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https://www.readbyqxmd.com/read/29139157/seronegative-granulomatosis-with-polyangiitis-presenting-with-multiple-cranial-nerve-palsies
#1
Eunhye Lee, Jynkyun Park, Seung Hong Choi, Sung-Hye Park
Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a rare systemic disease characterized by necrotizing granulomas and vasculitis that usually presents in the respiratory tract and renal system. Here, we report a case of anti-neutrophil cytoplasmic antibody (ANCA)-negative GPA with diagnostic difficulties. Neurologic involvement in GPA is not uncommon, with up to 50% of patients manifesting with neurologic defects; however, it is usually a late event. This patient was a 50-year-old man whose first manifestation was multiple neurologic defects without respiratory or renal symptoms...
November 14, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29136066/unsuspected-lymphomatoid-granulomatosis-in-a-patient-with-antisynthetase-syndrome
#2
Stephanie L Skala, Alexandra C Hristov, Johann E Gudjonsson, May P Chan
Clinical diagnosis of lymphomatoid granulomatosis (LYG) often is difficult, especially in patients with multiple comorbidities. We present a 60-year-old woman with worsening fatigue, night sweats, unintentional weight loss, and dyspnea of 2 weeks' duration. Her medical history was remarkable for recent radiation therapy for recurrent breast cancer and antisynthetase syndrome complicated by interstitial lung disease and controlled with azathioprine. Computed tomography showed ground-glass opacities and nodular infiltrates in all lung lobes, raising concern for radiation pneumonitis and drug toxicity...
October 2017: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/29127573/eosinophilic-granulomatosis-with-polyangiitis-complicated-by-subarachnoid-hemorrhage-and-coronary-vasculitis-a-case-report-and-review-of-the-literature
#3
REVIEW
Shogo Matsuda, Shuzo Yoshida, Youhei Fujiki, Hidetoshi Satomi, Tohru Takeuchi, Yoshinobu Hirose, Shigeki Makino, Shigeki Arawaka
Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by necrotizing vasculitis of small-sized vessels with extravascular granulomas and eosinophilic infiltration. The case of a 48-year-old Japanese woman with EGPA, who presented concurrently with subarachnoid hemorrhage (SAH) and coronary vasculitis, is reported. She initially presented with bronchial asthma, and then 8 months later she developed various symptoms caused by systemic eosinophilic vasculitis and was admitted to our hospital. Three days after admission, she started oral corticosteroid therapy, and her 2009 Five-Factor Score (FFS) was 0...
November 10, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29125145/the-2017-doyne-lecture-the-orbit-as-a-window-to-systemic-disease
#4
REVIEW
A A McNab
A very large number of disorders affect the orbit, and many of these occur in the setting of systemic disease. This lecture covers selected aspects of orbital diseases with systemic associations in which the author has a particular clinical or research interest. Spontaneous orbital haemorrhage often occurs in the presence of bleeding diatheses. Thrombosis of orbital veins and ischaemic necrosis of orbital and ocular adnexal tissues occur with thrombophilic disorders, vasculitis, and certain bacterial and fungal infections...
November 10, 2017: Eye
https://www.readbyqxmd.com/read/29124398/patient-perceptions-of-glucocorticoids-in-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis
#5
Joanna C Robson, Jill Dawson, Peter F Cronholm, Susan Ashdown, Ebony Easley, Katherine S Kellom, Don Gebhart, Georgia Lanier, Nataliya Milman, Jacqueline Peck, Raashid A Luqmani, Judy A Shea, Gunnar Tomasson, Peter A Merkel
Granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) are multisystem diseases of small blood vessels, collectively known as the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). This study explores the patient's perspective on the use of glucocorticoids, which are still a mainstay of treatment in AAV. Patients with AAV from the UK, USA, and Canada were interviewed, using purposive sampling to include a range of disease manifestations and demographics...
November 9, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29121188/complete-vision-loss-from-granulomatosis-with-polyangiitis
#6
Akosua A Nti, Grant T Liu
No abstract text is available yet for this article.
November 9, 2017: JAMA Ophthalmology
https://www.readbyqxmd.com/read/29121164/granulomatosis-with-polyangiitis-a-moral-impetus-for-change
#7
Marc Geoffrey Lubitz
No abstract text is available yet for this article.
November 9, 2017: JAMA Otolaryngology—Head & Neck Surgery
https://www.readbyqxmd.com/read/29119480/rheumatoid-factor-false-positivity-in-patients-with-anca-associated-vasculitis-not-having-medical-conditions-producing-rheumatoid-factor
#8
Jae-Seung Moon, Diane Da-Hyun Lee, Yong-Beom Park, Sang-Won Lee
We investigated the rate of rheumatoid factor (RF) false positivity at diagnosis and its influence on clinical and prognostic features and rheumatoid arthritis (RA) development during the follow-up in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients without RA or other medical conditions triggering RF false positivity. We reviewed the medical records of 128 AAV patients. We divided patients with AAV and each variant into two groups according to RF positivity and compared variables between the two groups...
