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https://www.readbyqxmd.com/read/28328827/clinical-study-on-single-organ-cutaneous-small-vessels-vasculitis-socsvv
#1
Maciej Pastuszczak, Magdalena Celińska-Löwenhoff, Joanna Sułowicz, Anna Wojas-Pelc, Jacek Musiał
Leukocytoclastic vasculitis (LCV) is a heterogenous group of disorders that may manifest as a mild disease isolated to the skin or be a part of life-threatening systemic vasculitis. According to the 2012 Chapel Hill Consensus Conference nomenclature, patients presenting symptoms of LCV confined only to the skin should be defined as suffering from a single-organ cutaneous small vessel vasculitis (SoCSVV). SoCSVV is a benign disease with a good clinical outcome but with a significant risk of relapse and skin ulcer formation...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28295009/-refractory-form-of-orbital-granulomatosis-with-polyangiitis-a-clinical-and-morphological-study
#2
E A Kogan, Ya O Grusha, D S Ismailova, P I Novikov, Yu V Abramova, A D Meshkov, E F Rizopulu
Orbital granulomatosis with polyangiitis (Wegener's granulomatosis, GPA), which is characterized by granulomatous inflammation with small-vessel vasculitis, can develop in local and generalized forms of the disease. The introduction of current immunosuppressive therapy regimens has improved the prognosis of the disease; however, there are immunosuppressive treatment-refractory forms of GPA, the morphology of which has been inadequately investigated. The paper describes a clinical case of refractory GPA involving the orbit, as evidenced by histological and immunohistochemical examinations...
2017: Arkhiv Patologii
https://www.readbyqxmd.com/read/28289826/polyarteritis-nodosum-of-the-breast-in-a-patient-with-history-of-bilateral-augmentation-mammoplasty
#3
Andrew Griffiths, Ashit Patel, Malcolm Z Roth
Polyarteritis nodosum belongs to a group of inflammatory disorders characterized by necrotizing vasculitis of small and medium-sized blood vessels. To date, there are 14 publications that document involvement of the breast. Our publication is the second study documenting polyarteritis nodosum of the breast in a patient who had previously had augmentation mammoplasty. Level of Evidence V This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www...
March 13, 2017: Aesthetic Plastic Surgery
https://www.readbyqxmd.com/read/28271648/henoch-sch%C3%A3-nlein-purpura-with-concurrent-cytomegalovirus-duodenitis
#4
Sae Bom Shin, Yeong Jin Choi, Jieun Lee, Bong Gye Kwak, Yong Hee Kim, Kyung Sun Ha, Jin Hyoung Kang
Henoch-Schönlein purpura (HSP) is a systemic vasculitis involving the small vessels with distinct clinical features. The etiology of HSP is diverse, and viral infection is one of the many predisposing factors. Cytomegalovirus (CMV) infection mostly affects immune-suppressed patients, but rarely patients with normal immunity can also be affected. Authors experienced a case of HSP patient, with underlying small-cell lung cancer (SCLC) with CMV duodenitis. This is a rare case of HSP diagnosed in SCLC patient with predisposing factor of CMV infection...
February 7, 2017: Infection & Chemotherapy
https://www.readbyqxmd.com/read/28255916/hospital-morbidity-database-for-epidemiological-studies-on-churg-strauss-syndrome
#5
Krzysztof Kanecki, Aneta Nitsch-Osuch, Paweł Gorynski, Patryk Tarka, Piotr Tyszko
Churg-Strauss syndrome or more accurately eosinophilic granulomatosis with polyangiitis (EGPA) is a small-vessel necrotizing vasculitis with a characteristic late-onset allergic rhinitis and asthma. The use of hospital morbidity database is an important element of the epidemiological analysis of this rare disease. The present study was undertaken to assess the incidence of EGPA and factors related to its epidemiology in Poland; the first analysis of the kind in Poland, enabling a comparison in the European context...
