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refractory anca vasculitis

Ibrahim Hatemi, Gulen Hatemi, Aykut F Çelik
PURPOSE OF REVIEW: Gastrointestinal system can be involved in primary and secondary vasculitides. The recent data regarding the pathophysiology, clinical findings, diagnosis, management, and outcome of gastrointestinal involvement in different types of vasculitis are reviewed. RECENT FINDINGS: Diagnosis of gastrointestinal vasculitis may be difficult and relies mostly on imaging, because biopsy samples are hard to obtain and superficial mucosal biopsies have a low yield...
September 28, 2016: Current Opinion in Rheumatology
Jun Zhang, Heng-Yuan Zhang, Shi-Zhi Chen, Ji-Yi Huang
Cholesterol embolism is a multisystemic disorder with clinical manifestations that resemble vasculitis. Anti-neutrophil cytoplasmic antibodies (ANCA) are a defining feature of ANCA-associated vasculitis, and the presence of ANCA in cholesterol embolism complicates its differential diagnosis and treatment. At present, the role of ANCA in cholesterol embolism remains unclear and no effective treatment is currently available. The present study reports the case of an Asian male who presented with spontaneous cholesterol embolism with proteinase 3 (PR3)-specific ANCA, subacute interstitial nephritis and late-developing skin lesions...
August 2016: Experimental and Therapeutic Medicine
Sheetal Malhotra, Hari Krishan Dhawan, Ratti Ram Sharma, Neelam Marwaha, Aman Sharma
Wegeners granulomatosis (WG) is an autoimmune, antineutrophil cytoplasmic antibody mediated necrotizing vasculitis involving renal, and upper and lower respiratory systems. Treatment relies on a combination of immunosuppressive drugs and tapering regimen of glucocorticoids. Therapeutic plasma exchange (TPE) has been recognized as a second line treatment. We report the successful use of TPE in combination with rituximab in achieving remission in a patient with WG (dialysis independent) not responding to conventional therapy...
June 2016: Indian Journal of Hematology & Blood Transfusion
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No abstract text is available yet for this article.
June 2016: Annals of Intensive Care
Jie Li, Liming Zhang, Wen Zhao, Min Zhu, Yi Xue, Huaping Dai
OBJECTIVE: To describe the clinical features of eosinophilic granulomatosis with polyangiitis (EGPA). METHODS: Patients who fulfilled the criteria for EGPA managed at the Beijing Chaoyang Hospital of Capital Medical University between May 2005 and Feb 2014 were retrospectively investigated. Patients' characteristics were compared according to antineutrophil cytoplasmic antibody (ANCA) status.Patients were followed up in outpatient service or by telephone to evaluate the treatment result...
March 15, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Jan Henrik Schirmer, Jan Phillip Bremer, Frank Moosig, Julia Ulrike Holle, Peter Lamprecht, Stefan Wieczorek, Sierk Haenisch, Ingolf Cascorbi
AIM: Correlation of outcomes of cyclophosphamide (CP) therapy in antineutrophil cytoplasmic antibody-associated vasculitis with genotype polymorphisms in prodrug activating cytochrome P450 enzyme genes CYP2C9 and CYP2C19. PATIENTS & METHODS: One hundred and ninety six patients with antineutrophil cytoplasmic antibody-associated vasculitis treated with CP, either as intravenous pulse or as daily oral medication, were included. Genotypes of CYP2C9 and CYP2C19 were correlated with clinical outcomes (leukopenia, infection, urotoxicity and treatment response)...
March 2016: Pharmacogenomics
Claudia Recillas-Gispert, Juan Carlos Serna-Ojeda, Luis Felipe Flores-Suárez
PURPOSE: The purpose of this descriptive study was to evaluate the clinical response to rituximab (RTX) in patients with scleritis due to granulomatosis with polyangiitis (GPA), in patients who had proved refractory to treatment with systemic glucocorticoids and immunosuppressive agents. METHODS: Retrospective analysis of interventional case series. Single referral center study. Eight patients (12 affected eyes) due to scleritis secondary to GPA, refractory to conventional treatment were included to receive RTX as therapy for remission induction...