November 8, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29112013/cancer-therapy-associated-lymphoproliferative-disorders-an-under-recognized-type-of-immunodeficiency-associated-lymphoproliferative-disorder
#9
Sergio Pina-Oviedo, Roberto N Miranda, L Jeffrey Medeiros
We describe the clinicopathologic features of 17 patients who had a hematologic malignancy of various types, were treated, and subsequently developed a lymphoproliferative disorder (LPD). There were 10 men and 7 women with a median age of 59 years (range, 36 to 83 y). The primary hematologic neoplasms included: 5 chronic lymphocytic leukemia/small lymphocytic lymphoma, 3 plasma cell myeloma, 2 acute monoblastic leukemia, and 1 case each of mixed-phenotype acute leukemia, chronic myeloid leukemia, splenic marginal zone lymphoma, follicular lymphoma, mantle cell lymphoma, T-cell prolymphocytic leukemia, and peripheral T-cell lymphoma...
November 3, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29105377/cryotherapy-to-treat-and-prevent-airway-stenosis-in-a-patient-with-granulomatosis-with-polyangiitis
#10
Chantal Y Spencer, Timothy J Harkin, Alfin G Vicencio
We report our use of cryotherapy delivered via flexible bronchoscopy in a 15-year old girl with granulomatosis with polyangiitis to both treat established airway stenosis and prevent multi-level progression of disease.
November 3, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29100647/subglottic-stenosis-in-granulomatosis-with-polyangiitis-the-role-of-laryngotracheal-resection
#11
Christina L Costantino, John L Niles, Cameron D Wright, Douglas J Mathisen, Ashok Muniappan
BACKGROUND: Granulomatosis with polyangiitis (GPA) is associated with development of subglottic stenosis in about one-fourth of all patients. Although endoscopic management is the primary treatment method for tracheobronchial stenosis, some patients have refractory disease, and tracheostomy is required. It is unclear if laryngotracheal resection and reconstruction (LTRR) can be safely performed in patients with GPA. METHODS: A retrospective review was performed of 11 patients with GPA undergoing LTRR...
November 1, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29099568/a-case-report-on-suspected-levamisole-induced-pseudovasculitis
#12
Tiffany Fan, Jeffrey Macaraeg, Toufik Mahfood Haddad, Holly Bacon, Duc Le, Mohsin Mirza, Carrie Valenta, Tammy Wichman
INTRODUCTION: Levamisole-induced pseudovasculitis should be considered in patients with inconsistent anti-neutrophil cytoplasmic antibodies (ANCA) pattern and history of cocaine use. CASE PRESENTATION: A 50-year-old man presented to the emergency department with symptoms of bilateral pulmonary emboli. His hospital course was complicated by multiple end organ failure, which improved dramatically with prednisone. Although he was diagnosed previously with granulomatosis with polyangiitis due to positive proteinase 3 (PR3), myeloperoxidase (MPO), perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCA) and cytoplasmic anti-neutrophil cytoplasmic antibodies (C-ANCA) markers, his longstanding cocaine use and history of skin ulcers, thrombotic events, and febrile illnesses suggested a diagnosis of levamisole-induced pseudovasculitis instead...
February 2017: WMJ: Official Publication of the State Medical Society of Wisconsin
https://www.readbyqxmd.com/read/29096807/eosinophilic-myocarditis-characteristics-treatment-and-outcomes
#13
Michela Brambatti, Maria Vittoria Matassini, Eric D Adler, Karin Klingel, Paolo G Camici, Enrico Ammirati
BACKGROUND: Eosinophilic myocarditis (EM) is an acute life-threatening inflammatory disease of the heart. Neither large case series nor clinical trials on this specific myocarditis have been reported. OBJECTIVES: Based on a systematic revision of all published histologically proven cases, this study aimed to describe the clinical presentation, treatment, and outcome of EM. METHODS: The study screened 443 manuscripts in MEDLINE and EMBASE on cases of EM published until June 2017...
November 7, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29093401/diffuse-alveolar-hemorrhage-developing-immediately-after-immunosuppressive-treatments-in-a-patient-with-granulomatosis-with-polyangiitis-who-had-pulmonary-nodules
#14
Chiaki Watanabe, Shoichi Fukui, Naoki Iwamoto, Toshimasa Shimizu, Masataka Umeda, Ayako Nishino, Tomohiro Koga, Shin-Ya Kawashiri, Kunihiro Ichinose, Yasuko Hirai, Mami Tamai, Hideki Nakamura, Tomoki Origuchi, Kazuhiro Tabata, Atsushi Kawakami
A 65-year-old man was diagnosed with granulomatosis with polyangiitis (GPA) based on the detection of high MPO-ANCA, vasculitis and granulomas in a lung biopsy specimen and crescentic glomerulonephritis in a kidney biopsy specimen. Soon after the initiation of intravenous methylprednisolone pulse therapy (mPSL pulse) and intravenous cyclophosphamide pulse therapy (IVCY), the patient experienced cough and hemoptysis. Based on emerging anemia and bilateral diffuse lung consolidation on computed tomography, we judged that diffuse alveolar hemorrhage (DAH) was complicated by GPA...
