241 results on '"Behcet Syndrome"'
Search Results
2. [Treatment-resistant oral ulcers in a 55-year-old man].
- Author
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Braun M, Ständer S, Vorobyev A, and Shimanovich I
- Subjects
- Male, Humans, Middle Aged, Oral Ulcer drug therapy, Behcet Syndrome
- Published
- 2024
- Full Text
- View/download PDF
3. [Behçet's disease]
- Author
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Alfred, Mahr
- Subjects
Behcet Syndrome ,Humans - Abstract
Behçet's disease
- Published
- 2022
4. [Inflammatory rheumatic diseases in migrants].
- Author
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Kötter I and Krusche M
- Subjects
- Humans, Rheumatic Fever complications, Transients and Migrants, Spondylarthritis complications, Arthritis, Rheumatoid complications, Familial Mediterranean Fever complications, Behcet Syndrome diagnosis
- Abstract
The immigration of people from other countries or even from other continents represents new challenges even for rheumatologists. Although all inflammatory rheumatic diseases that occur in this country also exist in the countries of origin of immigrants, the frequencies are different. Diseases such as familial Mediterranean fever (FMF) or Behçet syndrome (BS) are rare in western Europe but are often more frequent than rheumatoid arthritis (RA) and spondylarthritis (SPA) in North Africa and Mediterranean countries. Furthermore, FMF is associated with the occurrence of spondyloarthritis, which is then frequently negative for the human leucocyte antigen B27 (HLA-B27). There is also an association with BS. Rheumatic fever also still occurs relatively frequently especially in African countries, whereas it is almost eradicated in Europe. Possible differential diagnoses such as rheumatic symptoms in genetically determined anemia or infections such as human immunodeficiency virus (HIV) infections, hepatitis, tuberculosis and parasitosis need to be considered as they are all much more frequent in the countries of origin of immigrants than in northwestern Europe. Last but not least, the treatment situation with modern diagnostic and treatment modalities is different in the countries of origin of the migrants, either because these possibilities are unavailable due to limited resources or because the situation has dramatically deteriorated due to acute events, such as the recent war in Ukraine., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)
- Published
- 2023
- Full Text
- View/download PDF
5. Vaskulitis-Mimics
- Author
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Kötter, I. and Reinhold-Keller, E.
- Published
- 2019
- Full Text
- View/download PDF
6. [Behçet's syndrome : Current diagnostics and treatment]
- Author
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I, Kötter
- Subjects
Diagnosis, Differential ,Vasculitis ,Arthritis ,Behcet Syndrome ,Humans - Abstract
Behçet's syndrome (BS) in our latitude is a rare vasculitis of various vessels. It mainly consists of oral and genital aphthous ulcers, skin lesions, oligoarthritis and ocular manifestations, mostly a posterior uveitis with occlusive retinal vasculitis. This review article summarizes the diagnostic and classification criteria as well as possible differential diagnoses and current treatment recommendations.
- Published
- 2020
7. [Behçet's Syndrome]
- Author
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Theodoros, Xenitidis and Joerg Christoph, Henes
- Subjects
Behcet Syndrome ,Humans - Abstract
Behçet's syndrome (BS) is classified as a variable vessel vasculitis. The clinical picture is very diverse and usually requires interdisciplinary collaboration. Pathogenetically, BS seems to take a middle position between a polygenic autoinflammatory disease and an autoimmune disease. New EULAR recommendations were issued in 2018. The therapy depends on which organs are most affected. Since 2016, adalimumab has been approved for the treatment of posterior ocular involvement. Infliximab, interferon a2a, interleukin-1 antagonists and apremilast may be alternative therapies.Das Behçet-Syndrom gehört zu den Vaskulitiden variabler Gefäßgröße. Das klinische Bild ist sehr vielfältig und erfordert in der Regel eine interdisziplinäre Zusammenarbeit. Pathogenetisch scheint es eine Mittelstellung zwischen einer polygenetischen Autoinflammationserkrankung und einer autoimmunen Erkrankung einzunehmen. 2018 wurden neue EULAR-Empfehlungen herausgegeben. Die Therapie richtet sich nach der führenden Beteiligung der einzelnen Organe. Seit 2016 ist Adalimumab zugelassen für die Behandlung der hinteren Augenbeteiligung. Infliximab, Interferon a2a, Interleukin-1-Antagonisten und Apremilast können Therapiealternativen darstellen.
- Published
- 2020
8. [Therapy of Peripheral Artery Aneurysms]
- Author
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Georg, Jung, Maria-Elisabeth, Leinweber, Daphne, Gray, Wojciech, Derwich, Marc, Harth, and Thomas, Schmitz-Rixen
- Subjects
Blood Vessel Prosthesis Implantation ,Behcet Syndrome ,Angiography, Digital Subtraction ,Humans ,Popliteal Artery ,Arteries ,Aneurysm - Abstract
Peripheral arterial aneurysms are rare vascular pathologies characterised by focal dilatation of at least 1.5-fold relative to the diameter of the native vessel. Primary arterial aneurysms can affect any segment of the arterial trunk. Diagnostic tests include duplex sonography, CT and MR angiography, and digital subtraction angiography. It is essential that the influx and efflux to the aneurysm should be mapped as precisely as possible. The most common type of peripheral arterial aneurysms are popliteal artery aneurysms, which frequently occur with aorta aneurysms. Peripheral arterial aneurysms are more common in patients with connective tissue disease or autoimmune disease (Ehlers-Danlos syndrome, Marfan syndrome, Behçet's disease). The current gold standard for therapy is surgery, although endovascular therapies have also been described. As the condition is rare, there is insufficient evidence to compare these approaches. Results from several register studies and single centre studies in patients with popliteal aneurysms indicate that endovascular therapy can give similar outcomes to open therapy if patients are carefully selected. Nevertheless, open surgery remains the gold standard. Therapy of aneurysms of the extremities must consider individual treatment criteria, such as the morphology of the aneurysm, its anatomical localisation - particularly with respect to mobilise segments, vascular efflux and the patient's general condition.Periphere arterielle Aneurysmen sind seltene Gefäßerkrankungen, bei denen das betroffene Gefäßsegment auf mindestens das 1,5-Fache des nativen Gefäßdurchmessers dilatiert ist. Grundsätzlich können primäre Aneurysmen an sämtlichen Gefäßen auftreten. Die diagnostischen Verfahren umfassen neben der Duplexsonografie die CT- und MR-Angiografie sowie die digitale Subtraktionsangiografie. Von essenzieller Bedeutung ist es, im Rahmen der Diagnostik die Morphologie des Aneurysmas und die Zu- und Abstromverhältnisse möglichst exakt abzubilden. Die häufigste Entität peripherer Aneurysmen sind Poplitealarterienaneurysmen, die eine hohe Koinzidenz mit Aortenaneurysmen haben. Im Rahmen von Bindegewebserkrankungen oder Autoimmunerkrankungen können periphere arterielle Aneurysmen gehäuft auftreten (Ehlers-Danlos-Syndrom, Marfan-Syndrom, Morbus Behçet). Therapeutisch stehen als aktueller Therapiestandard offen chirurgische Verfahren zur Verfügung, aber auch endovaskuläre Therapieansätze sind beschrieben. Aktuell bietet die Literatur aufgrund der Seltenheit der Erkrankungen keine gute Evidenz zum Vergleich beider Verfahren. Für das Poplitealarterienaneurysma zeichnet sich aufgrund von mehreren Registerstudien und Singlecenterstudien ab, dass bei strenger Patientenselektion die endovaskuläre Therapie ein vergleichbares Outcome zur offenen Therapie bieten kann, wenngleich offene Chirurgie therapeutischer Standard bleibt. Für die Therapieplanung arterieller Aneurysmen an den Extremitäten müssen insbesondere individuelle Behandlungskriterien wie die Morphologie des Aneurysmas, die anatomische Lokalisation, besonders in Bezug auf bewegliche Segmente, der Gefäßabstrom und der Allgemeinzustand des Patienten berücksichtigt werden.
