Medical Codes
ICD-9-CM: 136, 136.1
Definition
Behçet's syndrome is a chronic disease characterized by a group of symptoms that affects multiple body systems. Although the musculoskeletal, gastrointestinal, and the central nervous system are most often involved, the syndrome can affect every system in the body in many different ways.
The classic three symptoms of Behçet's syndrome include ulcerations in the mouth (apthous ulcers), genital ulcers, and inflammation of an area around the pupil of the eye called the uvea. The syndrome may also affect the lungs, causing aneurysms; the joints, causing arthritis; the gastrointestinal system, causing ulcers or inflammatory bowel disease; and the brain, causing headaches, confusion, strokes, and personality changes. Optic disc damage (glaucoma) may cause blindness. The disease may also involve the meninges, which may develop into meningitis. The condition may cause memory loss and impaired speech, balance, and movement.
The disease generally is chronic, with outbreaks typically lasting several weeks, followed by periods of remission and relapse. Infections or environmental factors may cause onset of the disease.
Risk: Behçet's syndrome has a genetic predisposition, but not everyone with the gene (HLA-B51) will necessarily contract the disease. About 5% of cases are hereditary ("Behçet's Disease"). Behçet's syndrome usually affects individuals in their twenties or thirties, although it can occur at any age. The condition affects men twice as often as women ("NINDS").
Incidence and Prevalence: The disease is uncommon in the US. It affects approximately 15,000 to 20,000 people each year, with many more going undiagnosed ("What is Behçet's Disease?"). Behçet's syndrome is seen most frequently in Japan, Turkey, and Israel ("NINDS").
Source: Medical Disability Advisor
Diagnosis
History: Weight loss or headaches may precede onset of the disease. Some of the first symptoms individuals report include painful, recurrent sores in the mouth, followed by genital lesions. Eye inflammation also is common and can cause blurry vision. Individuals may also complain of ulcerated sores that may appear anywhere on the skin, most especially on the legs and upper torso. Varying symptoms may develop over a period of weeks or years and could include abdominal discomfort, uncontrolled eye movements (nystagmus), joint pain, joint stiffness and swelling, and convulsions.
Physical exam: The exam may reveal pus-filled blisters, often in the mouth or genital areas. Painful nodes may form in response to minor injuries. Inflammation of the iris (iritis), uvea (uveitis), or cornea (keratitis) of the eye may be seen, along with pus in the anterior chamber. The optic nerve may become inflamed, resulting in blindness. Blood vessels may protrude from inflammation (aneurysms), causing blockages (thrombophlebitis) or rupture.
Tests: For some individuals, placing a sterile or saline-filled needle in the skin will cause a positive reaction that looks like a rash or reddish circle surrounding the prick (pathergy test), indicating hypersensitivity. Laboratory tests may show elevated white blood cell counts, sedimentation rates, and antibody levels but will not specifically identify Behçet's syndrome.
Source: Medical Disability Advisor
Treatment
Behçet's syndrome is treated according to the symptoms manifested, since there is no known cure for the disease. Mouth rinses can be used to treat oral sores. Topical drugs (corticosteroids) may reduce inflammation created by the disease in areas such as the skin, eyes, and joints. High doses of oral corticosteroids may alleviate central nervous system symptoms and uveitis, which sometimes result from Behçet's syndrome. Immunosuppressive drugs help control the overactive immune system and thus reduce inflammation; they have serious side effects that require regular monitoring. Other drugs that attack and destroy certain types of cells (cytotoxic) may also be used for uveitis. Drugs that disperse blood clots (anticoagulants) may be given for thrombosis, another possible manifestation of the disease. Surgery may be required for extreme conditions, such as intestinal perforations, that may result from Behçet's syndrome.
Source: Medical Disability Advisor
Prognosis
Behçet's disease is chronic and incurable. Because of the many manifestations of the disease, it is difficult to characterize the ultimate outcome. Outbreaks usually last several weeks, occurring more frequently in early stages of the disease. Cumulative effects through the years may have serious consequences, the most common of which is eye damage and possible blindness.
Source: Medical Disability Advisor
Rehabilitation
Medical approaches generally are thought to be the most effective treatment for the numerous, varied symptoms of Behçet's syndrome. Individuals with joint pain and stiffness might benefit from rehabilitation therapy recommended for those afflicted with arthritis.
Source: Medical Disability Advisor
Complications
Possible severe complications include paralysis, blindness, strokes, heart trouble, and destruction of protective tissue surrounding the brain (aseptic meningitis).
Source: Medical Disability Advisor
Return to Work (Restrictions / Accommodations)
Individuals should avoid heat, humid conditions, and chemicals, which may irritate the skin and eyes. Depending on the severity and type of symptoms, larger computer screens might assist those with visual impairment. For individuals with joint pain or stiffness, easy accessibility to work stations may be needed. Keyboards and seating may need to be positioned to ease discomfort. Moderate exertion and rest periods may be necessary.
Source: Medical Disability Advisor
Failure to Recover
If an individual fails to recover within the expected maximum duration period, the reader may wish to consider the following questions to better understand the specifics of an individual's medical case.
Regarding diagnosis:
Does individual have a genetic predisposition to the syndrome?
Does individual have mouth ulcers?
Has individual had recent weight loss or headaches?
Has individual developed genital lesions?
Does individual have eye inflammation? Blurry vision?
Have blood vessels developed aneurysms from the inflammation?
Are ulcerated sores present on the upper torso and legs?
Is abdominal discomfort present? Swelling? Uncontrolled eye movement?
Does individual have joint pain or stiffness?
Does individual have convulsions?
Were blood tests done? Pathergy test?
Were conditions with similar symptoms ruled out?
Regarding treatment:
Was individual able to control symptoms?
Were corticosteroids helpful? Were cytotoxic drugs used?
Were anticoagulants necessary?
Was it necessary for individual to have surgery?
How long does an outbreak last? How frequent?
Regarding prognosis:
Can individual's employer accommodate any necessary restrictions?
Does individual have any conditions, such as heart trouble or aseptic meningitis that may affect ability to recover?
Have any complications developed such as paralysis, blindness, or stroke?
Source: Medical Disability Advisor
References
Cited
"Behcet's Disease." Johns Hopkins Vasculitis Center. American Psychiatric Association. 24 Sep. 2004
"What is Behcet's Disease?" American Behcet's Disease Association. 24 Sep. 2004
"NINDS Behcet's Disease Information Page." National Institute of Neurological Disorders and Stroke. National Institutes of Health (NIH). 24 Sep. 2004
Source: Medical Disability Advisor
No comments:
Post a Comment