December 2015: Graefe's Archive for Clinical and Experimental Ophthalmology
Leonardo Sales da Silva, Karla Valéria Miranda de Campos, Ana Karla Guedes de Melo, Danielle Christinne Soares Egypto de Brito, Alessandra Sousa Braz, Eutilia Andrade Medeiros Freire
The greater understanding of pathophysiology and behavior of systemic vasculitis, together with the development of therapeutic regimens with increasingly better safety and efficacy profiles, dramatically changed the prognosis of patients diagnosed with these clinical entities. Recently, the use of rituximab in the treatment of patients with ANCA-associated vasculitis in randomized clinical trials showed an important alternative in selected cases, especially patients refractory or intolerant to standard therapy with cyclophosphamide and corticosteroids...
November 2015: Revista Brasileira de Reumatologia
Vladimir Tesar, Zdenka Hruskova
INTRODUCTION: Antineutrophil cytoplasmic antibodies-associated vasculitis (AAV) is a group of autoimmune diseases characterized by the necrotizing inflammation of small vessels and associated with the antineutrophil cytoplasmic antibodies. Treatment of AAV can be divided into the induction phase aimed at achieving remission of the disease and the maintenance phase aimed at prevention of relapses. Long-term outcome of AAV dramatically improved with the introduction of cyclophosphamide...
2015: Expert Opinion on Pharmacotherapy
Rona M Smith
The introduction of glucocorticoids and cyclophosphamide has transformed ANCA associated vasculitis (AAV) from a fatal to a largely treatable condition. Over the past 40 years, considerable progress has been made in refining immunosuppressive regimens with a focus on minimising toxicity. As knowledge of the pathogenesis of AAV grows, it is mirrored by the availability of biological agents. Lymphocyte and cytokine targeted agents have been evaluated for the treatment of AAV and are entering the routine therapeutic arena with the potential to improve patient outcomes...
June 2015: La Presse Médicale
Petra M Pego, Inês Aguiar Câmara, José Pedro Andrade, João Matos Costa
INTRODUCTION: Polyarteritis nodosa (PAN) is a systemic necrotizing medium-size-vessel vasculitis with variable clinical manifestations. Diagnosis is confirmed by histology or angiography. The mainstay of treatment is corticosteroids alone or combined with cyclophosphamide (CYF). CASE REPORT: Seventy-one-year-old female, follow-up started in 1997 at the age of 56 for suspected relapsing febrile viral exanthema. Skin biopsy was performed and the diagnosis of lymphomatoid papulosis was made, with complete response to treatment with dapsone...
December 2013: Auto- Immunity Highlights
Katarzyna Wawrzycka-Adamczyk, Anna Zugaj, Anna Włudarczyk, Joanna Kosałka, Jan Sznajd, Stanisława Bazan-Socha, Jacek Musiał
INTRODUCTION: Recently, rituximab (RTX)--monoclonal antibody against the CD20 molecule on the surface of B-lymphocytes is used in the treatment of antineutrophil cytoplasmic antibody (ANCA) associated vasculitides. Efficacy of the drug administered in so-called lymphoma treatment protocol (4 x 375 mg/m2/week) has been shown not to be inferior to cyclophosphamide. However, some data published lately suggest that rituximab could also be effective in much lower doses, which could lead to reducing side effects, but above all, the cost of the therapy...
2014: Przegla̧d Lekarski
Pamela M K Lutalo, David P D'Cruz
The anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis (MPA) constitute a wide spectrum of clinical manifestations of systemic vasculitis which may range from limited disease to organ or life-threatening disease. The introduction of biologics for the management of severe ANCA-associated vasculitis and severe, relapsing disease refractory to conventional immunosuppressants, has significantly improved the clinical prognosis of these autoimmune disorders...
August 2015: International Immunopharmacology
Leilani Famorca, Marinka Twilt, Lillian Barra, Volodko Bakowsky, Susanne Benseler, David Cabral, Simon Carette, Navjot Dhindsa, Aurore Fifi-Mah, Michelle Goulet, Nader Khalidi, Majed Khraishi, Lucy McGeoch, Nataliya Milman, Christian Pineau, Kam Shojania, Regina Taylor-Gjevre, Tanveer Towheed, Judith Trudeau, Elaine Yacyshyn, Patrick Liang, Christian Pagnoux
OBJECTIVES: To study variations in Canadian clinical practice patterns for the management of ANCA-associated vasculitis (AAV) and identify points to consider for the development of national recommendations. MATERIAL AND METHODOLOGY: A 30-item needs assessment questionnaire was sent to all members of the Canadian Vasculitis network (CanVasc), Canadian Rheumatology Association (CRA), Canadian Thoracic Society (CTS) and Canadian Society of Nephrology (CSN). Respondent characteristics, practice patterns, concerns and expectations were analyzed...
