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https://www.readbyqxmd.com/read/28724596/severe-destructive-nasopharyngeal-granulomatosis-with-polyangiitis-with-superimposed-skull-base-pseudomonas-aeruginosa-osteomyelitis
#1
Mitchell S von Itzstein, Jithma P Abeykoon, Daniel D Summerfield, Jennifer A Whitaker
Skull base osteomyelitis in the setting of granulomatosis with polyangiitis (GPA) is rare and entails significant diagnostic challenges. We present a case of a 65-year-old Caucasian man with a history of rheumatoid arthritis, off immunosuppression for 18 months, who presented with 2 years of chronic headaches, severe fatigue, saddle nose deformity and 20-kilogram unintentional weight loss. Maxillofacial CT revealed an extensive destructive sinonasal and erosive skull base process. Laboratory evaluation showed equivocal elevation of antiproteinase 3 antibodies with negative antineutrophil cytoplasmic antibody panel...
July 19, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28713428/clinically-apparent-arterial-thrombosis-in-persons-with-systemic-vasculitis
#2
Alexander Tsoukas, Sasha Bernatsky, Lawrence Joseph, David L Buckeridge, Patrick Bélisle, Christian A Pineau
OBJECTIVE: To estimate the incidence rate of clinically apparent arterial thrombotic events and associated comorbidities in patients with primary systemic vasculitis. METHODS: Using large cohort administrative data from Quebec, Canada, we identified patients with vasculitis, including polyarteritis nodosa (PAN) and granulomatosis with polyangiitis (GPA). Incident acute myocardial infarctions (AMIs) and cerebrovascular accidents (CVAs) after the diagnosis of vasculitis were ascertained in the PAN and GPA group via billing and hospitalization data...
2017: International Journal of Rheumatology
https://www.readbyqxmd.com/read/28709761/central-nervous-system-involvement-in-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-report-of-26-patients-and-review-of-the-literature
#3
REVIEW
Raphaël André, Vincent Cottin, Jean-Luc Saraux, Gilles Blaison, Boris Bienvenu, Pascal Cathebras, Romain Dhote, Aurélie Foucher, Helder Gil, Joëlle Lapoirie, David Launay, Valentine Loustau, François Maurier, Edouard Pertuiset, Thierry Zénone, Jörg Seebach, Nathalie Costedoat-Chalumeau, Xavier Puéchal, Luc Mouthon, Loïc Guillevin, Benjamin Terrier
BACKGROUND: Although peripheral nervous system involvement is common in eosinophilic granulomatosis with polyangiitis (EGPA), central nervous system (CNS) manifestations are poorly described. This study aimed to describe CNS involvement in EGPA. PATIENTS AND METHODS: This retrospective, observational, multicenter study included patients with EGPA and CNS involvement affecting cranial nerves, brain and/or spinal cord. We also undertook a systematic literature review...
July 11, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28693060/-an-analysis-of-four-cases-of-misdiagnosed-primary-lymphocytic-hypophysitis
#4
Q Wei, L Zang, Y J Li, W J Gu, N Jin, Q H Guo, J Du, J M Ba, Z H Lyu, J M Lu, J T Dou, Y M Mu, G Q Yang
To improve the differential diagnosis of sellar region mass, 4 cases with sellar mass and misdiagnosed as lymphocytic hypophysitis (LYH) were reviewed retrospectively.The 4 patients (2 male and 2 female) aged 20-60 years old were all presented with symptoms of headache, polydipsia and polyuria.Biochemical studies confirmed the diagnoses of central diabetes insipidus and hypopituitarism.Head MRI scans showed LYH like image for all the cases, and, thus, high dose methylprednisolone pulse therapy (HDMPT) was applied to the patients...
July 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28689619/two-cases-of-granulomatosis-polyangiitis-presenting-with-strawberry-gingivitis-and-a-review-of-the-literature
#5
Grace Thompson, Nicola Benwell, Peter Hollingsworth, Andrew McLean-Tooke
Hyperplastic gingivitis is a rare manifestation of granulomatosis with polyangiitis (GPA). This gingivitis has a very distinctive clinical appearance (so-called Strawberry gingivitis) and when seen is virtually pathognomic for GPA. Gingivitis often precedes other organ involvement therefore making awareness of this manifestation particularly important to aid early diagnosis and treatment. Furthermore, histopathological findings of gingival specimens rarely reveal necrotizing granulomatous vasculitis, which is classically seen at other sites of involvement...
