The cause is unknown. There is no cure. Inflammatory eye disease can develop early in the disease course and lead to permanent vision loss in 20 percent of cases. Ocular involvement can be in the form of posterior uveitis , anterior uveitis , or retinal vasculitis. Anterior uveitis presents with painful eyes, conjuctival redness, hypopyon , and decreased visual acuity, while posterior uveitis presents with painless decreased visual acuity and visual field floaters. A rare form of ocular eye involvement in this syndrome is retinal vasculitis which presents with painless decrease of vision with the possibility of floaters or visual field defects.
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The cause is unknown. There is no cure. Inflammatory eye disease can develop early in the disease course and lead to permanent vision loss in 20 percent of cases. Ocular involvement can be in the form of posterior uveitis , anterior uveitis , or retinal vasculitis. Anterior uveitis presents with painful eyes, conjuctival redness, hypopyon , and decreased visual acuity, while posterior uveitis presents with painless decreased visual acuity and visual field floaters.
A rare form of ocular eye involvement in this syndrome is retinal vasculitis which presents with painless decrease of vision with the possibility of floaters or visual field defects. However, cases of acute optic neuropathy specifically anterior ischemic optic neuropathy have also been reported to occur.
Signs and symptoms of acute optic neuropathy include painless loss of vision which may affect either one or both eyes, reduced visual acuity, reduced color vision, relative afferent pupillary defect , central scotoma , swollen optic disc, macular edema , or retrobulbar pain.
Progressive optic atrophy may result in decreased visual acuity or color vision. Intracranial hypertension with papilledema may be present. Episcleritis may occur, which causes eye redness and mild pain, without a significant impact on vision.
GI manifestations include abdominal pain, nausea, and diarrhea with or without blood, and they often involve the ileocecal valve. Lung involvement is typically in the form of hemoptysis , pleuritis , cough, or fever, and in severe cases can be life-threatening if the outlet pulmonary artery develops an aneurysm which ruptures causing severe vascular collapse and death from bleeding in the lungs.
Arthritis is seen in up to half of people, and is usually a non-erosive poly or oligoarthritis primarily of the large joints of the lower extremities. CNS involvement most often occurs as a chronic meningoencephalitis. Lesions tend to occur in the brainstem, the basal ganglia and deep hemispheric white matter and may resemble those of MS. Brainstem atrophy is seen in chronic cases.
Neurological involvements range from aseptic meningitis to vascular thrombosis such as dural sinus thrombosis and organic brain syndrome manifesting with confusion, seizures, and memory loss. Sudden hearing loss Sensorineural is often associated with it. Pericarditis is a frequent cardiac manifestation. Arterial lesions pose a greater risk.
Most common arterial lesions are occlusions or stenosis and aneurysms or pseudoaneurysms. The cause is not well-defined, but it is primarily characterized by auto-inflammation of the blood vessels. Although sometimes erroneously referred to as a diagnosis of exclusion, the diagnosis can sometimes be reached by pathologic examination of the affected areas.
The primary mechanism of the damage is autoimmune, which by definition is an overactive immune system that targets the patient's own body. The involvement of a subset of T cells Th17 seems to be important. There does however seem to be a genetic component involved, as first degree relatives of the affected patients are often affected in more than the expected proportion for the general population.
Heat shock proteins HSPs are present in some bacteria and serve as a "danger signal" to the immune system. However, some HSPs share a similarity in bacteria and humans. Thus, it is sometimes known as Silk Road disease. However, this disease is not restricted to people from these regions. A large number of serological studies show a linkage between the disease and HLA-B Histological evaluation in a reported case of acute optic neuropathy demonstrated substitution of the axonal portion of the optic nerve with fibrous astrocytes without retinal changes.
There is no specific pathological testing or technique available for the diagnosis of the disease, although the International Study Group criteria for the disease are highly sensitive and specific, involving clinical criteria and a pathergy test.
A complete ophthalmic examination may include a slit lamp examination, optical coherence tomography to detect nerve loss, visual field examinations, fundoscopic examination to assess optic disc atrophy and retinal disease, fundoscopic angiography, and visual evoked potentials, which may demonstrate increased latency.
Optic nerve enhancement may be identified on Magnetic Resonance Imaging MRI in some patients with acute optic neuropathy. However, a normal study does not rule out optic neuropathy. Cerebrospinal fluid CSF analysis may demonstrate elevated protein level with or without pleocytosis. Imaging including angiography may be indicated to identify dural venous sinus thrombosis as a cause of intracranial hypertension and optic atrophy.
Despite the inclusive criteria set forth by the International Study Group, there are cases where not all the criteria can be met and therefore a diagnosis cannot readily be made. Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system. High-dose corticosteroid therapy is often used for severe disease manifestations. Interferon alpha-2a may also be an effective alternative treatment, particularly for the genital and oral ulcers  as well as ocular lesions.
