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https://www.readbyqxmd.com/read/29896335/acute-necrotizing-vasculitic-neuropathy-due-to-polyarteritis-nodosa
#1
Joe James, James Jose, Nallaveettil Kesavan Thulaseedharan
Necrotizing vasculitic neuropathy in polyarteritis nodosa can rarely present acutely and may mimic acute inflammatory neuropathies. A 53-year-old male presented with an acute neurological illness characterized by paresthesia and weakness of both lower limbs lasting six-days. He also had mild paresthesia of both hands. On examination, there were confluent, purpuric, and ecchymotic patches over the extensor aspects of both lower limbs, which were palpable. Neurological examination revealed grade II/V power with hypotonia and absent reflexes in the lower limbs...
May 2018: Oman Medical Journal
https://www.readbyqxmd.com/read/29895322/moyamoya-disease-in-a-moroccan-baby-a-case-report
#2
Abdelhafid Houba, Nisrine Laaribi, Mohammed Meziane, Abdelhamid Jaafari, Khalil Abouelalaa, Mustapha Bensghir
BACKGROUND: A stroke in a baby is uncommon, recent studies suggested that their incidence is rising. Moyamoya disease is one of the leading causes of stroke in babies. This condition is mostly described in Japan. In Morocco, moyamoya disease has rarely been reported and a few cases were published. We report a rare Moroccan case of a 23-month-old baby boy who presented with left-sided hemiparesis and was diagnosed as having moyamoya disease. CASE PRESENTATION: A 23-month-old full-term Moroccan baby boy born to a non-consanguineous couple was referred to our hospital with the complaint of sudden onset left-sided hemiparesis...
June 13, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29875539/a-case-of-whipple-s-disease-a-very-rare-cause-for-rapidly-progressive-dementia
#3
Sadanandavalli Retnaswami Chandra, Pawan Raj, Anupama Ramakanth Pai, Neeraja Reddy
Introduction: Whipple's disease (WD) is a very rare systemic disease caused by the gram-positive bacillus Tropherymawhippleii 1st described in the year 1907. It is a disease with multisystem involvement and high degree of suspicion is needed for diagnosis. However the classical (OMM)oculomasticatory (OFMM)oculofacial-skeletal myorhythmia clubbed with dementia, head ache and other neurologic features should deserve an attempt to confirm whenever possible and therapeutic trial as it is one of the treatable dementias...
May 2018: Indian Journal of Psychological Medicine
https://www.readbyqxmd.com/read/29808153/cardiogenic-shock-an-unusual-initial-presentation-of-churg-strauss-syndrome
#4
M Apirami, J A Pratheepan, T Kumanan, M Guruparan, G Selvaratnam
Churg-Strauss syndrome (CSS) is a rare autoimmune condition, characterized by necrotizing extravascular eosinophil rich granulomatous inflammation of the tissues and disseminated small-medium sized vessel vasculitis in a patient with bronchial asthma and tissue eosinophilia. Though pulmonary involvement is the predominant feature of CSS, extra pulmonary involvement, in particular, cardiac involvement, denotes an adverse outcome. Here we report a 50-year-old female who presented with cardiogenic shock due to an acute coronary event as the initial manifestation of CSS...
2018: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29776888/iga-vasculitis-as-a-presentation-of-human-immunodeficiency-virus-infection
#5
Anahy M Brandy-García, Jorge Santos-Juanes, Silvia Suarez, Luis Caminal-Montero
IgA vasculitis is a small-vessel vasculitis mediated by immune complexes. In clinical terms, it is characterized by palpable purpura in the lower limbs, joint involvement in the form of arthralgia or arthritis, and gastrointestinal and renal involvement (this will mark a poorer prognosis in adults). Infectious processes, mainly in the upper respiratory tract, are frequently found to be triggers. On the other hand, human immunodeficiency virus (HIV) causes immune dysfunction, which triggers hypergammaglobulinemia and can trigger autoimmune disorders...
