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"Small vessel vasculitis"

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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/28250302/henoch-sch%C3%A3-nlein-purpura-nephritis-associated-with-intravesical-bacillus-calmette-guerin-bcg-therapy
#3
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/28217966/pathogenesis-of-diffuse-alveolar-hemorrhage-in-murine-lupus
#4
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/28208969/acute-presentation-of-juvenile-dermatomyositis-with-subclinical-cardiac-involvement-a-rare-case
#5
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/28174414/a-case-of-henoch-schonlein-purpura-associated-with-rotavirus-infection-in-an-elderly-asian-male-and-review-of-the-literature
#6
REVIEW
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
#7
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/28152662/posterior-reversible-encephalopathy-syndrome-as-a-complication-of-henoch-sch%C3%A3-nlein-purpura-in-a-seven-year-old-girl
#8
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
#9
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
#10
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
https://www.readbyqxmd.com/read/28102817/validating-identification-of-patients-with-small-vessel-vasculitis-with-or-without-renal-involvement-using-administrative-healthcare-records
#11
Michelle M O'Shaughnessy, XingXing S Cheng, Maria E Montez-Rath, Richard A Lafayette, Wolfgang C Winkelmayer
No abstract text is available yet for this article.
January 19, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28094164/disorders-characterized-by-predominant-or-exclusive-dermal-inflammation
#12
Mark R Wick
Some cutaneous inflammatory disorders are typified by a predominant or exclusive localization in the dermis. They can be further subdivided by the principal cell types into lymphocytic, neutrophilic, and eosinophilic infiltrates, and mixtures of them are also seen in a proportion of cases. This review considers such conditions. Included among the lymphoid lesions are viral exanthems, pigmented purpuras, gyrate erythemas, polymorphous light eruption, lupus tumidus, and cutaneous lymphoid hyperplasia. Neutrophilic infiltrates are represented by infections, Sweet syndrome, pyoderma gangrenosum, and hidradenitis suppurativa, as well as a group of so-called "autoinflammatory" dermatitides comprising polymorphonuclear leukocytes...
December 14, 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28079316/clinical-characteristics-and-treatment-response-of-peripheral-neuropathy-in-the-presence-of-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome-experience-at-a-single-tertiary-center
#13
Hye Jin Cho, Sehyo Yune, Jin Myoung Seok, Eun Bin Cho, Ju Hong Min, Yeon Lim Seo, Byung Jae Lee, Byoung Joon Kim, Dong Chull Choi
BACKGROUND AND PURPOSE: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic small-vessel vasculitis accompanied by asthma, eosinophilia, and eosinophilic inflammation of various tissues including the peripheral nerves. This study investigated the clinical course and long-term outcomes of peripheral neuropathy in patients with EGPA. METHODS: Seventy-one patients with physician-diagnosed EGPA were identified at Samsung Medical Center between January 1995 and April 2014...
January 2017: Journal of Clinical Neurology
https://www.readbyqxmd.com/read/27940585/adult-primary-angiitis-of-the-central-nervous-system-isolated-small-vessel-vasculitis-represents-distinct-disease-pattern
#14
Hubert de Boysson, Grégoire Boulouis, Achille Aouba, Boris Bienvenu, Loïc Guillevin, Mathieu Zuber, Emmanuel Touzé, Olivier Naggara, Christian Pagnoux
OBJECTIVES: We aimed to identify whether presentations and outcomes in adult patients with isolated small-vessel primary angiitis of the CNS (PACNS) would differ from other patients with large/medium-vessel involvement. METHODS: In the French PACNS cohort, we compared the characteristics, treatments and outcomes of patients with isolated small-vessel disease (normal CT, MR and/or conventional angiograms, brain biopsy positive for vasculitis) with other patients who had large/medium-vessel involvement (vessel abnormalities on CT, MR or conventional angiograms)...
December 10, 2016: Rheumatology
https://www.readbyqxmd.com/read/27938815/acrally-distributed-dermatoses-vascular-dermatoses-purpura-and-vasculitis
#15
Jana Kazandjieva, Dimitar Antonov, Jivko Kamarashev, Nikolai Tsankov
Purpuric lesions appear in acral distribution in a variety of conditions and often provide clues to the clinical diagnosis. Purpuric means "hemorrhagic"-that is, the lesions do not blanch from pressure. This review focuses on dermatoses that produce hemorrhagic lesions in acral distribution from the large groups of the vasculitic diseases and their mimics. Cutaneous small vessel vasculitis is confined to the skin, involves mainly postcapillary venules, and has the hallmark manifestation of palpable purpura...
