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granulomatosis with polyangiitis

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https://www.readbyqxmd.com/read/28713428/clinically-apparent-arterial-thrombosis-in-persons-with-systemic-vasculitis
#1
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
#2
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/28689619/two-cases-of-granulomatosis-polyangiitis-presenting-with-strawberry-gingivitis-and-a-review-of-the-literature
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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/28637076/-clinical-course-of-an-early-childhood-onset-granulomatosis-with-polyangiitis
#13
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
#14
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
#15
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
#16
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...
June 16, 2017: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/28615072/chemokine-receptor-co-expression-reveals-aberrantly-distributed-th-effector-memory-cells-in-gpa-patients
#17
Lucas L Lintermans, Abraham Rutgers, Coen A Stegeman, Peter Heeringa, Wayel H Abdulahad
BACKGROUND: Persistent expansion of circulating CD4(+) effector memory T cells (TEM) in patients with granulomatosis with polyangiitis (GPA) suggests their fundamental role in disease pathogenesis. Recent studies have shown that distinct functional CD4(+) TEM cell subsets can be identified based on expression patterns of chemokine receptors. The current study aimed to determine different CD4(+) TEM cell subsets based on chemokine receptor expression in peripheral blood of GPA patients...
June 14, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28611282/the-4-plus-2-rituximab-protocol-makes-maintenance-treatment-unneeded-in-patients-with-refractory-anca-associated-vasculitis-a-10-year-observation-study
#18
Dario Roccatello, Savino Sciascia, Daniela Rossi, Mirella Alpa, Carla Naretto, Massimo Radin, Roberta Fenoglio, Simone Baldovino, Elisa Menegatti
BACKGROUND: ANCA associated vasculitides (AAV) often present with a chronic relapsing course. Relapse leads to increased immunosuppressive exposure and consequent toxicity. While two randomized controlled trials have shown rituximab (RTX) to be the most effective induction treatment in patients with relapsing disease, the optimal treatment duration and RTX dose remain debated. Whether to administer a maintenance dose to every patient, at a fixed time interval or on the basis of B cell count and ANCA titre or only when disease manifestations do occur is still debated as well...
May 23, 2017: Oncotarget
https://www.readbyqxmd.com/read/28608042/-granulomatous-reaction-pattern-of-the-skin-interstitial-granulomatous-dermatitis-lymphoma-vasculitis
#19
REVIEW
C Rose, K Holl-Ulrich
A particular diagnostic situation in the classification of a granulomatous dermatitis results when no circumscribed granulomas but instead a diffuse interstitial histiocytic inflammatory infiltrate, a granulomatous vasculitis or a neoplastic lymphocytic infiltrate is found. Interstitial granulomatous dermatitis was originally described in patients with arthritis. Later, it was recognized that there are also associations with other usually immunological diseases. Differentiating between interstitial granulomatous dermatitis and the interstitial form of granuloma annulare, early morphea and variants of borreliosis or scleromyxedema as well as interstitial granulomatous drug reaction can be very difficult...
June 12, 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28599871/a-case-report-of-granulomatosis-with-polyangiitis-with-granulomas-in-bone-marrow
#20
Isabel de la Cámara Fernández, Fernando Lozano Morillo, Luis Morillas López, Mario Martínez López
No abstract text is available yet for this article.
June 6, 2017: Reumatología Clinica
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