March 3, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28255551/multiple-perforations-and-fistula-formation-following-corticosteroid-administration-a-case-report
#6
Jing-Ni He, Zhong Tian, Xu Yao, Hang-Yu Li, Yun Yu, Yuan Liu, Jin-Gang Liu
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic small- and medium-sized-vessel vasculitis. The literature contains only a few reports of gastrointestinal perforation with this condition. We report a patient with EPGA treated with high-dose steroid who underwent emergency surgery for intestinal perforations. We performed a simple repair of the 11 perforations. Intestinal fistulas developed 8 d postoperatively; they healed well after 60 d of continuous washing and negative pressure suction...
February 16, 2017: World Journal of Clinical Cases
https://www.readbyqxmd.com/read/28250302/henoch-sch%C3%A3-nlein-purpura-nephritis-associated-with-intravesical-bacillus-calmette-guerin-bcg-therapy
#7
Hiroyuki Tsukada, Hiroshi Miyakawa
Henoch Schönlein purpura (HSP), also known as IgA vasculitis (IgAV), is a systemic small-vessel vasculitis that predominantly affects adolescents and is rare in adults. In many cases, the onset of HSP has been causally linked to an infectious disease. We encountered a case of HSP with severe renal involvement diagnosed by renal biopsy following bacillus Calmette-Guerin (BCG) therapy for bladder cancer. This is of clinical relevance, as intravesical BCG administration is becoming an established therapy for superficial bladder cancer and is supposed to be safe...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28243478/acute-hemorrhagic-edema-of-infancy-after-coronavirus-infection-with-recurrent-rash
#8
Hannah Chesser, Jeffrey M Chambliss, Eric Zwemer
Purpura, particularly when accompanied by fever, is a worrisome finding in children. Acute hemorrhagic edema of infancy (AHEI) is a benign type of small-vessel leukocytoclastic vasculitis that presents with progressive purpura and has an excellent prognosis. Patients with AHEI present with large, target-like purpuric plaques affecting the face, ear lobes, and extremities. While the rapid onset of these skin findings can be dramatic, the child with AHEI is usually well appearing with reassuring laboratory testing...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28228431/right-upper-quadrant-abdominal-pain-as-the-initial-presentation-of-polyarteritis-nodosa
#9
Ricardo Gago, Lee Ming Shum, Luis M Vilá
Polyarteritis nodosa (PAN) is a necrotising vasculitis that involves medium and small vessels. PAN generally presents with constitutional, cutaneous, neurological, renal and gastrointestinal manifestations. However, PAN initially involving a single organ/system is uncommon. Here, we present a 42-year-old man who was hospitalised because of severe right upper quadrant abdominal pain that started 2 months before. Physical examination was remarkable for right upper quadrant abdominal tenderness. Abdominopelvic CT showed lymphadenopathy but no hepatic, gallbladder, pancreatic, intestinal or renal abnormalities...
February 22, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28222228/neutrophil-to-lymphocyte-ratio-a-biomarker-for-predicting-systemic-involvement-in-adult-iga-vasculitis-patients
#10
Géza Róbert Nagy, Lajos Kemény, Zsuzsanna Bata-Csörgő
BACKGROUND: IgA vasculitis (IgAV) is a small-vessel leucocytoclastic cutaneous vasculitis, often associated with kidney and gastrointestinal (GI) manifestations. Although predictive factors for systemic involvement have been extensively studied in children, there is paucity in the literature regarding adult patients. Neutrophil-to-lymphocyte ratio (NLR) is an inflammatory marker, used to assess systemic inflammation in various diseases. OBJECTIVE: We sought to evaluate whether NLR can be used for predicting renal and GI involvement in adult IgA vasculitis patients...
February 21, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28217966/pathogenesis-of-diffuse-alveolar-hemorrhage-in-murine-lupus
#11
Haoyang Zhuang, Shuhong Han, Pui Y Lee, Ravil Khaybullin, Stepan Shumyak, Li Lu, Amina Chatha, Anan Afaneh, Yuan Zhang, Chao Xie, Dina Nacionales, Lyle Moldawer, Xin Qi, Li-Jun Yang, Westley H Reeves
OBJECTIVE: Diffuse alveolar hemorrhage (DAH) in lupus patients is >50% fatal. The cause is unknown. The pathogenesis of DAH in C57BL/6 mice with pristane-induced lupus, a model of human lupus-associated DAH, was examined. METHODS: Clinical/pathological and immunological manifestations DAH in pristane-lupus were compared with human DAH. Tissue distribution of pristane was examined by mass spectrometry. Cell types responsible for disease were determined by in vivo depletion using clodronate liposomes (CloLip) and anti-neutrophil monoclonal antibodies (GR1)...
