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https://www.readbyqxmd.com/read/29344060/neuro-ophthalmic-presentation-of-neuro-sweet-disease
#1
Padmaja Sudhakar, Stuart Tobin, William O Connor, Sachin Kedar
Acute febrile neutrophilic dermatosis (Sweet syndrome) is a systemic inflammatory condition usually associated with autoimmune or neoplastic processes and characterised by inflammatory dermatologic lesions such as erythematous plaques and papules associated with fever and leukocytosis. Neurological and ophthalmological involvement is rare. The authors describe an unusual case of Sweet syndrome associated with microscopic polyangiitis presenting with papilloedema, anterior uveitis, and skin rash. Years later, he developed acute posterior multifocal placoid pigment epitheliopathy...
August 2017: Neuro-ophthalmology
https://www.readbyqxmd.com/read/29336783/bronchiectasis-is-highly-prevalent-in-anti-mpo-anca-associated-vasculitis-and-is-associated-with-a-distinct-disease-presentation
#2
Antoine Néel, Alexandra Espitia-Thibault, Pierre-Paul Arrigoni, Christelle Volteau, Marie Rimbert, Agathe Masseau, Christian Agard, Fadi Fakhouri, Renan Liberge, Mohamed Hamidou
OBJECTIVES: To assess the prevalence of bronchiectasis in a Western cohort with ANCA-positive granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) and its correlations with disease presentation and outcome. METHODS: Retrospective study of ANCA-associated vasculitis (AAV) patients followed at Nantes University Hospital (2005-2015). Clinical, biological, and follow-up data were collected through chart review. Two experienced radiologists blinded to the clinical data interpreted chest high-resolution CTs according to the Feischner Society criteria...
December 7, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29328034/characteristics-of-articular-syndrome-in-systemic-vasculitis
#3
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/29279515/anti-neutrophil-cytoplasmic-antibody-associated-vasculitis-aav-restricted-to-the-limbs-a-case-report
#4
Yoshie Ojima, Kinya Sawada, Hiroshi Fujii, Tsuyoshi Shirai, Ayako Saito, Saeko Kagaya, Satoshi Aoki, Yoichi Takeuchi, Tomonori Ishii, Tasuku Nagasawa
A previously healthy 58-year-old man was admitted for muscle pain and weakness (manual muscle testing [MMT] of 4/4 for upper and lower limbs). We detected elevated levels of inflammatory makers and PR3-anti-neutrophil cytoplasmic antibody (ANCA). Subsequently, the muscle weakness rapidly progressed to an MMT of 2 for all limbs. Magnetic resonance imaging indicated muscle edema, and the CK level increased to 29,998 U/L. mPSL and cyclophosphamide pulse therapy improved the patient symptoms. MMT recovered to 4 for all limbs...
December 27, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29260039/intracerebral-hemorrhage-after-iv-tpa-for-stroke-as-early-symptom-of-anca-associated-vasculitis
#5
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
#6
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/29249740/a-case-of-infectious-enterocolitis-with-hyperammonemia
#7
Ken Otsuji, Satoko Simizu, Takeru Endo, Ayako Kanazawa, Hideaki Arai, Keiji Nagata, Nobuya Harayama, Shunichi Nihei, Keiji Aibara, Mitsumasa Saito, Masayuki Kamochi
Case reports of hyperammonemia due to urease-producing bacteria are found occasionally, but most of them are associated with urinary tract infections. We experienced a case of infectious enterocolitis with hyperammonemia in which the causative bacteria was speculated to be urease-producing bacteria. A Japanese woman in her 70s had been diagnosed with microscopic polyangiitis in a nearby hospital and was transferred to our hospital. Although the microscopic polyangiitis was relatively under control after treatment with steroids and rituximab, frequent diarrhea with hyperammonemia (324 µg/dl) appeared and she became comatose...