November 1, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29072665/-granulomatosis-with-polyangiitis-associated-with-the-cytoplasmic-antineutrophil-antibodies
#15
A I Kryukov, G P Bondareva, O M Kurbacheva, V A Utesheva
The objective of the present work was to analyze the clinical case of granulomatosis with polyangiitis associated with the presence of cytoplasmic antineutrophil antibodies. We considered the medical records of the patient presenting with this condition. It was shown that systemic vasculitis had a polymorphic clinical picture; its diagnostics and treatment encountered serious difficulties. It is concluded that the development of progressive perforation of the nasal septum and destructive changes in the intranasal and adjacent structures after the endonasal surgical intervention implies the necessity of the detailed analysis of the clinical and laboratory observations with the subsequent counselling by a rheumatologist and oncologist for the clarification of the diagnosis and the choice of adequate therapy...
2017: Vestnik Otorinolaringologii
https://www.readbyqxmd.com/read/29069513/a-cohort-study-of-comorbidity-in-patients-with-granulomatosis-with-polyangiitis
#16
Lin Li, Tuhina Neogi, Susan Jick
Objectives: To evaluate the associations between granulomatosis with polyangiitis (GPA) and a wide range of comorbidities in patients with GPA compared with population-based non-vasculitis patients. Methods: Using the UK-based Clinical Practice Research Datalink we identified 570 incident patients with GPA in 1990-2014, and for each case, we selected up to 10 random non-vasculitis patients matched for age, sex, practice and years of history before the date of first GPA diagnosis...
October 23, 2017: Rheumatology
https://www.readbyqxmd.com/read/29066732/associations-between-genetic-risk-variants-for-kidney-diseases-and-kidney-disease-etiology
#17
Sebastian Wunnenburger, Ulla T Schultheiss, Gerd Walz, Birgit Hausknecht, Arif B Ekici, Florian Kronenberg, Kai-Uwe Eckardt, Anna Köttgen, Matthias Wuttke
Chronic kidney disease (CKD) is a global health problem with a genetic component. Genome-wide association studies have identified variants associated with specific CKD etiologies, but their genetic overlap has not been well studied. This study examined SNP associations across different CKD etiologies and CKD stages using data from 5,034 CKD patients of the German Chronic Kidney Disease study. In addition to confirming known associations, a systemic lupus erythematosus-associated risk variant at TNXB was also associated with CKD attributed to type 1 diabetes (p = 2...
October 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29065902/treatment-of-orofacial-granulomatosis-a-case-report
#18
Maaz B Badshah, Saqib Walayat, Umair Ahmed, Sonu Dhillon, Sherri Yong, Sunanda Kane, Shoba Thievanayagam
BACKGROUND: Orofacial granulomatosis is a relatively recent term coined by Wiesenfield et al. in 1985 to define granulomatous lesions of oral mucosa without intestinal involvement. When it presents in a triad encompassing facial nerve palsy, lip swelling, and fissured or furrowed tongue it is called Melkersson-Rosenthal syndrome while monosymptomatic or oligosymptomatic forms are referred to as granulomatous cheilitis. It is an uncommon clinicopathologic entity which is distinct from classic Crohn's disease...
October 25, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29062247/frequency-of-mycobacterium-bovis-and-mycobacteria-in-primary-immunodeficiencies
#19
Ezgi Ulusoy, Neslihan Edeer Karaca, Güzide Aksu, Cengiz Çavuşoğlu, Necil Kütükçüler
AIM: Susceptibility to mycobacterial diseases is observed in some primary immunodeficiency diseases. In this study, we aimed to evaluate mycobacterial infections in primary immunodeficiency diseases. MATERIAL AND METHODS: Patients under follow-up by Ege University Pediatric Immunology Department for severe combined and combined immunodeficiencies, interleukin 12/ interferon gamma receptor deficiency, nuclear factor kappa-beta essential modulator deficiency and chronic granulomatosis disease were evaluated retrospectively in terms of the frequency and characteristics of mycobacterial infections using a questionnaire form for demographic properties, clinical features and laboratory tests...
September 2017: Türk Pediatri Arşivi
https://www.readbyqxmd.com/read/29061243/central-nervous-system-disease-in-antineutrophil-cytoplasmic-antibodies-associated-vasculitis
#20
REVIEW
Jonathan Graf
Central nervous system (CNS) disease is an uncommon but significant complication of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) and affects 3 primary areas of the CNS: the pituitary, the pachymeninges, and the CNS vasculature. Pituitary disease in uncommon, but hormonal deficiencies can be long lasting even in the face of excellent disease response. Chronic hypertrophic pachymeninigitis occurs in anti-proteinase 3-positive patients with systemic GPA and in anti-myeloperoxidase-positive patients with a milder and more limited form of the disease...
November 2017: Rheumatic Diseases Clinics of North America
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