2 English references Hypertrophic Osteoarthropathy
Summary Hypertrophic Osteoarthropathy (HOA) is a syndrome caused by thickening of soft tissue around bone and extensive periosteal new bone formation. The main clinical manifestations are clubbed fingers (toes), extensive periosteal new bone formation, joint pain and effusion.
Hypertrophic osteoarthropathy can be divided into primary and secondary. Primary hypertrophic osteoarthropathy, also known as familial hypertrophic osteoarthropathy and thick-skinned osteoarthropathy, is common in men, with a male-female ratio of 8.9: 1. About two-thirds of the patients developed the disease at birth or at the two peak periods of 15. It can be complicated with joint effusion, bone marrow fibrosis, gastrointestinal proliferative lesions, chromosome abnormalities and so on. Primary hypertrophic osteoarthropathy is a self-limiting disease, which is active in adolescence and enters asymptomatic stable period in adulthood. Secondary hypertrophic osteoarthropathy, also known as pulmonary hypertrophic osteoarthropathy, is often accompanied by obvious visceral diseases, and the onset age is generally older, mainly middle-aged and elderly people. It can be complicated with lung and pleural diseases, cardiovascular diseases and chest diseases. The development of secondary hypertrophic osteoarthropathy depends on its primary disease. If the primary disease is removed, the secondary hypertrophic osteoarthropathy can be alleviated or cured.
4 disease name hypertrophic osteoarthropathy
Hypertrophic osteoarthropathy in English.
6 alias of hypertrophic osteoarthropathy Primary familial secondary hypertrophic osteoarthropathy
7 classification of rheumatism > other types of rheumatism
8 ICD number M89.4
9 Epidemiological primary hypertrophic osteoarthropathy is more common in men, and the ratio of male to female is 8.9∶ 1. About two-thirds of patients get sick at birth or at the peak age of 15. The onset age of secondary hypertrophic osteoarthropathy is generally older, mainly middle-aged and elderly people, and it is more common.
10 Etiology 10. 1 The main cause is unknown. About14 patients have a positive family history, and the inheritance is transmitted by recessive or incomplete dominant genes, that is, autosomal dominant inheritance with different penetrance. It has also been reported that chromosomal abnormalities have no effect on the inheritance of this disease.
10.2 secondary diseases are mostly secondary to lung and pleural diseases, cardiovascular diseases and chest diseases.
(1) Lung or pleural diseases: often secondary to bronchogenic carcinoma, pleural mesothelioma, chronic lung abscess, empyema, bronchiectasis, emphysema, lung metastatic carcinoma and lung lymphoma. Squamous cell carcinoma with cancer cavity is more common in bronchial lung cancer. This disease has nothing to do with the size and volume of lung cancer.
(2) Cardiovascular diseases: often secondary to congenital cyanotic heart disease, pulmonary heart disease and bacterial endocarditis, such as tetralogy of Fallot, heterotopic aorta, Eisenmenger syndrome and congenital patent ductus arteriosus.
(3) Extrathoracic diseases: including all kinds of extrapulmonary malignant tumors without lung metastasis, such as pancreatic cancer, esophageal cancer and nasopharyngeal cancer. And liver cirrhosis and chronic ulcerative colitis caused by various reasons.
1 1 The pathogenesis of hypertrophic osteoarthropathy is not clear, but it has been recognized that hypertrophic osteoarthropathy is a special response to some disease states. There are several assumptions:
1 1. 1 body fluid theory Under normal circumstances, the lung can clear or inactivate certain factors from the organs or tissues of patients, but in the case of lung diseases, the lung cannot clear or inactivate such factors and make them enter the circulation, causing characteristic bone and soft tissue hyperplasia, but the existence of such factors has not been confirmed so far. Recently, many tumor-derived growth-promoting polypeptide factors have provided support for the development of this theory.
1 1.2 neurology believes that the diseased organ sends out impulses through the vagus nerve, which dilates the fingertip blood vessels through the reflex mechanism, clubbing. After vagotomy, the pain and signs can be alleviated, and the blood flow of the affected part is also reduced.
1 1.3 receptor theory in recent years, it has been found that glucocorticoid receptor and epidermal growth factor receptor increase in patients with hypertrophic osteoarthropathy, and the content of epidermal growth factor in urine increases. It is found that the changes of glucocorticoid receptor and epidermal growth factor receptor are related to the characteristic skin changes of the disease, and the increase of epidermal growth factor content in urine may be related to systemic changes such as subperiosteal new bone formation.
Some studies have also found that the blood flow in the lesion site of secondary hypertrophic osteoarthropathy increases, which is considered to be due to the increase of blood supply and the increase of hemoglobin concentration of deoxyhemoglobin, which leads to relative hypoxia of tissues and causes periosteal hyperplasia and ossification of hypertrophic osteoarthropathy. However, the primary hypertrophic osteoarthropathy has slow blood flow and local hypoxia, which is obviously different from the secondary hypertrophic osteoarthropathy, but the lesion is the same, and its mechanism is still unclear. Some people think it should belong to different diseases.
