OVERVIEW
Intestinal Behçet’s syndrome, first proposed by a Turkish dermatologist in 1937, is a systemic, chronic, vascular inflammatory disease, with the main clinical manifestations of recurrent oral ulcers, genital ulcers, ophthalmia, and skin lesions, and may also involve the blood vessels, the nervous system, the digestive tract, the joints, the lungs, the kidneys, the epididymis, and other organs. The disease has a certain genetic factor and shows an alternating course of recurrent episodes and remissions. Most patients have a favorable prognosis, while those with ocular, central nervous and large vessel involvement have a poor prognosis. Behçet’s syndrome combined with gastrointestinal ulcers is also known as intestinal Behçet’s syndrome, a special type of Behçet’s syndrome. Since Behçet’s syndrome has the highest incidence of ulcers in the oral mucosa, it was long ago hypothesized that ulcers would also occur in the gastrointestinal mucosa.
Etiology
The etiology of the disease is not clear, and there are theories of viral-bacterial anaphylaxis, autoimmunity, and socio-environmental factors. Immunologically, patients with this disease have an enlarged thymus as seen on mediastinal insufflation angiography, and pathohistology reveals a high percentage of thymic lymphoid follicles of up to 71%, hyperfunctioning wandering neutrophilic leukocytes in the peripheral blood, hyperactivity of lysosomal enzymes within the neutrophilic leukocytes, and a deficiency of immune complex suppressor T cells.
Symptoms.
The main symptoms of intestinal Behçet’s syndrome include recurrent ulcers of the oral mucosa; cutaneous symptoms such as erythema nodosum-like rash, subcutaneous thrombophlebitis, folliculitis-like rash, and a positive pinprick test; recurrent pus-filled iritis of the anterior chamber of the eye, retinitis, and chorioretinitis; and ulcers of the vulva. In addition, often combined with arthritis, epididymitis, as well as digestive, cardiovascular, central nervous system, respiratory system, urinary system and other symptoms as a secondary symptom of the disease. The complete form of the disease is characterized by the presence of all four main symptoms during the course of the disease. The incomplete form of the disease is characterized by the presence of three main symptoms, two main symptoms plus two secondary symptoms, or eye symptoms plus one other main symptom. Depending on the damage to the internal organ system, the disease is categorized into vascular, neurological, and gastrointestinal types.
1. Symptoms
(1) recurrent oral ulcers: several episodes per year, during which multiple painful red nodules appear on the buccal mucosa, tongue margin, lips, soft palate, etc., followed by the formation of ulcers with a diameter of 2-3 mm. some of them start with herpes, which subsides after 7-14 days without leaving any traces. There are also a few cases that last for several weeks without healing and end up with scarring, and the ulcers come and go. This symptom is seen in almost all patients and is the first symptom of the disease, it is considered to be a necessary symptom for the diagnosis of the disease.
(2) Skin lesions There is a high incidence of skin lesions with a wide variety of manifestations, including erythema nodosum, herpes, papules, acne-like rash, erythema multiforme, annular erythema, necrotizing tuberculous rash-like lesions, herpetic necrotizing vasculitis, and pyoderma gangrenosum. Patients may have 1 or more than 1 type of skin lesion. Skin signs of particular diagnostic value are erythema nodosum-like lesions and inflammatory reactions to tiny traumas (pinpricks).
(3) Ophthalmia The most common eye lesions are uveitis, or uveitis, and also retinitis due to vasculitis. Repeated episodes of these conditions can lead to severe visual impairment or even blindness. More male patients have uveitis than female.
(4) Recurrent vulvar ulcers are basically similar in nature to oral ulcers, except that they occur less frequently and in smaller numbers. They are commonly found on the labia majora and minora of female patients, followed by the vagina, as well as the scrotum and penis of male patients, and can also appear on the perineum or around the anus. The ulcers are deep and large, with severe pain and slow healing.
2. Characteristics of gastrointestinal lesions
Some patients with Behçet’s syndrome present with digestive symptoms, and the prevalence of patients presenting with gastrointestinal tract lesions is 20 to 50 years old, with a male-to-female ratio of 1.4:1, and more than half of them are incomplete. The main symptoms are right lower abdominal pain, abdominal mass, abdominal distension, belching, vomiting, diarrhea, and blood in stool. In severe cases, intestinal bleeding, intestinal paralysis, intestinal perforation, fistula formation. The ulcers of this disease can be seen in the whole digestive tract from the esophagus to the large intestine.
3. Other systemic symptoms
Arthropathy is common, and a few have arthralgias, with knee joints being the most commonly involved. Some patients have fever when the disease is active, mostly low-grade fever, sometimes high fever, may have weakness, myalgia, dizziness and other symptoms; some patients have visceral lesions caused by local vasculitis. Stenosis or aneurysm formation may occur when the large arteries are involved, and pulmonary vascular involvement manifests symptoms such as hemoptysis, shortness of breath, and pulmonary embolism. The nervous system may show meningoencephalitis, brainstem damage, benign intracranial hypertension, spinal cord damage, and peripheral neuropathy.
