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https://www.readbyqxmd.com/read/28724596/severe-destructive-nasopharyngeal-granulomatosis-with-polyangiitis-with-superimposed-skull-base-pseudomonas-aeruginosa-osteomyelitis
#1
Mitchell S von Itzstein, Jithma P Abeykoon, Daniel D Summerfield, Jennifer A Whitaker
Skull base osteomyelitis in the setting of granulomatosis with polyangiitis (GPA) is rare and entails significant diagnostic challenges. We present a case of a 65-year-old Caucasian man with a history of rheumatoid arthritis, off immunosuppression for 18 months, who presented with 2 years of chronic headaches, severe fatigue, saddle nose deformity and 20-kilogram unintentional weight loss. Maxillofacial CT revealed an extensive destructive sinonasal and erosive skull base process. Laboratory evaluation showed equivocal elevation of antiproteinase 3 antibodies with negative antineutrophil cytoplasmic antibody panel...
July 19, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28715943/introducing-treat-to-target-strategies-of-autoimmune-extrahepatic-manifestations-of-chronic-hepatitis-c-virus-infection
#2
Soledad Retamozo, Pilar Brito-Zeron, Luca Quartuccio, Salvatore De Vita, Manuel Ramos-Casals
The hepatitis C virus (HCV) is recognized as one of the hepatic viruses most often associated with extrahepatic manifestations (EHMs). It is currently accepted that cryoglobulinemic vasculitis (CV) is the key autoimmune extrahepatic disease associated with HCV infection. Therapeutic approaches have mainly been based on the use of old antiviral interferon (IFN)-based regimens and immunosuppressive therapies, often with an inadequate balance between therapeutic benefits and excess side effects. AREAS COVERED...
July 18, 2017: Expert Review of Clinical Pharmacology
https://www.readbyqxmd.com/read/28705782/biologics-in-myelodysplastic-syndrome-related-systemic-inflammatory-and-autoimmune-diseases-french-multicenter-retrospective-study-of-29-patients
#3
REVIEW
Arsene Mekinian, Guillaume Dervin, Nathanael Lapidus, Jean-Emmanuel Kahn, Louis Terriou, Eric Liozon, Eric Grignano, Jean-Charles Piette, Odile Beyne Rauzy, Vincent Grobost, Pascal Godmer, Jerome Gillard, Julien Rossignol, David Launay, Achille Aouba, Thierry Cardon, Laurence Bouillet, Jonathan Broner, Julien Vinit, Lionel Ades, Fabrice Carrat, Clementine Salvado, Eric Toussirot, Mathilde Versini, Nathalie Costedoat-Chalumeau, Jean Baptiste Fraison, Philippe Guilpain, Pierre Fenaux, Olivier Fain
BACKGROUND: Systemic inflammatory and autoimmune diseases (SIADs) associated with myelodysplastic syndromes are often difficult to treat. Corticosteroids are efficient but only usually at high doses. The use of biologics needs to be specified. METHODS: In a French multicenter retrospective study, we analyzed the efficacy and safety of biologics (tumor necrosis factor-α [TNF-α] antagonists, tocilizumab, rituximab and anakinra) for SIADs associated with myelodysplastic syndromes (MDSs)...
July 10, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28654132/henoch-sch%C3%A3-nlein-purpura-a-literature-review
#4
Liv Eline Hetland, Kjærsti Sørensen Susrud, Kim Hein Lindahl, Anette Bygum
Henoch-Schönlein purpura is the most common childhood vasculitis, but may also affect adults. This article reviews the literature since 2011 on advances in diagnosis, clinical disease manifestations, pathophysiology and treatment of Henoch-Schönlein purpura. The clinical manifestations are thought to arise from IgA depositions in blood vessel walls in the affected organs, mostly skin, gastrointestinal tract, joints and kidneys. Corticosteroids may be effective in rapid resolution of renal manifestations and treating joint and abdominal pain, but they are not proven effective for treating organ manifestations and complications, such as glomerulonephritis, bowel infarction or intussusception...
