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Vasculitis rituximab

Mitsuhiro Akiyama, Tsutomu Takeuchi
IgG4-related disease is a heterogeneous immune-mediated fibroinflammatory condition that can affect every single organ. This disease is more prevalent in the elderly (the mean age of patients is above 60 years) and the prevalence rate is estimated to be over 4.6 per 100,000 population. Before making a diagnosis, the exclusion of malignancies, lymphoma, anti-neutrophil cytoplasmic antibody-associated vasculitis, multicentric Castleman disease, and other mimickers is crucial for appropriate treatment. Broad management guidelines have been published emphasizing the need for prompt treatment and the use of glucocorticoids as first-line drug therapy for induction of remission...
March 15, 2018: Drugs & Aging
Alexandre Karras
Cryoglobulins are immunoglobulins that undergo reversible precipitation at low temperatures. They can induce systemic vasculitis, characterized by purpuric cutaneous lesions, arthritis, peripheral neuropathy, hypocomplementemia and glomerular disease. Renal pathology reveals membranoproliferative glomerulonephritis, with particularly intense mesangial cell proliferation and infiltration by macrophages, associated with intracapillary thrombi. This renal disease presents as a nephritic syndrome, with heavy proteinuria, haematuria severe hypertension and rapidly progressive kidney failure that can lead to end-stage renal disease...
March 12, 2018: Néphrologie & Thérapeutique
Eli M Miloslavsky, John L Niles, Zachary S Wallace, Frank B Cortazar, Ana Fernandes, Karen Laliberte, John H Stone
OBJECTIVE: Therapeutic advances in ANCA-associated vasculitis (AAV) have improved patient survival, but mortality rates remain higher than the general population. Glucocorticoids contribute to AAV morbidity and mortality. We examined whether an 8-week glucocorticoid course in combination with rituximab (RTX) would induce disease remission in patients with AAV. METHODS: Patients with active AAV received an 8-week prednisone taper and RTX 375mg/m2 weekly for 4 weeks...
February 3, 2018: Seminars in Arthritis and Rheumatism
Yushiro Endo, Tomohiro Koga, Midori Ishida, Yuya Fujita, Sosuke Tsuji, Ayuko Takatani, Toshimasa Shimizu, Remi Sumiyoshi, Takashi Igawa, Masataka Umeda, Shoichi Fukui, Ayako Nishino, Shin-Ya Kawashiri, Naoki Iwamoto, Kunihiro Ichinose, Mami Tamai, Hideki Nakamura, Tomoki Origuchi, Atsushi Kawakami
A 72-year-old Japanese woman diagnosed with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis was admitted to our hospital with hearing loss, temporal pain, and sudden blindness. We finally diagnosed recurrent granulomatosis with polyangiitis and initiated methyl-prednisolone pulse therapy (1,000 mg) followed by prednisolone (30 mg/day) and rituximab (RTX). After the third RTX administration, she developed bloody stools along with acute thrombocytopenia and low complement levels. We diagnosed rituximab-induced acute thrombocytopenia (RIAT), and her platelet counts spontaneously recovered...
March 9, 2018: Internal Medicine
Zdenka Hruskova, Vladimir Tesar
BACKGROUND: Rapidly progressive glomerulonephritis (RPGN) is characterized by a rapid deterioration of renal function and by extracapillary proliferation in >50% of glomeruli. The most common type of RPGN is "pauci-immune" glomerulonephritis caused by anti-neutrophil cytoplasmic antibodies-associated vasculitis (AAV). SUMMARY: The incidence of AAV increases with age and pauci-immune glomerulonephritis is the most common diagnosis found in renal biopsies in the elderly population...
January 26, 2018: Blood Purification
Stephen P McAdoo, Nicholas Medjeral-Thomas, Seerapani Gopaluni, Anisha Tanna, Nicholas Mansfield, Jack Galliford, Megan Griffith, Jeremy Levy, Thomas D Cairns, David Jayne, Alan D Salama, Charles D Pusey
Background: Current guidelines advise that rituximab or cyclophosphamide should be used for the treatment of organ-threatening disease in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV), although few studies have examined the efficacy and safety of these agents in combination. Methods: We conducted a single-centre cohort study of 66 patients treated with a combination of oral corticosteroids, rituximab and low-dose pulsed intravenous cyclophosphamide followed by a maintenance regimen of azathioprine and tapered steroid for the treatment of biopsy-proven renal involvement in AAV...