2017: Journal of UOEH
https://www.readbyqxmd.com/read/29213028/-acute-cholecystitis-due-to-microscopic-polyangiitis-a-case-report
#8
Masaya Kotsuka, Tadashi Tsukamoto, Akishige Kanazawa, Sadatoshi Shimizu, Yoshitaka Nakajima, Shintaro Kodai, Shigeaki Komatsu
A 78-year-old man with chief complaints of cough and lower limb numbness was admitted to our hospital. Serum myeloperoxidase-antineutrophil cytoplasmic antibody titer (MPO-ANCA) was elevated (48.8U/ml), and a diagnosis of microscopic polyangiitis (MPA) was made. After admission, the patient developed a fever and right upper quadrant pain. Ultrasonography and computed tomography revealed an acute cholecystitis of unknown cause, and laparoscopic cholecystectomy was performed. Histopathological examination of the resected gallbladder revealed necrotizing vasculitis along with the infiltration of eosinophils, lymphocytes, and plasma cells around the small arteries in the muscular layer of the gallbladder, which are characteristics of MPA...
2017: Nihon Shokakibyo Gakkai Zasshi, the Japanese Journal of Gastro-enterology
https://www.readbyqxmd.com/read/29185962/renal-outcome-of-kidney-transplantation-in-korean-recipients-with-anca-associated-vasculitis
#9
Eun Seong Park, Sung Soo Ahn, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
OBJECTIVES: We investigated renal outcome of kidney-transplantation in Korean recipients with biopsy-proven renal involvement of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in a single centre. METHODS: We reviewed the medical records of 144 Korean patients and included 3 female patients with microscopic polyangiitis (MPA) and one male patient with eosinophilic granulomatosis with polyangiitis (EGPA) in this study. We obtained clinical and laboratory data related to kidney-transplantation, analysed renal outcome of kidney-transplantation in 4 recipients with AAV and compared it with those of previous studies...
November 27, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29185957/the-utility-of-the-acr-eular-2017-provisional-classification-criteria-for-granulomatosis-with-polyangiitis-in-korean-patients-with-antineutrophil-cytoplasmic-antibody-associated-vasculitis
#10
Juyoung Yoo, Ho Jae Kim, Sung Soo Ahn, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
OBJECTIVES: We applied the ACR/EULAR 2017 provisional classification criteria for granulomatosis with polyangiitis (GPA) to 150 Korean patients with previously diagnosed antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and investigated how many patients with AAV were reclassified as GPA. METHODS: We included patients with 30 GPA, 30 eosinophilic GPA (EGPA) and 90 microscopic polyangiitis (MPA) patients. Patients can be classified as GPA, when the sum of scores is more than 5...
November 27, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29180123/childhood-versus-adult-onset-anca-associated-vasculitides-a-nested-matched-case-control-study-from-the-french-vasculitis-study-group-registry
#11
REVIEW
Michele Iudici, Christian Pagnoux, Pierre Quartier, Matthias Büchler, Ramiro Cevallos, Pascal Cohen, Claire de Moreuil, Philippe Guilpain, Alain Le Quellec, Jacques Serratrice, Benjamin Terrier, Loïc Guillevin, Luc Mouthon, Xavier Puéchal
OBJECTIVE: To investigate differences between childhood-onset ANCA-associated vasculitides (cAAVs) and matched adult-onset controls (aAAVs). METHODS: cAAV clinical pictures at onset and outcomes were compared to a randomly selected sample of aAAV patients from the French Vasculitis Study Group Registry. Cases and controls were matched for AAV (granulomatosis with polyangiitis [GPA], microscopic polyangiitis [MPA] or eosinophilic granulomatosis with polyangiitis [EGPA]), sex and year of enrollment...
November 24, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29167552/association-of-ets1-polymorphism-with-granulomatosis-with-polyangiitis-and-proteinase-3-anti-neutrophil-cytoplasmic-antibody-positive-vasculitis-in-a-japanese-population
#12
Aya Kawasaki, Keita Yamashita, Fumio Hirano, Ken-Ei Sada, Daisuke Tsukui, Yuya Kondo, Yoshitaka Kimura, Kurumi Asako, Shigeto Kobayashi, Hidehiro Yamada, Hiroshi Furukawa, Kenji Nagasaka, Takahiko Sugihara, Kunihiro Yamagata, Takayuki Sumida, Shigeto Tohma, Hajime Kono, Shoichi Ozaki, Seiichi Matsuo, Hiroshi Hashimoto, Hirofumi Makino, Yoshihiro Arimura, Masayoshi Harigai, Naoyuki Tsuchiya
ETS proto-oncogene 1, transcription factor (ETS1) is involved in various immune responses. Genome-wide association studies on systemic lupus erythematosus in Chinese populations identified the association of ETS1 polymorphism in 3' untranslated region, rs1128334A, which was associated with lower ETS1 expression. In view of substantial sharing of susceptibility genes across multiple autoimmune diseases, we examined whether ETS1 is associated with a rare autoimmune rheumatic disease, anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)...
