Kaplan syndrome
Introduction
Introduction to Kaplan Syndrome Caplan syndrome is also known as rheumatoid pneumoconiosis syndrome, rheumatoid arthritis - pneumoconiosis syndrome, and silicon deposition disease. In 1953, Caplan of the United Kingdom found that pneumoconiosis coal mine workers with rheumatoid arthritis had specific lung shadows, and later people called the disease Kaplan syndrome. Later, it has been confirmed that inhaled free silicic acid, other inorganic dusts such as silicate, iron, aluminum, etc. can also produce this syndrome. At present, silicon deposition disease combined with rheumatoid arthritis and characteristic shadows appear in the lungs are called Kaplan syndrome. basic knowledge The proportion of illness: 0.001% - 0.002% Susceptible people: no specific population Mode of infection: non-infectious Complications: hemoptysis pulmonary hypertension
Cause
Cause of Kaplan Syndrome
(1) Causes of the disease
It is not yet clear, considering the following factors:
Immune abnormality
Pulmonary nodules are rare in patients with rheumatoid arthritis who do not have silicosis. According to 28 patients reported by Walker (1966) in 12 years, pneumoconiosis has a certain promoting effect on pulmonary nodules forming rheumatoid joints.
2. Genetic factors Miall (1955)
An epidemiological survey of men over the age of 15 found that genetic quality plays an important role in the pathogenesis of rheumatoid arthritis and Kaplan syndrome.
(two) pathogenesis
1. Silicic acid deposited in the lungs can sensitize antibodies for a long time, leading to an adjuvant disease, which can promote immune response or is confined to the lungs and cause lung damage in rheumatoid arthritis, so It is believed that the silicosis adjuvant disease is caused by an immune abnormality, which may be caused by the release of antigenic substances by macrophages damaged by dust and the combination of rheumatoid arthritis and rheumatoid arthritis.
2. In recent years, due to the development of immunogenetics, the related role of HLA and disease susceptibility has been paid attention to. According to Wagner et al. (1979), the positive rate of HLA-BW45 in RF-positive Kaplan syndrome is significantly higher than that in the control group. The RF-negative Kaplan syndrome is negative for HLA-BW45.
3. Pathology Under the microscope, the central part of the lung lesion is composed of necrotic collagen fibers and dust.
From the center, there are active inflammatory layers composed of macrophages and multinucleated white blood cells, a grid of fibroblasts, a layer of infiltrates and connective tissue composed of numerous lymphocytes and plasma cells, and an occlusive endarteritis. There are many lymphocytes in the lumen, plasma cells and macrophages that disintegrate dust and phagocytosis. Black concentric rings can be seen by the naked eye. Calcification or cavities can occur in round lung damage. Pathologically, Kaplan syndrome and silicon The identification of progressive bulk fibrosis caused by stagnation is that the latter has no active inflammatory layer and rheumatoid arteritis, which is distinguished from silicon tuberculosis tuberculosis in that the former has active inflammatory layers and fibroblasts. Grid-like layer, but no tuberculosis can be found in the active inflammatory layer.
In addition to dust deposition, lung lesions of this syndrome have small intrapulmonary nodules similar to those of rheumatoid subcutaneous nodules.
Prevention
Kaplan syndrome prevention
Primary prevention
1 Do a good job in labor protection to avoid the invasion of dust, wind, cold and dampness.
2 Strengthen physical exercise, strengthen nutrition, regular life, feel comfortable, and improve autoimmune function.
3 Because the incidence of many patients has a certain relationship with bacterial or viral infections, timely and effective control of infection is an important means of preventing RA.
2. Secondary prevention
At present, there is no cure. The principle of treatment is to perform planned functional exercise on the joints under the condition of drug control pain, to prevent joint deformity and muscle atrophy. Immunosuppressive agents and surgical treatment also have certain effects on some patients.
(1) drug treatment: the treatment of rheumatoid arthritis (RA) should be personalized, according to different patients' different conditions, functional status and prognosis to implement different treatment options, and emphasize the early use of second-line drugs, commonly used drugs There are antibiotics, immunosuppressive agents, anti-inflammatory analgesics and related Chinese medicines.
(2) Exercise therapy: It is an important part of RA preventive intervention. The benefits of exercise therapy for RA patients include: increasing physical strength, improving local muscle endurance, increasing or maintaining joint mobility, increasing aerobic activity, and improving activity. Conditions, reduce the degree of disease activity, reduce the degree of pain, improve mood, improve sleep, reduce weight, the prescription of RA exercise therapy varies from person to person, graded, can not be uniform, see Table 2.