June 13, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28680302/eosinophilic-granulomatosis-with-polyangiitis-presented-as-acute-polyneuropathy-and-cerebral-vasculitis
#6
Il-Han Yoo, Sang Tae Choi, Seong-Ho Choi, Jeong-Min Kim, Suk-Won Ahn
Eosinophilic granulomatosis with polyangiitis (EGPA) is an immune related systemic disease that is caused by vasculitis affecting multiple organ systems. It is characterized by asthma, fever, eosinophilia, cardiac problems, renal injury, and peripheral neuropathy. In this report, we describe a patient with EGPA with concurrent cerebral infarction and acute polyneuropathy mimicking a Guillain-Barre syndrome (GBS). A 46-year-old man presented with rapidly progressing gait disturbance, muscular weakness, and tingling sensation in all four limbs...
June 2017: Experimental Neurobiology
https://www.readbyqxmd.com/read/28678392/adding-azathioprine-to-remission-induction-glucocorticoids-for-eosinophilic-granulomatosis-with-polyangiitis-microscopic-polyangiitis-or-polyarteritis-nodosa-without-poor-prognosis-factors-a-randomized-controlled-trial
#7
Xavier Puéchal, Christian Pagnoux, Gabriel Baron, Thomas Quémeneur, Antoine Néel, Christian Agard, François Lifermann, Eric Liozon, Marc Ruivard, Pascal Godmer, Nicolas Limal, Arsène Mékinian, Thomas Papo, Anne-Marie Ruppert, Anne Bourgarit, Boris Bienvenu, Loïck Geffray, Jean-Luc Saraux, Elisabeth Diot, Bruno Crestani, Xavier Delbrel, Laurent Sailler, Pascal Cohen, Véronique Le Guern, Benjamin Terrier, Matthieu Groh, Claire Le Jeunne, Luc Mouthon, Philippe Ravaud, Loïc Guillevin
OBJECTIVE: Most patients with nonsevere systemic necrotizing vasculitides (SNVs) achieve remission with glucocorticoids alone but one-third relapse within 2 years. This study aimed to determine whether azathioprine adjunction to glucocorticoids could achieve a higher sustained remission rate of newly diagnosed nonsevere eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis (MPA) or polyarteritis nodosa (PAN). METHODS: All patients included in this double-blind trial received glucocorticoids, gradually tapered over 12 months, and were randomized to receive azathioprine or placebo for 12 months, with stratification according to SNV (EGPA or MPA/PAN)...
July 5, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28674566/pseudotumoral-form-of-granulomatosis-with-polyangiitis
#8
Fadwa Mekouar, Naoual El Omri
No abstract text is available yet for this article.
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28668967/anatomy-revisited-hemodialysis-catheter-malposition-in-the-left-ascending-lumbar-vein
#9
Stefan Büttner, Sammy Patyna, Sarah Rudolf, Despina Avaniadi, Moritz Kaup, Helmut Geiger, Christoph Betz
In selected cases, cuffed tunneled catheters via the iliac vein are implanted as a last resort access for hemodialysis. To monitor the correct position, sonography of the inferior vena cava (IVC) is sufficient in most cases. Position control using an X-ray of the abdomen is not routinely recommended when femoral catheters are implanted. In this report, we describe the case of a 59-year-old patient on chronic hemodialysis due to granulomatosis with polyangiitis and complex shunt history with multiple shunt occlusions and revisions...
