Pediatric Wegener's Granulomatosis
Introduction
Introduction to pediatric Wegener's granulomatosis Pediatric Wegener's granulomatosis is a systemic disease characterized by more than one type, lower respiratory tract necrosis, granulomatous vasculitis, glomerulonephritis and vasculitis of other organs. The lesion involves small arteries, veins and capillaries. Clinically, it is often characterized by nasal and sinusitis, lung disease, and progressive renal failure. It was once considered a variant of nodular polyarteritis. basic knowledge Sickness ratio: 1-3% Susceptible people: children Mode of infection: non-infectious Complications: hemoptysis, renal insufficiency, convulsions in children, hemiplegia
Cause
Pediatric Wegener's granulomatosis
Causes
The cause of pediatric Wegener's granulomatosis is presumed to be related to autoimmunity and allergic reactions to unknown antigens. Since most patients have upper respiratory symptoms first, followed by 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 cause a whole disease.
Pathogenesis
The main pathological changes in children with Wegener's granulomatosis are characterized by necrotizing granulomas in the affected organs, including the nasal and sinus mucosa, the lower respiratory tract and the skin. Pulmonary infiltration and pulmonary hemorrhage can occur in the lungs. There are many types of vasculitis, such as segmental necrotizing vasculitis or pulmonary capillary vasculitis. The lesion invades the small arteries, venules, capillaries and surrounding tissues. Renal pathological changes were focal proliferative glomerulonephritis and necrotizing crescentic glomerulonephritis.
Pediatric Wegener's granuloma is involved in multiple organs, but most of them are initially affected by upper respiratory tract, such as rhinitis and sinusitis, followed by glomerulonephritis. This suggests that the virulence factor first enters the respiratory tract, induces an inflammatory response, and gradually expands to other tissues with vasculitis. There may be necrotizing granulomatous lesions in the upper or lower part of the respiratory tract, fibrosis of the small vessel wall, mononuclear cells in the whole layer, infiltration of epithelioid cells and multinucleated giant cells, and invasion of bone may cause damage in the lungs. The lesion may have a cavity, and the granuloma is also found in the maxilla, the ethmoid eyelid, and the like. Infarction and ulcer caused by extensive vasculitis cause saddle nose deformity and eyeball protrusion.
Renal lesions are characterized by necrotizing glomerulonephritis, which progresses to proliferative glomerulonephritis, fibrinoid degeneration and necrosis of early glomerular plexus, followed by vascular plexus and cystic epithelial hyperplasia and granuloma formation. Late renal failure shows sclerosing changes.
Systemic focal necrotizing vasculitis, mainly invading small arteries, arterioles, venules, capillaries and surrounding tissues, vascular cells infiltrating polymorphonuclear cells, fibrin-like degeneration, muscle layer and elastic fiber destruction, lumen The formation of thrombus, followed by tube wall necrosis, causes bleeding and the formation of small aneurysms. In addition to lung, extrarenal, skin, cardiovascular, digestive, nervous and other systems can be affected.
The pathology of pediatric Wegener's granulomatosis is vasculitis and non-infectious granuloma, which is effective for immunosuppressive agents. Patients have hypergammaglobulinemia and circulating immune complexes, and immunoglobulin deposits on the capillary wall. Autoimmune disease. Since active patients have been confirmed to have anti-neutrophil cytoplasmic antibodies (ANCA), cytoplasmic staining ANCA (cytoplasmic ANCA, c-ANCA), an anti-protease 3 (PR3) antibody, is highly specific to the disease ( 90% to 97%), related to the disease activity. After the disease is relieved, the c-ANCA titer decreases or disappears, and reappears when recurring.
Studies have shown that Wegner granulomatosis may be an abnormal hypersensitivity mediated by T cells involving one or more different immunopathological processes. Its pathogenesis mainly includes: 1 immune complex-mediated damage; 2 anti-endothelial cell antibody-mediated damage; 3 cell-mediated damage; 4 anti-lysosomal antibody-mediated damage. These four mechanisms may occur to some extent in Wegner's granulomatosis.
Prevention
Pediatric Wegener granuloma prevention
The occurrence of this disease may be related to drug allergies, micro-virus infections, etc., and should be actively prevented, especially the prevention and treatment of respiratory diseases, especially the symptoms of rhinitis in children should not be ignored.
The incidence of this disease is low, it is very common, easy to misdiagnosis and missed diagnosis. The annual incidence of Wegener's granulomatosis ranges from 0.5 to 8.5 parts per million. The symptoms of suspected granulomatosis should be carefully investigated.
It is necessary to clear away heat and detoxify, promote blood circulation and remove blood stasis. Qi and blood are weak, blood stasis and obstruction, Yiyiqi nourishing blood, phlegm and evil collaterals, qi stagnation and blood stasis, qi and blood circulation. Liver and kidney yin deficiency, liver yang sputum, should be yang yang yang, blood circulation.
Complication
Pediatric Wegener granulomatous complications Complications, hemoptysis, renal insufficiency, convulsions, hemiplegia
Can be complicated by hemoptysis, renal insufficiency, organic brain syndrome, mental symptoms, hemianopia, convulsions and hemiplegia, joint swelling and spleen swelling.