June 27, 2017: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/28638602/b-cell-depleting-therapy-with-rituximab-or-ofatumumab-in-immunoglobulin-a-nephropathy-or-vasculitis-with-nephritis
#5
Sigrid Lundberg, Emelie Westergren, Jessica Smolander, Annette Bruchfeld
BACKGROUND: Approximately 30% of adult patients with immunoglobulin A (IgA) nephropathy (IgAN) or IgA vasculitis with nephritis (IgAVN) develop end-stage renal disease during long-term follow-up. In particular, patients with nephritic-nephrotic syndrome have an increased risk of rapid progression. Conventional immunosuppressive therapy with corticosteroids (CSs) may be insufficient for disease control and is associated with a number of side effects. Rituximab (RTX) has been shown to be well tolerated and effective in a range of glomerular diseases, but there is little information on its therapeutic potential in IgAN...
February 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28631698/-hepatitis-c-virus-associated-cryoglobulinemic-vasculitis-a-20-year-experience-with-treatment
#6
T M Ignatova, L V Kozlovskaya, N B Gordovskaya, O A Chernova, S Yu Milovanova, P I Novikov, T P Nekrasova, T V Beketova, N A Mukhin
AIM: To summarize the experience of a multidisciplinary therapy hospital in treating patients with hepatitis C virus (HCV)-associated cryoglobulinemic vasculitis (CV). SUBJECTS AND METHODS: Seventy-two patients (mean age, 49.4±10.3 years) with HCV-associated CV were examined and followed up for an average period of 2.8±3.6 years. The efficiency of traditional (corticosteroids ± cyclophosphamide) and selective (rituximab) immunosuppressive therapy (IST) was estimated in 31 and 15 observations, respectively, and that of antiviral therapy (AVT) in 25...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28614191/anti-cd20-blocker-rituximab-in-kidney-transplantation
#7
Puneet Sood, Sundaram Hariharan
Rituximab is a chimeric anti-CD20 monoclonal protein used in various clinical scenarios in kidney transplant recipients. However, its evidence-based use there remains limited due to lack of controlled studies, limited sample size, short follow-up and poorly defined endpoints. Rituximab is indicated for CD20+ PTLD. It may be beneficial for treating recurrent MN and recurrent allograft ANCA vasculitis and possibly for recurrent FSGS. Rituximab, in combination with IVIG/PP, appears to decrease antibody level and increase the odds of transplantation in sensitized recipients...
June 13, 2017: Transplantation
https://www.readbyqxmd.com/read/28611282/the-4-plus-2-rituximab-protocol-makes-maintenance-treatment-unneeded-in-patients-with-refractory-anca-associated-vasculitis-a-10-year-observation-study
#8
Dario Roccatello, Savino Sciascia, Daniela Rossi, Mirella Alpa, Carla Naretto, Massimo Radin, Roberta Fenoglio, Simone Baldovino, Elisa Menegatti
BACKGROUND: ANCA associated vasculitides (AAV) often present with a chronic relapsing course. Relapse leads to increased immunosuppressive exposure and consequent toxicity. While two randomized controlled trials have shown rituximab (RTX) to be the most effective induction treatment in patients with relapsing disease, the optimal treatment duration and RTX dose remain debated. Whether to administer a maintenance dose to every patient, at a fixed time interval or on the basis of B cell count and ANCA titre or only when disease manifestations do occur is still debated as well...
May 23, 2017: Oncotarget
https://www.readbyqxmd.com/read/28552512/immune-response-to-pneumococcal-conjugate-vaccine-in-patients-with-systemic-vasculitis-receiving-standard-of-care-therapy
#9
Per Nived, Johanna Nagel, Tore Saxne, Pierre Geborek, Göran Jönsson, Lillemor Skattum, Meliha C Kapetanovic
AIM: To study the effect of standard of care therapy on antibody response and functionality following immunization with 13-valent pneumococcal conjugate vaccine (PCV13) in patients with primary systemic vasculitis compared to healthy controls. METHODS: 49 patients with vasculitis and 49 controls received a single dose (0.5ml) PCV13 intramuscularly. Ongoing treatments: azathioprine (AZA; n=11), cyclophosphamide (CYC; n=6), methotrexate (MTX; n=9), rituximab (n=3); anti-TNF (n=2), mycophenolate mofetil (n=2), prednisolone alone (n=15) and no active treatment (n=2)...
