osteitis deformity
Introduction
Introduction to malformation osteitis Osteostatitis (osteitis deformans) is a chronic progressive bone disease, with local bone tissue osteoclast and osteogenesis, bone resorption and reconstruction, osteoporosis and calcification coexist as pathological features, the disease is unknown, chronic Focal bone remodeling is abnormal. At first, the bone resorption in the lesion is increased, and then the compensatory new bone formation is increased, so that the woven bone and lamellar bone are embedded in the lesion, resulting in disordered bone structure, thickening of the bone, and fragility of the bone. And the blood vessels in the bone increase. Clinical manifestations of bone pain, bone deformity and fracture, the disease does not directly invade the joint, but bone deformity can cause secondary joint disease. basic knowledge The proportion of illness: the incidence rate is about 0.0002%-0.0005% Susceptible people: no special people Mode of infection: non-infectious Complications: primary hyperparathyroidism osteosarcoma arthritis depression
Cause
Causes of malformation osteitis
(1) Causes of the disease
The exact cause is unknown. There are data showing that patients have a higher frequency of HLA-DQW1; 15% to 30% have a family history, which is 7 times higher than that of the general population, and patients with positive family history have earlier onset and have a heavier condition; It is suggested that this disease may be related to certain viral infections. Viral shell samples have been found in the osteoclast nucleus and cytoplasm of the pathological part of the patient. According to the shape of the inclusion body, it appears to be a paramyxovirus family; this was found in in vitro studies. The virus can fuse with infected cells and form multinucleated giant cells; IL-6 can regulate this process, but intact virions have not been isolated, indirect immunofluorescence and immunoperoxidase staining have confirmed measles virus antigen in the nucleus and cytoplasm And respiratory syncytial virus antigens, clinical studies have found abnormal collagen in the skin of patients; some patients with retinal angioid streaks, elastic pseudo-yellow tumors and vascular calcification, suggesting that may be related to collagen metabolism, currently There are several kinds of doctrines.
Virus infection (35%):
It was found by electron microscopy that there is an RNA virus nucleocapsid in the cytoplasm and nucleus of osteoclasts in the lesion site. It is speculated that the paramyxovirus infection may be related to the disease. However, people are then in the bone. Similar structures have been found in osteoblasts of giant cell tumors and osteopetrosis. Other studies have found that measles virus and canine distemper virus infection may also be associated with this disease. The relationship between viral infection and malformation osteitis has not been Further confirmation and verification of the animal model were obtained.
Genetic (25%):
15% to 30% of patients have a positive family history, suggesting that the disease has a genetic predisposition. It has been reported that the disease is autosomal dominant, and it has been reported that this disease is associated with HLA-DQw1 antigen, but it has not been widely verified.
(two) pathogenesis
The bone structure and function of the lesion are closely related to the process of the lesion. The lesion process is generally divided into three phases: the early stage is mainly osteolytic, the later stage is mainly osteosclerosis, and the middle stage is a mixed type of two changes.
At the beginning of the lesion, huge multinucleated osteoclasts invade normal bone tissue, more than 50% of osteoclast nuclei of more than 50% of osteoarthritis, more than 20 of 10% of osteoclast nuclei, and some can have more than 100 In normal bone tissue, 50% of the number of osteoclast nuclei exceeds 3, and 10% of the number of osteoclast nuclei exceeds 5, and bone resorption is accelerated due to increased osteoclast activity accompanied by vascular enlargement and myeloplasmic fibrosis; At the same time of bone resorption, osteogenesis also increases compensatory, mainly in the disordered lamellar bone formation (mixing period), and finally only osteogenesis without bone resorption, new bone formation is irregular, arranged disorderly, forming weaving Bone (normal adult does not have braided bone except for fracture repair and high bone transition state), braided bone and lamellar bone inlay, the thickness and direction of the trabecular bone are very irregular, like a pile of chaos, a related to calcium nucleus The analysis showed that the bone conversion rate of the lesion was 46 times higher than that of the normal part.