May 15, 2018: Reumatología Clinica
https://www.readbyqxmd.com/read/29731283/atypical-presentation-of-incomplete-kawasaki-disease-a-peripheral-facial-nerve-palsy
#6
Moises Rodriguez-Gonzalez, Ana Castellano-Martinez, Alvaro A Perez-Reviriego
BACKGROUND: Kawasaki disease (KD) is a multisystem vasculitic disease. Coronary artery aneurysms (CAAs) are the most important and life-threatening complication of KD. Various neurologic complications have been described to occur in 1-30% of patients with KD, but peripheral facial nerve palsy (FNP) is rare (0.9%). CASE REPORT: We describe a 5-month-old male infant who presented to us with unilateral left infranuclear FNP in the convalescent phase (day 18 of illness) of incomplete KD...
May 3, 2018: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29713969/rituximab-for-refractory-rapidly-progressive-interstitial-lung-disease-related-to-anti-mda5-antibody-positive-amyopathic-dermatomyositis
#7
Ho So, Victor Tak Lung Wong, Virginia Weng Nga Lao, Hin Ting Pang, Ronald Man Lung Yip
To report our experience in using rituximab (RTX) for treating refractory rapidly progressive interstitial lung disease (RP-ILD) complicating anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab)-positive amyopathic dermatomyositis (ADM). Medical records of four ADM patients with refractory RP-ILD treated with RTX therapy were reviewed retrospectively. All four patients were tested positive for anti-MDA5 Ab and failed to respond to high-dose systemic steroid and other intensive immunosuppressive therapies...
July 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29672364/role-of-direct-immunofluorescence-in-cutaneous-small-vessel-vasculitis-experience-from-a-tertiary-center
#8
Khushboo Lath, Debajyoti Chatterjee, Uma Nahar Saikia, Biman Saikia, Ranjana Minz, Dipankar De, Sanjeev Handa, Bishan Radotra
Skin is commonly affected by vasculitic process and often subjected to biopsy. Cutaneous vasculitis can be either primary or part of a systemic vasculitic process. This study was conducted to evaluate the diagnostic utility of direct immunofluorescence (DIF) in determination of etiology of cutaneous vasculitis. All histologically proven cases of cutaneous vasculitis over the past two and half years were retrospectively analyzed along with their clinical and DIF findings (IgG, IgA, IgM, and C3). Within this study period, a total of 198 cases of small-vessel vasculitis were diagnosed based on skin biopsy and DIF findings...
April 17, 2018: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/29570133/small-vessel-vasculitis-in-herpes-zoster-discussion-of-current-aspects-of-varicella-zoster-virus-vasculopathy
#9
Barbara Burgard, Sigrun Smola, Thomas Vogt, Cornelia S L Müller
Varicella zoster virus (VZV) vasculopathy was initially described as herpes zoster ophthalmicus with contralateral hemiplegia in 1896. VZV is able to infect endothelial cells directly, leading to a thickened intima and vascular remodeling due to inflammation. Therefore, a spectrum of vasculopathies is induced, ranging from a discrete capillaritis-to-granulomatous vasculitis and obliterative angiitis. Cutaneous vasculitic changes sui generis with leukocytoclasia, cell debris, vascular damage, and endothelial swelling are very rare feature of herpetic infections in skin biopsies...
March 21, 2018: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/29565944/neuropathic-pain-in-acute-and-subacute-neuropathies-a-systematic-review
#10
Artemios Kin Artemiadis, Panagiotis Zis
BACKGROUND: Neuropathic pain (NP) is a common symptom caused by lesions or diseases of the somatosensory nervous system. Acute/subacute peripheral neuropathies (APN) are rare, however can be particularly painful. OBJECTIVES: The aim of this systematic review was to estimate the incidence of NP in APN and overview the various etiologies of such neuropathies. STUDY DESIGN: Systematic review. SETTING: Medline search. METHODS: We hand-searched Medline for observational studies published between 1995 and 2017...
