medical News
https://www.readbyqxmd.com/read/29321928/lung-cancer-development-in-the-patient-with-granulomatosis-with-polyangiitis-during-long-term-treatment-with-cyclophosphamide-first-documented-case
#2
Midori Toriyama, Etsuko Tagaya, Tomoko Yamamoto, Mitsuko Kondo, Yoji Nagashima, Jun Tamaoki
A 65-year-old man was diagnosed with granulomatosis with polyangiitis (GPA) at the age of 47, when cytoplasmic anti-neutrophil cytoplasmic antibody (C-ANCA) serology was positive, and he had multiple nodular shadows in both lungs. He had been treated with prednisolone, cyclophosphamide (CPA) and plasma exchange. At the age of 64, a nodular shadow was newly detected in the right lower lung field and serum tumour marker increased. Subsequent positron emission tomography/computed tomography scan demonstrated accumulations of fluorodexyglucose (FDG) in the same area, mediastinum lymph nodes, thoracic wall, right iliac bone, and right retroperitoneum...
February 2018: Respirology Case Reports
https://www.readbyqxmd.com/read/29321417/peripheral-ulcerative-keratitis-associated-with-granulomatosis-with-polyangiitis-emerging-despite-cyclophosphamide-successfully-treated-with-rituximab
#3
Yuya Fujita, Shoichi Fukui, Yushiro Endo, Sosuke Tsuji, Ayuko Takatani, Toshimasa Shimizu, Masataka Umeda, Ayako Nishino, Tomohiro Koga, Shin-Ya Kawashiri, Naoki Iwamoto, Kunihiro Ichinose, Mami Tamai, Hideki Nakamura, Tomoki Origuchi, Ryotaro Ueki, Masafumi Uematsu, Kaori Ishida, Kuniko Abe, Atsushi Kawakami
A 67-year-old Japanese man was diagnosed with granulomatosis with polyangiitis based on the presence of right maxillary sinusitis, proteinase 3 antineutrophil cytoplasmic antibody positivity, and right scleritis. A conjunctival biopsy specimen showed neutrophil-predominant infiltration around the vessels without granuloma. Because there was a risk of blindness, pulsed methylprednisolone and intravenous cyclophosphamide pulse therapy (IVCY) was started. However, it was ineffective, and peripheral ulcerative keratitis newly emerged...
January 11, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29319717/serum-25-hydroxyvitamin-d-levels-in-patients-with-granulomatosis-with-polyangiitis-association-with-respiratory-infection
#4
Mariana O Perez, Ricardo M Oliveira, Mauricio Levy-Neto, Valeria F Caparbo, Rosa M R Pereira
OBJECTIVES: To determine the possible association of serum 25-hydroxyvitamin D (25OHD) levels with disease activity and respiratory infection in granulomatosis with polyangiitis patients during two different periods: winter/spring and summer/autumn. METHODS: Thirty-two granulomatosis with polyangiitis patients were evaluated in the winter/spring, and the same patients (except 5) were evaluated in summer/autumn (n=27). The 25OHD levels were measured by radioimmunoassay...
December 2017: Clinics
https://www.readbyqxmd.com/read/29310745/polyps-grommets-and-eosinophilic-granulomatosis-with-polyangiitis
#5
F G Kavanagh, W Hasan, D A Smyth, J E Fenton
OBJECTIVE: To explore the link between nasal polyposis, refractory otitis media with effusion and eosinophilic granulomatosis with polyangiitis. METHODS: A retrospective observational study was carried out of patients diagnosed with refractory otitis media with effusion necessitating grommet insertion and who had nasal polyps. Patients were evaluated to determine if they fulfilled the diagnostic criteria of eosinophilic granulomatosis with polyangiitis. RESULTS: Sixteen patients (10 males and 6 females) were identified...
January 9, 2018: Journal of Laryngology and Otology
https://www.readbyqxmd.com/read/29303390/orbital-granulomatosis-with-polyangiitis-masquerading-as-invasive-fungal-sinusitis
#6
David Xu, Shawn R Lin, Laura Bonelli, Ben J Glasgow, Robert A Goldberg
A 55-year-old man presented with unilateral orbital inflammation and no light perception vision. Imaging revealed infiltrative enhancement of the optic nerve, orbit, and intracranial tissue. The case was suspicious for invasive fungal disease, but ultimate workup and orbital biopsy revealed granulomatosis with polyangiitis. The patient's inflammation resolved with corticosteroid and rituximab therapy. Granulomatosis with polyangiitis is a systemic vasculitis that can mimic a number of orbital pathologies.
January 5, 2018: Orbit
https://www.readbyqxmd.com/read/29302827/colonic-perforation-due-to-severe-cytomegalovirus-disease-in-granulomatosis-with-polyangiitis-after-immunosuppression
#7
REVIEW
Alessandra Soriano, Nazareno Smerieri, Stefano Bonilauri, Loredana De Marco, Alberto Cavazza, Carlo Salvarani
Granulomatosis with polyangiitis (GPA) is a small-vessel necrotizing granulomatous vasculitis typically involving upper airways, lungs, and kidneys, which may lead to end-organ damage and life-threatening complications. Major infections during GPA course represent a considerable concern in the management of the disease. Cytomegalovirus (CMV) infection and disease are rare but significant complications in the course of GPA being associated with high morbidity and mortality rates. Colonic perforation due to CMV colitis is exceedingly rare and has so far almost exclusively been documented in HIV, renal transplant, and systemic lupus erythematosus patients...
