Intestinal Behcet's disease
Introduction
Introduction to intestinal Behcet's disease Behcet's disease combined with gastrointestinal ulcers, also known as intestinal Behcet's disease, is a special type of Behcet's disease. basic knowledge The proportion of illness: 0.002% Susceptible people: no specific people Mode of infection: non-infectious complication:
Cause
Causes of intestinal Behcet's disease
(1) Causes of the disease
The etiology of this disease is still unclear. There are viruses, bacteria, allergies, home immunity, social environmental factors and other theories. From the perspective of immunity, the thymus enlargement can be seen in the mediastinal angiography of the patient, and the thymus lymphoid follicles are found by histopathology. The percentage is as high as 71%. In the peripheral blood, the migration function of neutrophils is increased, the lysosomal enzyme activity in neutrophils is increased, and immune complexes and inhibitory T cells are absent.
(two) pathogenesis
For inflammatory bowel diseases including Behcet's disease, luminol is used to induce PMA stimulation of peripheral blood neutrophils, and chemiluminescence studies are performed on the amount of photons emitted by phagocytosis. Significantly elevated, at this time, due to the occurrence of active oxygen, tissue damage including vascular endothelial cells is caused.
Pathologically, the intestinal ulcer of Behcet's disease is divided into necrotic, granuloma and mixed type, necrotic type is acute, subacute type, granuloma type is chronic disease, mixed type is between the two, microscopically visible intestinal mucosa Edema, mucosal lamina propria, and intestinal lymphatic vessels in the submucosal tissue. The vasculopathy of the intestine is an inflammatory change of the vasculature accompanying the ulcer. It is mainly characterized by hypertrophy of the intima, and the blood vessels of the submucosal tissue, especially the vein. Obviously, the lesion of this blood vessel has nothing to do with the size of the ulcer. Due to the passage of time, the vascular lesion of the acute phase ulcer (necrotic type) is lighter than that of the chronic ulcer (granulomatous type), and the deeper the ulcer, the blood vessel The more obvious the lesion, and the ulcer-free intestinal mucosa, the obvious vascular inflammatory changes are not seen. This vascular lesion is basically not much different from the vascular lesions of tuberculosis, Crohn's disease and ulcerative colitis.
Prevention
Enterococci prevention
Prevention: Treatment should be based on the cause.
Complication
Enterocutaneous disease complications Complication
Severe cases of intestinal bleeding, intestinal paralysis, intestinal perforation, fistula.
Symptom
Symptoms of intestinal Behcet's symptoms Common symptoms Dizziness erythematous rash intestinal paralysis intestinal perforation bloody acne-like rash intestinal bleeding intestinal deformity hernia visual impairment
The main symptoms of clinical manifestations include: recurrent ulcer of oral mucosa; nodular erythematous rash, subcutaneous thrombophlebitis, folliculitis-like rash, positive acupuncture test, etc.; recurrent anterior chamber purulent iritis , retina, choroiditis; genital ulcers, in addition, often combined with arthritis, epididymitis and digestive tract, cardiovascular, central nervous system, respiratory system, urinary system and other symptoms as a side symptom of the disease, the four main symptoms in the course of the disease All appear to be the complete type of the disease, with 3 main symptoms in the course of the disease, or 2 main symptoms plus 2 side symptoms, or symptoms of the eye plus one other main symptom for the disease. Incomplete type, divided into vascular type, neurological type, gastrointestinal type, etc. according to the damage of visceral system.
1. Main symptoms
(1) recurrent oral ulcer: several episodes per year, multiple painful red nodules appear in the buccal mucosa, tongue, lips, soft palate, etc., followed by ulcer formation, the diameter of the ulcer is generally 2 ~ 3mm, and some With herpes onset, after 7 to 14 days, it will resolve by itself, leaving no traces. There are also a few people who have been scarred for several weeks. The ulcers are one after another. This symptom is common in all patients and is the first symptom of this disease. It is It is considered to be an essential symptom for the diagnosis of this disease.
