Systemic lupus erythematosus

Systemic lupus erythematosus (SLE, Libman-Sacks disease) - a disease, that is characterized by immune-complex affect predominantly of connective tissue and its derivatives, with the affect of microvasculature vessels. This is systemic autoimmune disease in which the antibodies produced by human immune system damages the DNA of own healthy cells.

Disease was named due to its characteristic features - a rash on the cheeks and nasal bridge (affected area has butterfly shape), which in Middle Ages was reckoned as like wolf biting.

Patients usually complain for unwarranted rises in temperature, weakness, muscle pain, headaches, rapid fatigability. Of course, these symptoms occurs not only when SLE, but in combination with other, more specific, the possibility, that the patient suffers SLE increases.

Skin manifestations have 65% of SLE patients, they arises one of the first, but only 30-50% have «classic» rash on the cheeks in the shape of butterfly. Many patients turn out to have discoid lupus - thick red scaly patches on the skin. Nested hair loss and sores in the mouth and nose, in vagina may also be a manifestation of SLE. Most patients suffer from pain in the joints. More often suffer minor joints of hands and wrists.

When SLE it occurs LE-cell phenomenon, which is characterized by the appearance of LE-cells (cells of lupus erythematosus) - heterophilic leukocytes, containing phagocyte fragments of other cells cores (for SLE it is typical recognition of their own cells as foreign and formation of autoantibody against them, the destruction of these cells and phagocytosis). Anaemia is observed in half of the patients. Leukopenia and thrombocytopenia can be consequance of SLE as well as side effect of its therapy.

Pericarditis, myocarditis and endocarditis become evident in the part of the patients. Endocarditis during SLE has got noninfectious character (ednocarditis of Libmann-Sax) and becomes apparent in the injury of the mitral or tricuspid valve. Atherosclerosis develops more frequently and quicker in patients who suffer from SLE than in healthy people.

Very often painless hematuria or proteinuria is the only symptom. Frequency of the acute renal failure doesn’t exceed 5% due to the early diagnostics and timely therapy. The most serious injury of internal is caused by lupous nephritis. The frequency of its appearence depends on the character of the clinical course and activity. Most often kidneys are injured during acute and subacute clinical course. It occurs more seldom during the chronicity. Psychosises, encephalopathies, spasmodic syndrome, paresthesias, cerebrovasculitises can appear. All changes bear persivering course character.

Survival rate of the patients ten years later after diagnosing equals to 80%, 20 years later – 60%. The main causes of the death are lupus-nephritis, neurolupus, intercurrent infections.

Medical treatment of the Systemic lupus erythematosus provides for application of the glucororticosteroids, cytostatics, immunosuppressors, nonsteroidal resolvents, methods of the extracorporal detoxication (plasmapheresis, hemosorption, cryoplasmasorption)

One should consider SLE the most serious autoimmune disease because in this case immune attack is directed to the own DNA, which contains practically in all cells of the organism (except for erythrocytes and platelets). Figuratively speaking, all organism of the patient becomes alien for its own immune system. In this case transplantation of the high doses of the hematopoietic and mesenchymal stem cells with underlying of preliminary imunosuppression (tacrolimus) can replace the immune system by the new one eager to react sufficiently to patient’s DNA. In this case an inductive imunosuppression is a necessary condition for a successful treatment. In some cases it’s expedient to carry out chemotherapy with the destruction of the cells of the immune system with the following transplantation of the hematopoietic and mesenchymal stem cells for the assured replacement of the immune system, which needs special conditions, time and expenses.

Clinical example. Patient M., 36 years old, has been suffering from SLE more than 11 years. For the first time an illness was diagnosed in the clinic of the Russian Academy of Medical Sciences. Three years ago she was in reanimation in connection with the development of the acute renal failure with underlying of lupus-nephritis. She got efferential therapy (plasmasorption, hemosorption), pulse-theapy by prednisolone. Arriving to the Institute of cellular therapy she was on the monotherapy. Complaints at the time of the hospitalization: shifting pains in the small articulations of hands, constant low grade fever (37,1 – 37,3 C°), weakness, loss of the capacity for work, permanent fear of death. Patient was psychologically unbalanced, hypochondriacal. She “retired into her disease”. She had been examined and undergone treatment in 17 Ukrainian clinics as well as abroad. The diagnosis of the SLE was repeatedly confirmed by the presence of the LE-cells. Lupus-nephritis (creatinine of the blood – 167 micromol/litre) is the main visceral complication. The transplantation of the high doses of hematopoietic and mesenchymal stem cells was carried out at the Institute of cellular therapy. Within three months after the transplantation of the stem cells the body temperature has decreased to norm. Articular pains have disappeared. Patient’s interest in life has returned. There is no fear of death. She works actively. Creatinine of the blood – 76 micromole/litre. The patient continues to accept Plaquenil.