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Mpo and lupus

Bharat Kumar, Jennifer Strouse, Melissa Swee, Petar Lenert, Manish Suneja
INTRODUCTION: Hydralazine is an antihypertensive medication that has been associated with drug-induced lupus erythematosus (DIL) as well as ANCA-associated vasculitis (AAV). Although rare, early diagnosis is critical since drug cessation is the mainstay of therapy. This retrospective study aims to characterize the clinical, laboratory, and histopathologic features of this disease. METHODS: Once approval was obtained from the Institutional Review Board at the University of Iowa, all patients carrying a diagnosis of vasculitis (ICD9 code: 447...
January 12, 2018: Seminars in Arthritis and Rheumatism
K E Cervantes-Luevano, N Caronni, M C Castiello, E Fontana, G Piperno, A Naseem, P Uva, M Bosticardo, G E Marcovecchio, L D Notarangelo, M P Cicalese, A Aiuti, A Villa, F Benvenuti
BACKGROUND: Wiskott-Aldrich syndrome (WAS) is a rare primary immunodeficiency caused by mutations in WASp, a key regulator of cytoskeletal dynamics in hematopoietic cells. A high proportion of patients develop autoimmunity due to a breakdown in T and B cell tolerance. Moreover, excessive production of type-I interferon by plasmacytoid DCs contribute to autoimmune signs, however, the factors that triggers excessive innate activation have not been defined. OBJECTIVE: Neutrophils extracellular traps (NETs) emerged as major initiating factors in diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA)...
February 12, 2018: Journal of Allergy and Clinical Immunology
S G L da Silva, M T Terreri, T T O Abad, D Machado, F L A Fonseca, S Hix, F I Suano-Souza, R O S Sarni, C A Len
Objective This study sought to evaluate the effects of a nutritional intervention on the lipid metabolism biomarkers associated with cardiovascular risk, and their variation over time, in juvenile systemic lupus erythematosus (JSLE) patients. This study also investigated the relationships between these biomarkers and dietary intake, nutritional status, disease variables, and medication used. Methods A total of 31 10- to 19-year-old female adolescents with JSLE for at least six months were analyzed. The participants were randomly allocated to two groups: nutritional intervention or control...
January 1, 2018: Lupus
Chiara Tani, Sabrina Vagnani, Linda Carli, Francesca Querci, Anja A Kühl, Simone Spieckermann, Constanze Pamela Cieluch, Simone Pacini, Rita Fazzi, Marta Mosca
Objective: Pre-clinical and uncontrolled studies in patients with systemic lupus erythematosus (SLE) showed that mesenchymal stromal cells (MSCs) have a potential therapeutic role in refractory cases. The optimal therapeutic strategy in these patients remain to be elucidated. Our aim was to test the hypothesis that repeated administrations of 1×106 /kg body weight of allogenic MSCs, that is a significantly lower dosage with respect to the fixed 1×106 MSC used in animal models, can be effective in improving the clinical course of a murine SLE model...
November 30, 2017: International Journal of Stem Cells
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)...
January 2018: Journal of Human Genetics
Cui Li, Min-Lin Zhou, Dan-Dan Liang, Jing-Jing Wang, Jing Yang, Cai-Hong Zeng, Zhi-Hong Liu, Hai-Tao Zhang
OBJECTIVE: To assess the clinical features, pathological presentations, treatments and outcomes of lupus nephritis (LN) with anti-neutrophil cytoplasmic antibody (ANCA) positivity. DESIGN: A case-control study. METHODS: Patients (n=49) were retrospectively included from Jinling Hospital in China if presenting with biopsy-proven ANCA-positive LN between 1985 and 2008. Clinicopathological characteristics and outcomes were analysed and compared with those of a control group (n=1279)...
July 28, 2017: BMJ Open
F Pepe, S Disma, C Teodoro, P Pepe, G Magro
Kikuchi-Fujmoto disease (KFD), also known as "histiocytic necrotizing lymphadenitis", is a rare lymphadenitis of unknown origin, but with an excellent prognosis. It is more common in Asia, but isolated cases are also reported in America, Africa and Europe. The disease can have an acute or subacute course, usually develops in 1 to 3 weeks, with spontaneous resolution in 1-4 months. The main clinical sign is cervical lymphadenopathy, especially in the posterior cervical triangle with bulky and painful lymph nodes, usually affecting only one side; rare cases of generalized lymphadenopathy can be seen...
September 2016: Pathologica
Rujuan Dai, Catharine Cowan, Bettina Heid, Deena Khan, Zhihong Liang, Christine T N Pham, S Ansar Ahmed
Estrogen, a natural immunomodulator, regulates the development and function of diverse immune cell types. There is now renewed attention on neutrophils and neutrophil serine proteases (NSPs) such as neutrophil elastase (NE), proteinase 3 (PR3), and cathepsin G (CG) in inflammation and autoimmunity. In this study, we found that although estrogen treatment significantly reduced total splenocytes number, it markedly increased the splenic neutrophil absolute numbers in estrogen-treated C57BL/6 (B6) mice when compared to placebo controls...
