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https://www.readbyqxmd.com/read/28683778/unusual-acute-lupus-hemophagocytic-syndrome-a-test-of-diagnostic-criteria-a-case-report
#1
Wijetunga Mudalige Udai Akalanka Wijetunga, Ravindra Laxman Satarasinghe, Balasuriya Mudiyanselage Dayananda, Ganhewage Kokila Darshani
BACKGROUND: Hemophagocytic lymphohistiocytosis is an aggressive life-threatening syndrome of excessive immune activation. Hemophagocytic lymphohistiocytosis due to systemic lupus erythematosus is described as acute lupus hemophagocytic syndrome. Acute lupus hemophagocytic syndrome presenting with negative antinuclear antibody is uncommon. CASE PRESENTATION: A 57-year-old Sri Lankan woman presented with intermittent fever, weight loss, episodic confusion, and alopecia for 3 months...
July 7, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28683654/efficacy-and-safety-of-multitarget-therapy-with-cyclophosphamide-and-tacrolimus-for-lupus-nephritis-a-prospective-single-arm-single-centre-open-label-pilot-study-in-japan
#2
R Sakai, T Kurasawa, E Nishi, T Kondo, Y Okada, A Shibata, K Nishimura, K Chino, A Okuyama, H Takei, H Nagasawa, K Amano
Background Pulsed cyclophosphamide or mycophenolate mofetil for lupus nephritis has limited efficacy. We previously reported a case of mixed-class IV + V lupus nephritis successfully treated with cyclophosphamide and tacrolimus. This study assessed the efficacy and safety of multitarget therapy with cyclophosphamide and tacrolimus for the treatment of lupus nephritis. Methods In a prospective, single-arm, open label pilot study, we recruited 15 patients aged 18-64 years with active lupus nephritis who met the American College of Rheumatology criteria for a diagnosis of systemic lupus erythematosus (1997)...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28668085/utility-of-somatosensory-evoked-potentials-in-the-assessment-of-response-to-ivig-in-a-long-lasting-case-of-chronic-immune-sensory-polyradiculopathy
#3
Angelo Maurizio Clerici, Eduardo Nobile-Orazio, Marco Mauri, Federico Sergio Squellati, Giorgio Giovanni Bono
BACKGROUND: Chronic immune sensory polyradiculopathy (CISP) identifies a progressive acquired peripheral dysimmune neuropathy recognized as a chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) variant. We describe a young woman with a thirteen-year history of CISP with a belated variable response to intravenous immunoglobulin (IVIG) and an almost erratic anticipation of symptoms between IVIG cycles. The association of IVIG and corticosteroids, immunosuppressants, plasmapheresis, did not lead to clinical improvement and was characterized by significant side effects...
July 1, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28639592/current-status-of-lupus-nephritis
#4
REVIEW
Ajay Jaryal, Sanjay Vikrant
Systemic lupus erythematosus (SLE) is a systemic disease of unknown aetiology with variable course and prognosis. Lupus nephritis (LN) is one of the important disease manifestations of SLE with considerable influence on patient outcomes. Immunosuppression therapy has made it possible to control the disease with improved life expectancy and quality of life. In the last few decades, various studies across the globe have clarified the role, dose and duration of immunosuppression currently in use and also provided evidence for new agents such as mycophenolate mofetil, calcineurin inhibitors and rituximab...
February 2017: Indian Journal of Medical Research
https://www.readbyqxmd.com/read/28633752/rituximab-administration-in-a-patient-with-pemphigus-vulgaris-following-reactivation-of-occult-hepatitis-b-virus-infection
#5
Soheil Tavakolpour, Tahereh Soori, Pedram Noormohammadpour, Kamran Balighi, Hamidreza Mahmoudi, Maryam Daneshpazhooh
Immunosuppressive drugs are the milestone of treatment of autoimmune diseases, but they can lead to serious complications, including hepatitis B virus reactivation in HBV carriers as well as in patients with occult HBV infection (OBI). A 36-year-old man with OBI was diagnosed with pemphigus vulgaris. He was prescribed prednisolone and his hepatitis B surface antigen turned positive. Viral replication was successfully controlled by lamivudine and adefovir. Mycophenolate mofetil and intravenous immunoglobulin  were not effective in controlling the pemphigus vulgaris...