(3) Physical therapy: generally use superficial heat (hot compress, moist heat, wax therapy, 38 ~ 40 °C hot water local bath, Chinese medicine hot wash, hot compress), each time about 20min, if RA has peripheral nerve involvement or muscle Fascial pain, can be used to relieve pain by transcutaneous electrical nerve stimulation.
(4) Traditional Chinese medicine therapy:
1 Acupuncture: It has been reported that acupuncture can reduce the pain of patients with RA, reduce the erythrocyte sedimentation rate, and help the function to improve, but there are also invalid cases. Some scholars recommend refractory RA and have not received acupuncture treatment before. Trial treatment should be carried out at a professionally qualified acupuncturist;
2 massage: acupuncture massage as analgesic, reducing the muscle tension of the adjuvant therapy, is quite common in clinical;
3 Qigong: practice relaxation, and add benign self-suggestion to relieve pain and restore health, relax physical and mental relaxation, relieve pain symptoms, improve self-perception, and save energy consumption, cultivate vitality and enhance physical fitness;
4 Tai Chi Chuan: Simplify Tai Chi, even use only some of the simple movements to practice, pay attention not only to practice, but also to emphasize the practice of mind, in order to facilitate relaxation and training to calm the mind and cultivation, to promote spiritual health Whole body health.
(5) Relaxation therapy and mental health: Relaxation of mind and body helps to alleviate the pain of self-perception and reaction. Studies have shown that using Chinese-style relaxation therapy or using EMG biofeedback to guide muscle relaxation and mental relaxation can alleviate pain. In recent years, it has been reported that after patients with RA have appreciative music, their joint pain threshold is increased, and pain can be alleviated. If the pain or condition of RA patients is repeated and affects mental and psychological state, and depression, anxiety, etc., it is appropriate to use psychological counseling. "Cognitive-behavioral" treatment can relieve the pain of self-perception, change the feeling of pain, and improve the level of good feeling for the body and health.
(6) Surgical treatment:
1 synovectomy.
2 joint cleansing.
3. Three levels of prevention
Patients with functional status listed in grade 3 or 4 RA should be treated with orthoses and assistive devices, or orthopedic surgery to improve joint deformity. For patients with severe joint damage and joint dysfunction in the later stage, the condition is stable and selective. Artificial joint replacement is used to reconstruct joint function.
Complication
Complications of Kaplan syndrome Complications hemoptysis pulmonary hypertension
Can be complicated by hemoptysis, difficulty breathing, chest pain, pulmonary hypertension, right ventricular hypertrophy and right heart failure.
Symptom
Symptoms of Kaplan Syndrome Common Symptoms Joint swelling, difficulty breathing, rheumatoid arthritis, hemoptysis
1. The respiratory system has cough, coughing black or brown sputum, hemoptysis, difficulty breathing, chest pain.
2. The bones and joints may have symptoms and signs of migratory joint swelling and pain or other rheumatoid arthritis before or after pathological changes in the lungs. In some cases, joint symptoms may not be obvious.
Examine
Kaplan syndrome check
Immunological examination showed that rheumatoid factor was positive in 65% of cases, serum albumin decreased, 2 and gamma globulin increased, -globulin increased with the progression of the disease, and IgG and IgM increased.
Chest X-ray examination has characteristic shadows, and the lesions can be single or multiple, which are round or elliptical dense shadows, with clear edges and different sizes. The diameter is 0.5-1.5cm, and occasionally 3~5cm. Often in the middle and outer zone of the middle and lower lung fields, multiple lesions resemble metastases, but central necrosis forms thin-walled cavities, which generally have no fluid level, and a few may have calcification.
Diagnosis
Diagnosis and differentiation of Kaplan syndrome
Diagnosis According to the patient's occupation, there is a history of dust exposure, respiratory system performance such as difficulty breathing, characteristic X-ray changes, joint symptoms and rheumatoid factor positive, etc., can be clearly diagnosed.
It should be identified mainly with progressive bulk fibrosis (PMF) of silicon deposition disease, and the chest X-ray performance is different.
In addition, attention should be paid to the identification of tuberculosis, but pneumoconiosis can also be combined with tuberculosis. The symptoms of tuberculosis infection in this patient such as fever, weight loss and hemoptysis may not be obvious. It is necessary to pay attention to finding tuberculosis in the sputum.