The above pathological changes lead to an increase in bone fragility in the lesion site, which is prone to fracture.
Prevention
Malformation osteitis prevention
Primary prevention
Primary prevention measures are taken in response to the risk factors for their occurrence and development. In primary prevention, consideration should be given to changing risk factors that have a universal effect, as well as changing risk factors with special effects.
(1) Weight loss: For obese people, weight loss should be achieved by reasonable diet, exercise, etc. The data obtained by Framingham study suggest that obesity loses 5kg, which can reduce the risk of knee osteoarthritis in the next 10 years. %, therefore, weight loss is particularly important for the prevention of knee osteoarthritis.
(2) Prevention of joint damage: In the cultural and sports activities, pay attention to the prevention of joint damage such as shoulder, knee and ankle, so as to avoid increasing the risk of osteoarthritis in these joints in the future, especially to prevent more serious injuries.
(3) Prevention of occupational joint chronic strain: mainly to prevent excessive use of a joint. The method for athletes to avoid joint strain is to change the training method. It is not advisable to apply excessive exercise load to a joint for a long time. Training should be appropriate. Sites and equipment, if necessary, use personal protective equipment. The prevention of occupational strains should take into account the labor of different joint loads as much as possible, so that some joints that have been strained during labor can also be combined with work and rest. In addition, after labor, the overburdened joints are self-massage, home heat therapy (hot water soaking, moist heat, infrared) to improve the local blood circulation of the joints; pay attention to strengthening the joint movement muscle training on weekdays, and maintain the joints with powerful muscles. Stability and reduction of joint load, in general, prevention of chronic strain is not easy, and research needs to be strengthened.
(4) Hormone replacement therapy: hormone replacement therapy is given to menopausal women. It is observed that it has certain effect on prevention of knee osteoarthritis, but the prevention of osteoarthritis in the joint joint is not significant.
(5) Prevention of other diseases: Prevention and active treatment of diabetes and high blood pressure may be helpful in preventing osteoarthritis.
2. Secondary prevention
(1) Early diagnosis: The diagnosis of osteoarthritis is generally not difficult, but some atypical cases should be especially associated with rheumatoid arthritis, seronegative spondyloarthropathy, psoriatic arthritis, reiter syndrome, crystallization. Identification of arthritis and infectious arthritis should also be differentiated from secondary osteoarthritis.
(2) Early treatment:
1Adjust and change lifestyle: This is the most important measure for secondary prevention of osteoarthritis. Its purpose is to reduce the load on the affected joints and reduce or avoid further strain on the affected joints. This is for patients with knee and hip osteoarthritis. It is especially important that when asymptomatic osteoarthritis (only changes in joint structure seen by radiology) or mild symptomatic osteoarthritis is found, the patient should be instructed and asked to change the original inappropriateness. The lifestyle, taking knee osteoarthritis as an example, requires patients to:
A. Reduce the total amount of exercise per day: refers to walking, lower extremity exercise, running, etc., so that the knee and hip joints have a full rest, while avoiding fatigue in the joints and the whole body.
B. Avoid or reduce knee flexion: As above stairs, especially knee flexion will increase the pressure in the knee joint and increase the burden on the knee joint, and stimulate the diseased tissue to cause severe pain, which should be avoided.
C. Adjusting the type of work if necessary: If occupational labor is related to the above two items (the total amount of exercise is large, often knee-squatting, squatting, up and down stairs), the type of work should be adjusted to work on the above two requirements.
D. Reasonable diet: The purpose is to lose weight and lose weight (for obese patients).
2 medical gymnastics: the purpose is to maintain or improve the range of joint movement, increase muscle strength, thereby indirectly reducing joint load and improving the patient's exercise capacity. Recent studies have proved that appropriate medical gymnastics for patients with knee osteoarthritis can improve knee function. And help to relieve pain, the effect is better than single electrotherapy, medical gymnastics includes:
A. Joint gymnastics: maintain or increase joint mobility and prevent joint contracture. This kind of gymnastics should be fully active according to the movement axis of the joint itself (without causing pain), for example, the knee joint should be actively and fully flexed and stretched. motion.