Symptom
Pediatric Wegener's granulomatosis symptoms Common symptoms Nasal mucosal ulcer movement disorders Sensory disorder Hematuria chest pain Shortness of eyelids drooping nasal congestion Uveitis ecchymosis
Both men and women can occur, male: female is 3:2. The disease is characterized by sinusitis, lung infiltration, and triad of kidney disease. In the early stages of the disease, children often have non-specific systemic symptoms such as fever, fatigue, weight loss, muscle pain and joint pain. Many children also have some seasonal allergies. The upper respiratory symptoms then appear.
Respiratory symptoms: The most common nasal symptoms. 90% of patients have persistent chronic rhinitis or paranasal sinusitis, nasal congestion, sinus pain, purulent sputum and nosebleeds, which can invade the nasal septum, and even extend to the sputum, tongue, throat, throat, etc., ulceration. Inflammatory spread of the throat and tracheal obstruction, nasal and nasopharynx inflammation, can cause obstruction of the eustachian tube, otitis media, hearing impairment. When the lower respiratory tract spreads, the lungs are affected by cough and hemoptysis.
Lung involvement: 70% to 80% of patients with lung lesions, cough, hemoptysis, chest pain, shortness of breath, occasional massive hemoptysis, pneumothorax, and a large number of pleural effusion and atelectasis. About 34% of patients have migraine or multiple lung lesions. X-ray examination shows middle and lower lung nodules and infiltration, some are hollow, and some diameters can range from a few centimeters to small nodules, the number can be from 1 to 2 To multiple. Pleural effusion was seen in 20%, and lung function tests showed decreased lung capacity and diffuse function.
Skin manifestations: Half of the patients have skin manifestations, more common in the lower limbs, but the upper limbs, trunk and face can also be involved. Skin lesions have inflammation and necrotic nodules, fulminant purpura and gangrene, purpura lesions and nodules can also progress to ulcers, which form multiple necrotizing vasculitis secondary to skin blood vessels, occasionally fingertips Raynaud phenomenon.
Nervous system performance: 25% to 50% of patients may have neurological damage, polyneuritis, motor and sensory neurological disorders. Can also be caused by nasal or sinus granulomatosis invading adjacent nerve tissue causing ptosis, ophthalmoplegia: involving the posterior pituitary to cause diabetes insipidus. A few are epileptic seizures or mental disorders.
Examine
Pediatric Wegener's granuloma examination
There may be anemia, white blood cells and thrombocytosis. ESR is accelerating. C-reactive protein is increased. Urine routine examination showed proteinuria, hematuria and red blood cell casts. In patients with renal failure, blood urea nitrogen and creatinine increased. Creatinine clearance, qualitative urinary chemistry, urinary red blood cell and tubular sedimentation tests can be used to diagnose and follow up patients with glomerulonephritis.
X-ray examination showed multiple lung lesions, middle and lower lung nodules and non-specific interstitial infiltration, some were hollow, solitary masses, etc. Some diameters ranged from a few centimeters to small nodules, the number can be from 1 ~ 2 to multiple. Similar to pneumonia, tuberculosis, lung cancer, etc., pleural effusion can be seen, and the bronchial layer shows stenosis of the trachea or bronchi. The upper respiratory tract X-ray shows thickening of the sinus mucosa and bone destruction of the nose and sinuses.
Diagnosis
Diagnosis and diagnosis of pediatric Wegener's granuloma
According to the clinically severe sinusitis and lung X-ray photographs, there are nodular shadows or urinary examination with proteinuria. In hematuria, the disease should be highly suspected. The diagnosis is based on c-ANCA positive and Sinus, lung and kidney biopsy were further confirmed.
Diagnostic criteria: The clinical manifestations of the disease are divided into two categories:
Main performance
1 kidney damage;
2 Musculoskeletal changes.
2. There are 10 minor performances.
1 skin symptoms;
2 digestive tract symptoms;
3 peripheral neuropathy;
4 central nervous system changes;
5 hypertension;
6 heart involvement;
7 systemic symptoms;
8 lung involvement;
9 acute phase reactants positive, such as accelerated erythrocyte sedimentation rate, increased C-reactive protein;
10 hepatitis B surface antigen positive.
3. Histological changes with typical arteritis (full-thickness vasculitis with elastic fiber rupture) or angiography showing characteristic aneurysms.
5 of the above-mentioned performances, including at least one of the main manifestations, can be highly suspected of nodular polyarteritis; if there is evidence of histological changes, the disease can be diagnosed, and highly suspected cases, especially kidneys The affected person should start treatment at the same time as the biopsy.
Differential diagnosis
The disease must be differentiated from sarcoidosis and tuberculosis. Both c-ANCA are negative and must be differentiated from allergic granuloma. The latter may also have chronic sinusitis, but the patient has a history of asthma and peripheral blood. Eosinophilia, without respiratory destructive lesions, in addition to pulmonary hemorrhagic nephritis syndrome, systemic lupus erythematosus and nodular polyarteritis.