1 1.4 The pathological changes of skin are epidermal hypertrophy, slight tumor-like changes, dermal collagen fiber hyperplasia, hair follicle and sebaceous gland hyperplasia and hypertrophy, a small amount of inflammatory cells infiltration around, fibroblast proliferation, subcutaneous soft tissue edema, collagen tissue increase, periosteal arteriole wall thickening, mainly middle layer thickening, small blood vessels congestion in surrounding tissues and lymphocyte infiltration.
Bone changes include periosteal edema and inflammatory cell infiltration, followed by periosteal thickening, osteoid deposition, mineralization and new bone formation. Bone cortex is thickened by connecting with periosteal new bone.
Synovial changes include nonspecific inflammatory changes, congestion, edema, slight lining cell proliferation, inflammatory cell infiltration, and occasionally thickening of small blood vessels accompanied by fibrosis and pannus formation. Electron microscopic examination showed that electron dense substances were deposited under the intima of synovial tissue. No immune-mediated vascular injury was found by immunohistochemical technique.
Clinical manifestations of hypertrophic osteoarthropathy 12 The symptoms and signs of primary hypertrophic osteoarthropathy are often not completely consistent. Some patients have no symptoms at all, and they are not aware of clubbing. Others have obvious chronic bone pain before the appearance of clubbed fingers, mainly pain and deep, so they often can't clearly point out the specific parts and can't stick to their work. Clubbing finger is one of the most prominent clinical manifestations. Fingertips (toes) are spherical, and the circumference of normal nails is reduced 160 degrees. The thickness of fingers at the base of nail bed is greater than that of distal interphalangeal joint, and the circumference of nail bed base is greater than that of distal interphalangeal joint. Due to the hyperplasia and edema of soft tissue in nail bed, nail palpation has "swinging movement" Late skin thickening, nail bending, cyanosis, resulting in drumstick deformity. Some patients' hands and feet become thicker and thicker, and the length does not increase, but they are shovel-shaped or animal-palm-shaped.
Skin manifestations include sweaty hands and feet, greasy skin on face and scalp; There are more acne. The face is rough, the skin on the forehead and brow is thickened, and the forehead lines are horizontal and deep. The eye distance is widened, the upper eyelid is thick and drooping, the nose tip is enlarged, the nasolabial groove is deepened, and the upper lip is enlarged, showing the appearance of a lion's face. The scalp is thickened in the shape of a gyrus, with thick folds, obvious furrows and ridges, and vertical. This brain-like change of scalp skin is called scalp drooping. Some patients showed non-concave edema of lower limbs, similar to the change of rubber legs. Generally speaking, the skin changes of primary hypertrophic osteoarthropathy are more prominent and frequent. However, the skin changes of secondary hypertrophic osteoarthropathy are rare, and the symptoms and signs are also light.
About half of the patients have joint pain, swelling and joint effusion. Knee joints and ankle joints are common, but elbow joints, wrist joints, metacarpophalangeal joints and metatarsophalangeal joints can also be involved, which are generally asymmetrical. The pain is mainly at night, showing mild joint pain and even severe pain. Signs include local redness, fever, tenderness, swelling, joint effusion and limited activity, as well as painless joint effusion. Where there is not a lot of muscle coverage, the forearm or calf will thicken, and the wrist and ankle will also thicken due to the formation of new bone in the periosteum of long bone.
In addition to the above manifestations, patients with hypertrophic osteoarthropathy may also have fatigue, feminization of men, female-like distribution, bone marrow fibrosis, gastrointestinal proliferative lesions, chromosome abnormalities and so on.
13 complications of hypertrophic osteoarthropathy Primary hypertrophic osteoarthropathy can be complicated with joint effusion, myelofibrosis, gastrointestinal proliferative lesions and chromosomal abnormalities. Secondary hypertrophic osteoarthropathy can be complicated with lung and pleural diseases, cardiovascular diseases and chest diseases.
14 laboratory examination General laboratory examination showed that there was no abnormality except that erythrocyte sedimentation rate could be accelerated due to primary disease; Joint fluid is a small amount of viscous fluid, showing non-inflammatory changes.
Auxiliary examination 15 X-ray examination 15. 1 X-ray examination showed that the main X-ray changes of this disease were symmetrical periosteal new bone formation in different degrees of long bone and short bone. It can be parallel or layered, separated from the cortex by a linear semitransparent band, or it can be characterized by the fusion of periosteal new bone and protocortex without semitransparent band in the middle, wavy or extensive spinous periosteal osteophyte. It is more common in tibia, fibula, radius, ulna, metacarpal and metatarsal, and eventually involves all bones except skull, and develops into extensive ossification of ligament and interosseous membrane, occasionally leading to ankylosis of joints and spine. Reticular cortical thinning and osteoporosis. The sella turcica is normal. The X-ray manifestations of primary and secondary hypertrophic osteoarthropathy are consistent.