- Published
- 2019
9. [Diagnostic Strategy and Therapeutic Dilemma in Acute Posterior and Panuveitis]
- Author
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Justus G, Garweg and Juerg, Messerli
- Subjects
Diagnosis, Differential ,Behcet Syndrome ,Panuveitis ,Humans ,Uveitis, Posterior ,Immunosuppressive Agents - Abstract
Acute posterior and panuveitis mostly affect younger patients and affect both eyes in more than half of cases. Because of the severe consequences in the clinical course, rapid and broad differential diagnosis are critical steps. Permanent loss of vision after a delay in starting therapy and the initiation of ineffective treatment are both serious risks. The initial diagnostic classification is based on clinical presentation (anatomical localisation and type of inflammation) and clinical course and, secondarily, on the response to acute therapy. The aetiology is acute in as many as one third of cases. The most frequent acute posterior uveitis in immunocompetent persons is acute viral retinal necrosis. It is difficult to distinguish this clinically from Behçet uveitis, as long as there are no systemic manifestations. In patients with disease threatening the macula, high dose steroid therapy must be started no later than 24 hours after the start of antiviral and anti-parasitic acute therapy. Thus, misdiagnosis has therapeutic consequences. Moreover, the prognosis is favourably affected by aggressive treatment of acute posterior uveitis. Any delay in starting therapy increases infectious and inflammatory tissue damage, and increases the risk of involvement of the other eye and of other organs. On the other hand, the use of high doses of steroids, immunosuppressives and biological agents can lead to uncontrolled proliferation of the pathogen and relapses.Die breite Differenzialdiagnose der akuten posterioren und Panuveitis, die meist jüngere Individuen und in mehr als der Hälfte beide Augen betrifft, macht die rasche diagnostische Einordnung und die therapeutische Entscheidung wegen ihrer erheblichen Konsequenzen für den weiteren Verlauf zu kritischen Schritten. Das Risiko eines bleibenden Visusverlustes infolge eines verzögerten Therapiebeginns ist dabei ebenso bedeutsam wie das Einleiten einer nicht genügend wirksamen Therapie. Klinische Präsentation (anatomische Lokalisation und Entzündungstyp), Verlauf und sekundär das Ansprechen auf die Akuttherapie lassen eine erste diagnostische Eingrenzung zu, wobei eine infektiöse Ätiologie in bis zu einem Drittel der Fälle vorliegt. Die häufigste akute posteriore Uveitis bei immunkompetenten Patienten ist eine akute virale Netzhautnekrose. Diese lässt sich oft klinisch nicht sicher von einer Behçet-Uveitis unterscheiden, solange keine systemischen Manifestationen vorliegen. Bis zur definitiven diagnostischen Einordnung muss deshalb bei makulabedrohender Erkrankung spätestens 24 Stunden nach Beginn einer antiviralen und antiparasitären Akuttherapie eine hochdosierte Steroidtherapie begonnen werden. Den therapeutischen Konsequenzen der Fehleinschätzung der Erkrankung stehen prognostische Überlegungen gegenüber, die ein aggressives Vorgehen gerade bei akuten posterioren Uveitiden rechtfertigen: Jede Verzögerung des Therapiebeginns vermehrt die infektiöse und entzündliche Gewebsschädigung, erhöht das Risiko einer Affektion des Partnerauges und anderer Organe, wohingegen der Einsatz höherer Steroiddosen, Immunsuppressiva und Biologika zu unkontrollierter Erregerproliferation und zu Rezidiven führen kann.
- Published
- 2019
10. [Neonatal Behçet Disease]
- Author
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Katharina, Schuberth, Norbert, Kniess, Matthias, Johann Müller, and Stephan, Seeliger
- Subjects
Pregnancy ,Behcet Syndrome ,Infant, Newborn ,Humans ,Mothers ,Female ,Arteries ,Child ,Infant, Newborn, Diseases ,Autoantibodies - Abstract
Behçet's disease is a multi-system disease with inflammatory infestations, in particular of the small and middle arteries and veins. Histologically, the disease presents with the image of leukocytoclastic vasculitis. The etiology of Behçet disease is unknown, but genetic factors are discussed of playing a role in its pathogenesis. There is no known autoantibody, which could serve as a diagnostic tool. The disease usually affects patients in the 3. life decade and is rare in pediatrics. A very rare clinical form of Behçet's disease occurs during neonatal period. In this form life-threatening courses of the disease have been described.In this report a family with a diagnosed disorder of the mother, where the second child presented with ulcerations in the mucous membrane area during the first week of life, will be discussed. In this case, the question of a transfer of autoantibodies from the mother, which triggers the disease in the child, must be raised. The clinical progress will be compared to known cases in the literature in order to derive recommendations for a therapeutic procedure.In our patient, ANAs were transiently detected, which correlated to the mother's. Fortunately, the course of the disease was mild, so that no further treatment was necessary. But, potential life-threatening events should be always held in consideration. Here, steroids seem to be the treatment of choice.In the wake of the increasing immigration of pregnant Morbus Behçet patients from Mediterranean and from countries with higher prevalence of Morbus Behçet-positive mothers should be considered.Der Morbus Behçet ist eine Multisystemerkrankung mit entzündlichem Befall, v. a. der kleinen und mittleren Arterien und Venen. Histologisch zeigt sich das Bild einer leukozytoklastischen Vaskulitis. Die Ursachen des Morbus Behçet sind unbekannt. Genetische Faktoren werden in der Pathogenese diskutiert. Ein „klassischer“ Autoantikörper als Diagnostiktool ist nicht bekannt. Die Erkrankung betrifft normalerweise Patienten in der dritten Lebensdekade und ist in der Pädiatrie selten. Eine sehr seltene klinische Form des Morbus Behçet tritt bereits in der Neonatalperiode auf. Hier sind bei einer Manifestation lebensbedrohliche Verläufe beschrieben.Vorgestellt werden soll eine Familie mit bekannter Erkrankung der Mutter, bei der bereits das zweite Kind in der ersten Lebenswoche mit Ulzerationen im Schleimhautbereich auffiel. Die Frage einer Übertragung von Autoantikörpern, die dieses beim Kind triggert, muss in diesem Zusammenhang diskutiert werden. Dieser klinische Verlauf wird mit Verläufen aus der Literatur verglichen, um Empfehlungen zum therapeutischen Procedere ableiten zu können.Bei unserem Patienten ließen sich vorübergehend ANAs, die mit den mütterlichen korrelierten, nachweisen. Trotzdem zeigte sich ein milder Verlauf, der keine weiteren Interventionen bedurfte. An mögliche potentielle lebensbedrohliche Symptome sollte aber immer gedacht werden. Hierbei scheinen Steroide das Mittel der Wahl zu sein.Im Zuge der zunehmenden Immigration schwangerer Morbus Behçet-Patientinnen aus dem Mittelmeerraum und aus Ländern mit höherer Prävalenz, wie z. B. auch Japan, nach Deutschland sollte bei der Betreuung von Neugeboren Morbus Behçet-positiver Mütter an die Möglichkeit eines transienten neonatalen Morbus Behçet gedacht werden.