Thalidomide has also been used due to its immune-modifying effect. Early identification and treatment are essential. Response to ciclosporin , periocular triamcinolone, and IV methylprednisone followed by oral prednisone has been reported although relapses leading to irreversible visual loss may occur even with treatment. When symptoms are limited to the anterior chamber of the eye prognosis is improved.
Posterior involvement, particularly optic nerve involvement, is a poor prognostic indicator. Secondary optic nerve atrophy is frequently irreversible. Lumbar puncture or surgical treatment may be required to prevent optic atrophy in cases of intracranial hypertension refractory to treatment with immunomodulators and steroids. IVIG could be a treatment for severe  or complicated cases.
Surgical treatment of arterial manifestations of BD bears many pitfalls since the obliterative endarteritis of vasa vasorum causes thickening of the medial layer and splitting of elastin fibers. Therefore, anastomotic pseudoaneurysms are likely to form, as well as pseudoaneurysms at the site of the puncture in case of angiography or endovascular treatment; furthermore, early graft occlusion may occur.
For these reasons, invasive treatment should not be performed in the acute and active phases of the disease when inflammation is at its peak. Endovascular treatment can be an effective and safe alternative to open surgery, with less postoperative complications, faster recovery time, and reduced need for intensive care, while offering patency rates and procedural success rates comparable with those of surgery.
The syndrome is rare in the United States, Africa and South America, but is common in the Middle East and Asia, suggesting a possible cause endemic to those tropical areas. In the UK, it is estimated to have about 1 case for every , people.
Other fundoscopic findings include vascular sheathing The prevalence of this disease increases from North to South. It follows a more severe course in patients with an early age of onset particularly in patients with eye and gastrointestinal involvement.
The first modern formal description of the symptoms was made by H. Planner and F. Symptoms of this disease may have been described by Hippocrates in the 5th century BC, in his Epidemion book 3, case 7. From Wikipedia, the free encyclopedia. Archived from the original on 11 February Retrieved 29 May Archived from the original on 13 May Oxford Textbook of Vasculitis 3 ed.
OUP Oxford. Archived from the original on 10 September Philadelphia, Pa: American College of Physicians. Eur J Ophthalmol. J Clin Neuroophthalmol. Ring 23 August Steven S. Agabegi; Elizabeth Agabegi eds. Step-up to medicine 3rd ed. Archived from the original on 16 February Retrieved 28 March Genetics Research International. June Ocular Immunology and Inflammation. Annals of the Rheumatic Diseases. Semin Ophthalmol. Taylor J ed. The Cochrane Database of Systematic Reviews. Ann Rheum Dis.
J Rheumatol. Retrieved 19 November Arch Dermatol. Br J Ophthalmol. Isr Med Assoc J. Arthritis Rheum. A randomized, double-blind, placebo-controlled trial". Ann Intern Med. J Dermatol. Report of two cases". Allergy Asthma Immunol. Archived from the original on 4 July
Maladie de Behçet
C'est une affection ubiquitaire, mais elle est rare chez les noirs. These require the presence of recurrent oral ulcers plus two of the following: recurrent genital ulcerations, typical defined eye lesions, typical defined skin lesions or a positive pathergy test a skin hypersensitivity reaction to a non-specific physical insult; when positive, the response consists of a papule or pustule that develops after 24 to 48 hours at the site of a needle prick to the skin. This prevalence is probably affected by the type of study retrospective or prospective and regional and ethnic variations in disease expression. Psychiatric symptoms usually occur as incidental findings in some patients with neurological disease; they are misdiagnosed and mistreated.
What is Behçet’s?
Address for correspondence. Key-words: Budd-Chiari syndrome. Young adult. Adulto jovem. Budd-Chiari syndrome.
Cancer colique au cours de la maladie de Behçet
This system, which normally protects the body against infections by producing controlled inflammation, becomes over-active and results in unpredictable outbreaks of unwanted and exaggerated inflammation. This extra inflammation particularly affects blood vessels of all sizes, including arteries and veins. As a result, symptoms occur wherever there is a patch of inflammation; this can be anywhere where there is a blood supply — from the brain down to the feet. It is not because of any known infections, it is not necessarily hereditary, but can sometimes run in families, and it is not thought to be related to lifestyle, age, or where someone has lived or where they have been on holiday. It is not associated with cancer, but is found more frequently in people with certain tissue-type molecules and genes, also the potential link with this is not understood. It does not follow the usual pattern for autoimmune diseases and is better considered, for now, as a disease of inflamed blood vessels vasculitis — a vasculitic disease. There are several ways in which the immune system can be suppressed to an appropriate level to reduce the extra inflammation, and this suppresses the symptoms.