2017: PloS One
Mandy Sowa, Barbara Trezzi, Rico Hiemann, Peter Schierack, Kai Grossmann, Juliane Scholz, Valentina Somma, Renato Alberto Sinico, Dirk Roggenbuck, Antonella Radice
Rapidly progressive glomerulonephritis (RPGN) is mainly caused by anti-glomerular basement membrane (GBM) antibody-mediated glomerulonephritis, immune-complex or anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides and leads to rapid loss of renal function. Detection of ANCA and autoantibodies (autoAbs) to GBM and dsDNA enables early diagnosis and appropriate treatment of RPGN aiding in preventing end-stage renal disease.Determination of ANCA on neutrophils (ANCA) as well as autoAbs to myeloperoxidase (MPO-ANCA), proteinase 3 (PR3-ANCA), GBM, and dsDNA was performed by the novel multiplex CytoBead technology combining cell- and microbead-based autoAb analyses by automated indirect immunofluorescence (IIF)...
November 2016: Medicine (Baltimore)
Zahra Heidari, Hamidreza Mahmoudzadeh Sagheb, Nadia Sheibak
OBJECTIVE: Reports of increased level of Myeloperoxidase (MPO) in plasma and placental extracts of Systematic Lupus Erythematosus (SLE) has been proposed that MPO may have an important role in this pregnancy complication. In present study immunohistochemical MPO expression was investigated in placental samples of SLE women compared with normal controls. MATERIALS AND METHODS: Ten patients with SLE were recruited as case group. Control group was selected from mothers with normal uncomplicated pregnancies...
June 2016: Journal of Family & Reproductive Health
Namrata Singh, Duncan B Johnstone, Kayla A Martin, Italo Tempera, Mariana J Kaplan, Michael F Denny
Systemic lupus erythematosus (SLE) is an autoimmune disorder characterized by the development of autoantibodies that recognize components of the cell nucleus. The vast majority of lupus research has focused on either the contributions of immune cell dysfunction or the genetics of the disease. Because granulocytes isolated from human SLE patients had alterations in neutrophil nuclear morphology that resembled the Pelger-Huet anomaly, and had prominent mis-splicing of mRNA encoding the nuclear membrane protein lamin B receptor (LBR), consistent with their Pelger-Huet-like nuclear morphology, we used a novel mouse model system to test the hypothesis that a disruption in the structure of the nucleus itself also contributes to the development of lupus autoimmunity...
August 1, 2016: Disease Models & Mechanisms
Maria Luisa Fiorella, Matteo Gelardi, Andrea Marzullo, Elena Sabattini, Raffaele Fiorella
Kikuchi-Fujimoto's disease (KFD) is a rare, benign condition characterized by histiocytic necrotizing lymphadenitis predominantly in the cervical region and prolonged fever. It has a higher prevalence in the Japanese and Asian populations although it may be seen in a wide geographic distribution, but rare are the cases reported in Europe. While symptoms resolve spontaneously between 2 weeks and 2 months, complications are described in: CNS, eyes, respiratory tract, kidney and liver. KFD also described an association with systemic lupus erythematosus...
March 2017: European Archives of Oto-rhino-laryngology
Pierre-Andre Jarrot, Laurent Chiche, Baptiste Hervier, Laurent Daniel, Vincent Vuiblet, Nathalie Bardin, Daniel Bertin, Benjamin Terrier, Zahir Amoura, Emmanuel Andrés, Eric Rondeau, Mohamed Hamidou, Jean-Loup Pennaforte, Philippe Halfon, Eric Daugas, Bertrand Dussol, Xavier Puéchal, Gilles Kaplanski, Noemie Jourde-Chiche
The aim of the study was to report the clinical, biological, and pathological characteristics of patients with glomerulonephritis (GN) secondary to systemic lupus erythematosus (SLE)/antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) overlap syndrome.A nationwide survey was conducted to identify cases of SLE/AAV overlap syndrome. Data were collected from SLE and AAV French research groups. Inclusion criteria were diagnosis of both SLE and AAV according to international classification criteria and biopsy-proven GN between 1995 and 2014...
May 2016: Medicine (Baltimore)
Shuzo Sato, Makiko Yashiro, Naoki Matsuoka, Manabu Uematsu, Tomoyuki Asano, Hiroko Kobayashi, Hiroshi Watanabe, Hiromasa Ohira
Mixed connective tissue disease (MCTD) is characterized by a combination of clinical features of systemic lupus erythematosus, systemic sclerosis, and polymyositis with elevated antibodies to U1 small nuclear ribonucleoprotein (U1-RNP). MCTD is often accompanied by interstitial lung disease as pulmonary involvement. On the other hand, microscopic polyangiitis (MPA) is a systemic autoimmune disease characterized by the inflammation of small vessels (arterioles, capillaries, and venules) mainly affecting the lung and kidney...