June 22, 2017: Vaccine
https://www.readbyqxmd.com/read/28539536/remission-induction-therapy-with-rituximab-for-microscopic-polyangiitis-a-feasibility-study
#10
Ayako Saito, Yoichi Takeuchi, Saeko Kagaya, Yoshie Ojima, Hirotaka Fukami, Hiroyuki Sato, Ken Matsuda, Tasuku Nagasawa
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is systemic vascular inflammation. Microscopic polyangiitis (MPA) is a major type of AAV in Japan. MPA often affects the kidneys and lungs, leading to death if untreated. Induction therapy (i.e., initial treatment) for MPA has not been optimized, although methylprednisolone and cyclophosphamide are commonly used. Recently, rituximab (RTX) (a monoclonal antibody against the protein CD20) has also been used to treat refractory AAV. RTX at 375 mg/m(2)/week for 4 weeks (i...
2017: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28536934/-off-label-biological-therapies-in-patients-with-large-vessel-vasculitis-and-or-polymyalgia-rheumatica-safety-and-efficacy-analysis-of-a-nationwide-german-registry-graid2
#11
J C Henes, H Schulze-Koops, M Witt, H P Tony, F Mueller, M Grunke, M Czihal, T Dörner, F Proft
OBJECTIVE: To evaluate the safety and clinical outcome of biological therapies in patients with large vessel vasculitis (LVV) or polymyalgia rheumatica (PMR) refractory to standard of care therapy in a real-life setting in Germany. METHODS: GRAID 2 (German Registry in Autoimmune Diseases 2) is a retrospective, noninterventional, multicenter registry collecting data from all patients with inflammatory rheumatic diseases refractory to conventional therapy treated with an initial off-label biological between August 2006 and December 2013...
May 23, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28536920/clinicopathological-features-of-multiple-mononeuropathy-associated-with-systemic-lupus%C3%A2-erythematosus-a-multicenter-study
#12
Elodie Rivière, Fleur Cohen Aubart, Thierry Maisonobe, François Maurier, Christophe Richez, Bruno Gombert, Marie Gousseff, Daniel Adoue, Alexis Mathian, Miguel Hié, Julien Haroche, Zahir Amoura
Multiple mononeuropathy (MM) occurs rarely during systemic lupus erythematosus (SLE) but may lead to major disability. The aim of this study was to investigate the clinic-pathological presentations of MM during SLE, as well as long-term outcomes. We conducted a multicentric retrospective study that included patients receiving a diagnosis of MM during SLE. Ten patients were included (8 women and 2 men, median age at MM diagnosis: 40.4 years). SLE was diagnosed before MM in 9/10 patients (median time 8.2 years)...
June 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28508096/-off-label-biologic-therapy-of-anca-associated-and-non-anca-associated-small-vessel-vasculitis-efficacy-and-safety-analysis-of-a-national-registry-graid2
#13
N Venhoff, F Proft, H Schulze-Koops, J Holle, R E Voll, C Iking-Konert, A M Jacobi, J Henes, L Unger, O Kneitz, T Dörner, J Thiel
OBJECTIVE: To evaluate the clinical efficacy and safety of off-label biological therapies in patients with ANCA-associated vasculitis (AAV) and non-ANCA-associated small-vessel vasculitis (nAAV) in clinical practice. METHODS: The German Registry in Autoimmune Diseases 2 (GRAID2) is a national, retrospective, non-interventional, multicentre observational study (August 2006 until December 2013) on patients with autoimmune diseases refractory to standard immunosuppressive therapy treated with off-label biologicals...
May 15, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28507447/managing-refractory-cryoglobulinemic-vasculitis-challenges-and-solutions
#14
REVIEW
Predrag Ostojic, Ivan R Jeremic
Cryoglobulinemia is thought to be a rare condition. It may be an isolated disorder or secondary to a particular disease. According to immunoglobulin composition, cryoglobulinemia is classified into three types. In mixed cryoglobulinemia (types II and III), vascular deposition of cryoglobulin-containing immune complexes and complement may induce a clinical syndrome, characterized by systemic vasculitis and inflammation - cryoglobulinemic vasculitis (CryoVas). Most common clinical manifestations in CryoVas are skin lesions (orthostatic purpura and ulcers), weakness, peripheral neuropathy, Raynaud's phenomenon, sicca syndrome, membranoproliferative glomerulonephritis, and arthralgia and seldom arthritis...
