hemochromatotic osteoarthropathy
Introduction
Introduction to hemochromatosis osteoarthrosis The disease is a disease caused by abnormal iron metabolism caused by excessive iron content in the body, which is deposited in various internal organs and organs, and is clinically divided into two types: spontaneous and secondary. The former is caused by the imbalance of hereditary iron metabolism. It is characterized by excessive iron absorption, pathological iron deposition, and finally dysfunction of many organs. The liver, pancreas, heart and pituitary are most obvious. Its typical clinical manifestations are hepatomegaly, diabetes, skin pigmentation and sexual dysfunction. In some cases, there may be general weakness, weight loss, abdominal pain, dyspnea and edema. Secondary hemochromatosis is mostly secondary to long-term recurrence. A large number of blood transfusions (such as the treatment of aplastic anemia, the number of blood transfusions can be more than 100 times) and the previous treatment with iron. It can also be seen in hemolytic diseases, malnutrition, vitamin A deficiency and long-term diarrhea. In normal people, the total iron content in the body is 3 to 5 g. When iron metabolism is impaired, it may accumulate more than 25g, and 500mg of iron per 1000ml of whole blood during transfusion. It is generally believed that when the input iron exceeds 20 g, secondary hemochromatosis is about to occur. basic knowledge The proportion of illness: 0.001% - 0.003% Susceptible people: no special people Mode of infection: non-infectious Complications: bone cyst
Cause
Causes of hemochromatosis osteoarthrosis
Cause:
An abnormality in iron metabolism caused by spontaneous or secondary causes causes excessive iron content in the body.
Pathogenesis:
Mainly in the synovial cells, there is a large amount of hemosiderin deposition, some articular cartilage is calcified, and there is calcium pyrophosphate crystal in the joint fluid.
The mechanism by which iron in spontaneous hemochromatosis accumulates in the body is unclear. There are generally three theories:
1. Lack of xanthine oxidase in the liver.
2. Tissue protein variation with abnormal affinity for iron.
3. Intestinal protein secretion promotes or prevents iron absorption.
Hemochromatosis is rare in China, and only cases have been reported. In countries such as Europe and the United States, cirrhosis and iron over-deposition are often caused by excessive drinking, and the incidence is very high.
Prevention
Hemochromatosis osteoarthrosis prevention
There are no effective preventive measures for this disease.
Complication
Hemochromatosis osteoarthrosis complications Complications bone cyst
There are cyst formation and osteophyte formation under the metacarpophalangeal joint and the proximal interphalangeal joint.
Symptom
Hemochromatosis osteoarthrosis symptoms common symptoms joint swelling persistent pain liver large and hard skin atrophy and pigmentation morning stiffness
The disease often occurs in middle-aged and elderly people, especially in groups of people over 50 years old. Men are more than women, because women can regularly discharge iron in menstruation, so it is rare, but it can occur after menopause.
Clinically, the main manifestations of this disease are slow-moving polyarticular lesions, which are generally asymptomatic in the early stage, or have symptoms and signs similar to osteoarthrosis, such as pain, swelling, limited range of motion, etc.; often begin with the metacarpophalangeal joint And proximal interphalangeal joints, can also involve the hips, knees, ankles, feet, wrists, shoulders and other large joints, there are also individual patients involving the spine, the disease may have acute pain, joint activity dysfunction at the onset, more occurs In the hip joint, the period of 2 to 3 weeks, or even longer, gradually formed persistent pain, gradually worsening, other joints such as sputum, knee, hand joint joint acute pain, gradually transformed into persistent pain and morning joint stiffness The distribution of joint lesions has nothing to do with the work and trauma of the patient.
Joint swelling can be found during examination. In particular, the metacarpophalangeal joint has obvious bony enlargement, limited movement, and pain. The wrist, interphalangeal and metatarsophalangeal joints have similar changes, but are lighter, and many other joint activities. The scope is also limited. There may be a "rolling" sound when the knee joint is active. There is no synovial thickening or effusion, and there is no tendon lesion. The spine may have normal physiological curvature disappearing, activity is limited, and there is pain, severe type. All symptoms and signs may appear in the patient, and the individual may have only a few symptoms or even no joint symptoms.
Examine
Examination of hemochromatosis osteoarthrosis
Hemoglobin content and white blood cells, platelet count, and blood cell sedimentation rate were normal, rheumatoid factor was negative, macrophages containing iron particles were seen in the surrounding blood, liver function was normal, and some patients showed mild sulfonamide sodium excretion. Decreased amount, serum calcium, phosphorus content is normal, urine sugar and glucose tolerance test can be abnormal, serum iron content is increased, normal is 14.326.9mol/L (80150g/dl), serum transferrin saturation is increased (normal 30%), sometimes as high as 70% or even 100%.
On routine X-ray examination, cyst formation, bone erosion, joint space stenosis and osteophyte formation can be found under the joints of the metacarpophalangeal joint and the proximal interphalangeal joint. Cyst formation and erosion also occur in the wrist and lower ankle joints. A few distal interphalangeal joints may also change. The subtalar cysts first appear, usually located in the proximal part of the joint. It is common that the subtalar area of the metacarpal head has a round or oval small cyst with a diameter of 1 to 3 mm. There is a clear hardening zone around the periphery, followed by joint space stenosis and osteophyte formation. The joint space is not uniform, and the joint is most obvious. However, the joint space is not necessarily consistent with the cyst formation. Sometimes there are several joints. Lower cyst formation without joint space stenosis; there may be obvious joint space stenosis without cyst formation, osteophyte formation is generally a manifestation of severe joint involvement, but not necessarily with obvious joint space stenosis, there may be borders in the wrist bone Clear cysts, 5 to 6 mm in diameter, often multiple; there are also 2 to 3 cysts in the same bone, common in the head bone, hook bone, scaphoid and lunate bone.
Some patients may have changes in the distal ankle joint and distal ulnar. There may be several cysts and bone erosion at the distal end of the ulna and at the styloid process. In a few cases, cysts may appear on the temporal side of the joint, even at the triangular ligament. Shows opacity shadows.
The X-ray manifestations of the larger joints such as the knee and the hip are mainly cartilage opacity, which is due to cartilage calcification or iron deposition.
Diagnosis
Diagnosis and identification of hemochromatosis osteoarthrosis
The diagnosis of hemochromatosis, in addition to the age of onset, symptoms, signs, especially liver, diabetes, sexual dysfunction and skin pigmentation, serum iron increased, especially the transferrin saturation increased significantly, and found in the surrounding blood When macrophages containing iron particles are diagnosed, liver biopsy can be clearly diagnosed when suspicious patients or clinical manifestations are not obvious.
This disease is sometimes confused with rheumatoid arthritis and should be identified. Because of the pyrophosphate crystals in the joint fluid, it should also be differentiated from cartilage calcium deposition, that is, pseudo gout; due to hemorrhagic disease, osteoarthrosis has a special lesion distribution. And the performance of imaging, it is not difficult to identify.