- Published
- 2018
11. [Not Available]
- Author
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Ina, Kötter
- Subjects
Diagnosis, Differential ,Europe ,Evidence-Based Medicine ,Treatment Outcome ,Behcet Syndrome ,Practice Guidelines as Topic ,Anti-Inflammatory Agents ,Humans ,Glucocorticoids ,Immunosuppressive Agents - Abstract
Die Prävalenz bei deutschstämmigen Patienten ist 0,9/100 000, bei türkischstämmigen 77/100 000. HLA-B51 ist mit der Augenbeteiligung assoziiert, Männer haben schwerere Verläufe. Neben HLA-B51 ist auch HLA-A26 mit der Erkrankung assoziiert. Zusätzlich gibt es Assoziationen mit Mutationen im IL12/23-Rezeptor-Gen. Der M. Behçet wird inzwischen als intermediäre, partiell autoinflammatorische Erkrankung betrachtet. In den neuen EULAR-Empfehlungen wird für die schwere Augenbeteiligung Adalimumab gleichrangig mit Infliximab und Interferon-alpha empfohlen. Adalimumab ist für die schweren (nicht anterioren) Uveitiden inklusive der bei M. Behçet zugelassen. Infliximab wird als Primärtherapie bei ZNS-Beteiligung empfohlen. Bei Thrombosen im Rahmen der Erkrankung muss vorrangig die Entzündungsaktivität beherrscht werden, eine Antikoagulation wird nicht mehr bzw. nur noch vorübergehend empfohlen. Apremilast wird aktuell in Studien für die mukokutanen Manifestationen untersucht und wird für diese ebenfalls bereits empfohlen.
- Published
- 2017
12. [Uveitis in Behçet's Syndrome in Germany - Prognosis During Current Therapy].
- Author
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Stübiger N, Deuter C, and Doycheva D
- Subjects
- Germany, Humans, Prognosis, Behcet Syndrome, Retinal Vasculitis, Uveitis
- Abstract
Behçet's syndrome (BS) is an inflammatory systemic disease associated with multiple organ manifestations. Major symptoms include oral and genital ulcerations, skin lesions and uveitis. Diagnosis of Behçet's syndrome is based on clinical findings. A typical for ocular Behçet's syndrome is recurrent non-granulomatous panuveitis with occlusive retinal vasculitis. Treatment used to be based only on the use of systemic corticosteroids and conventional immunosuppressives, but most often this treatment did not appear to be sufficient. In consequence, prognosis of ocular BS was very poor over a long period of time - especially if occlusive retinal vasculitis was present. In recent years, the introduction of biologicals has revolutionised the therapeutic regime and consecutively visual prognosis of ocular Behçet's syndrome has dramatically improved., Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Georg Thieme Verlag KG Stuttgart · New York.)
- Published
- 2020
- Full Text
- View/download PDF
13. [Morbus Behçet or inflammatory bowel disease--a diagnostic and therapeutic dilemma]
- Author
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S, Schack, M, Weber, P, Oelzner, M, Mireskandari, D, Antonov, and A, Stallmach
- Subjects
Adult ,Diagnosis, Differential ,Male ,Treatment Outcome ,Gastrointestinal Agents ,Behcet Syndrome ,Clinical Decision-Making ,Humans ,Symptom Assessment ,Inflammatory Bowel Diseases - Abstract
We present the case of a 43-year old caucasian male suffering from a condition initially diagnosed as colitis ulcerosa. For 2 years Azathioprine and anti-TNF-alpha antibodies were used for treatment without convincing benefit but with serious adverse events. After the first occurrence of complex accompanying symptoms like oral and scrotal ulcerations, arthritis and scratch-induced skin lesions the differential diagnosis of a Morbus Adamantiades-Behçet with intestinal evolvement was considered. After introduction of a parenteral Ciclosporin medication, which was later switched to Tacrolimus and Azathioprin, a remission could be achieved that lasted for several months. When a drug-induced acute kidney injury occurred, the regime was changed to Golimumab and a delayed but significant improvement was achieved. To separate Morbus Adamantiades-Behçet from inflammatory bowel disease is of some difficulty, demands interdisciplinary cooperation and is the basis for a successful therapy.
- Published
- 2015
14. [Systemic Adamantiades-Behçet's disease: adverse effects of an otherwise successful therapy with interferon-α-2a]
- Author
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Ioannis, Karagiannidis and Christos C, Zouboulis
- Subjects
Adult ,Hyperprolactinemia ,Treatment Outcome ,Behcet Syndrome ,Anti-Inflammatory Agents ,Humans ,Interferon-alpha ,Female ,Interferon alpha-2 ,Oral Ulcer ,Mastodynia ,Recombinant Proteins - Abstract
Adamantiades-Behçet's disease is an immune-mediated vasculitis with relapsing course. It is characterised by the classic clinical trias of oral aphthous ulcers, genital ulcers and uveitis.A 37-year-old woman suffered from systemic Adamantiades-Behçet disease with recurrent uveitis, oral ulcers, genital ulcers, arthralgia, erythema nodosum and folliculitis.Longterm interferon-α-2a (IFNα-2a) led to reduction of the clinical manifestations except for occasional occurrence of oral ulcers. One year after initiation of treatment however, the patient developed symptomatic hyperprolactinemia of unknown etiology.Even in otherwise successful treatment with IFNα-2a possible side effects and complications of treatment can affect the course. Mastodynia and hyperprolactinemia have not yet been described as potential side effects of IFNα-2a.
- Published
- 2015
15. Zerebrale Vaskulitis
- Author
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Berlit, P.
- Published
- 2004
- Full Text
- View/download PDF
16. [Behçet disease]
- Author
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Nina, Weber, Dominik, Schaer, and Florence, Vallelian
- Subjects
Adult ,Male ,Hematologic Tests ,Transverse Sinuses ,Behcet Syndrome ,Anti-Inflammatory Agents ,Anticoagulants ,Prognosis ,Diagnosis, Differential ,Sinus Thrombosis, Intracranial ,Humans ,Interdisciplinary Communication ,Cooperative Behavior ,Drug Monitoring ,Tomography, X-Ray Computed ,Immunosuppressive Agents ,Magnetic Resonance Angiography - Published
- 2013
17. [Behcet's disease--ophthalmological and general aspects: Part 2: Therapy]
- Author
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U, Pleyer, D, Hazirolan, S, Winterhalter, and N, Stübiger
- Subjects
Behcet Syndrome ,Anti-Inflammatory Agents ,Humans - Abstract
Behcet's disease (also called Admantiades-Behcet syndrome) is a chronic vasculitis. The disease is characterized by exacerbations and remissions of symptoms and organ manifestations and may produce only mild mucocutaneous lesions, whereas ocular lesions can cause blindness. In addition, involvement of the gastrointestinal tract, central nervous system (CNS) and large blood vessels is sometimes life-threatening. Cyclosporin A is the only agent for treatment of ocular lesions registered in Germany; however, the neurotoxicity and nephrotoxicity restrict usage of the drug. In patients suffering from severe uveitis, biologics have been a breakthrough. Interferon (IFN) alpha therapy has shown significant efficacy for intraocular inflammation. Monoclonal antibodies to TNF-alpha and interleukin-1 have been successful in clinical trials and are approved in some countries. This article summarizes the current state of knowledge and emphasizes the important role of the ophthalmologist in the therapy of Behcet's disease.
- Published
- 2013
18. [Retinal vein occlusion. Diagnosis or symptom?]