October 5, 2017: Journal of Human Genetics
https://www.readbyqxmd.com/read/29165307/cutaneous-sarcoidosis-during-rituximab-treatment-for-microscopic-polyangiitis-an-uncommon-adverse-effect
#13
Leonardo Pescitelli, Giacomo Emmi, Lara Tripo, Linda Lazzeri, Maria Letizia Urban, Elena Silvesri, Margherita Vannucchi, Francesca Prignano
No abstract text is available yet for this article.
November 22, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/29142188/a-case-of-microscopic-polyangiitis-with-subarachnoid-hemorrhage-and-cardiovascular-complications
#14
Sae Aratani, Yukinao Sakai, Shuichi Tsuruoka
Microscopic polyangiitis (MPA) is a primary systemic vasculitis that predominantly affects small and medium vessels. MPA is rarely complicated with central nervous system or cardiovascular disease. We report a very rare case of MPA complicated with cerebral infarction, cardiovascular disease, and fatal subarachnoid hemorrhage in a 54-year-old man. During the first six days of hospitalization the patient was diagnosed with rapid progressive glomerulonephritis (RPGN), cerebral infarction, and unstable angina...
2017: Journal of Nippon Medical School, Nippon Ika Daigaku Zasshi
https://www.readbyqxmd.com/read/29124398/patient-perceptions-of-glucocorticoids-in-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis
#15
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/29119480/rheumatoid-factor-false-positivity-in-patients-with-anca-associated-vasculitis-not-having-medical-conditions-producing-rheumatoid-factor
#16
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/29082297/distinct-pathogenesis-in-nonsystemic-vasculitic-neuropathy-and-microscopic-polyangiitis
#17
Mie Takahashi, Haruki Koike, Shohei Ikeda, Yuichi Kawagashira, Masahiro Iijima, Atsushi Hashizume, Masahisa Katsuno, Gen Sobue
OBJECTIVE: To investigate the mechanisms of vasculitis in nonsystemic vasculitic neuropathy (NSVN) and microscopic polyangiitis (MPA), focusing on complement- and antineutrophil cytoplasmic antibody (ANCA)-associated pathogenesis. METHODS: Sural nerve biopsy specimens taken from twenty-four patients with NSVN and 37 with MPA-associated neuropathy (MPAN) were examined. Twenty-two patients in the MPAN group tested positive for ANCA. RESULTS: Immunostaining for complement component C3d deposition showed more frequent positive staining of epineurial small vessels in NSVN than in MPAN (p = 0...
November 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/29061243/central-nervous-system-disease-in-antineutrophil-cytoplasmic-antibodies-associated-vasculitis
#18
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
https://www.readbyqxmd.com/read/29016646/methotrexate-versus-cyclophosphamide-for-remission-maintenance-in-anca-associated-vasculitis-a-randomised-trial
#19
Federica Maritati, Federico Alberici, Elena Oliva, Maria L Urban, Alessandra Palmisano, Francesca Santarsia, Simeone Andrulli, Laura Pavone, Alberto Pesci, Chiara Grasselli, Rosaria Santi, Bruno Tumiati, Lucio Manenti, Carlo Buzio, Augusto Vaglio
OBJECTIVES: The treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is based on remission-induction and remission-maintenance. Methotrexate is a widely used immunosuppressant but only a few studies explored its role for maintenance in AAV. This trial investigated the efficacy and safety of methotrexate as maintenance therapy for AAV. METHODS: In this single-centre, open-label, randomised trial we compared methotrexate and cyclophosphamide for maintenance in AAV...
2017: PloS One
https://www.readbyqxmd.com/read/28988280/echocardiographic-features-in-patients-with-anca-associated-vasculitis-within-3%C3%A2-months-before-and-after-diagnosis
#20
Sung Soo Ahn, Eun Seong Park, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
We investigated the all-inclusive echocardiographic features in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) within 3 months before and after diagnosis. We reviewed the medical records of 89 AAV patients taking echocardiography and 35 age- and gender-matched controls. We collected clinical and laboratory data and echocardiographic results. We compared the variables between patients with AAV of each variant and controls and among those with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA)...
December 2017: Clinical Rheumatology
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