February 14, 2018: Nephrology, Dialysis, Transplantation
Abhilash Koratala, Xu Zeng
Membranoproliferative glomerulonephritis associated with mixed cryoglobulinemia is the most common form of kidney disease observed in relation to hepatitis C virus (HCV) infection. Rituximab, a monoclonal antibody against CD20, is an effective treatment for severe and/or refractory HCV-related vasculitis and may evade the need for dialysis as in our patient.
February 2018: Clinical Case Reports
Amrita Padda, Elena Schiopu, Justin Sovich, Vincent Ma, Ajjai Alva, Leslie Fecher
BACKGROUND: Immune check point inhibitors (ICIs) have emerged as a new therapeutic paradigm for a variety of malignancies including metastatic melanoma. As the use of ICIs expand, immune-mediated adverse events are becoming a common occurrence. CASE PRESENTATION: We describe the first reported patient with small vessel vasculitis, manifested by digital ischemia, following treatment with high dose Ipilimumab for resected stage IIIB/C melanoma. This patient received high dose steroids, five-day intravenous (IV) Epoprostenol protocol, botulinum toxin injections, and Rituximab 375 mg/m2 weekly for four cycles...
February 12, 2018: Journal for Immunotherapy of Cancer
Yasunori Minami, Masatoshi Kudo
Cryoglobulins are produced by the over-stimulated immune system of patients with hepatitis C virus (HCV). HCV-induced cryoglobulinemic vasculitis primarily affects small-sized vessels of systemic organs. The most common symptoms are purpura, joint pain, and peripheral neuropathy or Raynaud phenomenon. The blood tests of patients with HCV may exhibit raised erythrocyte sedimentation rate and C-reactive protein levels, positive rheumatoid factor and cryoglobulins, and decreased complement levels. Therapeutic options for HCV-induced cryoglobulinemic vasculitis, include anti-virals, Rituximab, steroids, immunosuppressant drugs, and plasma exchange...
February 2018: Brain and Nerve, Shinkei Kenkyū No Shinpo
Sohail Abdul Salim, Tauqeer Yousuf, Asha Patel, Tibor Fülöp, Mohit Agarwal
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disease characterized by multiple organ system involvement, including renal disease, with low complement levels. We report the case of a 31-year-old woman who presented with nonspecific symptoms including fatigue, diarrhea, macular rash and abdominal pain with acute renal failure leading to end-stage kidney disease. Laboratory results showed hematuria, nephrotic range proteinuria, worsening creatinine and low C1q levels. Left kidney biopsy showed proliferative glomerulonephritis with crescent formation...
February 2018: American Journal of the Medical Sciences
Daniel J Wallace
The story of how anti TNFs went from bench to bedside in 5 years is an inspirational, satisfying narrative that changed the lives of millions of patients suffering from rheumatoid arthritis and the spondyloarthropathies. Rituximab received approval for ANCA positive vasculitis on the basis of a single study with fewer than 200 subjects. Unfortunately, attempts to obtain approval for drugs for systemic lupus erythematosus (SLE) has been a 10 billion dollar failed effort that yielded only belimumab, and has been disappointing for Sjogren's syndrome...
January 30, 2018: Arthritis & Rheumatology
Marion Haubitz
In patients with ANCA-associated vasculitis renal involvement is frequently seen and the severity of renal manifestation is very important for therapeutic strategies and prognosis. Clinically rapid loss of renal function, nephritic sediment and proteinuria in a non-nephrotic range are characterizing a focal segmental necrotizing pauci-immune glomerulonephritis with extrarenal proliferations. Induction treatment depends on the severity of manifestations. With a normal renal function methotrexate can be used in combination with steroids...
January 2018: Deutsche Medizinische Wochenschrift
Divi Cornec, Brian F Kabat, John R Mills, Melissa Cheu, Amber M Hummel, Darrell R Schroeder, Matthew D Cascino, Paul Brunetta, David L Murray, Melissa R Snyder, Fernando Fervenza, Gary S Hoffman, Cees G M Kallenberg, Carol A Langford, Peter A Merkel, Paul A Monach, Philip Seo, Robert F Spiera, E William St Clair, John H Stone, David R Barnidge, Ulrich Specks
Objectives: To study the determinants of the pharmacokinetics (PK) of rituximab (RTX) in patients with ANCA-associated vasculitis (AAV) and its association with clinical outcomes. Methods: This study included data from 89 patients from the RTX in AAV trial who received the full dose of RTX (four weekly infusions of 375 mg/m2). RTX was quantified at weeks 2, 4, 8, 16 and 24, and summarized by computing the trapezoidal area under the curve. We explored potential determinants of the PK-RTX, and analysed its association with clinical outcomes: achievement of remission at 6 months, duration of B-cell depletion and time to relapse in patients who achieved complete remission...
