Systemic lupus erythematosus - systemic inflammatory disease associated with the production of autoantibodies and immune complexes to the organism's own tissues.
The predominant age of the disease - 20-40 years. The prevailing gender - female
Causes of
Environmental factors. It is believed that viruses, toxins and drugs may be the cause of systemic lupus erythematosus. In some cases, patients with systemic lupus erythematosus exhibit antibodies to Epstein - Barr known phenomenon of "molecular masking" lupus autoantigens and viral proteins.
Hormonal influences. Systemic lupus erythematosus develops mainly in women of childbearing age, but hormonal factors may have a greater impact on the manifestation of the disease, than to its appearance.
Genetic features. The role of genetic factors confirms connection of systemic lupus erythematosus with hereditary deficiency of certain components of the immune system.
manifestations of lupus
- Skin lesions: discoid lesion - pockets shaped like a coin with red edges, thinning and discoloration in the center. Redness of the skin of the nose and cheekbones of the butterfly type (redness of the cheeks and the nose backs). Hypersensitivity to light - skin rash as a result of an unusual response to sunlight. It is also possible hair loss, hives.
- Mucous membranes: inflammation of the mouth, erosion.
- The defeat of the joints: pain in the joints.
- The defeat of the muscles: pain, muscle weakness.
- Lung - shortness of breath, pain when breathing.
- heart damage.
- Kidney involvement.
- Headache, reminding migraineThat does not pass after taking pain medication, mood disorders.
Diagnostics
- General blood analysis
- antinuclear antibodies
- Detection of blood in LE-cells
The diagnostic criteria of the American Rheumatism Association
The diagnosis of systemic lupus erythematosus is considered significant in the presence of 4 or more criteria (sensitivity - 96%, specificity - 96%).
- The rash on the cheeks: redness fixed (flat or elevated) in the zygomatic projections having a tendency to spread to the nasolabial area.
- Discoid rash: red erect plaques with adherent skin scales.
- Fotodermatit: skin rash that occurs as a result of unusual reaction to sunlight.
- Ulcers in the oral cavity: ulcers oral or nasopharyngeal, usually painless.
- Arthritis (joint disease).
- Pleurisy.
- Pericarditis.
- Kidney involvement.
- Convulsions: in the absence of medication, or metabolic disorders (uremia, ketosis, electrolyte imbalance).
- Psychosis: in the absence of medication or electrolyte abnormalities.
- Hematologic Disorders: leukopenia <4,0h109/ L (registered 2 or more times) or lymphopenia <1,5x109/ L (registered 2 or more times) or thrombocytopenia <100x109/ L (not connected with medication).
- Anti-DNA: antibodies to native DNA at an elevated titer.
- Increased titer of antinuclear antibodies detected by indirect immunofluorescence or a similar method in any period of the disease in the absence of medication, causing lupus-like syndrome.
lupus treatment
The basis of treatment constitute glucocorticoid hormones. The highest dose used in the acute course, exacerbation of the disease and high activity. The average dose of 1-1.5 mg / kg / day. (In terms of prednisolone).
The starting dose is selected to reduce the activity of the process, solving the problem individually. When III degree of activity is dose prednisolone 60-40 mg, at II - 30-40 mg, with I - 15-20 mg. If the first two days of the patient's condition has not improved, the dosage is increased by 20-30%.
Treatment of lupus in a maximum dose of hormone is conducted to achieve clinical benefit (4-6 weeks), then reduce the dose of not more than 1/2 of prednisolone tablets a week. At very high doses it can begin to decrease high dose - 5 mg / week. The maintenance doses are used for a number of years - 2.5-5 mg / day.
The smaller the dose needed to maintain remission, the better the prognosis. Prolonged treatment with hormones possible complications occurrence alimentary canal ulcers, diabetes diabetes, Cushing syndrome - Cushing, mental disorders, electrolyte metabolism, osteoporosis, Activation of a chronic infection and others.
With the ineffectiveness of hormones necessary appointment cytostatic immunosuppressants. Frequently used azathioprine and cyclophosphamide at a dose of 1-2 mg / kg. 15 mg of methotrexate per week. Mycophenolate mofetil 1.5-2 g / day. Cyclosporin 2.5-4 mg / kg / day. The treatment course of 6-8 weeks followed by a maintenance dose is maintained for many months. Improvement of cytostatics in the treatment occurs after 4-6 weeks.
Lupus is contraindicated sun exposure.
The diet must observe a low fat content, a high content of polyunsaturated fatty acids, calcium and vitamin D.
