Wegener's granulomatosis
Introduction
Introduction to Wegener's granuloma Wegenersgranulomatosis is characterized by progressive necrotizing granuloma and extensive small vasculitis, which mainly affects the respiratory tract, kidney, skin and other organs to produce corresponding clinical manifestations. basic knowledge The proportion of illness: 0.005% Susceptible population: age of onset is 30 to 50 years old Mode of infection: non-infectious Complications: staphylococcal infection
Cause
Wegener granulomatosis
It is not clear that since most patients have upper respiratory symptoms and glomerulonephritis, some people think that the slow-separated protein after upper respiratory tract infection can become an allergen, causing the body to develop an allergic reaction and a whole disease. Long-term survival was reported with trimethoprim (TMP) and iodomethylisoxazole (SMZ), suggesting that the disease is associated with microbial infections, and half of the cases are positive for rheumatoid factor, with high gamma globulinemia and circulation. Immune complex related, and there are cellular immune interventions in autoimmune diseases. Many data have found that patients with active Wegener granulomatosis have anti-leukocyte autoantibodies, anti-SSA and anti-SSB antibodies, treated with immunosuppressive agents, when the disease is relieved, Serum anti-neutrophil cytoplasmic antibody (ANCA) can disappear and reappear when the disease recurs, indicating that ANCA is related to the pathogenesis of this disease. A few cases have finally developed into malignant lymphoma, so whether the disease is a malignant tumor The response has yet to be further explored.
Prevention
Wegener granuloma prevention
First, level prevention
1. Strengthen nutrition and enhance physical fitness.
2. Prevent and control infection and improve autoimmune function.
3. Avoid wind and cold, avoid too tired, avoid alcohol and tobacco, avoid spicy food.
4. Protect the eyes and nose during outdoor activities.
Second, secondary prevention
Early diagnosis, understanding of the nose and nose infection, good clinical observation, early detection of damage to various systems, early treatment, mainly control of the eye, nasal infection.
Third and third level prevention
Pay attention to lung, kidney, heart and skin lesions, and pay attention to the occurrence of secondary Staphylococcus aureus infection. In addition, the nervous system and digestive system may also be involved. The application of traditional Chinese medicine may have the effects of regulating immunity, clearing away heat and detoxifying, promoting blood circulation and removing blood stasis. .
Complication
Wegener granulomatous complications Complications staphylococcal infection
The main complication of this disease is Staphylococcus aureus infection.
Symptom
Wegener's granulomatosis symptoms Common symptoms Chest pain nasal mucosa ulcer uveitis eyelid droop keratitis no urinary nephrotic syndrome nasal septum perforation nasal congestion sensory disorder
Both men and women can occur, male: female is 3:2, more than half of the age of onset is 30 to 50 years old, most patients have fever, weight loss, fatigue and joint pain, myalgia, etc., typical patients have upper respiratory fatigue, lung and Three symptoms of kidney disease.
1. At the beginning of the disease, there are often persistent rhinitis or sinusitis symptoms of upper respiratory tract, such as nasal overflow, purulent sputum, nasal discharge or even no necrotic tissue. Rough and uneven granules on the nasal septum and nasal septum are attached with scars. Severe nasal septum perforation, nasal bone destruction to form saddle nose, a small number of cases of acute throat obstruction, lower respiratory symptoms, such as cough, small amount of hemoptysis, often secondary bacterial infection, severe cases can occur a large number of alveolar hemorrhage, Difficulty breathing or even respiratory failure.
2. Kidney damage is almost common in all cases. Proteinuria, red blood cells and tubular urine usually appear within half a year after the onset of the disease. When the condition worsens, it is accompanied by high blood pressure, which can lead to renal failure. If there is no kidney disease, the disease cannot be ruled out.
3. Skin and mucous membrane damage is found in 60% of cases, manifested as purpura, hemorrhagic herpes, nodules, invasive plaques and ulcers, etc., 1/4 of cases of lesions appear in the early stages of the disease, manifested as necrotizing papules and blisters, often Symmetrically distributed in the extremities and buttocks, damage to the anterior pyoderma can sometimes be used as an early manifestation.
4. Eye damage accounts for about 20% to 60% of cases, which may be caused by granuloma and vasculitis, including keratoconjunctivitis, corneal scleral ulcer, granulomatous scleritis, optic nerve vasculitis, retinal arteritis, Eyeballs and other protrusions.
5. Ear damage accounted for about 1/4 of the cases, due to granulomatosis caused by the middle ear cavity, sinus sinus destruction, nasal ulcers, eustachian tube occlusion caused by exudative or suppurative otitis media, hearing loss, dizziness, etc. .
6. The nervous system lesions account for about 1/5. The vasculitis of the nervous system is the main cause of symptoms, including polyneuritis, motor-induced neurological disorders, etc. It can also be caused by nasal or sinus granulomatosis invading adjacent nerve tissue, causing ptosis. Ophthalmoplegia: involving the posterior pituitary gland to cause diabetes insipidus.
7. The cardiovascular system is affected by about 15%, which is manifested as pericarditis, myocarditis, arrhythmia, etc. In the advanced stage, hypertension and heart failure may occur.