B. Isometric exercises: make the muscles of the same length contraction (static tension, do not cause joint movement), which is an effective way to strengthen muscle strength and prevent disuse muscle atrophy, such as knee osteoarthritis The isometric contraction exercise of the cephalic muscle to strengthen the quadriceps muscle, the isometric contraction lasts 5s each time, and then relaxes and can be repeated 30 to 40 times.
C. Stretching: stretching the muscles and tendons around the joints, preventing contractures, and improving muscle coordination, improving gait in patients with lower extremity osteoarthritis.
D. Endurance exercise: Generally, the bicycle is fixed on the fixed knee, and the appropriate endurance exercise is carried out under the weight of the knee joint. The time is usually no more than 8 to 10 minutes. It can also be used for swimming, walking on the ground, etc., but it is not suitable for uneven roads. Or walk on the slopes.
E. Note: When there is an acute exacerbation or severe pain in osteoarthritis, the medical gymnastics is suspended, or only a few muscles of equal length contraction are practiced.
3 joint protection: Take a series of simplified, effortless, action to reduce the load on the joints to complete daily activities, so that the affected joints will not be strained.
4 Antioxidant nutrient intake: It has been observed that chondrocytes may react with reactive oxygen species (ROS), and ROS promote degenerative damage processes, using micronutrient antioxidants to defend against This damage process, in Framingham's Knee Osteoarthritis Study (1996), found that vitamins C, E, and beta carotene help reduce the risk of developing the disease (as seen in radiology), vitamin C, E It also helps to prevent pain. In addition, it has been observed that vitamin C not only plays its role as an antioxidant, but also contributes to the biosynthesis of prostaglandin in cartilage, thereby inhibiting the biological process of cartilage destruction; vitamin E has The effect of alleviating synovial inflammation is beneficial to the process of changing osteoarthritis. Therefore, vitamin C and E can be used as secondary prevention of osteoarthritis.
5 quit smoking: clinical observations found that obesity, high blood pressure, smoking, poor mental state (depression, boredom, etc.), will promote the symptoms of osteoarthritis, should be dealt with these risk factors for triggering symptoms, including smoking cessation.
6 drug treatment: the use of drugs to treat both the symptoms and the symptoms, on the one hand to alleviate the symptoms, while limiting the development of the pathological process of the disease itself, play a secondary prevention role? Although this issue is different from experts, many people tend to Clinically, not only anti-inflammatory analgesics that have been used for many years, but also drugs that alter the course of osteoarthritis can be used as follows:
A. Calcitonin (human calcitonin, salmon calcitonin): Calcitonin is an active polypeptide secreted by thyroid follicular cells, which inhibits osteoclast activity, inhibits bone resorption, and facilitates bone repair. It is used for the treatment of paget disease, which can reduce the serum alkaline phosphatase activity of paget disease, reduce the excretion of hydroxyproline in urine, reduce the local blood flow of bone disease, and have good analgesic effect. Although the molecular structure of calcitonin and human calcitonin are different, the pharmacological effects are similar, the drug source is sufficient and the price is cheap, but the long-term use is easy to produce neutralizing antibody and the drug effect is reduced or allergic reaction occurs. Calcitonin 100U /d, subcutaneous injection, continuous use for 3 to 6 months, can reduce bone pain, improve blood biochemical abnormalities, such as no special toxic side effects, can reduce the maintenance of treatment for 1 to 2 years, common side effects have nausea after injection, Skin flushing and diarrhea, etc., generally do not need to stop the drug, the drug can be relapsed if the drug is stopped for a long time.