2017: Journal of Inflammation Research
https://www.readbyqxmd.com/read/28495143/central-and-peripheral-nervous-system-immune-mediated-demyelinating-disease-after-allogeneic-hematopoietic-stem-cell-transplantation
#15
M I Stefanou, F Bischof
OBJECTIVE: We aimed to evaluate clinical and diagnostic features of central and peripheral immune-mediated demyelinating disease (CPID) in allogeneic hematopoietic stem cell transplantation (aHSCT) recipients. BACKGROUND: CPID refers to the late-onset, immune-mediated neurological complications following aHSCT, when other frequent differential diagnoses have been ruled out, and when symptoms and signs of systemic GvHD manifestations are absent. METHODS: Case records at the University of Tuebingen, between 2001 and 2015, were screened to identify patients with CPID after aHSCT...
June 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28491854/mpo-anca-associated-necrotizing-glomerulonephritis-in-rheumatoid-arthritis-a-case-report-and-review-of-literature
#16
Mário Góis, Ana Messias, Dulce Carvalho, Fernanda Carvalho, Helena Sousa, João Sousa, Fernando Nolasco
BACKGROUND: Renal involvement in rheumatoid arthritis (RA) is common and has a negative impact on patient survival. Only few cases have been reported of necrotizing glomerulonephritis (GN) associated with myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) in patients with RA. CASE PRESENTATION: We report a patient with RA who developed a necrotizing GN associated with ANCA-MPO, treated with rituximab (RTX). A 55-year-old man with a 27-year history of RA under secukinumab was referred to our nephrology clinic with worsening renal function associated with microhematuria and proteinuria...
March 2017: Journal of Nephropathology
https://www.readbyqxmd.com/read/28487867/non-ischemic-cardiomyopathy-after-rituximab-treatment-for-membranous-nephropathy
#17
Wisit Cheungpasitporn, Stephen L Kopecky, Ulrich Specks, Kharmen Bharucha, Fernando C Fervenza
Rituximab is an anti-CD20 monoclonal antibody frequently used for the treatment of non-Hodgkin's lymphoma, chronic lymphocytic leukemia (CLL), rheumatoid arthritis (RA), and anti-neutrophilic cytoplasmic antibody (ANCA)-associated vasculitis. In addition, rituximab has recently been increasingly used as an off-label treatment in a number of inflammatory and systemic autoimmune diseases. It is advised that rituximab infusion may cause infusion reactions and adverse cardiac effects including arrhythmia and angina, especially in patients with prior history of cardiovascular diseases...
2017: Journal of Renal Injury Prevention
https://www.readbyqxmd.com/read/28471627/rituximab-as-a-second-line-treatment-for-lymphocytic-vasculitis-of-the-central-nervous-system
#18
Saar Anis, Amir Sharabi, Yair Mina, Ainat Klein, Emanuela Cagnano, Ori Elkayam, Tanya Gurevich
No abstract text is available yet for this article.
October 2016: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28457680/-hypocomplementemic-urticarial-vasculitis
#19
M Jachiet, B Flageul, J-D Bouaziz, M Bagot, B Terrier
Hypocomplementemic urticarial vasculitis (HUV), called anti-C1q vasculitis in the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides, is a rare systemic vasculitis of unknown etiology, affecting small vessels. HUV is characterized by urticarial lesions, hypocomplementemia and systemic manifestations, mostly musculoskeletal and ocular, but also gastrointestinal, pulmonary and kidney involvement. Anti-C1q antibodies are detected in only half of the patients, and low C1q seems to represent a more sensitive marker...
April 27, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28454112/the-challenge-of-treating-hepatitis-c-virus-associated-cryoglobulinemic-vasculitis-in-the-era-of-anti-cd20-monoclonal-antibodies-and-direct-antiviral-agents
#20
REVIEW
Dario Roccatello, Savino Sciascia, Daniela Rossi, Laura Solfietti, Roberta Fenoglio, Elisa Menegatti, Simone Baldovino
Mixed cryoglobulinemia syndrome (MC) is a systemic vasculitis involving kidneys, joints, skin, and peripheral nerves. While many autoimmune, lymphoproliferative, and neoplastic disorders have been associated with this disorder, hepatitis C virus (HCV) is known to be the etiologic agent in the majority of patients. Therefore, clinical research has focused on anti-viral drugs and, more recently, on the new, highly potent Direct-acting Antiviral Agents (DAAs). These drugs assure sustained virologic response (SVR) rates >90%...
June 20, 2017: Oncotarget
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