- Author
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J, Matlach and W, Göbel
- Subjects
Male ,Retinal Vasculitis ,Treatment Outcome ,Anti-Infective Agents ,Behcet Syndrome ,Retinal Vein Occlusion ,Humans ,Middle Aged - Abstract
Behçet's disease is a chronic recurrent systemic vasculitis and can affect nearly all organ systems. Eye involvement is characterized by uveitis with occlusive retinal vasculitis. Oral aphthous ulcers are the major primary symptom and arthritis, genital ulcerations and skin lesions are common extraocular manifestations. Gastrointestinal and vascular symptoms or manifestations of the central nervous system are less frequently seen. Biologic agents are used alone or in combination with immunosuppressive or cytotoxic drugs to prevent relapse as the prognosis is poor in patients with retinal vasculitis.
- Published
- 2013
19. [Behçet's disease - ophthalmological and general aspects part I : etiology, pathogenesis and diagnostics]
- Author
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U, Pleyer, D, Hazirolan, S, Winterhalter, and N, Stübiger
- Subjects
Behcet Syndrome ,Humans ,Ophthalmologic Surgical Procedures ,Ophthalmic Solutions - Abstract
Behçet's disease (also known as morbus Behcet or Admantiades-Behcet syndrome) is a chronic vasculitis mainly characterized by recurrent mucocutaneous lesions and sight threatening uveitis. It may also involve joints, vessels of all sizes and the central nervous system. Because of its severe morbidity and considerable mortality early diagnosis and treatment is important. Treatment and prognosis of this disorder have profited considerably in recent years following the introduction of biologic agents. This article summarizes the current state of knowledge and emphasizes the important role of the ophthalmologist in the diagnosis and therapy of Behçet's disease.
- Published
- 2012
20. [Behçet's disease]
- Author
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I, Kötter, T, Xenitidis, G, Fierlbeck, S, Schanz, A, Melms, M, Horger, U, Ernemann, and C, Deuter
- Subjects
Behcet Syndrome ,Cyclosporine ,Humans ,Steroids ,Immunosuppressive Agents - Abstract
Behçet's disease is a systemic disorder with the histopathological correlate of leukocytoclastic vasculitis. Pathogenetically, besides a strong genetic component participation of the innate immune system and an autoinflammatory component are discussed. The disease is most common in countries along the former silk route but in Germany the disease is rare (prevalence approximately 0.6/100,000). Oral aphthous ulcers are the main symptom, followed by skin manifestations, genital ulcers and oligoarthritis of large joints. Severe manifestations, threatening quality of life and even life itself, are the gastrointestinal manifestations which often perforate, arterial, mainly pulmonary arterial aneurysms which cause life-threatening bleeding, CNS manifestations and ocular disease, which with occlusive retinal vasculitis often leads to blindness. For milder manifestations low-dose steroids and colchicine are used, for moderate manifestations such as arthritis or ocular disease not immediately threatening visual acuity, azathioprin or cyclosporin A are combined with steroids. For severe manifestations, interferon-alpha, TNF-antagonists or cytotoxic drugs are recommended. Interleukin 1 (IL-1) antagonists are currently being examined in clinical studies.
- Published
- 2012
21. [Extraocular manifestations of Behcet's disease]
- Author
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I, Kötter, T, Xenitidis, G, Fierlbeck, S, Schanz, A, Melms, M, Horger, U, Ernemann, and C, Deuter
- Subjects
Male ,Arthritis ,Behcet Syndrome ,Humans ,Vasculitis, Leukocytoclastic, Cutaneous - Abstract
Behcet's disease is a multisystem disorder with the histopathological correlate of leukocytoclastic vasculitis. The classification criteria for the disease include the presence of recurrent oral aphthous ulcers combined with at least two other manifestations, such as genital aphthous ulcers, skin manifestations (mostly erythema nodosa or pseudofolliculitis) and ocular manifestations (panuveitis or posterior uveitis with retinal vasculitis). A positive pathergy test is regarded as pathognomonic for the disease and a sterile papulopustule occurs after a sterile needle prick of the forearm. However, this test is positive in only 15% of the patients. The prognosis of Behcet's disease becomes unfavorable when vital organs are involved. This is the case for involvement of the central nervous system which occurs in 10% of patients, arterial and pulmonary arterial aneurysms and gastrointestinal involvement, which clinically and histopathologically is difficult to differentiate from inflammatory bowel disease but tends to perforate. Oligoarthritis, which occurs in approximately 50% of the patients, causes problems concerning the differential diagnosis from classical forms of spondyloarthritis. Behcet's disease is associated with HLA-B51 in 50-80% of the cases depending on the country of origin of the patient. The prognosis becomes unfavorable if the disease manifests in young male patients. The treatment of extraocular manifestations depends on the aggressiveness. Milder manifestations are treated with low dose prednisolone and steroid sparing immunosuppressants, such as azathioprine or cyclosporine A. In cases with more severe manifestations, such as central nervous system (CNS) involvement cyclophosphamide or TNF antagonists and in selected cases also interferon alpha can be considered.
- Published
- 2012
22. [Epidemiology of ocular involvement in Adamantiades-Behçets disease]
- Author
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C E, Kneifel, A-K, Köhler, A, Altenburg, C C, Zouboulis, and L, Krause
- Subjects
Male ,Adolescent ,Eye Diseases ,Behcet Syndrome ,Infant, Newborn ,Infant ,Comorbidity ,Young Adult ,Risk Factors ,Child, Preschool ,Germany ,Prevalence ,Humans ,Female ,Child - Abstract
Epidemiological studies demonstrate a prevalence of Adamantiades-Behçets disease (MAB) in the range of 0.12-420 per 100,000 inhabitants with the highest rates in Istanbul, Turkey and the lowest rates in the USA. Ophthalmological data on the prevalence of ocular involvement are limited for MAB in Germany because most epidemiological studies are based on rheumatological or dermatological data. Berlin is the city with the highest number of non-native German inhabitants and its multiethnic character renders it uniquely appropriate for epidemiological studies on MAB. This article summarizes the most important epidemiological data of 140 patients (63 female and 77 male) with a mean follow-up of 6.4 years (range 0.5-22 years) which we have recently published. The mean age was 23 years at the first manifestation and 32 years when the fully developed disease was recorded. The mean age at onset of ocular involvement was 30 years, 56% of patients developed ocular involvement, which was the first manifestation in 8.6% and the second manifestation in 19.3%. More than half the patients developed ocular involvement. The calculated prevalence of ocular involvement in MAB is 1.77/100,000 inhabitants for the population of Berlin.
- Published
- 2012
23. [Treatment of Behçet's disease yesterday and today]
- Author
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C M E, Deuter, M, Zierhut, D, Doycheva, and I, Kötter
- Subjects
Biological Products ,Behcet Syndrome ,Humans ,Blindness ,Immunosuppressive Agents - Abstract
Uveitis due to Behçet's disease belongs to those types of intraocular inflammation with a poor visual prognosis. Despite early treatment with corticosteroids and immunosuppressants, patients often become blind. With the so-called biologicals, highly effective drugs are now available which very rapidly lead to quiescence of intraocular inflammation and which successfully avoid further uveitis relapse. Recently, an interdisciplinary group of experts on behalf of the EULAR developed nine recommendations for the treatment of Behçet's disease. Two of these address ocular involvement. Compared with TNF alpha antagonists, interferon alpha offers the advantage that even after cessation of treatment a high percentage of patients remain in remission. This article provides an overview on current medical treatment of ocular Behçet's disease.
- Published
- 2012
24. [Comments on the pathogenesis of Behçet's disease. A key to understanding new therapies?]
