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Marie-Nathalie Kolopp-Sarda, Pierre Miossec
Cryoglobulins are immunoglobulins precipitating in cold condition. They are classified in 3 types according to the Brouet classification and may lead to vasculitis of small and medium size vessels. Vasculitis is related to vessel obstruction by monoclonal cryoglobulin aggregates in type I cryoglobulins and immune complex deposition in type II and III mixed cryoglobulins. This phenomenon is favored by low temperature, especially in skin, joints, and peripheral nerves, or increased cryoglobulin concentration in kidneys...
March 8, 2018: Autoimmunity Reviews
Omer Karadag, David J Jayne
Polyarteritis nodosa (PAN) is a rare form of primary systemic vasculitis with heterogeneous presentations, treatments and disease course. Historical approaches to classification and diagnostic terminology are reviewed. Since differentiation of PAN from microscopic polyangiitis (MPA) and other ANCA vasculitides by the Chapel Hill conference statements, and with hepatitis associated PAN defined as a secondary vasculitis, the phenotyping and subclassification of PAN has received little attention. Monogenic disorders similar to PAN have been described (familial Mediterranean fever, Adenosine Deaminase-2 deficiency), and cutaneous PAN and single organ vasculitis, discussed...
February 20, 2018: Clinical and Experimental Rheumatology
W Ennis James, Efstratios Koutroumpakis, Biplab Saha, Alireza Nathani, Leahruth Saavedra, Recai M Yucel, Marc A Judson
BACKGROUND: Compared to pulmonary sarcoidosis, sarcoidosis without lung involvement may involve other immunopathologic mechanisms and be associated with other demographic and clinical features. METHODS: This was a retrospective analysis of clinical data collected in real time on 1,686 patients with biopsy-proven sarcoidosis from two large university sarcoidosis outpatient clinics in the United States. We compared differences in demographics characteristics and clinical presentation between pulmonary and non-pulmonary sarcoidosis (NPS)...
February 14, 2018: Chest
Christian Dejaco, Elisabeth Brouwer, Justin C Mason, Frank Buttgereit, Eric L Matteson, Bhaskar Dasgupta
The fields of giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) have advanced rapidly, resulting in a new understanding of these diseases. Fast-track strategies and improved awareness programmes that prevent irreversible sight loss through early diagnosis and treatment are a notable advance. Ultrasonography and other imaging techniques have been introduced into routine clinical practice and there have been promising reports on the efficacy of biologic agents, particularly IL-6 antagonists such as tocilizumab, in treating these conditions...
October 2017: Nature Reviews. Rheumatology
Hasan Yazici, Emire Seyahi, Gulen Hatemi, Yusuf Yazici
The presence of symptom clusters, regional differences in disease expression and similarities with, for example, Crohn's disease suggest multiple pathological pathways are involved in Behçet syndrome. These features also make formulating disease criteria difficult. Genetic studies have identified HLA-B*51 to be the important genetic risk factor. However, the low prevalence of HLA-B*51 in many patients with bone fide disease, especially in non-endemic regions, suggests other factors must also be operative in Behçet syndrome...
January 3, 2018: Nature Reviews. Rheumatology
Athanase D Protogerou, Efthimia G Nasothimiou, Petros P Sfikakis, Athanasios G Tzioufas
Vascular inflammation in small to large veins and arteries contributes substantially to mortality above that of the general population in Behçet's disease. Recent data verified also the presence of accelerated classical subclinical arterial damage (atheromatosis, arteriosclerosis, arterial hypertrophy) even in patients free of overt vascular complications, and may be complementary to that of vasculitis. Early detection of such vascular damage might provide helpful pathophysiological insight and potentially even guide treatment management...
November 2017: Clinical and Experimental Rheumatology
Federica Maritati, Roberta Fenoglio, Evangeline Pillebout, Giacomo Emmi, Maria L Urban, Rossana Rocco, Maria Nicastro, Monia Incerti, Matteo Goldoni, Giorgio Trivioli, Elena Silvestri, Aladdin J Mohammad, David Jayne, Per Eriksson, Mårten Segelmark, Pavel Novikov, Helen Harris, Dario Roccatello, Augusto Vaglio
OBJECTIVE: Adult-onset IgA vasculitis (Henoch-Schönlein) (IgAV) is a rare systemic vasculitis characterized by IgA1-dominant deposits. The treatment of adult-onset IgAV is controversial and is based on the combination of glucocorticoids and immunosuppressive agents, but many patients have refractory or relapsing disease despite treatment. Rituximab (RTX) is a B cell-depleting antibody of proven efficacy in antineutrophil cytoplasmic antibody-associated vasculitis. We undertook this study to test the efficacy and safety of RTX in a multicenter cohort of patients with adult-onset IgAV...