Methylprednisolone pulse therapy is carried out for the following indications: rapidly progressive glomerulonephritis, Young age, high immunological activity. Pulse therapy - not "despair therapy", as a component of intensive care programs.
Besides classical pulse therapy (methylprednisolone 15-20 mg / kg of body weight / per day for 3 consecutive days) pulse therapy administered repeatedly at intervals of several weeks. Pulse therapy may be enhanced by cyclophosphamide 1g intravenously on day 2 of treatment. After the pulse therapy with prednisone dosage should be reduced slowly.
Intravenous immunoglobulin G preparation is normal polyspecific immunoglobulin sera obtained from not less than 5,000 donors. Standard drugs are Sandoglobulin, Octagam. Immunoglobulin administered as a "last resort" to 0.5 g per 1 kg body weight for 5 consecutive days. In patients with lupus nephritis immunoglobulin assignment requires great caution because of the danger of sharp progression of renal failure. There allergic reactions such as fever, rash, febrile reactions, nausea and dizziness. Absolute contraindication to the use of intravenous immunoglobulin is an immunoglobulin deficiency A.
plasmapheresis. In modern plasmapheresis is carried out by using a centrifuge or membrane technology, the removal of one procedure of 40-60 ml / kg of plasma. Recommended plasmapheresis courses consist of 3-6 procedures carried out consecutively or at short intervals.
Plasmapheresis in patients with systemic lupus erythematosus is shown as an acute intervention in complicated cryoglobulinemia, hyperviscosity syndrome and thrombocytopenic purpura. Plasmapheresis may be used as an additional highly effective for conditions directly threaten life: fulminant vasculitis polimieloradikulonevrit, cerebral coma, hemorrhagic pneumonitis. Justified connection plasmapheresis in cases of lupus nephritis that is resistant to the hormones and tsitotoksikam.
Additional sorption treatment distinguish selective and nonselective removal from circulation pathological protein structures. For nonselective relates construction, which is based on physico-chemical properties of the activated carbon. In addition to the direct removal of the CEC, cytokines and autoantibodies, heavy stimulates idiotypic activity, phagocytosis, and enhances the sensitivity of cellular receptors to medicines. In the treatment of patients with SLE GS has the same indications as the PF. Selective sorption carried out by means of selective immunosorbents (biological or chemical) capable of specifically removing the RF, antibodies to DNA and CEC.
Experimental studies in this area show a high efficiency immunosorbents SLE patients with high immunologic activity. Side effects of extracorporeal therapies are usually limited to transient hypovolemia and chills, contraindication is a peptic ulcer in the acute stage, uterine bleeding, intolerance heparin.
Despite the sometimes fantastic results in critical situations in SLE, PF and GE as an independent method of treatment, they rarely find their place in the planned therapy. Their use is largely constrained by the development of the so-called syndrome of ricochet arising immediately after the procedure and is characterized by recurrent clinical activity and a sharp increase in the level of antibodies and CIC. Suppression activity of antibody-b-lymphocyte syndrome and prevention of rebound achieved by sequential, simultaneous application of PD and PT MP-CP. Intensive Care Unit Synchronization in a months-long program, perhaps superior to all known methods for the treatment of SLE with a poor vital prognosis.
There are several methods of synchronous intensive care:
- Plasmapheresis 3-6 A series of sequential treatments with a further short course megadoses of CF intravenously;
- Starting series PD procedures (usually 3) in synchronism with the intravenous administration of 1 g of CP and MP 3 g and further one PD procedure in 1-3 months. throughout the year, in synchronism with the 1 g and 1 g TF MF.
The second synchronous circuit intensive therapy is more convincing, since it provides program control during the year. Software assignment combination PD and PT MP and CP shown primarily SLE patients with a poor prognosis of life, due to the start of systemic lupus erythematosus in adolescence and young adulthood, the rapid development of nephrotic syndrome, rapidly progressive type of jade resistant hypertension and the development of life-threatening conditions (cerebral crisis, transverse myelitis, hemorrhagic pneumonitis, thrombocytopenia, and others.).
In recent years, aggressive treatments are no longer "despair therapy", with emergency departments and urgent situations. The planned appointment of these methods can significantly improve long-term vital prognosis in a significant proportion of patients with SLE. In the short term it is obvious emergence of new schemes and programs of intensive therapy of SLE, e.g., TIG and sync FS, immunoadsorption and Cy shock doses of interferon preparations and anticytokine antibodies.
Autologous stem cell transplantation is proposed for the treatment of severe SLE.
The prognosis for survival is significantly reduced in the presence of antiphospholipid syndrome.