B. bisphosphonate complex: Hydroxyethylene diphosphonate disodium salt has a strong inhibition of osteolysis, oral dose is 5 ~ 10mg / (kg · d), continuous treatment should not exceed 6 months, and then discontinued 3 to 6 months, according to the condition needs to proceed to the next course of treatment, because the use of this drug for 6 months can generally receive significant effects, such as continued use of drugs will inhibit bone mineralization, inhibit new bone formation, or cause bone pain Another new bisphosphonate alendronate has been tried clinically and can be administered parenterally, with comparable efficacy to the disodium hydroxydiphosphonate.
C. Anti-inflammatory and analgesic drugs: This is a palliative drug to relieve pain symptoms. There are many drugs to choose from, but for patients with high risk factors (such as heart, kidney, liver dysfunction, stomach, 12 fingers) Intestinal ulcers), anti-inflammatory painkillers with high-risk side effects should not be used.
7 physical factor treatment: mainly used for anti-inflammatory and pain relief, relieve muscle spasm.
8 Psychotherapy: psychological counseling for the existence of depression and anxiety, health education, psychological state improvement helps prevent and control pain.
Complication
Malformation osteitis complications Complications primary hyperparathyroidism osteosarcoma arthritis depression
1. A small number of cases coexist with primary hyperparathyroidism.
2. Osteosarcoma and other tumor osteosarcoma are the most serious complications of this disease. About 0.3% of patients develop osteosarcoma, which is about 30 times higher than normal group. Some patients may also have other malignant tumors other than osteosarcoma such as fiber. Sarcoma, chondrosarcoma, etc., it is worth noting that the site of osteosarcoma of this disease is not consistent with the original lesion, such as the spine rarely occurs, and the humerus and facial bone often occur, once diagnosed as osteosarcoma, its survival The period is often less than one year.
3. There are often complications such as low back pain, arthritis and neck pain.
4. In addition, Paget patients are often accompanied by psychological disorders. In the United States, a survey of 2 000 patients with malformation osteitis found that 47% of patients were associated with depression.
5. The disease will also be complicated by abnormal bone reconstruction, hearing loss and so on.
Symptom
Malformations of osteoarthritis common symptoms elbow joint extension... Osteoporosis bone pain at birth, small skull, pelvic pain, sleepiness, deafness, hydrocephalus, palpitations, heart failure
About 10% to 20% of patients with this disease have no clinical symptoms, and are often found by X-ray examination for other diseases. The symptomatic patients mainly have the following performances:
1. Pain, bone deformity and fracture pain are the main complaints of this disease. Nearly 80% of cases show pain, and nearly half of them show joint pain. Joint pain is the most common complication of this disease. Caused by arthritis, common in the knee joint, hip joint and spine, about 17% of patients show single bone, mainly in the tibia and fibula, or multi-bone, the latter symptoms are often more dangerous than the former, generally Dull pain or burning pain, obvious at night and rest, occasionally sharp pain or radiation-like pain, weight can make the lower limbs, spine and pelvic pain worse.
This disease can involve any bone in the human body. The most commonly involved bones are pelvis, lumbar vertebrae, femur, thoracic vertebrae, humerus, skull, humerus and humerus. The deformity of long bones of the extremities can cause bone bending, resulting in secondary bone and joint. Inflammation, the deformity of the facial bone may not be obvious, but it can also lead to the removal of the teeth, the denture installation is difficult, etc., the skull can cause deafness and cranial nerve damage in time, and the skull base can cause the flat skull base and the skull. Bottom implicit, hydrocephalus, vertebral basilar artery insufficiency, etc., spinal involvement can cause spinal cord injury, nerve root compression symptoms and cauda equina syndrome.
The bone of the disease is brittle and can cause fractures after spontaneous or minor trauma, especially in the long bones of the extremities, often transverse fractures.