March 2018: Pain Physician
https://www.readbyqxmd.com/read/29564582/novel-mutation-in-cecr1-leads-to-deficiency-of-ada2-with-associated-neutropenia
#11
Funda Erol Cipe, Cigdem Aydogmus, Nina K Serwas, Gonca Keskindemirci, Kaan Boztuğ
PURPOSE: Adenosine deaminase 2 (ADA2) have been reported to cause vasculitic diseases and immunodeficiency recently. Patients present with stroke episodes and rashes mimicking polyarteritis nodosa (PAN). We report a patient who has been followed up with severe neutropenia and found an unexpectedly revealed novel mutation in CECR1 affecting ADA2. METHODS: We reviewed medical records and clinical history of the patient. No mutations in other known neutropenia genes such as ELA, G6PC3, HAX1, AP3B1, LAMTOR2, VPS13B, VPS45, GFI1, JAGN1, or WAS could be detected...
April 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29536184/coccidioidal-meningitis-a-review-on-diagnosis-treatment-and-management-of-complications
#12
REVIEW
Royce Johnson, Jackie Ho, Pete Fowler, Arash Heidari
PURPOSE OF REVIEW: This article summarizes the diagnosis and treatment of coccidioidal meningitis (CM) and its complications. An overview of current and prospective pharmacologic treatment options and monitoring parameters is provided. A consensus has not been reached regarding universally accepted therapeutic serum levels for azoles because of insufficient evidence. We describe the preferred therapeutic drug level ranges that our institution uses to monitor azole therapy. RECENT FINDINGS: Ho et al...
March 13, 2018: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/29530330/reducing-glucocorticoid-duration-in-anca-associated-vasculitis-a-pilot-trial
#13
Eli M Miloslavsky, John L Niles, Zachary S Wallace, Frank B Cortazar, Ana Fernandes, Karen Laliberte, John H Stone
OBJECTIVE: Therapeutic advances in ANCA-associated vasculitis (AAV) have improved patient survival, but mortality rates remain higher than the general population. Glucocorticoids contribute to AAV morbidity and mortality. We examined whether an 8-week glucocorticoid course in combination with rituximab (RTX) would induce disease remission in patients with AAV. METHODS: Patients with active AAV received an 8-week prednisone taper and RTX 375mg/m2 weekly for 4 weeks...
February 3, 2018: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29486145/testing-and-reporting-antineutrophil-cytoplasmic-antibodies-anca-in-treated-vasculitis-and-non-vasculitic-disease
#14
REVIEW
Judy Savige, Michelle Trevisin, Wendy Pollock
Testing for antineutrophil cytoplasmic antibodies (ANCA) is performed to diagnose or exclude small vessel vasculitis, and, in treated patients, to monitor disease activity. However testing is also undertaken to assist with the diagnosis of other autoimmune diseases and some infections. Most laboratories use the same assays for all sera regardless of the testing indications. The International Consensus Statement on ANCA Testing and Reporting recommended screening for ANCA by indirect immunofluorescence (IIF) and confirming IIF-positive sera in antigen-specific ELISAs for both proteinase 3 (PR3) and myeloperoxidase (MPO)...
February 24, 2018: Journal of Immunological Methods
https://www.readbyqxmd.com/read/29468099/effect-of-gamma-knife-radiosurgery-and-programmed-cell-death-1-receptor-antagonists-on-metastatic-melanoma
#15
Nathan Nordmann, Molly Hubbard, Tyler Nordmann, Paul W Sperduto, H Brent Clark, Matthew A Hunt
Learning objectives To evaluate radiation-induced changes in patients with brain metastasis secondary to malignant melanoma who received treatment with Gamma Knife radiosurgery (GKRS) and programmed cell death 1 (PD-1) receptor antagonists. Introduction  Stereotactic radiosurgery and chemotherapeutics are used together for treatment of metastatic melanoma and have been linked to delayed radiation-induced vasculitic leukoencephalopathy (DRIVL). There have been reports of more intense interactions with new immunotherapeutics targeting PD-1 receptors, but their interactions have not been well described and may result in an accelerated response to GKRS...