January 10, 2018: Rheumatology
Sian K Alexander, Maria Di Cicco, Ute Pohl, Alberto Cifelli
A 73-year-old man presented with three episodes of dysphasia and disinhibited behaviour, a single seizure and transient ischaemic attack-like events characterised by right arm and/or leg weakness. These episodes were separated by month-long asymptomatic intervals. Medical history included rheumatoid arthritis, which was clinically quiescent on leflunomide.Repeated cerebrospinal fluid examination showed a persistent lymphocytosis with mildly reduced glucose and elevated protein; oligoclonal bands and viral PCR were negative...
January 12, 2018: BMJ Case Reports
Eva Schrezenmeier, David Jayne, Thomas Dörner
The unique contributions of memory B cells and plasma cells in kidney diseases remain unclear. In this review, we evaluate the clinical experience with treatments directed at B cells, such as rituximab, and at plasma cells, such as proteasome inhibition, to shed light on the role of these two B lineage compartments in glomerular diseases. Specifically, analysis of these targeted interventions in diseases such as ANCA-associated vasculitis, SLE, and antibody-mediated transplant rejection permits insight into the pathogenetic effect of these cells...
January 11, 2018: Journal of the American Society of Nephrology: JASN
Gizem Ayan, Sinem Nihal Esatoglu, Gulen Hatemi, Serdal Ugurlu, Emire Seyahi, Melike Melikoglu, Izzet Fresko, Huri Ozdogan, Sebahattin Yurdakul, Vedat Hamuryudan
Rituximab (RTX) is becoming a standard treatment for patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) but heterogeneity exists regarding its use. We present our uncontrolled experience with RTX in patients with refractory AAV and also the results of a systematic review of non-randomized studies on RTX in AAV patients. We retrospectively reviewed the records of AAV patients treated with RTX following an inadequate response to immunosuppressives between 2011 and 2015. The systematic review covered all English articles listed in PubMed until June 2017...
January 10, 2018: Rheumatology International
David Xu, Shawn R Lin, Laura Bonelli, Ben J Glasgow, Robert A Goldberg
A 55-year-old man presented with unilateral orbital inflammation and no light perception vision. Imaging revealed infiltrative enhancement of the optic nerve, orbit, and intracranial tissue. The case was suspicious for invasive fungal disease, but ultimate workup and orbital biopsy revealed granulomatosis with polyangiitis. The patient's inflammation resolved with corticosteroid and rituximab therapy. Granulomatosis with polyangiitis is a systemic vasculitis that can mimic a number of orbital pathologies.
January 5, 2018: Orbit
Miaoli Lin, Stephen D Anesi, Lina Ma, Aseef Ahmed, Karen Small, C Stephen Foster
PURPOSE: To describe the clinical characteristics, therapies, visual outcomes and prognoses of patients with retinal vasculitis associated with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). DESIGN: Retrospective case series. METHODS: Patients diagnosed with retinal vasculitis associated with AAV and at least 6-months of follow-up were included. Demographic data, systemic and ocular features, best corrected visual acuity at the initial visit and latest visit, fluorescein angiography (FA) and indocyanine green angiography (ICGA) findings, therapy regimen and outcome were collected from the Massachusetts Eye Research and Surgery Institution (MERSI) database from 2006 to 2017...
December 16, 2017: American Journal of Ophthalmology
Shunsuke Furuta, Takao Sugiyama, Takeshi Umibe, Yuko Kaneko, Koichi Amano, Kazuhiro Kurasawa, Daiki Nakagomi, Masaki Hiraguri, Hideki Hanaoka, Yasunori Sato, Kei Ikeda, Hiroshi Nakajima
INTRODUCTION: Antineutrophil cytoplasm antibody-associated vasculitis (AAV) is a form of systemic vasculitis. The current standard induction therapy with the combination of high-dose glucocorticoids and cyclophosphamide or rituximab has high remission rates of 80%-90%. However, it is also associated with various side effects, including death due to infection or cardiovascular disease. There is an unmet medical need of a new therapy to reduce side effects. METHODS AND ANALYSIS: This is a phase IV multicentre, open-label, randomised controlled trial that aims to evaluate the efficacy and safety of a new remission induction regimen with the combination of low-dose glucocorticoids and rituximab...
December 14, 2017: BMJ Open
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