- Author
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U, Pleyer, D, Hazirolan, and N, Stübiger
- Subjects
Behcet Syndrome ,Models, Immunological ,Humans ,Heat-Shock Proteins ,Immunosuppressive Agents - Abstract
The etiology and pathogenesis of Behçet's syndrome remains unclear. It has been postulated that an infectious agent may induce an immune-mediated, generalized vasculitis in genetically predisposed individuals (HLA-B51 +). There is accumulating evidence that an abnormality of the innate and adaptive immune response, e.g. directed against heat shock proteins plays an important role in Behçet's syndrome. This article is intended to evaluate the more recent knowledge on this disorder and evaluate new therapeutic options.
- Published
- 2012
25. [Typical and atypical ocular manifestations of Behçet's disease]
- Author
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N, Stübiger and U, Pleyer
- Subjects
Behcet Syndrome ,Iritis ,Humans ,Macular Edema - Abstract
The first ocular symptom in 50-87% of patients with Behçet's disease (BD) is unilateral anterior uveitis. As a characteristic sign of ocular BD the appearance of hypopyon iritis was originally described by Adamantiades and Behçet, but nowadays, probably due to an earlier and more aggressive treatment, this rarely occurs. In the further course of the disease up to 75% of BD patients develop a bilateral chronic relapsing form of posterior uveitis or panuveitis. In addition occlusive retinal vasculitis, which involves arteries as well as veins, is frequently present. A typical complication consists of a cystoid macular edema, which is, besides the retinal vasculitis, responsible for the limited prognosis of visual acuity.
- Published
- 2012
26. [Epidemiology and clinical aspects of Adamantiades-Behçet disease in Gemany. Current data]
- Author
-
A, Altenburg, A, Mahr, C, Maldini, C E, Kneifel, L, Krause, I, Kötter, T, Stache, N G, Bonitsis, and C C, Zouboulis
- Subjects
Adult ,Aged, 80 and over ,Male ,Adolescent ,Turkey ,Behcet Syndrome ,Middle Aged ,Young Adult ,Risk Factors ,Germany ,Prevalence ,Humans ,Female ,Registries ,Child ,Aged - Abstract
Of the 721 documented patients in the German Registry for Adamantiades-Behçet disease (registered charity), 258 were of German and 308 of Turkish descent, along with 30 other countries of origin. The prevalence in Germany is 0.9:100,000. Manifestation of the disease was predominantly in the third decade of life (median age: 26.5 years) and in 10.7% the disease onset was under 16 years of age. The full clinical picture developed on average in 2.9 years (median 3 months). Patients of Turkish descent showed androtopism in contrast to those of German descent (female:male 1.9:1), which was also detected in the whole collective in a ratio of 1.4:1. In 12.4% there was a family history with differences between German and Turkish patients (3.8% versus 14.6%) as well as in patients with disease onset in young and adult age (25.0% versus 7.3%). Most frequent features included oral aphthae (98.5%), cutaneous lesions (81%), genital ulcers (64.7%), ocular manifestations (51.6%), arthritis (52.4%) and positive pathergy test (30.8%). Turkish patients suffered significantly more often from eye manifestations compared with Germans, while in German patients prostatitis/epididymitis and gastrointestinal involvement were more frequently documented. As serious complications arose blindness 6.8%, meningoencephalitis 4.0%, severe arthritis 2.6%, fatal outcome 1.2%, hemoptysis 1.1% and gastrointestinal perforation 0.5%. The HLA-B5 antigen was positive in 58.1% and showed an association with eye manifestations. The relative risk of HLA-B5 positive individuals is high in both Germans (6.57) and Turks (5.81).
- Published
- 2012
27. [Clinical results after cataract surgery in patients with Behçet's disease]
- Author
-
O, Saygili, P, Szurman, S, Gieselmann, C, Deuter, M S, Spitzer, S, Thaler, K U, Bartz-Schmidt, and E, Yoeruek
- Subjects
Adult ,Lenses, Intraocular ,Male ,Behcet Syndrome ,Prednisolone ,Anti-Inflammatory Agents ,Interferon-alpha ,Cataract Extraction ,Interferon alpha-2 ,Middle Aged ,Perioperative Care ,Recombinant Proteins ,Young Adult ,Postoperative Complications ,Azathioprine ,Humans ,Female ,Immunosuppressive Agents ,Retrospective Studies - Abstract
Behçet's disease is a systemic vasculitis disorder of unknown aetiology. Ocular involvement, especially with vasculitis, is detected in up to 80 % of the cases. Anterior segment involvement such as cataract is also seen in the follow-up of patients who are then treated surgically. In this study, we aimed to analyze the outcomes of cataract surgery in patients with Behçet's disease retrospectively.The records of 9 patients (12 eyes) with Behçet's disease who underwent phacoemulsification with IOL implantation in 11 eyes and extracapsular cataract extraction (ECCE) with IOL implantation in one eye between June 2001 and September 2009 were evaluated retrospectively. The visual outcome and complications were analysed.The mean follow-up was 33.8 months (range 3 to 88 months). The mean preoperative LogMAR BCVA was 1.15 ± 0.53 (95 % CI: 0.81 - 1.49) and increased to 0.36 ± 0.32 (95 % CI: 0.15 - 0.56) at last medical visit (p0.001). The most frequent postoperative complication was posterior capsular opacification, which developed in 2 eyes (17 %). Other complications were mild fibrinous reaction in 1 eye (8 %).The outcomes of cataract surgery in patients with Behçet's disease were satisfactory. The great majority of the patients regained and retained a good visual outcome and had fewer postoperative complications.
- Published
- 2011
28. [Autoinflammatory diseases as cause of wound healing defects]
- Author
-
R, Löhrer, R, Eming, N, Wolfrum, T, Krieg, and S A, Eming
- Subjects
Arthritis, Infectious ,Wound Healing ,Inflammasomes ,Behcet Syndrome ,DNA Mutational Analysis ,Hereditary Autoinflammatory Diseases ,Pyoderma Gangrenosum ,Diagnosis, Differential ,Acne Vulgaris ,Skin Ulcer ,Humans ,Immunologic Factors ,Stomatitis, Aphthous ,Mevalonate Kinase Deficiency ,Immunosuppressive Agents ,Interleukin-1 - Abstract
Ulcerations of the skin and mucosal membranes are a common feature of autoinflammatory diseases. They can give raise to chronic wound healing defects and should be considered in the differential diagnosis of chronic skin ulcers. The increased activation of the innate immune system in the absence of an apparent provocation for inflammation is a hallmark of autoinflammatory diseases. Mutations and alterations of signaling pathways regulating the innate immune response to physical trauma/tissue damage result into an unrestrained activation of the inflammasome, which leads to increased activation of Interleukin-1. Uncontrolled recruitment and activation of myeloid effector cells within the wound site lead to the release of potent proteases that cause the degradation of structural components of the skin. The majority of these diseases respond well to immunosuppressive and immunomodulatory treatment regimes. Therapeutic resistance converts the acute inflammatory response into a chronic and non-resolving inflammatory process that leads to tissue degeneration. In this article we will focus on the review of those autoinflammatory diseases that often display ulcerative cutaneous and aphthous lesions including pyoderma gangrenosum, Behçet disease, PAPA syndrome and hyperimmunoglobulinemia D with periodic fever syndrome (HIDS). Furthermore, the article will be complemented by an overview of those inflammatory diseases that are associated with non-ulcerative cutaneous manifestations.
