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A J Mohammad, A Hot, F Arndt, F Moosig, M-J Guerry, N Amudala, R Smith, P Sivasothy, L Guillevin, P A Merkel, D R W Jayne
BACKGROUND: Conventional treatment of eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss) with glucocorticoids, with or without additional immunosuppressive drugs, is limited by partial efficacy, frequent toxicity and a high relapse rate. Rituximab is a licensed treatment for granulomatosis with polyangiitis and microscopic polyangiitis and is of potential benefit to patients with EGPA. METHODS: Patients with EGPA who received rituximab as single or repeated courses were identified from four vasculitis centres...
February 2016: Annals of the Rheumatic Diseases
Lindsay Lally, Robert Spiera
This article provides an update on the diagnosis and management of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides, granulomatosis with polyangiitis (formerly Wegener), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss). Focus is on new schemes of classification and the importance of ANCAs in the diagnosis and prognosis of these systemic vasculitides. Current therapeutic strategies consisting of glucocorticoids in conjunction with conventional or biologic agents for both induction of remission and remission maintenance are outlined...
2015: Rheumatic Diseases Clinics of North America
Lindsy Forbess, Serguei Bannykh
Polyarteritis nodosa (PAN) is a systemic disease, but variants are cutaneous PAN and single-organ disease. Histologic confirmation of vasculitis in medium-sized arteries is desirable, and biopsies should be obtained from the symptomatic and least invasive sites. Angiography can show multiple microaneurysms in the viscera. Treatment includes high-dose corticosteroids, which are combined with immunosuppressive agents when internal organs are involved and with life-threatening disease. Once remission is achieved, maintenance agents are initiated...
2015: Rheumatic Diseases Clinics of North America
Ora Singer
Cogan and Behcet syndromes are considered large vessel vasculitides. Both are rare diseases, with varied clinical manifestations affecting multiple organ systems. Although both have hallmark symptoms (ocular and vestibuloauditory inflammation in Cogan syndrome and aphthous ulcers in Behcet syndrome), neither has confirmatory diagnostic testing. Delayed diagnosis can result in poor outcomes. In both syndromes, large vessel arterial inflammation may result in severe morbidity and mortality. Treatment strategies in both syndromes vary based on organ system involvement and severity of manifestations...
2015: Rheumatic Diseases Clinics of North America
(no author information available yet)
No abstract text is available yet for this article.
January 2015: Nature Reviews. Rheumatology
Olga Vera-Lastra, Jesús Sepúlveda-Delgado, María del Pilar Cruz-Domínguez, Gabriela Medina, Moisés Casarrubias-Ramírez, Luis E Molina-Carrión, Luis F Pineda-Galindo, Arturo Olvera-Acevedo, Claudia Hernández-Gonzalez, Luis J Jara
The objectives of this study are to compare the initial clinical, laboratory, and imaging features in primary central nervous system vasculitis (PCNSV) vs secondary central nervous system vasculitis (SCNSV) and follow up after treatment with intravenous cyclophosphamide (IV-CYC) plus glucocorticosteroids (GCS): methylprednisolone (MP). Neurological, laboratory, and neuroimaging findings were analyzed in PCNSV and SCNSV patients. Cerebral biopsy (CB) was performed in nine patients. Both groups received at onset MP plus IV-CYC for 6 months, followed by bimonthly IV-CYC plus prednisone (PND) for 12 months...
April 2015: Clinical Rheumatology
Ben Mulhearn, Ian N Bruce
The use of IVIG to treat a wide variety of immune-driven diseases has grown rapidly, although the mechanism of action is not completely understood. Increasing demand for IVIG coupled with concerns regarding potential transmissible agents has led to worldwide supply shortages. National agencies have therefore produced guidelines for its use, with the latest England and Wales guideline being published in 2011. Due to the rarity of the rheumatic diseases, the evidence for IVIG use has been shown to be lacking in some areas and promising in others...
March 2015: Rheumatology
Pia Isomäki, Ilkka Junttila, Krista-Liisa Vidqvist, Markku Korpela, Olli Silvennoinen
OBJECTIVE: Many cytokines involved in RA activate the Janus kinase-signal transducer and activator of transcription (JAK-STAT) pathways. Therapeutic drugs that inhibit these pathways are being developed for RA. To investigate disease-related alterations in the activity of JAK-STAT pathways in RA, we studied the expression and activation of STAT1 and STAT3 in unstimulated and cytokine-stimulated cells and determined the levels of circulating cytokines. METHODS: The expression of STAT1 and STAT3 mRNA in peripheral blood (PB) and SF T cells and monocytes was studied in RA patients and healthy volunteers by RT-PCR...
June 2015: Rheumatology
Josef S Smolen, Robert Landewé, Ferdinand C Breedveld, Maya Buch, Gerd Burmester, Maxime Dougados, Paul Emery, Cécile Gaujoux-Viala, Laure Gossec, Jackie Nam, Sofia Ramiro, Kevin Winthrop, Maarten de Wit, Daniel Aletaha, Neil Betteridge, Johannes W J Bijlsma, Maarten Boers, Frank Buttgereit, Bernard Combe, Maurizio Cutolo, Nemanja Damjanov, Johanna M W Hazes, Marios Kouloumas, Tore K Kvien, Xavier Mariette, Karel Pavelka, Piet L C M van Riel, Andrea Rubbert-Roth, Marieke Scholte-Voshaar, David L Scott, Tuulikki Sokka-Isler, John B Wong, Désirée van der Heijde
In this article, the 2010 European League against Rheumatism (EULAR) recommendations for the management of rheumatoid arthritis (RA) with synthetic and biological disease-modifying antirheumatic drugs (sDMARDs and bDMARDs, respectively) have been updated. The 2013 update has been developed by an international task force, which based its decisions mostly on evidence from three systematic literature reviews (one each on sDMARDs, including glucocorticoids, bDMARDs and safety aspects of DMARD therapy); treatment strategies were also covered by the searches...
March 2014: Annals of the Rheumatic Diseases
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