June 20, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/28625079/retrospective-analysis-of-nephritis-response-and-renal-outcome-in-a-cohort-of-928-egyptian-lupus-nephritis-patients-a-university-hospital-experience
#6
M Momtaz, A Fayed, M Wadie, S M Gamal, S A Ghoniem, N Sobhy, N M Kamal Elden, W M Hamza
Aim We aim to describe the pattern of response to treatment in a cohort of Egyptian lupus nephritis (LN) patients and to define variable prognostic factors. Methods We retrospectively analyzed records of 928 systemic lupus erythematosus (SLE) patients (898 females, 30 males) with biopsy-confirmed LN seen between 2006 and 2012 at Cairo University hospitals. Results Our study involved 928 SLE patients with a mean age of 26.25 ± 6.487 years, mean LN duration at time of renal biopsy 6.48 ± 4.27 months, mean SLEDAI 28...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28612005/renal-limited-lupus-like-nephritis-how-much-of-a-lupus
#7
Asmaa S Abu Maziad, Jose Torrealba, Mouin G Seikaly, Jared R Hassler, Allen R Hendricks
Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by development of autoantibodies to nuclear and cytoplasmic antigens. A small subset of SLE patients who had the typical clinical features of SLE was reported to show persistently negative antinuclear antibody tests. Our report describes a 5-year-old male who presented with histopathological findings suggestive of lupus nephritis with no clinical signs or symptoms of SLE and negative autoantibodies. He was treated with corticosteroids, mycophenolate mofetil, and monthly intravenous cyclophosphamide...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28604554/severe-ocular-myositis-after-ipilimumab-treatment-for-melanoma-a-report-of-2-cases
#8
Anna Pushkarevskaya, Ulf Neuberger, Antonia Dimitrakopoulou-Strauss, Alexander Enk, Jessica C Hassel
Ipilimumab binds and blocks cytotoxic T-lymphocyte-associated antigen-4, causing enhanced T-cell reaction, antitumor response, and significant improvement of the overall survival of patients with metastatic melanoma. Patients treated with ipilimumab can develop immune-related adverse effects, primarily dermatitis, colitis, hepatitis, and hypophysitis. Although, in phase I-III studies, 64.2% of all patients suffered from immune-related adverse effects, ocular adverse effects occurred in 1.3% only. In the cases reported below, 2 patients with metastatic melanoma developed severe ocular myositis after treatment with ipilimumab...
June 9, 2017: Journal of Immunotherapy
https://www.readbyqxmd.com/read/28584258/cathepsin-s-inhibition-combines-control-of-systemic-and-peripheral-pathomechanisms-of-autoimmune-tissue-injury
#9
Maia Tato, Santhosh V Kumar, Yajuan Liu, Shrikant R Mulay, Solange Moll, Bastian Popper, Jonathan N Eberhard, Dana Thomasova, Arne Christian Rufer, Sabine Gruner, Wolfgang Haap, Guido Hartmann, Hans-Joachim Anders
Cathepsin(Cat)-S processing of the invariant chain-MHC-II complex inside antigen presenting cells is a central pathomechanism of autoimmune-diseases. Additionally, Cat-S is released by activated-myeloid cells and was recently described to activate protease-activated-receptor-(PAR)-2 in extracellular compartments. We hypothesized that Cat-S blockade targets both mechanisms and elicits synergistic therapeutic effects on autoimmune tissue injury. MRL-(Fas)lpr mice with spontaneous autoimmune tissue injury were treated with different doses of Cat-S inhibitor RO5459072, mycophenolate mofetil or vehicle...
June 5, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28525589/bone-marrow-involvement-in-systemic-lupus-erythematosus
#10
Emilie Chalayer, Nathalie Costedoat-Chalumeau, Odile Beyne-Rauzy, Jacques Ninet, Stephane Durupt, Jacques Tebib, Bouchra Asli, Olivier Lambotte, Martine Ffrench, Christian Vasselon, Pascal Cathébras
BACKGROUND: Besides peripheral cytopenias, bone marrow abnormalities, such as fibrosis, pure red cell aplasia, and aplastic anemia have been reported in patients with systemic lupus erythematosus (SLE), suggesting that bone marrow may be a target organ in SLE. Our objective was to describe this bone marrow involvement. METHODS: This registry is a nationwide retrospective study. Centers provided data concerning medical history, SLE manifestations, type of hematologic disorder, treatments and outcome...