15.2 radionuclide examination 99mTcMDP bone imaging is more sensitive than X-ray, and often shows the symmetrical bone salt metabolism of distal limbs.
16 the diagnosis of hypertrophic osteoarthropathy is mainly based on the progressive periosteal osteogenesis, clubbed fingers (toes) and hypertrophy of the skin of the head, face and limbs, and the first two items are the most important. Male patients, with younger onset age, can't find any primary diseases clinically, and are considered as primary hypertrophic osteoarthropathy. The onset age is older, with arthropathy or bone pain as the main manifestations. Those with primary diseases of lung, pleura, heart, liver, blood and intestine and no positive family history should be considered as secondary hypertrophic osteoarthropathy. Generally speaking, the skin changes of secondary hypertrophic osteoarthropathy are rare. Hypertrophic osteoarthropathy before lung tumor is difficult to distinguish. The longest interval between the diagnosis of hypertrophic osteoarthropathy and the diagnosis of lung tumor can be 18 months. At this time, it is difficult to diagnose secondary hypertrophic osteoarthropathy and long-term clinical follow-up is needed.
Primary hypertrophic osteoarthropathy can be divided into three types: ① Complete type: periosteal hyperostosis, clubbed fingers (toes), facial hypertrophy, and brain-like scalp. ② Incomplete type: there are periosteal hyperostosis, clubbed fingers (toes) and facial hypertrophy, but there is no brain-like scalp change. ③ Mild: clubbed fingers (toes), facial and/or scalp changes, little or no periosteum osteogenesis. Incomplete type is very common in clinic. Whether the above classification is also applicable to secondary hypertrophic osteoarthropathy remains to be seen.
17 The differential diagnosis of hypertrophic osteoarthropathy has typical clubbing fingers, and there is no problem in the diagnosis. Sometimes, other manifestations of hypertrophic osteoarthropathy include skin manifestations before clubbing fingers, so it needs to be differentiated from the following diseases:
17. 1 acromegaly acromegaly can be confused with hypertrophic osteoarthropathy, such as thick hands and feet, thick skin and rough face. However, there is no periosteal new bone formation of long bone and short bone in this disease, and the thick hands and feet only thicken and widen, without obvious increase, and the head circumference does not increase significantly. Growth hormone and serum inorganic phosphorus increase during active period, and most butterflies are caused by pituitary tumors.
17.2 acromegaly of thyroid gland has clubbed fingers (toes), malignant exophthalmos and mucinous edema in front of tibia. X-ray examination showed that there was new bone formation under the periosteum of metacarpal bone, mostly caused by hyperthyroidism treatment. This disease has an obvious history of hyperthyroidism and can be used for differentiation.
17.3 Endometrial osteoporosis is mainly manifested as cortical thickening and medullary cavity narrowing caused by endosperm hyperplasia, but the transverse diameter of bone does not increase, and the skull is often involved to cause cranial plate thickening and plate barrier closure, without clubbing fingers and skin changes, which is different from hypertrophic osteoarthropathy.
Others need to be differentiated from diseases such as rheumatoid arthritis, deformed osteitis and syphilis.
18 treatment of hypertrophic osteoarthropathy At present, there is no exact treatment for hypertrophic osteoarthropathy. Non-steroidal anti-inflammatory drugs or analgesics can be used for pain symptoms. Hyperhidrosis can be treated with beta blockers or sympathectomy. Plastic surgery is feasible when facial skin hyperplasia affects appearance and function. All treatments can't change the course of the disease. For secondary hypertrophic osteoarthropathy, active treatment of the primary disease, such as resection of lung tumor or correction of cardiovascular malformation, can alleviate hypertrophic osteoarthropathy. If clubbed fingers have existed for more than a few months, the changes of connective tissue may not be recovered.
19 Prognosis Primary hypertrophic osteoarthropathy is a self-limited disease, which is active in adolescence and adolescence, and enters a asymptomatic stable period in adulthood. The development of secondary hypertrophic osteoarthropathy depends on its primary disease. If the primary disease is removed, the secondary hypertrophic osteoarthropathy can be alleviated or cured.
Prevention of hypertrophic osteoarthropathy. Eliminate, reduce or avoid pathogenic factors, improve living environment and space, develop good living habits, prevent infection, pay attention to food hygiene, and rationally distribute meals. Avoid cold and humidity.
2. Pay attention to exercise, increase the body's disease resistance, don't be overtired, over-consume, quit smoking and drinking. Keep a balanced mind and overcome anxiety and tension.
3. Early detection, early diagnosis, early treatment, establish the confidence to overcome the disease, and insist on treatment.
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