sarcoidosis in children
Introduction
Introduction to pediatric sarcoidosis Sarcoidosis, also known as sarcoidosis, is an unexplained cause of granulomatous lesions in various organs such as the lungs, joints, lymph nodes, skin, eyes, liver, spleen, muscles and brain. basic knowledge The proportion of illness: 0.001% Susceptible people: children Mode of infection: non-infectious Complications: bullae, pneumothorax, bronchiectasis, diabetes insipidus, hematuria
Cause
Causes of pediatric sarcoidosis
(1) Causes of the disease
Although the cause is unknown, according to the analysis of most cases, it seems to be related to infection or exposure to toxic substances, but no pathogens were found.
(two) pathogenesis
Normal or enhanced humoral immunity, IgM, IgA and IgG increased, T-helper lymphocytes infiltrated around and within the acute stage granuloma. After the lesion subsided, T lymphocytes decreased, but the remaining lymphocytes were mainly T-suppressor cells.
The pathological feature is chronic granulomatous inflammation without necrosis of cheese. Any organ can be invaded, but the most common lung lesions are seen. Most miliary granulomas can be seen in both lungs, ranging from a few millimeters to 1-2 cm. Sexual granuloma, epithelioid cells in the center of the lesion, macrophages and multinucleated giant cells (Langerhans giant cells), surrounded by monocytes, lymphocytes and fibroblasts infiltrating, lymphocytes and giants in granulomas Phagocytes can release various mediators such as IL-1, IL-2, interferon and other cytokines to maintain this granuloma. In the active stage of the disease, the lymphocytes in and around the granuloma are mostly auxiliary T (CD4+). After the lesion subsides, it can be restored to normal tissue, but about 20% of the fibrosis occurs around the granuloma. The lung lesions can be seen in the peripheral part of the lung parenchyma. The macrophages in the granuloma of the disease can produce 1,25. -(OH)2-D3, such a substance having vitamin D2 activity is generally produced by the kidney, and hypercalcemia and hypercalciuria are seen in the disease due to excessive production of active vitamin D.
Prevention
Pediatric sarcoidosis prevention
The cause is unknown, and the focus should be on preventing infection and preventing exposure to toxic substances.
Complication
Pediatric sarcoidosis complications Complications, bullous, pneumothorax, bronchiectasis, diabetes insipidus, hematuria
Pulmonary bullae may occur in severe pulmonary lesions, pneumothorax and bronchiectasis may develop into fibrosis, causing pulmonary insufficiency and heart failure, ocular lesions may cause severe visual impairment, complicated with diabetes insipidus, and Adison ( Addison) syndrome, occasionally proteinuria, pyuria, hematuria, hypercalcemia, renal failure and so on.
Symptom
Symptoms of pediatric sarcoidosis Common symptoms Uveitis, parotid gland swelling, joint pain, weight loss, rash, urinary arrhythmia, intrapulmonary lesions, heart failure, synovial thickening
Symptoms vary greatly depending on the affected organ, slow onset, cough, fatigue, weight loss, bone and joint pain, etc., mostly due to lymphadenopathy, parotid swelling, rash or iritis, uveitis For the visit, the following symptoms can be seen depending on the affected organ:
1. Infiltrating the lungs in childhood mainly affects the lungs. The symptoms are not obvious at the onset. Cough can occur. Most of the lesions are found on X-ray examination. The lymph nodes around the hilar and trachea are swollen, and miliary nodules can be seen in the lungs. Dot-like shadows are sometimes difficult to distinguish from miliary tuberculosis. When granuloma is swollen in the alveoli, it may appear as flocculent shadows. Occasionally, chest pain may occur. When the hilar lymph nodes are swollen, there may be shortness of breath. In severe cases, the lungs may have large lungs. Blisters, pneumothorax and bronchiectasis, the above lesions can be naturally absorbed, and some can develop into fibrosis. In some cases, lung function is extensively destroyed, causing pulmonary insufficiency and heart failure.
