fibrotic syndrome
Introduction
Introduction to fibrosis syndrome Fibrosis syndrome, also known as multifocal sclerosis, is a non-infectious granulomatosis disease. It is characterized by progressive fibrosis and hyperplasia of multiple organ systems. Among them, hydronephrosis caused by fibrosis around the ureter is the most common, followed by fibroproliferative mediastinal inflammation and fibroseptic cholangitis, which may also involve other organ systems. This syndrome can occur in several organ systems, and can also be manifested as an organ involvement. basic knowledge The proportion of illness: 0.0002% Susceptible people: more often in adults Mode of infection: non-infectious Complications: liver
Cause
Causes of fibrotic syndrome
(1) Causes of the disease
Most cases are idiopathic, and a few cases are secondary.
(two) pathogenesis
At present, the pathogenesis of this disease is autoimmune, infectious allergic reaction and tumor antigen sensitization theory.
The fibrotic syndrome lesions are thickened and have a rubbery sensation. The peritoneum is followed by the humerus and the rectum. It can also be seen in the upper mediastinum and thyroid. The early pathological changes are a chronic inflammation. Infiltration, microscopic examination of scattered fat islands, surrounded by inflammatory infiltrates, containing lymphocytes, monocytes, plasma cells, a small number of multinucleated giant cells and small fibroblasts, scattered between the inflammatory foci, adipose tissue inflammation, As the disease progresses, typical granulomatous changes can occur gradually. At this time, fibroblasts increase more obviously, capillary proliferation, collagen fibers are formed, but inflammatory cells are reduced. In some cases, vasculitis changes may occur, ie, Granuloma inflammation occurs in the wall of the small vein. The pathological changes in the later stage are mainly fibrous tissue hyperplasia. The original tissue has reduced or lacked cellular components, and the vascular tissue is significantly reduced. Occasionally, calcification is observed.
Prevention
Fibrosis syndrome prevention
1. Early detection and early diagnosis of primary disease, other symptoms should be considered whether it is related to malignant tumors, establish confidence in the fight against disease, adhere to treatment.
2. Conservative treatment should be taken as much as possible to wait for the formation of collateral circulation, relieve symptoms, and avoid death caused by surgery.
3. Prevention of streptococcal infection is an important part of autoimmune rheumatic diseases and co-morbidity.
Complication
Complications of fibrotic syndrome Complications
Can be complicated by renal insufficiency, occasionally visible gastrointestinal bleeding, but also complicated with superior vena cava obstruction, intraorbital pseudotumor, sclerosing cholangitis, progressive hepatomegaly.
Symptom
Symptoms of fibrotic syndrome Common symptoms constipation abdominal pain retroperitoneal fibrosis dysphagia bladder fibrosis diarrhea nausea mediastinal fibrosis eyeball prominent asphyxia
1. Early retroperitoneal fibrosis disease, mild retroinflammatory infiltration around the retroperitoneal aorta and mesentery, patients can be asymptomatic, diagnosis can only rely on biopsy, with the progress of fibrosis, can appear back, both rib pain, body Discomfort, weight loss, fever, etc., sometimes clinical manifestations of severely wasting disease, may be life-threatening, when fibrotic masses squeeze the retroperitoneal organs, there may be compression symptoms, such as ureteral shift, obstruction, urinary retention, but Symptoms caused by ureteral lesions can not be detected until renal insufficiency occurs. Fibrosis can occur in the inferior vena cava, but rarely causes inferior vena cava obstruction. Digestive symptoms are nausea, vomiting, upper abdominal pain or total abdominal pain. Occasionally, gastrointestinal bleeding, constipation or diarrhea can be seen, and the abdominal examination can touch the mass.
2. Mediastinal fibrosis If the mediastinum and anterior mediastinal collagen tissue form a dense fiber bundle, it can compress the aorta, trachea and pericardium, etc., but mainly manifested by the symptoms caused by superior vena cava obstruction, ie, face, neck, upper limb and conjunctiva. Swelling and congestion occurred, resulting in non-depressed edema in these areas and venous dilatation in the neck and upper limbs.
3. Other organ system fibrosis The fibrosis of this disease involves the thyroid gland. Riedel thyroiditis is characterized by varying degrees of asphyxia, dysphagia or anterior cervical discomfort. The thyroid is normal or moderately swollen during physical examination. Large, hard texture, severe adhesion to surrounding tissues, normal or low thyroid function, fibrosis, such as involving the sputum, forming a pseudotumor within the iliac crest, unilateral ocular protrusion can occur.
Examine
Fibrosis syndrome examination
1. Blood routine and erythrocyte sedimentation rate may have mild anemia, erythrocyte sedimentation rate, and individual cases may have sickle cell anemia.
2. Some cases of urine may have pyuria and leukocytosis.
3. Biochemical tests often have BUN, Cr rises, and there may be biochemical changes in renal insufficiency such as elevated blood potassium.
4. Immunological examination of patients with primary biliary cirrhosis can be seen with high titers of anti-mitochondrial antibodies, anti-liver specific protein antibodies, anti-smooth muscle antibodies, anti-nuclear antibodies, anti-dsDNA antibodies, etc., blood immunoglobulins It is an increase in IgM.
5. X-ray examination of retroperitoneal fibrosis can only be diagnosed by intravenous pyelography or retrograde pyelography. The pyelography is characterized by ureter-pyel hydrops (either bilateral or unilateral), the ureter deviates to the medial, and the renal pelvis expands. External ureteral compression signs, when there is upper mediastinal fibrosis, angiography can show obstruction of the superior vena cava and its branches.
Diagnosis
Diagnosis and differentiation of fibrotic syndrome
Due to the lack of localization and specific clinical features, the diagnosis is difficult. Only by pyelography or superior vena cava angiography can the initial diagnosis be made, and then laparotomy or open chest biopsy can confirm the diagnosis.
Differential diagnosis
This syndrome needs to be differentiated from Budd-Chiari syndrome and ankylosing spondylitis.
1. Bu-Ka syndrome is the upper varicose veins of the trunk caused by intrahepatic vein occlusion, abdominal pain, ascites, varicose veins under the xiphoid, no varicose veins in the neck and upper extremities, hepatic venography shows hepatic vein occlusion .
2. Ankylosing spondylitis is characterized by lower back pain. The lower back is stiff in the morning, the lesions gradually develop upwards, and finally the kyphosis is straight. X-ray examination shows that the ankle joint gap disappears, the anterior longitudinal ligament is calcified, and the vertebral body bone Loose, finally appeared in the bamboo-like spine, no abnormal findings in intravenous pyelography.
