Lupus erythematosus

Lupus erythematosus is a systemic chronic polysyndrome disease of connective tissue and vessels that develops in connection with the genetically conditioned imperfection of immunoregulatory processes.

Etiology. The value of a viral infection is assumed against the background of genetically determined immunity disorders.

Pathogenesis: the formation of circulating autoantibodies, of which the most important diagnostic and pathogenetic significance are antinuclear antibodies; The formation of circulating immune complexes, which, deposited on the basal membranes of various organs, cause their damage and inflammation. This pathogenesis of nephritis, dermatitis, vasculitis, etc. This hyperreactivity of humoral immunity is associated with impaired cellular immunoregulation. Recently, the importance of hyperestrogenemia, accompanied by a decrease in the clinic of circulating immune complexes, etc. has been attached. Family-genetic predisposition has been proved. Mostly young women and adolescent girls are ill. The provoking factors are: insolation, pregnancy, abortion, childbirth, the onset of menstrual function, infection (especially in adolescents), drug or postvaccic reaction.

Symptoms, course. The disease begins gradually with recurrent polyarthritis, asthenia. Less common is acute (high fever, dermatitis, acute polyarthritis). Further, there is a recurrent course and a characteristic polysyndromicity.

Polyarthritis, polyarthralgia is the most frequent and early symptom of the disease. Mostly small joints of the hands are affected mainly in wrist, ankle, and rarely knee joints. A non-erosive type of polyarthritis is characteristic, even in the presence of deformation of interphalangeal joints, which develops in 10 - 1 5% of patients with chronic course. Erythematous rashes on the skin of the face in the form of a "butterfly", in the upper half of the chest in the form of "decollete," on the limbs - is also a frequent sign of systemic lupus erythematosus. Polyserositis is considered a component of the diagnostic triad, along with dermatitis and polyarthritis. It is observed in almost all patients in the form of bilateral pleurisy and (or) pericarditis, less often perihepatitis and (or) perisplenitis.

Characteristic defeat of the cardiovascular system. Usually, pericarditis develops, to which myocarditis joins. Comparatively often there is a warty endocarditis of Liebman-Sachs with mitral, aortic and tricuspid valves. Signs of the defeat of the vessels are included in the picture of the lesion of individual organs. Nevertheless, it should be noted the possibility of the development of Raynaud's syndrome (long before the typical picture of the disease), the defeat of both small and large vessels with the corresponding clinical symptoms.

Lesions of the lungs can be associated with a major disease in the form of lupus pneumonitis, characterized by coughing, shortness of breath, and inaudible wet wheezing in the lower parts of the lungs. X-ray examination in such patients reveals an increase and deformation of the pulmonary pattern in the basal parts of the lungs; At times, focal-like tones can be detected. Since pneumonitis usually develops against the background of the current polyserositis, the described radiologic symptomatology is supplemented by a high standing diaphragm with signs of pleurodiaphragmal and pleuropericardial fusion and discoid atelectasis (linear shadows parallel to the diaphragm).

In the study of the gastrointestinal tract, aphthous stomatitis, dyspeptic syndrome and anorexia are noted. Pain abdominal syndrome can be associated with the involvement of the peritoneal process of the peritoneum, as well as the actual vasculitis - mesenteric, splenic, and others. Segmental ileitis develops less often. The defeat of the reticuloendothelial system is expressed in the increase in all groups of lymph nodes - a very frequent and early sign of the systemic nature of the disease, as well as in the increase in the liver and spleen. Actually lupus erythematous hepatitis develops extremely rarely. However, the increase in the liver may be due to cardiac insufficiency in pancarditis or pronounced exudate pericarditis, as well as the development of fatty liver.

Lupus diffuse glomerulonephritis (lupus nephritis) develops in half of patients usually during the generalization of the process.

There are different variants of kidney damage - urinary syndrome, nephritic and nephrotic. For the recognition of lupus nephritis, intravital puncture biopsy with immunomorphological and electron microscopic examination of the kidney biopsy is of great importance. Development of renal pathology in patients with relapsing articular syndrome, fever and persistently elevated ESR requires the exclusion of lupus genesis of nephritis. It should be remembered that almost every fifth patient with nephrotic syndrome has systemic lupus erythematosus.

The defeat of the neuropsychic sphere occurs in many patients in all phases of the disease. At the beginning of the disease - asthenovegetative syndrome, later develop signs of damage to all parts of the central and peripheral nervous system in the form of encephalitis, myelitis, polyneuritis. Characteristic is the defeat of the nervous system in the form of meningoencephalomy lopolyradiculoneuritis. Less common are epileptiform seizures. Possible hallucinations (auditory or visual), delusional conditions, etc.