(2) skin lesions: high incidence of skin lesions, up to 80% to 98%, a variety of manifestations, nodular erythema, herpes, papules, acne-like rash, polymorphous erythema, ring erythema, necrotizing tuberculosis Such lesions, bullous necrotizing vasculitis, pyoderma, etc., patients may have one or more types of skin lesions, especially the diagnostic value of the skin signs are nodular erythematous lesions and small trauma (acupuncture After the inflammatory reaction.
(3) Ophthalmitis: The most common eye lesions are uveitis or uveitis, and retinitis caused by vasculitis. Repeated episodes of these conditions can lead to severe visual impairment or even blindness. Male patients have There are more ophthalmia than women.
(4) recurrent vulvar ulcer: similar to oral ulcer traits, but the number of occurrences is small, the number is also small, the common is the female patient's large, small labia, followed by the vagina, male scrotum and penis, can also appear Around the perineum or anus, about 75% of patients have this symptom, the ulcer is deep, the pain is severe, and the healing is slow.
2. Gastrointestinal lesions The symptoms of digestive system in patients with Behcet's disease account for 21.5% to 60%, and the age of patients with gastrointestinal lesions is 20 to 50 years old. The ratio of male to female is 1.4:1, more than half is Incomplete type, the main symptoms are right lower abdominal pain, abdominal mass, abdominal fullness, hernia, vomiting, diarrhea, blood in the stool, etc., severe cases of intestinal bleeding, intestinal paralysis, intestinal perforation, fistula formation, etc. From the esophagus to the large digestive tract of the large intestine, Pushan et al analyzed 719 patients with Behcet's disease, including 13 cases (1.8%) of stomach and duodenal ulcer and 18 cases (2.5%) of multiple ulcers at the end of ileum. 5 cases of blood in the stool (0.7%), 36 cases (5%), Wang Hongjin analysis of 89 cases of Behcet's disease, 28 cases of digestive system involvement (31.5%), of which 28 cases (31.5%) of oral ulcers, esophagus 3 Case (3.4%), 1 case (1.1%) in the stomach, 1 case (1.1%) in the duodenum, 4 cases (4.5%) in the ileocecal area, 2 cases (2.2%) in the transverse colon, and 3 cases (3.4%) in the rectum There were 7 cases (7.9%) of anus, 131 cases of intestinal type Behcet's disease analyzed by horses, etc. 105 cases (80.2%) of lesions at the end of ileum and cecum, suggesting that this part is a good site, white bird report hand Of the 66 patients, the lesions were 36.4% in the small intestine, 45.6% in the small intestine, 16.6% in the colon, and 1.5% in the stomach and duodenum. More than half of these cases were due to massive blood in the stool, or as acute abdomen. Symptoms, perforation, intestinal obstruction and emergency surgery for appendicitis, 40% of surgical cases found perforation of intestinal ulcers, recurrence after surgery, anastomotic fistula formation, wound dehiscence, etc., requiring 33.9% of reoperations, often repeated remission It alternates with recurrence and does not heal.
3. Other systemic symptoms Arthropathy is common, a few have joint swelling, more common in knee joint involvement, some patients have fever during disease activity, more common in low heat, high fever, fatigue, muscle pain, dizziness, etc. Symptoms, some patients with visceral lesions due to local vasculitis, stenosis or aneurysm may occur when the aorta is involved, pulmonary vascular involvement is characterized by hemoptysis, shortness of breath, pulmonary embolism and other symptoms, meningeal encephalitis, brain stem damage, Benign intracranial hypertension, spinal cord damage and peripheral neuropathy.
Examine
Examination of intestinal Behcet's disease
There is no specific serological examination of Behcet's disease, sometimes mild globulin is elevated, erythrocyte sedimentation rate is mild, moderate increase is fast, about 40% anti-PPD antibody is increased, and leukocyte antigen HLA-B51 is positive.
1. Acupuncture reaction is the only specific test with strong specificity in this disease. Patients receive pustules or folliculitis in acupuncture 24 to 48 hours after venipuncture, intramuscular or intradermal injection, and the surrounding area is red. It is a positive reaction to acupuncture.