2. Hyperplasia of blood vessels in the bone and skin of the angiogenic lesions causes the local temperature to rise. The excessively formed blood vessels in the skull lesion can obtain blood from the external carotid artery, causing the superficial temporal artery to become rough and flex, causing insufficient blood supply to the brain (vascular blood stealing) Syndrome), can be expressed as drowsiness, solitude and apathy, etc. Similarly, such as vascular steal syndrome in the vertebral artery can lead to lower extremity palsy and lateral paralysis, when the disease involves more than 35% of the bones of the whole body It can cause congestive heart failure with high blood output, which is not common.
The diagnosis of this disease mainly depends on clinical manifestations, laboratory tests and imaging examinations.
Examine
Examination of deformity osteitis
Laboratory biochemical indicators for the diagnosis of this disease, except for other causes of metabolic bone disease and judgment of efficacy is important, because this disease affects bone absorption and formation, therefore, biochemical indicators reflecting bone resorption and formation often rise high.
1. Indicators reflecting bone resorption such as urinary pyridinium cross-linking and deoxypyridinoline cross-linking can be elevated.
2. Reflecting bone formation indicators are blood alkaline phosphatase or bone-derived alkaline phosphatase and urinary hydroxyproline. These indicators can be increased. When only blood alkaline phosphatase is detected, attention should be paid to the interference of liver disease. Alkaline phosphatase detection is the most effective indicator for monitoring disease activity and judging efficacy. When the lesions are wide, especially when the skull is invaded, the alkaline phosphatase rises more than 10 times the upper limit of the normal reference value.
3. Other indicators for determining metabolic bone disease such as blood calcium, phosphorus, magnesium, etc. often in the normal range of elevated blood calcium should pay attention to the presence or absence of malignant tumors, primary hyperparathyroidism or long-term bed rest, the active period of the disease Some patients have elevated blood parathyroid hormone and normal blood calcium.
X-ray examination X-ray performance has certain characteristics, such as the early X-ray of skull lesions as the most marginal osteoporosis area of the diseased skull area, which develops from the outer plate to the inner plate, and the lesion is surrounded by osteosclerosis zone. In the advanced stage, the bone layer is thickened between the lamellar bone and the braided bone to form an irregular or cotton-like bone shadow. When the outer panel appears loose, the inner panel can be expressed as a sclerotic image, which is the X-ray characteristic of the disease. One of the long bone lesions is often the translucent area where the cortical bone is first affected, and then the cystic area appears in the bone sponge, which makes the cortex phenotype a double contour. At the junction between the initial area of the lesion and the normal area, a V-shaped or "visible" can be seen. The flaming-shaped bone-dissolving zone is caused by bone resorption. After the lesion enters the repairing stage, the V-shaped boundary zone is covered by the repaired tissue, and a multi-layered periosteal bone is formed, which makes the backbone thicker and is extensively distributed along the line of force. Striped or reticular trabecular alignment, resulting in long bone bending, gross deformity.
5. Bone scan can be used to understand the extent and extent of the lesion, but it is generally not used for diagnosis. When the bone scan suggests a single bone lesion, it should be noted that other injuries such as fractures, infections, malignant tumors, etc., due to the constant lesion area of the disease, Changes in the condition of the patient who may or may not be associated with the disease during long-term follow-up may be judged and identified by the patient's initial bone scan data.
Diagnosis
Diagnosis and differentiation of deformity osteitis
diagnosis
Diagnosis can be based on medical history, clinical symptoms, and laboratory tests.
Differential diagnosis
The diseases that need to be identified with this disease are as follows:
1. Complications of the disease Arthritis needs to be differentiated from degenerative arthritis secondary to pelvic and lower extremity malformations.
2, the disease needs to be identified with poor bone fiber structure, the latter mostly in adolescents, with bone lesions, pain, dysfunction and bow deformity as symptoms, often accompanied by waist, hip, thigh skin pigmentation; X-ray image onset Long bones often occur in the metaphysis. The diseased medullary cavity is inflated and osteolytic, the cortical bone is thin, the thickness is different, the lesion boundary is clear, and there is no periosteal reaction.