May 19, 2017: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/28513247/use-of-nonbiologic-treatments-in-antihistamine-refractory-chronic-urticaria-a-review-of-published-evidence
#11
Jesper Grønlund Holm, Ilya Ivyanskiy, Simon Francis Thomsen
BACKGROUND: Knowledge of effectiveness and safety of the nonbiologic, nonantihistamine treatments used for chronic urticaria is important as in some cases the principal guideline-recommended drug; omalizumab, has limited effect, side effects or is too expensive or unavailable. Herein, we systematically review the evidence for the use of the nonbiologic treatments in antihistamine-refractory chronic urticaria. METHODS: We performed a systematic review of the literature using PubMed and Webofscience and identified studies that reported use of one or more of the nonbiological, nonantihistamine treatment options for chronic urticaria...
May 31, 2017: Journal of Dermatological Treatment
https://www.readbyqxmd.com/read/28508388/positive-clinical-outcome-in-a-patient-with-recalcitrant-bullous-pemphigoid-treated-with-rituximab-and-intravenous-immunoglobulin
#12
T Nguyen, A R Ahmed
A 41-year-old white man was treated for bullous pemphigoid (BP) for 4 years, using high-dose prednisone as well as ciclosporin and mycophenolate mofetil. Sustained clinical improvement was not observed. He suffered several serious side effects. Consequently, he was treated with a combination of rituximab (RTX) and intravenous immunoglobulin (IVIg). He received 12 infusions of RTX in 6 months and monthly IVIg until the end of the therapy. Within 5 weeks of this therapy, appearance of new lesions ceased. Within 8 weeks, all previous lesions resolved and previous medications were discontinued...
May 15, 2017: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/28481421/immunomodulatory-treatment-other-than-corticosteroids-immunoglobulin-and-plasma-exchange-for-chronic-inflammatory-demyelinating-polyradiculoneuropathy
#13
REVIEW
Mohamed Mahdi-Rogers, Ruth Brassington, Angela A Gunn, Pieter A van Doorn, Richard Ac Hughes
BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a disease that causes progressive or relapsing and remitting weakness and numbness. It is probably caused by an autoimmune process. Immunosuppressive or immunomodulatory drugs would be expected to be beneficial. This review was first published in 2003 and has been updated most recently in 2016. OBJECTIVES: To assess the effects of immunomodulatory and immunosuppressive agents other than corticosteroids, immunoglobulin, and plasma exchange in CIDP...
May 8, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28471902/recurrent-optic-neuritis-associated-with-mog-antibody-seropositivity
#14
Thashi Chang, Patrick Waters, Mark Woodhall, Angela Vincent
INTRODUCTION: Optic neuritis (ON) can be the first manifestation of autoimmune central nervous system diseases such as multiple sclerosis and neuromyelitis optica, but it can also occur as an isolated monophasic or relapsing disease. A proportion of these patients have antibodies against myelin oligodendrocyte glycoprotein (MOG). We report a case of recurrent ON with MOG antibodies in a South Asian patient. CASE REPORT: A 50-year-old Sri Lankan female with a previous history of 2 episodes of steroid-responsive ON presented with a 20/120 visual impairment of her right eye...
May 2017: Neurologist
https://www.readbyqxmd.com/read/28439062/a-rare-case-of-necrotizing-myopathy-and-fibrinous-and-organizing-pneumonia-with-anti-ej-antisynthetase-syndrome-and-ssa-antibodies
#15
Muhammad Kashif, Divya Arya, Masooma Niazi, Misbahuddin Khaja
BACKGROUND Idiopathic inflammatory myopathies are autoimmune disorders that can involve the skin, joints, muscles, and lungs. The most common of these disorders are dermatomyositis, polymyositis, overlap syndrome, and inclusion body myositis. Necrotizing autoimmune myopathy is an idiopathic inflammatory myopathy that is rarely associated with Sjögren's syndrome. The most common lung findings associated with anti-EJ antisynthetase syndrome are nonspecific interstitial pneumonia and usual interstitial pneumonia; this condition is rarely associated with fibrinous and organizing pneumonia...