- Published
- 2011
29. [Arthritis, erythema nodosum and genital ulcerations. Behçet disease]
- Author
-
C, Arnold, S, Revaz, and J, Dudler
- Subjects
Diagnosis, Differential ,Male ,Erythema Nodosum ,Arthritis ,Behcet Syndrome ,Skin Ulcer ,Humans ,Genital Diseases, Male ,Middle Aged - Abstract
Behcet's disease is a disease of unknown etiology resting in between vasculitis, spondyloarthropathy and autoinflammatory diseases. If his predilection for the population originating from the Silk Road is well known, as are its cutaneous, ocular and vascular manifestations, this case illustrates the non-specificity of those manifestations, the diagnostic difficulties and the importance of routinely assessing for eyes and bowel diseases in this type of patient.
- Published
- 2011
30. [Janus-faced?: Effects and side-effects of interferon therapy in ophthalmology]
- Author
-
N, Stübiger, S, Winterhalter, U, Pleyer, D, Doycheva, M, Zierhut, and C, Deuter
- Subjects
Cytotoxicity, Immunologic ,Retinal Vasculitis ,Multiple Sclerosis ,Dose-Response Relationship, Drug ,Eye Diseases ,Behcet Syndrome ,Interferon-alpha ,Interferon-beta ,Lymphocyte Activation ,Macular Edema ,Uveitis ,Antibody Formation ,Animals ,Humans ,Hypersensitivity, Delayed ,Follow-Up Studies - Abstract
Interferon alpha (IFN-α) and interferon beta (IFN-β) are naturally occurring cytokines, which belong to the type I interferons and share the same receptor leading to very similar therapeutic effects. The immunomodulatory effect of type I interferons includes modulation of antibody production, inhibition of lymphocyte proliferation, inhibition of delayed-type hypersensitivity and enhancement of T-cell and NK-cell cytotoxicity. An increasing number of open clinical studies and case reports have demonstrated the efficacy of IFN-α for severe ocular inflammation in patients with Behçet's disease and of interferon-β, which has been used mainly for the treatment of multiple sclerosis.
- Published
- 2011
31. 21-year-old female patient with oral aphthosis and skin lesions
- Author
-
K, Muehlenberg, C, Metzler, and K H, Wiedmann
- Subjects
Diagnosis, Differential ,Young Adult ,Recurrence ,Behcet Syndrome ,Skin Diseases, Papulosquamous ,Humans ,Female ,Stomatitis, Aphthous ,Fluorometholone - Published
- 2011
32. [Management of Behçet's disease : A systematic literature review for the European League Against Rheumatism evidence-based recommendations for the management of Behçet's disease]
- Author
-
I, Kötter
- Subjects
Clinical Trials as Topic ,Evidence-Based Medicine ,Behcet Syndrome ,Humans ,European Union - Published
- 2010
33. [Fever and aphthae--Behçet disease simulating an infection]
- Author
-
Holger, Neye, Peter, Rauh, Christine, Uhle, Daniel, Ensberg, Lizzy, Willert, and Steffen, Rickes
- Subjects
Diagnosis, Differential ,Male ,Young Adult ,C-Reactive Protein ,Behcet Syndrome ,Prednisolone ,Humans ,Stomatitis, Aphthous ,Fever of Unknown Origin ,Referral and Consultation ,Immunosuppressive Agents ,Ultrasonography - Published
- 2009
34. [Chlorambucil treatment of Behçet's syndrome. Retrospective evaluation of two cases]
- Author
-
Andreas, Eichhorn, Martin, Siepmann, and Wilhelm, Kirch
- Subjects
Erythema Nodosum ,Behcet Syndrome ,Humans ,Prednisone ,Chlorambucil ,Retrospective Studies - Abstract
Behçet's syndrome rarely occurs in North America and Central Europe (incidence: 1 : 500,000), whereas it is more frequently seen in Japan and Mediterranean countries (incidence: 1 : 10,000). The diagnosis is based on the detection of symptoms and clinical signs. Orogenital aphthosis, anterior and posterior uveitides that frequently cause loss of vision are considered to be primary symptoms. Dermatologic manifestations, i.e., erythema nodosum, vascular lesions (angio-Behçet's syndrome), gastrointestinal ulcers and neurologic involvement, can be observed. HLA B5 is found in some of the patients with Behçet's syndrome. Administration of chlorambucil, a cytotoxic compound, is an effective form of treatment of symptoms and complications of Behçet's syndrome.The present article describes the course of a female and a male patient who were 39 and 23 years old when Behçet's syndrome was diagnosed for the first time. Treatment with chlorambucil was started in the early 1990s and continued for a period of 9 1/2 and 3(3/4) years, respectively, with the symptoms remitting during and after this therapy. Approximately 10 years after the start of treatment with chlorambucil, the patients' symptoms changed. The female patient who was first diagnosed having Behçet's syndrome at the age of 39 years developed rheumatoid arthritis with joint destruction. Her symptoms could be controlled in the long term by oral administration of prednisone, at doses below the Cushing threshold combined with methotrexate. The male patient who was first diagnosed having Behçet's syndrome at the age of 23 years developed systemic vasculitis remitting during low-dose treatment with prednisone.Immunosuppressive therapy with chlorambucil administered over several years often induces remission of Behçet's syndrome. However, both case reports indicate that symptoms can change from Behçet's syndrome to systemic vasculitis or rheumatoid arthritis.
- Published
- 2009
35. [EULAR recommendations for the management of Behçet's disease. Report of a task force of the European Standing Committee for International Clinical Studies Including Therapeutics (ESCISIT)]
- Author
-
I, Kötter
- Subjects
Europe ,Rheumatology ,Behcet Syndrome ,Practice Guidelines as Topic ,Humans - Abstract
The aim of the EULAR/ESCISIT initiative was to develop evidence-based recommendations for the management of Behçet's disease (BD), supplemented where necessary by expert opinions and taking already published data into consideration. The current article briefly summarises the results in German and comments on them.The multidisciplinary expert committee consisted of nine rheumatologists, three ophthalmologists, one internist, one dermatologist, and one neurologist, representing six European countries, Tunisia and Korea. One patient representative was also present. Problem areas and related keywords for a systematic literature search were identified. A systematic literature research was performed using MedLine and the Cochrane library up to December 2006. Consequently, 40 initial statements were generated based on the literature research. These yielded the final recommendations which resulted from two blind Delphi rounds of voting.Nine recommendations were developed for the management of different aspects of BD. The strength of each recommendation was determined by the level of evidence and the expert's opinions. The level of agreement for each recommendation was determined using a visual analogue scale for both the whole committee and for each individual viewpoint of the subgroup, who considered themselves experts in that field of BD. There was excellent concordance between the level of agreement of the whole group and the "experts in the field".Recommendations relating to eye, skin, and mucosal disease, as well as arthritis are mainly evidence-based, while recommendations on vascular disease and neurological and gastrointestinal involvement are based largely on expert opinions and uncontrolled evidence from open trials and observational studies. The need for further properly designed controlled clinical trials is apparent.