July 1, 2017: Blood Purification
https://www.readbyqxmd.com/read/28666565/a-novel-strategy-with-combined-assays-for-detection-of-anti-neutrophil-cytoplasmic-antibody-anca-in-clinically-anca-negative-granulomatosis-with-polyangiitis-patients
#10
Kaori Tateyama, Satoru Kodama, Kan Kishibe, Yasuaki Harabuchi, Masashi Suzuki
OBJECTIVE: Granulomatosis with polyangiitis (GPA) that is localized to the upper airway presents a diagnostic challenge because of a tendency towards anti-neutrophil cytoplasmic antibody (ANCA)-negativity. The purpose of this study was to investigate whether positivity of ANCA detection might be elicited with combined use of enzyme-linked immunosorbent assay (ELISA) kits. METHODS: Twenty-nine serum samples obtained from GPA patients were used in this study. In addition to routine biochemical investigation for ANCA, tests for detecting PR3-, MPO-ANCAs, and minor ANCAs were performed with commercially available ELISA kits...
June 27, 2017: Auris, Nasus, Larynx
https://www.readbyqxmd.com/read/28663599/palate-perforation-differentiates-cocaine-induced-midline-destructive-lesions-from-granulomatosis-with-polyangiitis
#11
M Trimarchi, S Bondi, E Della Torre, M R Terreni, M Bussi
Cocaine abuse occasionally causes extensive destruction of the osteocartilaginous structures of the nose, sinuses and palate, which mimics the clinical picture of other diseases associated with necrotising midfacial lesions. The differentiation of cocaine-induced midline destructive lesions (CIMDL) and limited granulomatosis with polyangiitis (GPA) may be difficult, particularly if patients do not readily admit substance abuse. We studied 10 patients with CIMDL and palate perforation referred to our Unit between 2002 and 2015...
June 30, 2017: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/28663246/cardiogenic-shock-from-coronary-vasculitis-in-granulomatosis-with-polyangiitis
#12
Vikram Raghunathan, Aryeh Pelcovits, Daniel Gutman, Gerardo Carino
Granulomatosis with polyangiitis (GPA) is a systemic vasculitis characterised by necrotising inflammatory changes in small-sized and medium-sized vessels and granuloma formation. It most commonly involves the kidneys and respiratory tract, but it can present with widespread manifestations involving any organ system. Rarely, it causes coronary vasculitis which can precipitate a severe cardiomyopathy. Here, we report a patient who presented in cardiogenic shock requiring vasopressors and was found to have extensive myocardial ischaemia secondary to coronary vasculitis...
June 29, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28650889/occult-renal-granulomatous-inflammatory-lesions-in-granulomatosis-with-polyangiitis-detected-by-18f-fdg-pet-ct
#13
Zhanli Fu, Meng Liu, Ziao Li, Jin Zhang, Qian Li
A 33-year-old woman with a 7-month history of granulomatosis with polyangiitis F-FDG PET/CT scan due to low-grade intermittent fever and elevation of serum inflammatory markers, which revealed multiple hypermetabolic lesions in bilateral kidneys. Biopsy of a lesion showed granulomatosis with polyangiitis, consistent with renal involvement of granulomatosis with polyangiitis.
June 24, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28645447/colitis-as-a-form-of-presentation-of-eosinophilic-granulomatosis-with-polyangiitis
#14
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/28645431/bronchocentric-granulomatosis-in-an-immunocompetent-case
#15
Cengiz Özdemir, Sinem Nedime Sökücü
No abstract text is available yet for this article.
June 20, 2017: Archivos de Bronconeumología
https://www.readbyqxmd.com/read/28637076/-clinical-course-of-an-early-childhood-onset-granulomatosis-with-polyangiitis
#16
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
#17
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
#18
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
#19
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...
August 2017: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/28619741/saddle-nose-and-bilateral-cauliflower-ear-deformities-with-pyoderma-gangrenosum-like-ulcers-cavitary-pulmonary-lesions-digital-gangrene-and-pulselessness-in-a-young-female
#20
Sweta Subhadarshani, Vishal Gupta, Anurag Chahal, Kaushal K Verma
We report a young female who presented with saddle-nose and bilateral cauliflower ear deformities along with pyoderma gangrenosum-like ulcers, digital gangrene and pulselessness. Subsequently, she was found to have bilateral conductive hearing loss, a corneal opacity, mild aortic regurgitation and radiological evidence of cavitary changes in lungs and aortoarteritis. Our patient had a constellation of symptoms which posed a diagnostic challenge. Finally, a diagnosis of relapsing polychondritis with several unusual features was made...
June 15, 2017: BMJ Case Reports
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