January 2018: Arthritis & Rheumatology
Cord H Sunderkötter, Bernhard Zelger, Ko-Ron Chen, Luis Requena, Warren Piette, J Andrew Carlson, Jan Dutz, Peter Lamprecht, Alfred Mahr, Elisabeth Aberer, Victoria P Werth, David A Wetter, Seiji Kawana, Raashid Luqmani, Camille Frances, Joseph Jorizzo, J Richard Watts, Dieter Metze, Marzia Caproni, Erkan Alpsoy, Jeffrey P Callen, David Fiorentino, Peter A Merkel, Ronald J Falk, J Charles Jennette
OBJECTIVE: The objective of this project was to prepare a dermatologic addendum to the 2012 Chapel Hill consensus conference nomenclature to address vasculitides affecting the skin. The goal was to standardize names and definitions for cutaneous vasculitis. METHODS: A nominal group technique with a facilitator was used to reach a consensus using multiple face-to-face meetings, e-mail discussions, and teleconferences. RESULTS: Standardized names, definitions and descriptions are provided for cutaneous components of systemic vasculitides (e...
November 14, 2017: Arthritis & Rheumatology
Jack Kingdon, Joseph Roscamp, Shirish Sangle, David D'Cruz
Relapsing polychondritis (RPC) is a rare autoimmune rheumatic disorder that is traditionally classified as a systemic vasculitis. It is characterized by inflammation of cartilage, and typical presenting features include chondritis of the nasal bridge, auricular chondritis, ocular inflammation and involvement of the bronchial tree. Its rarity often leads to considerable delay in establishing a diagnosis and poses a significant management challenge to clinicians, as no conventional guidelines exist. This review summarizes the clinical features of RPC and provides guidance for rheumatologists on making the diagnosis and assessing organ involvement...
November 6, 2017: Rheumatology
Sara Monti, Alberto Floris, Cristina Ponte, Wolfgang A Schmidt, Andreas P Diamantopoulos, Claudio Pereira, Jennifer Piper, Raashid Luqmani
Colour duplex sonography (CDS) of temporal arteries and large vessels is an emerging diagnostic tool for GCA. CDS can detect wall oedema, known as a halo, throughout the length of the vessel and shows higher sensitivity compared with biopsy. Specificity reaches 100% in case of bilateral halos. A positive compression sign has been demonstrated to be a robust marker with excellent inter-observer agreement. The assessment of other large vessels, particularly the axillary arteries, is recognized to further increase the sensitivity and to reliably represent extra-cranial involvement in other areas...
February 1, 2018: Rheumatology
Haner Direskeneli
Takayasu's arteritis (TAK) is a rare, chronic, large-vessel vasculitis (LVV) that predominantly affects aorta, its major branches and the pulmonary arteries. Recent controversial issues in the diagnosis, disease assessment and prognosis in TAK are discussed in this review. In recent years, conventional angiography, the standard method for the initial diagnosis, seems to have been replaced by the new imaging modalities, such as MRI and 18F-FDG-PET. Less invasive techniques (CT/MRI) are now suggested first, compared to conventional angiography, and MRI is preferable to CT with less contrast load/radiation...
March 2017: Clinical and Experimental Rheumatology
Giuseppe Germanò, Sara Monti, Cristina Ponte, Niccolo' Possemato, Roberto Caporali, Carlo Salvarani, Pierluigi Macchioni, Nicolò Pipitone
Large-vessel vasculitides comprise giant cell arteritis and Takayasu's arteritis. In both conditions, early changes consist of transmural inflammation of the arterial wall, while later complications include lumen changes, such as stenoses or aneurysms. Colour Doppler sonography has the ability to depict the arterial wall as well as the lumen, and is therefore ideally suited both to diagnose early vasculitis and to monitor patients over time. In this review article, we addressed the following issues: 1) the role of colour Doppler sonography in the diagnosis of large-vessel vasculitis and its common pitfalls; 2) whether colour Doppler sonography can increase the yield of temporal artery biopsy in giant cell arteritis; 3) the role of colour Doppler sonography in monitoring patients with LVV over time; and 4) how colour Doppler sonography performs compared to other imaging techniques...
March 2017: Clinical and Experimental Rheumatology
Massimo Galli, Letizia Oreni, Francesco Saccardo, Laura Castelnovo, Davide Filippini, Piero Marson, Maria Teresa Mascia, Cesare Mazzaro, Laura Origgi, Elena Ossi, Maurizio Pietrogrande, Piero Pioltelli, Luca Quartuccio, Salvatore Scarpato, Salvatore Sollima, Agostino Riva, Paolo Fraticelli, Roberta Zani, Dilia Giuggioli, Marco Sebastiani, Piercarlo Sarzi Puttini, Armando Gabrielli, Anna Linda Zignego, Patrizia Scaini, Clodoveo Ferri, Salvatore De Vita, Giuseppe Monti
OBJECTIVES: To investigate the clinical and laboratory patterns of HCV-unrelated cryoglobulinaemic vasculitis (CV), and the factors influencing its outcome. METHODS: Prospective study of all anti-HCV and HCV-RNA negative patients with CV who have been observed since January 2004 in 17 centres participating in the Italian Group for the Study of Cryoglobulinaemias (GISC). RESULTS: 175 enrolled were followed up for 677 person-years. The associated conditions were primary Sjögren's syndrome (21...