- Published
- 2008
36. [Neuro-Behçet's disease: MR-imaging findings]
- Author
-
M, Horger, O, Maksimovic, I, Kötter, and U, Ernemann
- Subjects
Behcet Syndrome ,Brain ,Demyelinating Autoimmune Diseases, CNS ,Image Enhancement ,Cerebral Veins ,Magnetic Resonance Imaging ,Brain Ischemia ,Diagnosis, Differential ,Diffusion Magnetic Resonance Imaging ,Spinal Cord ,Image Processing, Computer-Assisted ,Humans ,Vasculitis, Central Nervous System ,Magnetic Resonance Angiography - Published
- 2008
37. [Adamantiades-Behçet's disease. Clinical review]
- Author
-
Stefan, Störk, Christian, Kneitz, Eva-B, Bröcker, Caroline, Hoyer, Georg, Ertl, and Christiane E, Angermann
- Subjects
Adult ,Male ,Behcet Syndrome ,Age Factors ,Infant, Newborn ,Middle Aged ,Prognosis ,Thalidomide ,Recurrence ,Prevalence ,Humans ,Female ,Child ,Colchicine ,Immunosuppressive Agents ,Randomized Controlled Trials as Topic - Abstract
Behçet's disease is a chronic relapsing systemic vasculitis of unknown etiology, affecting predominantly oral and genital mucocutaneous tissues and also the eyes. The disease is spread worldwide with a higher prevalence rate in countries along the ancient Silk Route, but it is rare (1-10/100,000) in Central and Northern Europe. Genetic, environmental, immunologic, inflammatory and rheologic factors are involved in the pathogenesis and the course of the disease. Any vascularized organ may be affected. Eye involvement is frequent, and may eventually result in loss of vision. Further important complications are cerebral manifestations, thrombotic syndromes, and arterial aneurysms with a high risk of rupture. Diagnosis and therapy of Behçet's disease are best managed by an interdisciplinary team. Skin lesions may be controlled by systemic treatment with colchicine, alternatively with dapsone, and in severe cases with thalidomide. Active systemic disease should be treated more aggressively using immunosuppressants. Despite advances in treatment relapses are still frequent, and systemic disease remains associated with an adverse prognosis.
- Published
- 2008
38. A case of Adamantiades-Behçet disease with ischemic optic neuritis (posterior optic neuropathy)
- Author
-
Satoko, Shima, Keisuke, Nishimura, Kei-ichi, Yamanaka, Arata, Hakamada, Ken-ichi, Isoda, Ichiro, Kurokawa, Koji, Toyoda, and Hitoshi, Mizutani
- Subjects
Adult ,Erythema Nodosum ,Optic Neuritis ,Treatment Outcome ,Behcet Syndrome ,Anti-Inflammatory Agents ,Humans ,Female ,Methylprednisolone - Abstract
Adamantiades-Behçet disease (ABD) may present with cutaneous and ophthalmologic findings. A 29-year old woman complained of fever and general fatigue, along with erythema nodosum and vesiculo-pustular lesions on the legs, acneiform lesions, genital ulcerations and painful oral ulcers. She also complained of reduced visual acuity, visual disturbance and blurred vision in the left eye. Her left visual acuity was 6/20. Light reflex in the left eye was reduced. The relative afferent pupillary defect (RAPD) was positive in the left eye where a central scotoma was present. The vitreous was clear; the optic disc, macula, retina and iris were all normal. Uveitis was not observed. The patient was diagnosed with ischemic optic neuritis (posterior optic neuropathy) with ABD. Histopathological findings taken from a blister on the leg showed subepidermal bulla, dense dermal neutrophil infiltration, and extravasation of erythrocytes, suggesting leukocytoclastic vasculitis. She was treated orally with high-dose corticosteroids (methylprednisolone 500 mg/d) for three days. Her general condition and ophthalmic symptoms resolved completely. Optic neuropathy with ABD is very rare; we know of two previous cases [1, 2] of ABD with ischemic posterior optic neuritis.
- Published
- 2007
39. [Vasculo-Behcet's disease: imaging findings]
- Author
-
C, Thomas, I, Kötter, P, Aschoff, and M, Horger
- Subjects
Adult ,Diagnosis, Differential ,Male ,Sex Factors ,Behcet Syndrome ,Positron-Emission Tomography ,Age Factors ,Prevalence ,Humans ,Female ,Radiography, Thoracic ,Tomography, X-Ray Computed ,Magnetic Resonance Angiography - Published
- 2007
40. [Arthralgia and hemoptysis. Diagnosis by a look at the mouth]
- Author
-
Peter, Stiefelhagen
- Subjects
Adult ,Diagnosis, Differential ,Male ,Hemoptysis ,Behcet Syndrome ,Humans ,Stomatitis, Aphthous ,Pulmonary Embolism ,Arthralgia ,Fever of Unknown Origin - Published
- 2007
41. [Massive hemorrhage in a 28-year-old male patient with intestinal Behçet's disease]
- Author
-
M, Waldner, C, Schimanski, W, Klaas, F, Bittinger, M, Pitton, M, Korenkov, R, Kiesslich, M, Neurath, P, Galle, and S, Kanzler
- Subjects
Adult ,Diagnosis, Differential ,Male ,Behcet Syndrome ,Humans ,Gastrointestinal Hemorrhage - Abstract
A 28-year-old man was admitted to the emergency room of our hospital after syncope and acute gastrointestinal bleeding. On the basis of numerous oral and genital ulcerations as well as uveitis anterior and erythema nodosum, the diagnosis of Behçet's disease had been confirmed previously. The bleeding focus could not be detected by endoscopy. During the next days recurrent massive hemorrhages occurred in spite of immunosuppressive therapy with steroids. Angiography revealed a circumscribed bleeding source in the colon transversum near the left colonic flexure, which was treated by superselective coil embolization. A massive hemorrhage reoccurred and required a surgical approach with a Hartmann procedure. Histology depicted bleeding into the submucosa and mucosa and an ulcer in this area as well as lymphatic infiltration of the mucosa. Immunosuppressive therapy was intensified with azathioprine and resulted in a dramatic improvement of the oral and genital ulcers. In addition, no further gastrointestinal hemorrhage occurred. Due to its higher prevalence in patients from eastern Asia, Behçet's disease poses an important differential diagnosis of intestinal hemorrhage in patients of this descent.
- Published
- 2006
42. [So you become the aphthae master]
- Author
-
Waldtraud, Paukstadt
- Subjects
Adult ,Diagnosis, Differential ,Male ,Ointments ,Recurrence ,Behcet Syndrome ,Smoking ,Anti-Infective Agents, Local ,Humans ,Female ,Stomatitis, Aphthous ,Child ,Anti-Bacterial Agents - Published
- 2006
43. [Fever of unknown origin in the 21st century. 2. Non-infectious diseases (autoimmune diseases)]
- Author
-
T, Schneider, C, Loddenkemper, M, Rudwaleit, H, Lode, and M, Zeitz
- Subjects
Adult ,Male ,Behcet Syndrome ,Incidence ,Lymphoma, Non-Hodgkin ,Age Factors ,Adenocarcinoma ,Middle Aged ,Prognosis ,Fever of Unknown Origin ,Autoimmune Diseases ,Familial Mediterranean Fever ,Diagnosis, Differential ,Heart Neoplasms ,Pregnancy Complications ,Pregnancy ,Germany ,Neoplasms ,Population Surveillance ,Colonic Neoplasms ,Humans ,Female ,Heart Atria ,Myxoma ,Still's Disease, Adult-Onset - Published
- 2005
44. [Intraocular inflammations. A diagnostic and therapeutic challenge]
- Author
-
U, Pleyer
- Subjects
Diagnosis, Differential ,Uveitis ,Behcet Syndrome ,Chronic Disease ,Cyclosporine ,Humans ,Interferon-alpha ,Vision, Low ,Interferon alpha-2 ,Prognosis ,Antiviral Agents ,Immunosuppressive Agents ,Recombinant Proteins - Published
- 2005
45. [Adamantiades-Behcet's disease]
- Author
-
L, Krause
- Subjects
Retinal Vasculitis ,Dose-Response Relationship, Drug ,Tumor Necrosis Factor-alpha ,Behcet Syndrome ,Anti-Inflammatory Agents ,Antibodies, Monoclonal ,Drug Administration Schedule ,Infliximab ,Receptors, Tumor Necrosis Factor ,Etanercept ,Adrenal Cortex Hormones ,Immunoglobulin G ,Azathioprine ,Panuveitis ,Cyclosporine ,Drug Therapy, Combination ,Immunosuppressive Agents - Abstract
Adamantiades-Behcet's disease (ABD) is a chronic recurrent vasculitis whose aetiology is still unclear. The first description goes back to B. Adamantiades und H. Behcet. The disease mainly occurs in Mediterranean, Middle and Far Eastern countries. However, it is not limited to these regions and can be found worldwide, and, as a result of increasing human mobility, is steadily becoming more significant. Clinical findings reveal recurrent oral and/or genital ulcers, arthritis and skin involvement in the form of erythema nodosum and superficial thrombophlebitis. Relapsing ocular involvement is one of the major manifestations in ABD and can be found in 60-80% of patients, resulting in retinal vasculitis, neuropathy or panuveitis. Eye involvement can lead to blindness in the affected eye in 20-50% of cases. Steroids, cyclosporin A und azathioprin are available to treat affected eyes. Studies of new treatment modalities including interferon-alpha or TNF antibodies are under way.