2. Endoscopic findings of intestinal ulcers of Behcet's disease occur in the ileocecal area. Colonoscopy is most meaningful (Fig. 1). Ulcers occur mostly on the opposite side of the mesenteric attachment. They are round, small and deep ulcers. The tendency of multiple hair and perforation, careful observation, small colonoscopy is helpful for finding small intestine ulcer, small intestine ulcer and large intestine ulcer have different appearances, small intestine ulcer is small and deep, often multiple, mucous membrane is concentrated to ulcer, ulcer surrounding peripheral bulge is not obvious, ulcer For the acute ulcer with a rounded shape with a very clear edge, there is no white moss attached to the bottom of the ulcer, most of which is below 2cm, and there are also diameters of 2 to 3cm. Endoscopically, the mucosa of the central part of the opposite ulcer is obviously concentrated. The ulcer forms a prominent bulge, which is a ring-shaped embankment. The ulcer is irregularly depressed. It looks like the shape of Borrmann type 2 or Borrmann type 3 cancer. It is difficult to distinguish the form of ulcer from the simple ulcer of ileocecal area.
3. X-ray examination often found mucosal ulceration in the ileocecal area (Fig. 2). The mucosa of the intestinal tube of the lesion can be narrowed and the tension of the small intestine and colon is increased.
Diagnosis
Diagnosis and identification of intestinal Behcet's disease
Diagnostic criteria
Intestinal Behcet's disease usually has the symptoms of Behcet's disease first. Oral mucosal ulcers and vulvar ulcers are the most common. Later, intestinal lesions appear again. The digestive tract has symptoms such as abdominal pain due to multiple ulcers. The diagnosis of intestinal lesions is mainly By endoscopic and barium enema examination, the endoscope sees the intestinal mucosa, especially in the ileocecal part, with a round or nearly circular single or multiple ulcers with distinct edges, which are characteristically changed, and the bottom of the ulcer is mostly covered with yellow and white moss. Because of the deformation of the intestine, the observation of the ulcer is often not easy. X-ray examination often finds the ulcer in the ileocecal area. In the case of more than half of the cases of intestinal Behcet, there are no eye symptoms, so ask in detail when diagnosing. History, it is often difficult to diagnose a small number of patients with initial intestinal ulcers.
Differential diagnosis
1. The cause of simple ulcer is unknown, the round or oval deep ulcer of the ileocecal area is good, the histology is non-specific inflammation, the oral ulcer with abscess, the vulvar ulcer, the ophthalmitis and other clinical specific manifestations.
2. Non-specific ulcerative colitis ulcers develop from the rectum to the whole colon, and have continuity, superficial, diffuse, non-specific characteristics, mucosal congestion, edema, chronic pseudopolyps formation A large number of neutrophils accumulate at the bottom of the intestine gland, forming a characteristic small crypt abscess and crypt abscess formation and rupture to form mucosal erosion or superficial ulcer, generally limited to mucosa or submucosa, crypt abscess is non-specific ulcerative More meaningful features of colitis.
3. Crohn's disease occurs in the ileocecal area, which is a segmental lesion. The typical mucosa has a "paving stone" sign. The inflammatory changes in the entire intestinal wall are characteristic features. The intestinal wall or mesenteric lymph nodes are visible. Typical granuloma changes, the center is epithelial cells, multinucleated giant cells and fibrous structure, no cheese-like necrosis, has diagnostic significance, can be combined with oral ulcers, ophthalmia, skin erythema and other parenteral manifestations, but vulvar ulcers are rare.
4. Intestinal tuberculosis Intestinal tuberculosis is secondary to open tuberculosis; the lesion mainly involves ileocecal, sometimes involving adjacent colon, tuberculin test positive, lack of Behcet's mouth ulcer, vulvar ulcer, ophthalmia and other clinical specific manifestations For those who have difficulty in identification, it is recommended to observe the efficacy of anti-tuberculosis treatment first. Surgery can be performed by surgical indications. The pathological histology of mesenteric and mesenteric lymph nodes can be diagnosed with caseous necrotizing granuloma.
5. Acute appendicitis with acute abdominal pain, often with metastatic right lower abdominal pain, tenderness is limited to Mai's point, blood leukocyte count is significantly increased, generally no oral ulcers, white vulvar ulcers, ophthalmia and other clinical manifestations can be identified, Sometimes a laparotomy is needed to confirm the diagnosis.
6. Others should be differentiated from amyloidosis, ischemic enteritis, colonic diverticulitis.