Examination
1. Acupuncture reaction
It is the only test with high specificity for this disease at present. The appearance of pustules or folliculitis with peripheral redness at the site of the needle puncture 24-48 hours after the patient receives venipuncture, intramuscular injection or intradermal injection is called a positive reaction to needle puncture.
2. Endoscopic manifestations
In Behçet’s syndrome, intestinal ulcers are found in the ileocecal region, and colonoscopy is the most meaningful. The ulcers mostly occur on the opposite side of the mesenteric attachment and are round, small and deep ulcers with a tendency to be multiple and perforate. Careful observation of the small bowel microscope is helpful in detecting small bowel ulcers, which have a different morphologic appearance than ulcers of the large bowel. Small intestinal ulcers are small and deep, often multiple, the mucosa is concentrated to the ulcer, the peripheral elevation of the ulcer is not obvious, the ulcer is a very clear edge of the rounded chisel-like acute ulcers, at the bottom of the ulcer is not attached to the white moss, mostly in 2 cm or less, and there are also large in diameter to 2-3 cm; endoscopy can be seen to the center of the ulcer of the mucosa is obviously concentrated, the periphery of the ulcer to form a clear elevation, for the ring embankment-like.
3.X-ray examination
X-ray examination is often found in the ileocecal region of the mucosa concentrated ulcer niche shadow. Stenosis of the mucosa of the intestinal canal in the lesion area as well as increased tension in the small intestine and colon may occur.
Diagnosis
Behçet’s syndrome of the intestinal type usually begins with symptoms of Behçet’s syndrome, most commonly oral mucosal ulcers and vulvar ulcers, followed by intestinal lesions and abdominal pain in the digestive tract due to multiple ulcers. The diagnosis of intestinal lesions mainly relies on endoscopy and barium enema examination. Endoscopy is characterized by single or multiple round or nearly round ulcers with clear margins, especially in the ileocecal region. Most of the ulcers are covered with yellowish-white moss at the bottom, and the ulcers are often not easy to observe because of the distortion of the intestinal tube. x-ray examination often reveals concentrated ulcerated niches in the ileocecal region of the mucosa, and more than half of the cases of intestinal-type Behçet’s syndrome do not present with ocular symptoms, so it is necessary to take a detailed medical history in making the diagnosis. In the minority of patients who initially present with intestinal ulcers, it is often difficult to confirm the diagnosis. There are no specific serologic tests for Behçet’s syndrome, but sometimes there is a mild elevation of globulin and a mild to moderate increase in blood sedimentation.
Treatment
1. General treatment
During the active period, activities should be restricted, adequate rest should be provided, and liquid diet should be given, which should be changed to nutrient-rich and less residue diet when the condition improves. For the acute stage of severe abdominal pain and blood in stool, be absolutely quiet, give parenteral central intravenous nutrition or enteral nutrition agent. Pay attention to correct water and electrolyte balance disorders, anemia can be transfused, hypoproteinemia appropriate supplementation of human serum protein. Antibiotic treatment is not indicated for general cases, but for severe cases with secondary infection, antibiotic treatment should be actively carried out. Give broad-spectrum antibiotics, intravenous administration, combined with metronidazole is effective for anaerobic infections.
2.Drug treatment
There is no specific drug therapy for intestinal-type Behçet’s syndrome.
(1) Aminosalicylic acid preparation Sulfasalazine (SASP) is a commonly used drug in the treatment of this disease.
(2) Glucocorticoids Corticosteroids are usually used when inflammation is evident or when they are not effective against aminosalicylic acid preparations.
(3) Immunosuppressive drugs are used in chronic active cases with poor therapeutic effect of glucocorticoid or dependence on glucocorticoid, and the dosage of glucocorticoid can be gradually reduced or even discontinued with the addition of this kind of drugs, and the following immunosuppressive drugs can be chosen as one of the following: cyclophosphamide (CTX), azathioprine (AZP) or mercaptopurine (6-MP), and the use of its side effects should be noted.
(4) Other drugs Colchicine can be applied when ophthalmia occurs, and hormonal ointment can be applied to oral ulcers, in addition to levamisole and transfer factor, which have been tried with mixed clinical evaluations.
3.Surgical treatment
Cases of intestinal perforation should be operated urgently. Surgical resection is also advocated for those who have obvious abdominal pain, abdominal masses and deep ulcers, and those who are ineffective in conservative treatment through internal medicine. Because of the high recurrence rate after surgery, the indications should be carefully grasped. Surgery is usually performed for ileocaecal resection or right hemicolectomy, and most postoperative recurrences are on the ileal side, so it has been suggested that the ileum should be adequately resected.