February 19, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28215926/-acute-pancreatitis-as-the-presenting-feature-of-an-iga-vasculitis-an-unusual-presentation
#12
L Fertitta, N Noel, F Ackermann, N Lerolle, S Benoist, L Rocher, O Lambotte
INTRODUCTION: IgA vasculitis is a systemic small vessel leukocytoclastic vasculitis characterized by skin purpura, arthritis, abdominal pain and nephritis. Most of the abdominal complications are due to edema and hemorrhage in the small bowel wall, but rarely to acute secondary pancreatitis. CASE REPORT: Here, we report a 53-year-old woman who presented with acute pancreatitis and, secondarily, developed skin purpura and arthritis at the seventh day of the clinical onset...
February 16, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28208969/acute-presentation-of-juvenile-dermatomyositis-with-subclinical-cardiac-involvement-a-rare-case
#13
Rhythm Khera, Shrayash Khare, Shailendra Kumar Singh
Cardiac involvements are common in patients with Dermatomyositis, most of which are not severe enough to present definite or readily observable symptoms. However, Cardiovascular (CVS) manifestations constitute a major cause of death in these patients. The most frequently reported clinically evident of CVS manifestations in-patient of dermatomyositis are Congestive Heart Failure (CHF), conduction aberrations, that may predispose to complete heart block and coronary artery disease. The principal pathophysiological mechanisms that may produce these cardiac manifestations involve coronary artery disease as well as small vessels vasculitis of the myocardium...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28208913/eosinophilic-granulomatosis-with-polyangiitis-egpa-and-hepatitis-b-infection-a-rare-association
#14
Nidhi Kaeley, Narula Kamakshi
Eosinophilic Granulomatosis with Polyangiitis (EGPA) or Churg-Strauss syndrome is a rare systemic illness which is characterized by necrotizing inflammation of small and medium sized vessels. The prominent features include asthma, eosinophilia, transient pulmonary infiltration and systemic vasculitis. Various triggering factors have been reported as putative aetiological agents of Churg-Strauss syndrome. They include infections, allergic desensitization, vaccinations like hepatitis B and influenza vaccination, cocaine abuse, drugs like leukotriene receptor antagonists and exposure to pigeons...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28174414/a-case-of-henoch-schonlein-purpura-associated-with-rotavirus-infection-in-an-elderly-asian-male-and-review-of-the-literature
#15
Chen Tang, Daphne Scaramangas-Plumley, Cynthia C Nast, Zab Mosenifar, Marc A Edelstein, Michael Weisman
BACKGROUND Henoch-Schönlein purpura (HSP), a small vessel vasculitis mediated by deposition of immune-complexes containing IgA in the skin, gut, and glomeruli, often presents with abdominal pain, purpuric rash in the lower extremities and buttocks, joint pain, and hematuria. The disease most commonly targets children but can affect adults who tend to have a worse prognosis. CASE REPORT We discuss a case of HSP in an elderly Chinese male who presented with severe proximal bowel inflammation, vasculitic rash, and proteinuria; he was found to have positive stool rotavirus and giardia...
February 8, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28164561/frequency-of-foxp3-regulatory-t-cells-in-the-blood-of-chronic-hepatitis-c-patients-with-immune-mediated-skin-manifestations-relationship-to-hepatic-condition-and-viral-load
#16
Carmen Farid, Wafaa El Sheikh, Rania Swelem, Engy El-Ghitany
BACKGROUND: Chronic hepatitis C (CHC) infection causes a wide range of immune mediated hepatic and extrahepatic manifestations. Cutaneous manifestations constitute a major portion of the latter group. Host immune response to the virus - particularly regulatory T lymphocytes - is important in determining the outcome of the infection and the possibility of extrahepatic manifestations. The present study aims to investigate the relationship between immune-mediated cutaneous manifestations of CHC infection and the frequency of CD4+CD25 high FOXP3+ T regulatory lymphocytes...