June 2016: Tohoku Journal of Experimental Medicine
Sierra C Simmons, Maxwell L Smith, April Chang-Miller, Mira T Keddis
Lupus nephritis (LN) is a serious and common complication of systemic lupus erythematosus (SLE) that predisposes to significant morbidity and mortality. Studies show that prompt diagnosis and treatment improves patient survival. We present a case of a 49-year-old female with an atypical presentation of LN who initially presented with new-onset hypertension, edema, arthritis, serositis and recently diagnosed leukocytoclastic vasculitis who later developed acute kidney injury, hematuria and nephrotic syndrome...
2015: American Journal of Nephrology
Shira Ginsberg, Itzhak Rosner, Gleb Slobodin, Nina Boulman, Michael Rozenbaum, Lisa Kaly, Ofrat Katz Beyar, Doron Rimar
Anti-tumor necrosis factor (TNF) agents have become central players in the management of autoimmune and rheumatic disease. With the wide use of anti-TNF agents today, we have become aware of rare autoimmune complications as systemic lupus erythematosus and psoriasis, yet rarely has large vessels vasculitis been described. We herein describe a case of cytoplasmic anti-neutrophil cytoplasmic antibody (c-ANCA) (with myeloperoxidase (MPO) antibodies)-associated large vessel vasculitis (aortitis) that developed during anti-TNF treatment for ankylosing spondylitis...
January 2016: Clinical Rheumatology
Yoshikazu Abe, Tomoaki Shima, Yasumori Izumi, Mineaki Kitamura, Hiroshi Yamashita, Yoshika Tsuji, Osamu Sasaki, Chie Maeda, Chieko Kawahara, Ai Torisu, Yuuki Kawaguchi, Hirokazu Kurohama, Masahiro Ito, Nozomi Iwanaga, Atsushi Kawakami, Toshiyuki Nakayama, Takashi Taguchi, Kiyoshi Migita
A 63-year-old Japanese woman with a 30-year history of systemic lupus erythematosus developed macrohematuria and massive proteinuria after seroconversion of myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA). A renal biopsy indicated focal proliferative lupus nephritis (class III A/C) with a fibrous crescent formation. Methylprednisolone pulse therapy (500 mg, 3 successive days) was administered because of progressive proteinuria. Steroid therapy did not suppress the progressive proteinuria; therefore, tacrolimus was added as an alternative immunosuppressive therapy, resulting in the improvement of proteinuria and renal impairment...
2015: Internal Medicine
Sigrid M C Broekaert, Almut Böer-Auer, Katrin Kerl, Ilka Herrgott, Xenia Schulz, Gisela Bonsmann, Randolf Brehler, Dieter Metze
BACKGROUND: Neutrophilic urticarial dermatosis (NUD) comprises a particular autoinflammatory condition within the spectrum of aseptic neutrophilic dermatoses characterized by a distinct urticarial eruption clinically and a neutrophilic dermatosis histopathologically. OBJECTIVE: In this study, we reviewed skin biopsies of lesional skin of patients seen in our outpatient clinic for autoimmune dermatoses and in allergy department from 1982 to 2014 that fulfilled these criteria...
January 2016: American Journal of Dermatopathology
Mirjana Gajic-Veljic, Branka Bonaci-Nikolic, Branislav Lekic, Dusan Skiljevic, Jasmina Ciric, Svetlana Zoric, Biljana Stojimirovic, Milos Nikolic
OBJECTIVE: To study the role of deoxyribonuclease (DNase) I activity and ANCA in propylthiouracil (PTU)-induced lupus-like syndrome (LLS). METHODS: We compared 36 SLE patients with 17 PTU-induced LLS patients diagnosed from 2008 to 2014. We studied ANCA profile (MPO, PR3, lactoferrin, CTG, elastase, bactericidal/permeability-increasing protein), anti-dsDNA, anti-ENA, anti-nucleosome, anti-histone, anti-C1q, anti-aCL, complement components, cryoglobulins and serum DNase I activity...
November 2015: Rheumatology
Peter C Grayson, Carmelo Carmona-Rivera, Lijing Xu, Noha Lim, Zhong Gao, Adam L Asare, Ulrich Specks, John H Stone, Philip Seo, Robert F Spiera, Carol A Langford, Gary S Hoffman, Cees G M Kallenberg, E William St Clair, Nadia K Tchao, Steven R Ytterberg, Deborah J Phippard, Peter A Merkel, Mariana J Kaplan, Paul A Monach
OBJECTIVE: To discover biomarkers involved in the pathophysiology of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) and to determine whether low-density granulocytes (LDGs) contribute to gene expression signatures in AAV. METHODS: The source of clinical data and linked biologic specimens was a randomized controlled treatment trial in AAV. RNA sequencing of whole blood from patients with AAV was performed during active disease at the baseline visit and during remission 6 months later...
July 2015: Arthritis & Rheumatology
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