April 25, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28413686/cytokine-profiles-of-amyopathic-dermatomyositis-with-interstitial-lung-diseases-treated-with-mycophenolate
#16
Masachika Hayashi, Ami Aoki, Katsuaki Asakawa, Takuro Sakagami, Toshiaki Kikuchi, Toshinori Takada
A 59-year-old Japanese man diagnosed with interstitial lung disease associated with amyopathic dermatomyositis with anti-melanoma differentiation-associated gene 5 (MDA-5) antibodies was treated with intravenous methyl prednisolone (PSL) 1000 mg, oral PSL 1 mg/kg, and oral cyclosporin 200 mg daily. His respiratory condition worsened after treatment with two times of intravenous cyclophosphamide and another steroid pulse therapy as well as PSL and cyclosporin. Addition of mycophenolate mofetil (MMF), 1...
July 2017: Respirology Case Reports
https://www.readbyqxmd.com/read/28405598/clearance-of-bk-virus-nephropathy-by-combination-antiviral-therapy-with-intravenous-immunoglobulin
#17
Kathy Kable, Carmen D Davies, Philip J O'connell, Jeremy R Chapman, Brian John Nankivell
BACKGROUND: Reactivation of BK polyoma virus causes a destructive virus allograft nephropathy (BKVAN) with graft loss in 46%. Treatment options are limited to reduced immunosuppression and largely ineffective antiviral agents. Some studies suggest benefit from intravenous immunoglobulin (IVIG). METHODS: We evaluated effectiveness of adjuvant IVIG to eliminate virus from blood and tissue, in a retrospective, single-center cohort study, against standard-of-care controls...
April 2017: Transplantation Direct
https://www.readbyqxmd.com/read/28384208/comparison-of-renal-response-to-four-different-induction-therapies-in-japanese-patients-with-lupus-nephritis-class-iii-or-iv-a-single-centre-retrospective-study
#18
Hironari Hanaoka, Tomofumi Kiyokawa, Harunobu Iida, Kana Ishimori, Yukiko Takakuwa, Takahiro Okazaki, Hidehiro Yamada, Daisuke Ichikawa, Sayuri Shirai, Junki Koike, Shoichi Ozaki
The recent recommendations for the management of lupus nephritis suggest that racial background should be considered while choosing induction therapy. However, the responses to different induction regimens have been poorly studied in Japanese population. Here, we assessed the renal response to different induction therapies in Japanese patients with lupus nephritis class III or IV. The records of 64 patients with biopsy-proven lupus nephritis class III or IV were retrospectively evaluated according to therapy received: monthly intravenous cyclophosphamide (IVCY), the Euro-lupus nephritis trial (ELNT) protocol-IVCY, tacrolimus (TAC), or mycophenolate mofetil (MMF)...
2017: PloS One
https://www.readbyqxmd.com/read/28295192/state-of-the-art-how-i-manage-immune-thrombocytopenia
#19
REVIEW
Nichola Cooper
The management of patients with immune thrombocytopenia (ITP) is rapidly evolving. Over the last 15 years, a number of novel treatments have improved practice, with many steroid-sparing agents and a reduction in the progression to splenectomy. Although this has improved clinical care, many therapeutic challenges remain. There is no diagnostic test, no biomarkers to direct treatment and few comparative studies to help management decisions. Development of up to date guidelines is difficult with little high-grade evidence...
April 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28293459/refractory-optic-perineuritis-due-to-granulomatosis-with-polyangiitis-successfully-treated-with-methotrexate-and-mycophenolate-mofetil-combination-therapy
#20
Yoshitaka Kimura, Kurumi Asako, Hirotoshi Kikuchi, Hajime Kono
Optic perineuritis is an uncommon inflammatory disorder of the optic sheath that causes visual loss or eye pain. There are few case reports of optic perineuritis associated with granulomatosis with polyangiitis. Herein we report the case of a 37-year-old male with granulomatosis with polyangiitis and who presented with headache, blurred vision in the right eye, diplopia, and numbness in the right forehead. Brain magnetic resonance images (MRI) findings revealed hypertrophic pachymeningitis and refractory optic perineuritis...
March 2017: European Journal of Rheumatology
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