March 2017: Clinical and Experimental Rheumatology
Francesco Ciccia, Aroldo Rizzo, Angelo Ferrante, Giuliana Guggino, Stefania Croci, Alberto Cavazza, Carlo Salvarani, Giovanni Triolo
Giant cell arteritis (GCA) is an inflammatory chronic disease occurring exclusively in elderly individuals. Until recently, the disease has been considered a unique disease resulting from the interaction in the walls of susceptible arteries, between an unknown infectious agents with local dendritic cells (DCs), activated CD4 T cells and effector macrophages. Recent evidence has shown that this view was too simplistic and has clarified many of the pathogenetic aspects of the disease. Many genetic studies recently published have identified different new genes, including cytokines, adhesion molecules and regulators of innate immunity, as crucial players in the development and progression of GCA...
July 2017: Autoimmunity Reviews
Dario Roccatello
This Special Issue of Autoimmunity Reviews constitutes summaries of presentations at the 20th International Meeting on Immunopathology and Orphan Diseases, held in Torino, Italy, 25-28th January 2017. As such, these presentations represent the state of the art on the pathophysiology of autoimmune diseases as well as the most recent insights into the management of these pathologic conditions. The latter includes both the optimal use of established drugs and approaches as well as novel knowledge on the means and consequences of targeted blocking of molecules or cellular mechanisms...
October 2017: Autoimmunity Reviews
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...
January 2017: Current Opinion in Rheumatology
John H Stone, Katie Tuckwell, Sophie Dimonaco, Micki Klearman, Martin Aringer, Daniel Blockmans, Elisabeth Brouwer, Maria C Cid, Bhaskar Dasgupta, Juergen Rech, Carlo Salvarani, Georg Schett, Hendrik Schulze-Koops, Robert Spiera, Sebastian H Unizony, Neil Collinson
BACKGROUND: Giant-cell arteritis commonly relapses when glucocorticoids are tapered, and the prolonged use of glucocorticoids is associated with side effects. The effect of the interleukin-6 receptor alpha inhibitor tocilizumab on the rates of relapse during glucocorticoid tapering was studied in patients with giant-cell arteritis. METHODS: In this 1-year trial, we randomly assigned 251 patients, in a 2:1:1:1 ratio, to receive subcutaneous tocilizumab (at a dose of 162 mg) weekly or every other week, combined with a 26-week prednisone taper, or placebo combined with a prednisone taper over a period of either 26 weeks or 52 weeks...
July 27, 2017: New England Journal of Medicine
Alessandra Soriano, Francesco Muratore, Nicolò Pipitone, Luigi Boiardi, Luca Cimino, Carlo Salvarani
Giant cell arteritis (GCA) is the most common form of vasculitis in individuals aged 50 years and over. GCA typically affects large and medium-sized arteries, with a predilection for the extracranial branches of the carotid artery. Patients with GCA usually present with symptoms and signs that are directly related to the artery that is affected, with or without constitutional manifestations. The most dreaded complication of GCA is visual loss, which affects about one in six patients and is typically caused by arteritis of the ophthalmic branches of the internal carotid artery...
August 2017: Nature Reviews. Rheumatology
Eric L Matteson, Christian Dejaco
This issue provides a clinical overview of polymyalgia rheumatica, focusing on risk factors, diagnosis, treatment, and practice improvement. The content of In the Clinic is drawn from the clinical information and education resources of the American College of Physicians (ACP), including MKSAP (Medical Knowledge and Self-Assessment Program). Annals of Internal Medicine editors develop In the Clinic in collaboration with the ACP's Medical Education and Publishing divisions and with the assistance of additional science writers and physician writers...
May 2, 2017: Annals of Internal Medicine
Alexandra Audemard-Verger, Benjamin Terrier, Agnès Dechartres, Johan Chanal, Zahir Amoura, Noémie Le Gouellec, Patrice Cacoub, Noémie Jourde-Chiche, Geoffrey Urbanski, Jean-François Augusto, Guillaume Moulis, Loic Raffray, Alban Deroux, Aurélie Hummel, Bertrand Lioger, Mélanie Catroux, Stanislas Faguer, Julie Goutte, Nihal Martis, François Maurier, Etienne Rivière, Sébastien Sanges, Aurélie Baldolli, Nathalie Costedoat-Chalumeau, Mélanie Roriz, Xavier Puéchal, Marc André, Christian Lavigne, Boris Bienvenu, Arsène Mekinian, Elie Zagdoun, Charlotte Girard, Alice Bérezné, Loïc Guillevin, Eric Thervet, Evangéline Pillebout
OBJECTIVE: Data on adult IgA vasculitis (Henoch-Schönlein) (IgAV) are scarce. This survey was designed to better define the clinical spectrum of IgAV and efficacy of treatments in a French patient population. METHODS: Data on clinical characteristics, histologic features, and treatment response from 260 patients with IgAV included in a French multicenter retrospective survey were analyzed. Efficacy data were compared using different statistical models. RESULTS: The mean ± SD age of the patients with IgAV at diagnosis was 50...
September 2017: Arthritis & Rheumatology
2017-06-16 16:25:49
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