January 4, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29298788/granulomatosis-with-polyangiitis-an-unusual-cause-of-acute-liver-injury
#8
David Owen Rees, Anu Gunavardhan, David Andrew Glover
Granulomatosis with polyangiitis (GPA) causing liver injury is rare. Where liver biopsies have been taken findings are not always typical and diagnosis can be challenging. Here, we present a case of a 58-year-old male who on admission to hospital was found to have acute liver injury. Diagnosis of liver involvement in GPA is supported by liver histology and the resolution of hepatitis after commencement of immunosuppressive treatment.
January 3, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29288039/immunoglobulin-g4-related-chronic-rhinosinusitis-a-pitfall-in-the-differential-diagnosis-of-granulomatosis-with-polyangiitis-rosai-dorfman-disease-and-fungal-rhinosinusitis
#9
Yingshi Piao, Yuan Zhang, Changli Yue, Chengshuo Wang, Luo Zhang
Immunoglobulin G4 (IgG4)-related chronic rhinosinusitis (CRS) has recently been proposed to be a new clinical entity of nasal disease, with no consensually agreed criteria for diagnosis. Moreover, the pathological features if IgG4-related CRS often overlap with other sinonasal inflammatory and autoimmune diseases such as Granulomatosis with Polyangiitis (GPA), Rosai-Dorfman disease (RDD) and Fungal Rhinosinusitis (FRS). We aimed to explore the specific similarities and differences in clinicopathologic features between IgG4-related CRS, and GPA, RDD and FRS, in order that these conditions can be diagnosed more accurately...
December 26, 2017: Human Pathology
https://www.readbyqxmd.com/read/29280820/gastroduodenal-artery-aneurysm-in-a-patient-with-granulomatosis-with-polyangiitis
#10
Claude Bachmeyer, Samuel Haddad, Michel Djibré, Benjamin Langman, Sophie Georgin-Lavialle, Xavier Amiot
No abstract text is available yet for this article.
December 27, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29279498/granulomatosis-with-polyangiitis-complicated-by-hypertrophic-pachymeningitis-presenting-with-simultaneous-multiple-intracerebral-hemorrhages
#11
Eri Kato, Koichiro Tahara, Haeru Hayashi, Aki Shoji, Hiroaki Mori, Tetsuji Sawada
Central nervous system (CNS) involvement in granulomatosis with polyangiitis (GPA), including pachymeningitis and CNS vasculitis, is uncommon. Although intracerebral hemorrhage (ICH) has been reported in GPA, simultaneous multiple ICH (SMICH) is rare. We describe the case of a 50-year-old woman with a history of a limited form of GPA with chronic pachymeningitis who presented with acute-onset headache accompanied by nausea and vomiting, and who developed consciousness impairment. Computed tomography revealed bilateral subcortical ICH...
December 27, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29274703/clinical-significance-of-neutrophil-lymphocyte-ratio-in-patients-with-granulomatosis-with-polyangiitis
#12
Nouran M Abaza, Eiman M Abd El-Latif, Tamer A Gheita
OBJECTIVE: To determine to neutrophil-to-lymphocyte ratio (NLR) in granulomatosis with polyangiitis (GPA) patients and to study its relation to disease manifestations and activity. METHODS: The study included 44 GPA patients and 44 matched age and sex controls. Full history taking, thorough clinical examination with more attention to ocular examination, laboratory and radiological investigations were considered. Disease activity was assessed using the Birmingham Vasculitis Activity Score (BVAS)...
December 20, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/29260039/intracerebral-hemorrhage-after-iv-tpa-for-stroke-as-early-symptom-of-anca-associated-vasculitis
#13
Neda Zarghami Esfahani, Daniel M Anderson, Connie Pieper, Harold P Adams
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare diseases characterized by a necrotizing small-vessel vasculitis and circulating ANCA that comprise granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (EGPA). Acute ischemic stroke (AIS) can be a manifestation of central nervous system (CNS) involvement in these diseases. Furthermore, intracerebral hemorrhage (ICH) is a potential complication of these necrotizing vasculitides...
December 2017: ENeurologicalSci
https://www.readbyqxmd.com/read/29258731/characteristics-and-visual-outcome-of-refractory-retinal-vasculitis-associated-with-antineutrophil-cytoplasm-antibody-associated-vasculitides
#14
Miaoli Lin, Stephen D Anesi, Lina Ma, Aseef Ahmed, Karen Small, C Stephen Foster
PURPOSE: To describe the clinical characteristics, therapies, visual outcomes and prognoses of patients with retinal vasculitis associated with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). DESIGN: Retrospective case series. METHODS: Patients diagnosed with retinal vasculitis associated with AAV and at least 6-months of follow-up were included. Demographic data, systemic and ocular features, best corrected visual acuity at the initial visit and latest visit, fluorescein angiography (FA) and indocyanine green angiography (ICGA) findings, therapy regimen and outcome were collected from the Massachusetts Eye Research and Surgery Institution (MERSI) database from 2006 to 2017...