The laboratory data are of diagnostic importance: the determination of a large number of LE cells, high titers of antibodies to DNA, especially to native DNA, to deoxyribonucleoprotein, Sm antigen.

In acute course, after 3-6 months, lupus-nephritis is often detected as a nephrotic syndrome. In the subacute course, a wavy form with involvement in the pathological process of various organs and systems and a characteristic polysyndromicity. Chronic course of the disease for a long time is characterized by the reductions of polyarthritis and (or) polyserositis, syndromes of discoid lupus, Reynaud; Only in 5 - 10th year the characteristic polysyndromicity gradually develops. In accordance with the clinical and laboratory characteristics, three degrees of activity of the process are distinguished; High (AND! Degree), moderate (AND degree) and minimal (! Degree).

Treatment. Patients need continuous multi-year complex treatment. The best results with the development of persistent clinical remission - with early treatment. In chronic and subacute flow and activity, nonsteroidal anti-inflammatory drugs and aminoquinoline derivatives are shown. The first are recommended for articular syndrome. It is important to select a drug taking into account its individual effectiveness and tolerance: voltaren (orthophen) 50 mg 2 to 3 times a day, indomethacin 25 to 50 mg 2 to 3. Once a day, brufen 400 mg 3 times a day, hingamin (chloroquine, delagil) 0.25-0.5 grams per day for 10-14 days, and then 0.25 g / day for several months. With the development of diffuse lupus-nephritis, plaquenyl is used successfully with 0.2 g 4-5 times a day for a long time under the control of the dynamics of the urinary syndrome.

In acute course from the very beginning, and with subacute and chronic course at III, II degree of activity of the pathological process, glucocorticosteroids are shown. The initial dose of these drugs should be sufficient to reliably suppress the activity of the pathological process. Prednisolone in a dose of 40-60 mg / day is prescribed for acute and subacute flow with a degree of activity and the presence of nephrotic syndrome or meningoencephalitis. With the same variants of flow with the II degree of activity, as well as in chronic course with III and II degree of activity, the suppressive dose should be 30-40 mg, and at the first degree of activity 15-20 mg / day The treatment with prednisolone in the overwhelming dose is carried out before the onset Pronounced clinical effect (according to the reduction in clinical and laboratory activity indicators). When the effect is achieved, the dose of prednisolone is slowly reduced.

One of the most important tasks and the key to the effectiveness of therapy are the selection of the lowest dose, which allows maintaining clinical and laboratory remission. Prednisolone in a maintenance dose of 5 to 10 mg / day is prescribed for several years. To reduce the side effect of glucocorticoids, it is recommended to combine this therapy with potassium preparations, anabolic steroids, diuretics and hypotensive drugs, tranquilizers, antiulcer measures. The most serious complications: steroid ulcer, septic infections, tuberculosis, candidiasis, psychosis.

In the aggressive course of the disease, high titer of autoantibodies, immune complexes, plasmapheresis is successfully used.

If glucocorticoids are ineffective, immunosuppressants (alkylating series or azathioprine) are prescribed. Indications for the appointment of cytotoxic drugs (usually in combination with moderate doses of corticosteroids) are as follows; 1) And! The degree of activity in adolescents and in the climacteric period; 2) nephrotic and nephritic syndromes; 3) the need to quickly reduce the suppressive dose of prednisolone due to the severity of side effects (rapid and significant increase in body weight, excessive arterial hypertension, steroid diabetes, severe osteoporosis with signs of spondylopathy); 4) the need to reduce the maintenance dose of prednisolone, if it exceeds 15-20 mg / day.

The most commonly used azathioprine (imarant) and cyclophosphamide (cyclophosphamide) at a dose of 1-3 mg / kg (100-200 mg / day) in combination with 30 mg of prednisolone. In this dose, the drug is prescribed for 2 - 2.5 months usually in the hospital, then recommend a maintenance dose (50-100 mg per day), which is given for several months and even 1 - a year or more.

To ensure the safety of treatment, careful monitoring of the blood test is required to prevent pancytopenia; It is necessary to avoid the attachment of infectious complications, dishepsic complications; When cyclophosphamide is taken, the risk of developing hemorrhagic cystitis can be reduced by administering an abundant drink (2 liters of fluid and more per day).

Since the ballroom needs long-term treatment after discharge from the hospital, they must be under the supervision of a therapist or rheumatologist in a polyclinic. In order to improve the tolerability of long-term corticosteroid therapy in polyclinic conditions, it is recommended delagil of 0.25 g / day and B vitamins, ascorbic acid in the form of a spring-autumn course. Patients are shown treatment in sanatoria of local type (cardiological, rheumatological). Climatological, physiotherapeutic treatment is contraindicated, since ultraviolet irradiation, insolation and hydrotherapy can cause exacerbation of the disease.