December 13, 2017: Curēus
https://www.readbyqxmd.com/read/29465615/peripheral-vasculitic-neuropathy-associated-with-minocycline-use
#16
Min K Kang, Rajesh K Gupta, Jayashri Srinivasan
We describe 2 patients presenting with multiplex mononeuritis, associated with skin manifestation, secondary to minocycline-induced vasculitis. One of the cases is associated neither with lupus nor polyarteritis nodosa. An extensive laboratory workup ruled out any possible underlying immunologic disorder. Electrodiagnostic studies were conducted to show axonal neuropathy in patchy and multifocal distribution consistent with multiplex mononeuritis. This diagnosis was confirmed with nerve biopsy. Withdrawing from the offending medication, minocycline, improved the patients' clinical condition and the quantitative serological measures...
March 2018: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/29465365/polyarteritis-nodosa-revisited-a-review-of-historical-approaches-subphenotypes-and-a-research-agenda
#17
REVIEW
Omer Karadag, David J Jayne
Polyarteritis nodosa (PAN) is a rare form of primary systemic vasculitis with heterogeneous presentations, treatments and disease course. Historical approaches to classification and diagnostic terminology are reviewed. Since differentiation of PAN from microscopic polyangiitis (MPA) and other ANCA vasculitides by the Chapel Hill conference statements, and with hepatitis associated PAN defined as a secondary vasculitis, the phenotyping and subclassification of PAN has received little attention. Monogenic disorders similar to PAN have been described (familial Mediterranean fever, Adenosine Deaminase-2 deficiency), and cutaneous PAN and single organ vasculitis, discussed...
February 20, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29465347/an-increased-major-vessel-uptake-by-18f-fdg-pet-ct-in-nih-criteria-clinical-inactive-patients-with-takayasu-s-arteritis
#18
Qing Han, Qiang Liang, Fei Kang, Jing Wang, Zhenbiao Wu, Ping Zhu
OBJECTIVES: The commonly adopted method of defining active disease in Takayasu's arteritis (TAK) is the definition used by the US National Institutes of Health (NIH). A gold standard in imaging techniques for assessing disease activity in TAK has not been clearly established and the creation of practical and valid tools represents a challenge. To assess whether 18F-FDG-PET/CT and NIH criteria show a good level of agreement in assessing disease activity of TAK patients. METHODS: 18F-FDG-PET/CT was performed in 17 patients with TAK...
February 7, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29446067/headache-linked-to-intracranial-hypertension-and-hypertrophic-pachymeningitis-as-the-initial-and-dominant-presentation-of-granulomatosis-with-polyangiitis-case-report-and-review-of-the-recent-literature
#19
Marianthi A Arnaoutoglou, Chrysostomos G Xerras, Ioannis K Kalevrosoglou, Vasileios D Rafailidis, Konstantinos P Notas, Thomas I Tegos
OBJECTIVE: The objective of this article is to report a rare case of headache as the initial symptom of granulomatosis with polyangiitis (GPA) and to review the recent literature. BACKGROUND: Granulomatosis with polyangiitis is a rare, systemic, autoimmune disease of unknown etiology. GPA has a wide spectrum of clinical symptomatology, including involvement of the nervous system, even as the initial manifestation. Symptoms of the peripheral nervous system used to dominate the clinical symptomatology...
April 2018: Headache
https://www.readbyqxmd.com/read/29420247/macrophages-and-cytotoxic-t-cells-infiltrate-the-destructed-mitral-tissue-in-kawasaki-disease
#20
Yuichiro Sugitani, Kenji Furuno, Katsuo Sueishi, Toshiro Hara
Kawasaki disease (KD) is an acute febrile systemic vasculitic syndrome especially affecting medium-sized arteries, including the coronary artery. Inflammation may involve all organs, and valvulitis is one of the cardiovascular complications that occurs in the acute phase of KD. However, details regarding the mechanism are unclear. An infant developed KD and severe mitral regurgitation with deformity and prolapse of the mitral tissue and underwent mitral valvotomy 1 year later. Histopathological study was conducted, and infiltrating cells consisted of mainly macrophages and cytotoxic T cells were found in resected mitral valve tissue...
February 2, 2018: BMJ Case Reports
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