December 1, 2016: Clinical Laboratory
https://www.readbyqxmd.com/read/28163812/acute-urinary-retention-secondary-to-urethral-involvement-of-granulomatosis-with-polyangiitis
#17
Patrick T Anderson, Stephanie Gottheil, Manal Gabril, Lillian Barra, Nicholas Power
Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis) is a systemic necrotizing vasculitis of small- and medium-sized blood vessels, primarily affecting the upper and lower respiratory tracts, as well as the kidneys. Urogenital manifestations of GPA are exceedingly rare and usually respond well to systemic immunosuppressive therapy. Here, we present a case of a 36-year-old female presenting with acute urinary obstruction secondary to urethral GPA involvement in the immediate postpartum period...
January 2017: Canadian Urological Association Journal, Journal de L'Association des Urologues du Canada
https://www.readbyqxmd.com/read/28152662/posterior-reversible-encephalopathy-syndrome-as-a-complication-of-henoch-sch%C3%A3-nlein-purpura-in-a-seven-year-old-girl
#18
Daiane Dos Santos, Felipe Welter Langer, Tatiane Dos Santos, Giordano Rafael Tronco Alves, Marisa Feiten, Walter Teixeira de Paula Neto
Introduction Henoch-Schönlein purpura is a multisystem small vessel vasculitis. Neurologic manifestations are uncommon. Posterior reversible encephalopathy syndrome is a rare complication of Henoch-Schönlein purpura with typical clinical and neuroimaging findings that occurs most commonly in the setting of severe hypertension and renal injury. Case presentation A seven-year-old girl was admitted to our institution presenting with clinical and laboratory findings suggestive of Henoch-Schönlein purpura. Glucocorticoid therapy was initiated, but five days following her admission, she developed altered consciousness, seizures, arterial hypertension, and cortical blindness...
January 1, 2017: Scottish Medical Journal
https://www.readbyqxmd.com/read/28134583/pathology-of-antineutrophil-cytoplasmic-antibody-associated-pulmonary-and-renal-disease
#19
REVIEW
Maxwell L Smith
CONTEXT: -Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis commonly presents with pulmonary and renal involvement that may present diagnostic challenges. OBJECTIVES: -To highlight the updates in the classification of small vessel vasculitis, present the patterns of pulmonary and renal pathology in which ANCA-associated vasculitis is included in the differential diagnosis, analyze the screening and specific antineutrophil cytoplasmic antibody testing methods in the clinical laboratory, compare and contrast the 3 major ANCA-associated vasculitis diseases, and review the pathophysiologic mechanisms of tissue injury in this setting...
February 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28115215/damaging-heterozygous-mutations-in-nfkb1-lead-to-diverse-immunologic-phenotypes
#20
Meri Kaustio, Emma Haapaniemi, Helka Göös, Timo Hautala, Giljun Park, Jaana Syrjänen, Elisabet Einarsdottir, Biswajyoti Sahu, Sanna Kilpinen, Samuli Rounioja, Christopher L Fogarty, Virpi Glumoff, Petri Kulmala, Shintaro Katayama, Fitsum Tamene, Luca Trotta, Ekaterina Morgunova, Kaarel Krjutškov, Katariina Nurmi, Kari Eklund, Anssi Lagerstedt, Merja Helminen, Timi Martelius, Satu Mustjoki, Jussi Taipale, Janna Saarela, Juha Kere, Markku Varjosalo, Mikko Seppänen
BACKGROUND: The nuclear factor κ light-chain enhancer of activated B cells (NF-κB) signaling pathway is a key regulator of immune responses. Accordingly, mutations in several NF-κB pathway genes cause immunodeficiency. OBJECTIVE: We sought to identify the cause of disease in 3 unrelated Finnish kindreds with variable symptoms of immunodeficiency and autoinflammation. METHODS: We applied genetic linkage analysis and next-generation sequencing and functional analyses of NFKB1 and its mutated alleles...
January 21, 2017: Journal of Allergy and Clinical Immunology
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