2. Lymph node enlargement In addition to mediastinal lymphadenopathy, there may be superficial lymph nodes enlargement, no tenderness, no adhesion, increased liver and spleen, and parotid swelling is a prominent symptom.
3. About 1/3 of patients with rash have special skin changes, more common in children under 4 years old, generally more severe, showing a variety of sarcomatoid granuloma, more common in the face, children with such a rash has a poor prognosis, The rash may be waxy miliary nodules or larger papules, and some may be mossy, raised or flat smooth purple-red macules or papules, up to 1 cm in diameter, and nodular erythema is common. Especially seen in girls, often early symptoms of this disease, rash can appear itching.
4. Eye symptoms Eye lesions can cause severe visual impairment, keratitis, iritis, iridocyclitis, uveitis (ophthalmitis) and glaucoma are more common, occasional retinitis or retinal hemorrhage, lacrimal gland Even if it is violated, there may be many symptoms such as tears, which may eventually cause uveal fibrosis and cause visual impairment.
5. Salivary gland enlargement A specific symptom of this disease is the painless enlargement of the parotid gland and other salivary glands, combined with uveitis, facial nerve palsy and fever, and is known as "the uveal parotid fever".
6. Skeletal and joint lesions Skeletal changes are more common in the hands and feet, but very common symptoms, mild pain and limited movement, X-ray film showed multiple cystic changes, joint synovial thickening, tendon sheath involvement, but no bone Loose changes, unlike rheumatoid arthritis, infants under 1 year of age rarely develop, but the main symptoms are arthritis, rash and eye symptoms, much like rheumatoid arthritis.
7. Other symptoms such as invading the myocardium or pericardium may have corresponding symptoms. In addition to pulmonary heart disease, common arrhythmia, such as muscle aggression, may appear muscle atrophy or pseudohypertrophy, occasionally neurological symptoms appear facial paralysis, also It may involve endocrine glands, such as diabetes invagination invading the pituitary; Addison syndrome may occur in the adrenal cortex, and renal disease is rare in children with sarcoidosis. Occasionally, proteinuria, pyuria, hematuria Or intermittent occurrence of diabetes and granular casts, some patients with hypercalcemia, the severity and duration of which may have a relationship with renal function, renal failure with hypercalcemia, a small number of sick children have nasal congestion, Deaf, deafness and nocturia.
Examine
Examination of pediatric sarcoidosis
Most of the findings in the laboratory are non-specific, high proteinemia, albumin to globulin ratio decreased, erythrocyte sedimentation rate increased, peripheral blood eosinophils increased, urinary calcium increased, occasionally increased blood calcium, plasma angiotensin converting enzyme (ACE) increased concentration, etc.
About 40% to 60% of children with X-ray can be seen with hilar lymphadenopathy, or with increased lung texture, chest TV can be seen in general X-ray can not be found in hilar lymphadenopathy.
Diagnosis
Diagnosis and diagnosis of pediatric sarcoidosis
According to the clinical symptoms and X-ray examination of multiple organ infiltration, if there are pulmonary symptoms and rash, ophthalmitis or arthritis should be highly suspected of this disease, lymph nodes, rash and other biopsy should be done for suspicious cases, if you see non- Case-like granuloma can be diagnosed. If the lung symptoms are mainly open chest biopsy or tracheal lung biopsy, bronchoalveolar lavage, even if the lung lesions are not serious, a large number of lymphocytes can be seen in the lavage fluid, mainly T lymphocytes, in recent years, lung scans can be found in chest lesions.
Similar chronic diseases such as tuberculosis, Hodgkin's and non-Hodgkin's lymphoma, connective tissue disease, hepatitis, and histoplasmosis should be excluded, and ocular symptoms should be differentiated from herpetic conjunctivitis.
Lymph nodes around the hilar and trachea, miliary nodules or reticular shadows in the lungs, sometimes difficult to identify with miliary tuberculosis, the main symptoms of infants under 1 year old are arthritis, rash and eye symptoms, much like rheumatoid Arthritis should be noted for identification.