December 16, 2017: American Journal of Ophthalmology
https://www.readbyqxmd.com/read/29250311/an-unusual-presentation-of-eosinophilic-angiocentric-fibrosis
#15
Joel Hardman, Christopher Toon, Arjuna Nirmalananda
Eosinophilic angiocentric fibrosis (EAF) is a rare, benign condition affecting the respiratory mucosa and is generally characterized by a locally destructive growth. We present a case of a lady with a saddle nose deformity that had for many years been treated as granulomatosis with polyangiitis (GPA), of which saddle nose deformity is a classic feature. At the time of surgery, she was found to have subglottic stenosis another classic feature of GPA, however, histology demonstrated EAF. We discuss the difference between the two conditions and highlight the importance of making the correct diagnosis...
December 2017: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/29239918/anti-neutrophil-cytoplasmic-antibody-negative-central-nervous-system-granulomatosis-with-polyangiitis-and-its-clinical-characteristics
#16
Zhihua Chen, Yifeng Miao, Hui Wu, Ran Wang, Zhiyi Zhou, Shilei Zhang, Longtian Chen, Yongming Qiu
Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous vasculitis occasionally affecting central nervous system (CNS), and GPA patients with initial CNS symptoms are even rarer, whose diagnosis is further confused by an absence of positive antineutrophil cytoplasmic antibody. The authors described the characteristics of antineutrophil cytoplasmic antibody -negative GPA with CNS onset in a patient and discussed on its management, which may contribute to future diagnosis and treatment of patients with similar conditions...
December 12, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29238021/-granulomatosis-with-polyangiitis-complicated-with-gastrointestinal-perforation-a-case-report-and-review-of-literature
#17
Takao Kiboshi, Kentaro Isoda, Koichi Furukawa, Tomoyuki Wakahara, Kenichiro Otani, Kayo Ueda, Junichi Konma, Kazuhiro Teramura, Nozomi Ueno, Hiroshi Fujiwara, Takeshi Shoda
  A 51-year-old man was detected nasal bleeding, multiple pulmonary nodule and mass, urinalysis abnormality, renal involvement and high titer of proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA), and was suspected of granulomatosis with polyangiitis and initiated with steroid pulse therapy. On the day after the start of steroid pulse therapy, generalized peritonitis due to ileal perforation occurred, and emergency ileectomy and peritonitis surgery were performed. Induction therapy with steroid pulse therapy, plasma exchange and intravenous cyclophosphamide therapy (IVCY) and maintenance therapy with glucocorticoid and azathioprine led to good therapeutic outcomes...
2017: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29230559/increased-histopathological-yield-for-granulomatosis-with-polyangiitis-based-on-nasal-endoscopy-of-suspected-active-lesions
#18
Olga Beltrán Rodríguez-Cabo, Edgardo Reyes, Jorge Rojas-Serrano, Luis Felipe Flores-Suárez
PURPOSE: To present the results of an endoscopic and histopathologic evaluation of suspected nasal active granulomatosis with polyangiits (GPA) lesions, describe them as seen by the ENT specialist, and propose a guide for tissue sampling of the nasal cavity to improve the yield of confirmatory histology. METHODS: Randomly selected patients seen from December 1997-October 2007 had a thorough endoscopic nasal evaluation, preceded by careful cleansing of the nasal cavity...
December 11, 2017: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/29225784/diagnosing-alpha-1-antitrypsin-deficiency-the-first-step-in-precision-medicine
#19
REVIEW
Craig P Hersh
Severe alpha-1 antitrypsin (AAT) deficiency is one of the most common serious genetic diseases in adults of European descent. Individuals with AAT deficiency have a greatly increased risk for emphysema and liver disease. Other manifestations include bronchiectasis, necrotizing panniculitis and granulomatosis with polyangiitis. Despite the frequency and potential severity, AAT deficiency remains under-recognized, and there is often a delay in diagnosis. This review will focus on three recent updates that should serve to encourage testing and diagnosis of AAT deficiency: first, the publication of a randomized clinical trial demonstrating the efficacy of intravenous augmentation therapy in slowing the progression of emphysema in AAT deficiency; second, the mounting evidence showing an increased risk of lung disease in heterozygous PI MZ genotype carriers; last, the recent publication of a clinical practice guideline, outlining diagnosis and management...
2017: F1000Research
https://www.readbyqxmd.com/read/29200025/granulomatosis-with-polyangiitis-in-a-young-adult-with-down-syndrome-therapeutic-challenges-and-clues-to-immunopathogenesis
#20
Kofi A Mensah, Victoria Malek Pascha, Gilbert Moeckel, Abhijeet Danve
No abstract text is available yet for this article.
December 1, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
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