2015: Open Rheumatology Journal
A Parrot, M Fartoukh, J Cadranel
Alveolar hemorrhage occurs relatively rarely and is a therapeutic emergency because it can quickly lead to acute respiratory failure, which can be fatal. Hemoptysis associated with anemia and pulmonary infiltrates suggest the diagnosis of alveolar hemorrhage, but may be absent in one third of cases including patients in respiratory distress. The diagnosis of alveolar hemorrhage is based on the findings of a bronchoalveolar lavage. The causes are numerous. It is important to identify alveolar hemorrhage due to sepsis, then separate an autoimmune cause (vasculitis associated with antineutrophil cytoplasmic antibody, connective tissue disease and Goodpasture's syndrome) with the search for autoantibodies and biopsies from readily accessible organs, from a non-immune cause, performing echocardiography...
April 2015: Revue des Maladies Respiratoires
Javier Loricera, Ricardo Blanco, José L Hernández, Trinitario Pina, M Carmen González-Vela, Miguel A González-Gay
Standard therapeutic schemes for vasculitis are usually associated with numerous side effects and uneven clinical response. However, recent advances in understanding of the pathogenesis of these systemic diseases have resulted in the development of a group of biologic agents potentially useful in patients with vasculitis. Thus, anti-tumor necrosis factor-α drugs may be effective in patients with refractory Kawasaki disease but have failed to do so in giant cell arteritis, and their role in Takayasu arteritis is yet unclear...
August 2015: International Immunopharmacology
H Timlin, S M Lee, R L Manno, P Seo, Duvura Geetha
OBJECTIVES: Advancing age is a risk factor for treatment-related side effects and mortality in AAV patients treated with cyclophosphamide (CYC) and glucocorticoids (GC) for remission induction. The efficacy and safety of rituximab (RTX) in elderly AAV patients has not been well described. METHODS: We performed a single center retrospective review of 31 consecutive AAV patients aged 60 or more at the time of RTX use for remission induction. All patients received RTX with GC for remission induction...
August 2015: Seminars in Arthritis and Rheumatism
A V Marzano, Y Balice, S Tavecchio, C Desimine, A Colombo, E Berti
Granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Wegener's granulomatosis and Churg-Strauss Syndrome respectively, are systemic granulomatous vasculitides affecting small- and medium-sized blood vessels. Both GPA and EGPA are included within the group of antineutrophilic cytoplasmic antibodies (ANCA)-associated vasculitides, on the basis of the detection of such autoantibodies in a significant proportion of affected patients. Two main settings of GPA, possibly overlapping each other, are recognized: a localized form, which is limited to the upper airways but is highly relapsing and refractory, and a diffuse form, which is initially more severe but then less commonly recurrent...
April 2015: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
Sebastián Andrés Muñoz, Ignacio Javier Gandino, Alberto Omar Orden, Alberto Allievi
BACKGROUND: The general consensus is that for patients with EGPA with poor prognosis, intensive therapy with both GC and CF is indicated. The maintenance of remission is made with GC and AZA. A considerable number of patients with EGPA are refractory to first line therapy, experience dose-limiting side effects or relapse. In clinical trials, RTX was effective for the treatment of ANCA-associated vasculitis. However, patients with a diagnosis of EGPA were not included. OBJECTIVE: to review and analyze the published literature regarding the use of RTX in the treatment of EGPA...
May 2015: Reumatología Clinica
Hiroko Nagafuchi, Tatsuya Atsumi, Kazuhiro Hatta, Eri Muso, Mitsuhiro Takeno, Hidehiro Yamada, Shoichi Ozaki
OBJECTIVES: The safety and efficacy of rituximab were examined in a multicenter open-label pilot study in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Japan. METHODS: Patients with refractory AAV were administered a rituximab infusion at a weekly dose of 375 mg/m(2) for 4 weeks. All patients also received oral daily prednisolone. The primary outcome was complete remission, which was defined as a Birmingham Vasculitis Activity Score (BVAS) of 0 or 1...
July 2015: Modern Rheumatology
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