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Unexplained Fever Associated With Cutaneous Manifestations
Published in Benedict Isaac, Serge Kernbaum, Michael Burke, Unexplained Fever, 2019
Fever may be found in the following conditions: Behcet’s syndrome is a chronic disorder, characterized by recurrent ulcerations of the mouth and genitalia, associated with uveitis and follicular or nonfollicular pustules on the genitalia and trunk; fever may be moderate or severe during periods of activity.Melkerson-Rosenthal syndrome is a recurrent and chronic condition manifested by a diffuse swelling of the upper lip, facial palsy, and scrotal tongue (the latter may precede attacks of edema); mild fever may accompany episodes of the cutaneous lesions.
High-Dose Chemotherapy with Haematopoietic Stem Cell Transplantation in Primary Systemic Vasculitis, Behcet’s Disease and Sjogren’s Syndrome
Published in Richard K. Burt, Alberto M. Marmont, Stem Cell Therapy for Autoimmune Disease, 2019
Christoph Fiehn, Manfred Hensel
The international experience with HSCT in autoimmune diseases was presented at an international meeting in October 2000 in Basel, Switzerland and is described in the present book. At the Basel meeting, data on 390 patients from all over the world were reported. This combined international data base included 260 patients from the EBMT/EULAR Basel European/Asian database, 87 from North America (55 from the IBMTR), 39 from Australia and 4 others.1 Experience with PSV remains anecdotal at present. Within this database, 9 cases of systemic vasculitis including Behcet’s syndrome were reported. In 3 cases of Wegener’s granulomatosis, initially complete remissions were observed. Two of them relapsed at 2 and 3 years, respectively. In two of three patients with cryoglobulinemia, with variable vasculitic features, complete responses were reported (Table 5). Three patients with Behcet’s syndrome were treated by HSCT, two of them in our institution. In one case there was a relapse following autologous HSCT, and this patient then received an allogeneic HSCT from her HLA-identical brother.34a For Sjogren’s syndrome, only one patient was reported. However, there are several reports of HSCT in patients with Sjogren’s syndrome and consecutive lymphoma. Unfortunately, in these reports, mainly no response or only short term response of the autoimmune disease, in contrast to the lymphoma, was described.35,36 However, one paper reports a lasting remission of nonerosive polyarthritis in Sjogren’s syndrome after HSCT for lymphoma.37
Clinical Diagnosis of Pulmonary Hemorrhage
Published in Philip T. Cagle, Timothy C. Allen, Mary Beth Beasley, Diagnostic Pulmonary Pathology, 2008
Behcet’s syndrome is a chronic relapsing multisystem illness characterized by oral and genital ulceration, cutaneous vasculitis, arthritis, glomerulonephritis, and meningoencepalitis. Pulmonary hemorrhage can result from small-vessel vasculitis, large-vessel vasculitis with arterial aneurysms that can erode into bronchi and pulmonary artery occlusion associated with pulmonary infarction.
Myocardial strain: speckle tracking or early disease tracking?
Published in Acta Cardiologica, 2022
Subclinical cardiac dysfunction is frequently detected in systemic inflammatory diseases involving many organs, including Behçet’s syndrome. In addition to the effect of systemic inflammation on the vascular bed, direct endothelial damage of vasculitides is also considered to contribute to circulatory system damage. Obviously, by using more sensitive methods, such as perfusion cardiac magnetic resonance, these changes can be more readily detected [2]. On the other hand, there are 3 important questions that need to be answered in this type of disease. 1) Are subclinical changes progressive and do they affect the disease prognosis? 2) What are the findings that warrant close monitoring of cardiac involvement and 3) How often and by which tools should the cardiac function be evaluated if these are “at-risk” patients? Although increased cardiovascular mortality due to ischaemic heart diseases is a well-known complication in some systemic inflammatory diseases such as rheumatoid arthritis and systemic lupus erythematosus, the prognostic impact of subclinical cardiac dysfunction detected in many diseases including Behçet's syndrome is largely unknown [3]. We can reach out to the answers to these questions only with long-term follow-up studies. From this perspective, early identification of high-risk groups such as patients with Neuro Behcet syndrome may enable us to reach these answers in a more focussed way with less resource needs.
Proprotein convertase subtilisin/kexin type 9 is associated with atherosclerosis in patients with Behcet’s disease
Published in Clinical and Experimental Hypertension, 2022
Rabia Aydogan Baykara, Pinar Diydem Yilmaz, Mevlüt Hakan Göktepe, Cengiz Kadiyoran, Mustafa Ogul, Adem Kucuk, Medine Cumhur Cüre, Erkan Cüre
The study included 58 patients who applied to the Turgut Özal University, Faculty of Medicine, Physical Medicine and Rehabilitation and Rheumatology outpatient clinic and met the criteria of Behcet’s Syndrome International Study Group (20). These patients and 58 age- and sex-matched healthy volunteers were recruited as the control group. Patients with known cardiovascular disease (coronary artery disease, hypertension, valvular disease, and heart failure), chronic metabolic and endocrine diseases (diabetes mellitus, hyperlipidemia, and chronic kidney disease), neurological diseases (cerebrovascular disease, demyelinating diseases), other rheumatological and autoimmune diseases, malignancy, substance abuse, chronic steroid use, pregnancy, patients under the age of 18 were excluded from the study. Patients signed the informed consent form prepared under the Declaration of Helsinki. The study submitted to Turgut Özal University, Clinical Research Ethics Committee (Approval No: 2021/75, 30.09.2021) was accepted with protocol number.
Investigation of dissolved cellulose in development of buccal discs for oromucosal drug delivery
Published in Pharmaceutical Development and Technology, 2018
Emrah Yildir, Erica Sjöholm, Maren Preis, Poonam Trivedi, Jani Trygg, Pedro Fardim, Niklas Sandler
Inflammatory, atrophic, and ulcerative conditions such as oral lichen planus, aphthous stomatitis, erythema multiforme, and Behcet’s syndrome appear at the oral cavity. Among these conditions, recurrent aphthous stomatitis (RAS) is one of the most common painful oral mucosal conditions seen in the oromucosal area (Preeti et al. 2011; Belenguer-Guallar et al. 2014). RAS can occur as single or multiple shallow painful ulcers on buccal, labial, and tongue mucosa and can be caused by microbial, nutritional, immunological, genetic factors, and psychosocial stress (Akintoye and Greenberg 2014). Currently, many different types of drugs have been tried for the treatment topically and systematically. Some of the anti-inflammatory agents used in topical therapies are glucocorticoids (triamcinolone acetonide, fluocinonide, etc.), antimicrobials (chlorhexidine gluconate), and anti-TNF-alpha agents (pentoxifylline, thalidomide, colchicine, etc.) (Ship 1996; Scully and Porter 2008). Among these substances, systemically or topically applied corticosteroids such as triamcinolone acetonide have been studied to reduce and heal the ulcers sustainably (Zegarelli et al. 1960; Browne et al. 1968; Miles et al. 1993; Ahn et al. 2002; Ghalayani et al. 2017). In addition to symptom alleviating substances, drug compounds with local anesthetic effects such as articaine, lidocaine hydrochloride or benzydamine hydrochloride have been studied and used to relieve oral mucosal pains (Ship et al. 1960; Epstein et al. 2001; Malamed et al. 2001; Wolf and Otto 2015).