- Published
- 2005
46. [Behçet's disease with gastrointestinal involvement without evidence for chronic inflammatory bowel disease]
- Author
-
M, Philipper, B, Schumacher, A, Donner, and H, Neuhaus
- Subjects
Adult ,Diagnosis, Differential ,Male ,Treatment Outcome ,Behcet Syndrome ,Azathioprine ,Chronic Disease ,Humans ,Gastrointestinal Hemorrhage ,Inflammatory Bowel Diseases ,Methylprednisolone ,Immunosuppressive Agents - Abstract
A case of a 32-year-old male patient with lower gastrointestinal bleeding is reported as an initial manifestation of Behçet's disease. The early diagnosis of this chronic-systemic disease is often very difficult, in particular with regard to the differentiation from chronic inflammatory bowel diseases. In addition, lower gastrointestinal bleeding is a rare manifestation of Behçet's disease. An immunosuppressive therapy was initiated and the course of the disease with recurrent oral ulcerations gave evidence for the diagnosis. Further bleeding episodes or other gastrointestinal symptoms have not been observed during a follow-up period of 36 months. The diagnostic problems and the therapeutic management of the disease are discussed in relation to neighbouring entities.
- Published
- 2004
47. [Differential diagnosis of chronic inflammatory diseases of the central nervous system. Cerebrospinal fluid diagnosis and immunological parameters]
- Author
-
D, Reske and H-F, Petereit
- Subjects
Inflammation ,Sjogren's Syndrome ,Sarcoidosis ,Central Nervous System Diseases ,Behcet Syndrome ,Chronic Disease ,Lupus Vasculitis, Central Nervous System ,Humans ,Guidelines as Topic ,Syndrome ,Practice Patterns, Physicians' - Abstract
A number of neurological syndromes may be evoked by involvement of the nervous system due to systemic diseases such as lupus erythematosus, sarcoidosis, Behcet's disease, and Sjogren's syndrome. Because of different treatment strategies, it is important to distinguish between these different diseases. Neither clinical signs nor additional analyses such as serological findings or cerebrospinal fluid analysis are able to differentiate between the diseases with certainty. Nevertheless, diagnosis may finally be made taking all findings together. Here we compare typical clinical and cerebrospinal fluid findings in neurosarcoidosis, neurolupus, neuro-Behcet, and nervous system involving Sjogren's syndrome, with special emphasis on those findings allowing differentiation of the respective diseases.
- Published
- 2004
48. [Ocular involvement in Behçet's disease: first 5-year-results for visual development after treatment with interferon alfa-2a]
- Author
-
C M E, Deuter, I, Kötter, I, Günaydin, M, Zierhut, and N, Stübiger
- Subjects
Adult ,Male ,Retinal Vasculitis ,Time Factors ,Behcet Syndrome ,Visual Acuity ,Interferon-alpha ,Interferon alpha-2 ,Prognosis ,Recombinant Proteins ,Treatment Outcome ,Panuveitis ,Humans ,Female ,Immunosuppressive Agents ,Follow-Up Studies - Abstract
Behçet's disease (BD) is a systemic vasculitis of unknown origin. Up to now ocular involvement has a poor visual prognosis irrespective of immunosuppressive treatment. Now recent open studies have shown that interferon alfa-2a (IFN alfa-2a) is very effective in the treatment of ocular BD. The purpose of this study was to evaluate the long-term development of visual acuity (VA) in patients with severe ocular BD who were treated with IFN alfa-2a.We included 15 eyes of 9 patients with an active panuveitis and/or retinal vasculitis due to BD refractory to immunosuppressive treatment. VA before initiation of IFN-therapy was compared to VA at the end of the follow-up time.Mean follow-up time was 68.8+/-10.6 months. Mean duration of IFN-treatment was 40.6+/-17.0 months. With the exception of one patient IFN alfa-2a could be discontinued in complete remission of ocular symptoms. During the follow-up time 10 eyes showed an increase of VA of two lines or more. In 5 eyes VA remained stable. There was no decrease of VA in any eye. If a macular edema was present, a quick response to IFN alfa-2a was seen. No eye developed a pale optic disk during follow-up.Compared to conventional immunosuppressants, interferon alfa-2a seems to be much more effective to prevent a loss or decrease of VA over a long period of time in patients with severe ocular BD.
- Published
- 2004
49. [The Greek and the Turk: Benediktos Adamantiades (1875-1965) and Hulûsi Behçet (1889-1948)]
- Author
-
W, Keitel and Hulûsi, Behçet
- Subjects
Greece ,Turkey ,Behcet Syndrome ,Iritis ,Humans ,History, 19th Century ,History, 20th Century ,Philately - Published
- 2003
50. [Behçet's disease: uveitis-therapy with interferon alpha2a - prospective clinical study in 33 patients]
- Author
-
N, Stübiger, I, Kötter, C, Deuter, and M, Zierhut
- Subjects
Adult ,Male ,Behcet Syndrome ,Interferon-alpha ,Pilot Projects ,Interferon alpha-2 ,Middle Aged ,Recombinant Proteins ,Uveitis ,Treatment Outcome ,Humans ,Female ,Prospective Studies ,Fluorescein Angiography - Abstract
Behçet's disease (BD) is a multisystem vasculitis of unknown origin. Visual outcome in BD is bad irrespective of therapy. We investigated in a prospective, open clinical trial wether interferon alpha2a (IFN alpha2a) is effective as single agent in severe ocular BD.33 patients (11 female, 22 male) with severe ocular BD (posterior uveitis/panuveitis), fulfilling the Criteria of the "International Study Group of Behçet's Disease", were treated with IFN alpha2a. Other immunosuppressive drugs were stopped at the beginning of interferon-therapy. Efficacy was evaluated by the uveitis scoring system (Ben Ezra et al.), visual acuity and BD activity score (Rigby et al.).In 32 (97 %) patients complete remission of the inflammatory changes occured. Mean posterior uveitis score fell from 3.7 to 0.4, visual acuity rose from 0.5 to 0.9, and mean BD activity score fell from 5.1 to 3.0 during a mean observation period of 28.6 months. There was only one non-responder. Side effects were hair loss (69 %), depression (36 %), itching (33 %), fibromyalgia (10 %), and the development of autoantibodies (21 %). Fever, arthralgia and headache occured in all patients, but only during the first weeks of therapy.IFN alpha2a seems to be very effective in ocular BD. Controlled randomized studies are warranted in order to prove the efficacy of IFN alpha2a in ocular BD and to compare it with other, established treatments, such as azathioprine or cyclosporin A.
- Published
- 2002
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