8. Digestive system affected, manifested as oral cavity, intestinal mucosa size, different depths of ulcers, abdominal pain, diarrhea, blood in the stool, occasional mumps, hepatitis, pancreatitis reported, the disease is often progressive development The prognosis is poor. The early respiratory symptoms begin. After the systemic system is involved, the kidneys are the most common. They are usually issued within half a year. Once renal failure occurs, it is the main cause of death. Therefore, before the patient has renal damage. Early treatment with active treatment is the key to delaying the course of the disease. A small number of patients can only affect the lungs or skin, the gastrointestinal tract, lack of glomerulonephritis and upper respiratory tract lesions, and are limited to Wegener's granulomatosis, which is benign and typical. The relationship between intragranular granuloma is unclear.
Examine
Wegener's granuloma examination
White blood cell
Eosinophils often increase, chronic renal insufficiency often have small cell anemia, increased erythrocyte sedimentation rate, especially when the system is involved, urine protein appears, red blood cells suggest kidney involvement, renal function and kidney damage The lesions were consistent, some cases were positive for rheumatoid factor, r-globulin increased, circulating immune complexes increased, serum complement was normal or slightly elevated, anti-SSA, SSB antibody, anti-smooth muscle antibody positive.
In recent years, many literatures have reported that serum anti-neutrophil granule antibody (ANCA) is a specific antibody for this disease by indirect immunofluorescence using neutrophils as an antigen substrate. It is bright, coarse-grained, and active. The sensitivity of the period is 70% to 100%, and the specificity is 86%, which can be used as an indicator for diagnosing the disease and monitoring activity.
2. X-ray inspection
Chest radiograph shows multiple lung lesions, mostly non-specific interstitial infiltration in the early stage, followed by invasive, nodular, or even hollow lesions, solitary masses, etc., similar to pneumonia, tuberculosis, lung cancer, etc., a small number of patients may be due to granulation The ablation of the airway forms atelectasis, the bronchial layer shows stenosis of the trachea or bronchi, the upper respiratory tract shows thickening of the sinus mucosa, and even the bone and sinus bone destruction.
3. Pathological examination
Upper airway lesion biopsy can show vasculitis or necrotizing granuloma, negative biopsy and exclude the disease, repeated examination can increase the positive rate, renal biopsy can be seen in some cases of glomerular focal, segmental, necrotic glomerulus Nephritis, where fibrin deposits are often found in areas of active damage and necrosis.
Diagnosis
Diagnosis and diagnosis of Wegener's granuloma
diagnosis
The diagnosis is based on a combination of clinical and histopathological examinations:
1. A necrotizing granuloma occurs in the upper or lower respiratory tract.
2. Lung, the skin has primary focal necrotizing vasculitis.
3. Focal necrotizing tubular nephritis.
In order to be able to diagnose early, check the following conditions and repeat the pathological examination if necessary:
1. Chronic rhinitis and / or sinusitis with mucosal erosion or granulation tissue hyperplasia.
2. Eye, oral mucosal ulcer, necrosis or granuloma.
3. There are variable nodular shadows or holes in the lungs.
4. The skin has purpura, nodular invasive plaque, necrosis, ulcers and so on.
The standards proposed by the American College of Rheumatology in 1990 are available for reference: 1 nasal or stomatitis (painful or painless oral ulcers, bloody nasal secretions), 2 chest radiographs showing nodules, fixed pulmonary infiltration or cavity formation, 3 urine Liquid microscopic hematuria (red blood cells > 5 / high power field) or red blood cell cast, 4 tissue thrombus showing arterial wall or arteries and small arteries around the tissue and granulomatous inflammation changes, in line with the above two or more criteria, can be diagnosed as Wegener's granuloma has a sensitivity of 88.2% and a specificity of 92%.
Differential diagnosis
What needs to be diagnosed is:
1. Nodular polyarteritis, mainly segmental involvement of small and medium arteries with inflammatory and necrotic lesions, no granulomatous lesions, multiple organs and organs, clinical manifestations, often subcutaneous nodules, hypertension, abdomen Symptoms, early kidney damage, and respiratory fatigue are often not affected.
2. Lymphomatoid granulomatosis, systemic vascular infiltration and vascular central necrotizing granulomatosis, the upper respiratory tract is often not involved, the lesion mainly involves the lung, skin, nerve and renal interstitial, lymphocytes, plasma cells Tissue cells, atypical lymphocytes infiltrate, although granulomatous vasculitis, but granulomatous damage is often not obvious, vasculitis is also atypical leukocyte disruption or fibrin-like necrosis.
3. Midline malignant reticulosis, a destructive lesion of the nose and face, usually does not involve the lungs, pathologically mainly coagulative necrosis, pleomorphic cell infiltration, also visible atypical lymphocytes, without vasculitis And granuloma.
4. Pulmonary hemorrhagic nephritis syndrome (Goodpasture syndrome), kidney and lung biopsy immunofluorescence antibody method can detect anti-kidney small basement membrane antibody, and can detect circulating anti-GBM antibody, can be differentiated from Wegener granuloma.
