What is arthropathy in hemochromatosis?
Hemochromatosis (bronze diabetes) develops as a result of metabolic iron-containing pigments and the deposition of hemosiderin in the tissues and organs with their subsequent dystrophic changes and dysfunction, including the joints.
Sick more often men aged 40 years and older. The rarer incidence of women is due to the periodic removal of iron from the body during menstruation. There are primary (idiopathic) and secondary forms of hemochromytosis. Peplegal gog.ma gmohpomataza is a nasal disease associated with histocompatibility antigens HLA A3 and B14.
Secondary hemochromatosis occurs as a result of an overload of the body with iron due to the consumption of food rich in iron, chronic anemia, cirrhosis of the liver, multiple blood transfusions. If this does not mark tissue lesions, the disease is usually referred to as hemosiderosis.
Pathogenesis during arthropathy in hemochromatosis
In hemochromatosis, there is a significant increase in the serum iron content and an increase in the amount of protein bound to iron in the plasma. Along with this, hemosiderin deposition is detected in various organs and tissues. In liver biopsy specimens, iron deposits are found in hepatic parenchymal cells (in idiopathic form) or in reticuloendothelial cells (in secondary form). Deposits of hemosiderin and melanin are found in the skin (melasma), as well as in the intestinal mucosa, heart, bone marrow, pancreas to other organs, followed by the development of fibrosis and impaired function (liver cirrhosis, diabetes mellitus).
Iron can also be found in the urinary sediment, mainly in its idiopathic form, especially after loading with iron-containing substances.
In articular tissues, hemosiderin deposits are found in the synovial membrane, as well as in articular cartilage cells (chondrocytes). In 50% of patients, hemochromatosis is accompanied by chondrocalcinosis with the deposition of calcium crystals in the articular cartilage and the synovial membrane of the large joints, symphysis and wrist joints, including the characteristic calcification of the triangular ligament. Impregnation of articular cartilage with hemosiderin and calcium crystals causes its degenerative changes with the subsequent development of chronic arthropathy, resembling arthrosis.
Primary hemochromatosis is often complicated by diffuse osteoporosis, osteophytosis of the affected joints. In this regard, of interest is severe osteoporosis, which complicates hemochromatosis, which is observed in the population of South Africa who consumes foods rich in iron. This siderosis often occurs concurrently with vitamin C deficiency, which is also one of the factors causing osteoporosis.
The pathogenesis of arthritis that occurs during hemochromatosis before the development of degenerative changes in the articular tissues is unclear. With the simultaneous presence of chondrocalcinosis, patients may experience pseudo-gouty attacks of acute arthritis, the pathogenesis of which is associated with the rapid transition of calcium pyrophosphate crystals from cartilage to synovial fluid and the development of acute synovitis.
As for the reasons for the private combination of hemochromatosis and chondrocalcinosis, A. Gordon believes that this is due to the fact that ionized iron can inhibit the activity of the pyrophosphatase enzyme and lead to an increase in the level of calcium pyrophosphate in the joint.
Prolonged impregnation of articular cartilage with hemosiderin and calcium crystals leads to its dystrophy and gradual degeneration with fibrillation, destruction and fragmentation, compaction of the subchondral bone and the development of osteophytes, just as it happens with arthrosis.
Symptoms of Arthropathy with hemochromatosis
The lesion of the joints is one of the main, and sometimes the only clinical manifestation of hemochromatosis. However, in most cases, chronic arthropathy develops in patients who already have the characteristic features of hemochromatosis — skin pigmentation, liver enlargement and cirrhosis, signs of diabetes, myocardiodystrophy in some cases with myocardial insufficiency.
Arthropathy often begins with a lesion of the proximal interphalangeal joints of the second and third fingers. Such localization, chronic course and frequent development of erosive changes in the ulnar styloid process make this early syndrome similar to rheumatoid arthritis. In the future, the distal interphalangeal and wrist, elbow and knee, hip (rare), shoulder joints may be affected. Slight pain, some limitation of movements and deformities, similar to those observed during arthrosis, are noted. On the background of this chronic progressive arthropathy with the simultaneous presence of pyrophosphate arthropathy, acute attacks of pseudogout are observed.
Diagnosis of Arthropathy with hemochromatosis
Radiographic changes are identical to those in arthrosis – narrowing of the joint space, thickening of the subchondral bone, multiple cysts. However, fewer osteophytes, but often diffuse osteoporosis are detected. When combined with chondrocalcinosis, radiograph shows characteristic calcification of articular cartilage.
The diagnosis of hemochromatosis is established on the basis of characteristic skin pigmentation, the presence of liver cirrhosis, hyperglycemia and chronic arthropathy with localization of the process in large joints and proximal interphalangeal joints of the hands and confirmed by histological examination of skin biopsies (accumulation of melanin in the basal layer of the skin), synovial lymphatic lymphatic lymphitis, and livers. in hepatocytes), as well as the presence of elevated levels of iron in serum. However, it should be borne in mind that in the early stage of primary hemochromatosis, the iron content in the serum may be normal. An important diagnostic role is played by the desferal test — an increased excretion of iron in the urine after a load of desferal.
In the early stage of the disease should be a differential diagnosis with rheumatoid aoitis. For hemohypathy, there is no inflammatory changes in the joints and a predominantly degenerative process in the joints on roentgenograms, as well as the presence of such typical signs as skin pigmentation, etc. The articular syndrome in hemochromatosis is sometimes difficult to distinguish from that in deforming osteoarthrosis, if you do not take into account uncharacteristic for the latter lesion of the metacarpal and carpal joint, the styloid process, mild osteophytosis on radiographs. In doubtful cases, the diagnosis is decided on the basis of histological examination of biopsy specimens of the skin and synovial membrane, which can detect hemosiderin deposits, and an increase in the content of serum iron.
Treatment of Arthropathy with hemochromatosis
The most effective method is periodic hemorrhage, plasmapheresis, hemosorption in order to remove excess iron from the body, as well as limiting the administration of iron with food. Effective is the use of the drug Desferal (deferoxamine), which promotes the removal of iron from the body (500-1000 mg intramuscularly 1-2 times a day for 2-3 months, 3-4 times a year). Prolonged use of this drug causes remission and stabilization of the process. As symptomatic agents that reduce pain and inflammation in the joints, ARS is used (indomethacin, voltaren, brufen, etc.) In the presence of diffuse osteoporosis, it is necessary to limit the physical load on the joints, to prescribe calcium drugs (up to 1-2 g per day for 20 days each months) and phosphorus (1.5 g / day), as well as anabolic steroids (nerobol tablets 0.005 g 1-2 times a day), or methandrostenolone (0.025-0.05 g / day), or retabolil 0 , 05 g intramuscularly 1 time per week, as well as vitamin Da (calciferol 2,000-4,000 IU / day).
Recently, for treatment of osteoporosis, calcitrin 2-3 U per day are used, in severe forms up to 5 U per day subcutaneously or intramuscularly during a month (repeated course not earlier than two months later), as well as calcitonin 50-100 ME every other day or ossin (sodium fluoride) 60-80 mg per day. Preparations are used for a long time, for years. Under their influence, pain is reduced and the calcium metabolism in the body is normalized.
In severe lesions of the hip joint with severe articular deformities, surgical treatment is superficial and limited in mobility.