January 2017: Clinics in Dermatology
https://www.readbyqxmd.com/read/27919708/early-interstitial-lung-disease-in-microscopic-polyangiitis-case-report-and-literature-review
#16
Marcos García-Nava, Heidegger Mateos-Toledo, Ana Patricia Georgina Guevara-Canseco, Cesar Eduardo Infante-González, Diego Alberto Reyes-Nava, Emilio Estrada-Castro
Microscopic polyangiitis (MPA) is a systemic disease included in the Chapel Hill 2012 Classification as necrotizing vasculitis affecting capillaries, venules and arterioles. It usually expresses antineutrophil cytoplasmic antibodies (ANCA) and has a perinuclear immunofluorescence pattern and correlation with anti-myeloperoxidase (MPO) antibodies. Capillaritis with alveolar hemorrhage is the most common manifestation of lung disease. Interstitial lung disease (ILD) is uncommon, with usual interstitial pneumonia being the predominant pattern...
December 2, 2016: Reumatología Clinica
https://www.readbyqxmd.com/read/27863214/type-ii-natural-killer-t-cells-that-recognize-sterol-carrier-protein-2-are-implicated-in-vascular-inflammation-in-the-rat-model-of-systemic-connective-tissue-diseases
#17
Yusuke Nishioka, Madoka Yamaguchi, Ai Kawakami, Maya Munehiro, Sakiko Masuda, Utano Tomaru, Akihiro Ishizu
We previously generated a rat model that developed systemic connective tissue diseases, including synovitis, myositis, and small-vessel vasculitis (SVV), and established a vascular endothelial cell-reactive T-cell clone, VASC-1, from the model. VASC-1 was determined to be a type II natural killer T-cell clone. In this study, we attempted to identify the antigen recognized by VASC-1. The monkey-derived cell line COS-7 was used because VASC-1 does not bind naturally to COS-7, although the amino acid sequences are well conserved between monkey CD1d and rat CD1d...
January 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/27829255/-pulmonary-manifestations-of-vasculitis
#18
S von Vietinghoff
The variable symptoms and signs of pulmonary vasculitis are a diagnostic and therapeutic challenge. Vasculitis should be considered in rapidly progressing, severe and unusual manifestations of pulmonary disease. Clinical examination of other organ systems typically affected by vasculitis such as skin and kidney and autoantibody measurements are complementary approaches to manage this situation. Pulmonary involvement is common in small vessel vasculitis including anti-GBM disease (Goodpasture syndrome) and the ANCA-associated vasculitides...
November 2016: Pneumologie
https://www.readbyqxmd.com/read/27826099/post-translational-modified-proteins-are-biomarkers-of-autoimmune-processes-netosis-and-the-inflammatory-autoimmunity-connection
#19
REVIEW
Maurizio Bruschi, Andrea Petretto, Roberta Bertelli, Maricla Galetti, Alice Bonanni, Federico Pratesi, Paola Migliorini, Giovanni Candiano, Augusto Vaglio, Gian Marco Ghiggeri
Basic research is showing new mechanisms involved in early immune responses and Neutrophil Extracellular Trap (NET) formation (or NETosis) is of key importance as first line defense against bacteria, virus and protozoa. Enzymatic modification of arginine in citrulline in histones is the prerequisite of NETosis being it necessary for decondensation and extrusion of DNA from cells; it is conceivable that other post translational modifications may occur during this event. There is consensus in considering that post translational modified proteins may elicit an autoimmune response that leads to the formation of autoantibodies...
January 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/27825424/radiological-and-clinical-features-of-eosinophilic-granulomatosis-with-polyangiitis
#20
Xiao-Li Xu, Wei Song, Xin Sui, Lan Song, Qian-Ni DU, Xiao Wang
Eosinophilic granulomatosis with polyangiitis(EGPA),also known as Churg-Strauss syndrome,is a clinically rare small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) and the hypereosinophilic syndromes (HESs),characterized by asthma,disseminated necrotizing vasculitis,extravascular granulomas,peripheral eosinophilia,and tissue eosinophilia. This article reviews the pathology,imaging,and clinical features of EGPA.
October 10, 2016: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
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