Lupus nephritis is one of the most common complications of systemic lupus erythematosus. SLE is an autoimmune disease caused by the appearance in the body of antibodies that perceive “native” proteins as foreign. As a result, aseptic inflammation develops in different parts of the body. Including in the kidneys.
Definition
Lupus nephritis, or lupus nephritis, is a serious kidney disease with systemic lupus erythematosus. The prevalence of this disease in a population is an average of forty people per one hundred thousand people. Most often, representatives of the beautiful half from the age of twenty to forty are sick. Most often, pathology occurs in the Afro-Caribbean population.
The disease can be caused by a variety of factors: from excessive passion for tanning, to genetic disorders, so it is extremely important to pay attention to the symptoms in time and go to the doctor. After all, the sooner treatment is started, the better the prognosis for the life and health of the patient.
Etiology
Lupus nephritis can be triggered by constant prolonged insolation (hobby for tanning or living in sunny places), allergies to drugs, permanent stress, and even pregnancy (the fetus is perceived as a foreign organism and the immune system begins to attack mother cells).
In addition, the development of the disease is affected by genetic tendencies, the presence of hormonal imbalance, frequent viral diseases (viruses integrate into the cells of the body, leaving their antigens on their surface and thus provoke an immune response). The chance of developing a disease in close relatives is several times higher than the population average.
Pathogenesis
Lupus nephritis is part of a large symptom complex that develops with systemic lupus erythematosus. Autoantibodies primarily develop to native DNA and its combination with histones, to complement system proteins and cardiolipin. The reason for this aggression is a decrease in tolerance to their own antigens, defects in B and T-lymphocytes.
The development of nephritis is directly associated with the fact that antigen-antibody complexes are tropic to the tissues of the renal tubules. As soon as such a protein molecule attaches to the surface of the cell, it triggers a cascade of biochemical reactions, as a result of which the active substances that melt the cells are released. This, in turn, causes an inflammatory reaction, which only exacerbates the destruction.
Pathomorphology
Lupus nephritis in SLE can have various morphological manifestations. At autopsy of patients, there is a change in the membranes of the glomeruli of the kidneys, active division of their cells, expansion of the mesangium, sclerosis of vascular loops and more. These manifestations can be simultaneously in one or in several glomeruli.
The most specific for lupus nephritis is fibrinoid necrosis of the capillaries of the loop of Henle, as well as histologically detected karyopyknosis and karyorexis (separation and lysis of the cell nucleus). In addition, pathognomonic is a change in the basal membranes of the glomeruli in the form of “wire loops” and the presence of hyaline thrombi in the lumen of the capillaries as a result of deposition of immune complexes.
Classification
Clinically and morphologically, several stages can be distinguished that pass lupus nephritis. The World Health Organization classification is as follows:
- First class: the glomeruli have a normal structure.
- Second class: there are only changes in mesangium.
- Third class: glomerulonephritis with the defeat of half of all glomeruli.
- Fourth grade: diffuse glomerulonephritis.
- Fifth grade: membranous glomerulonephritis.
- Sixth grade: sclerosing glomerulonephritis.
There is also Serov's classification, in which he distinguishes focal, diffuse, membranous, mesangioproliferative, mesangiocapillary and fibroplastic glomerulonephritis.
Symptoms
Membranous lupus nephritis has both obligate and optional symptoms. One of its mandatory manifestations is proteinuria, that is, the presence of protein in the urine. You can also often find hematuria, leuko - and lymphocyturia. These signs indicate the presence of an inflammatory process in the kidneys and may not only be with SLE.
With the progression of the autoimmune process, the symptoms of renal failure increase, manifested by an increase in the level of creatinine in the blood and urine, weakness, lethargy of the patient, soporous conditions.
Slowly progressive and rapidly progressive nephritis is isolated. If the disease develops slowly, then urinary and nephrotic syndrome prevail. In addition, lupus nephritis can occur in an inactive or latent form when only minor proteinuria is present from all the symptoms.
The rapidly progressing lupus nephritis is very similar to classic glomerulonephritis. Renal failure is growing rapidly, there is macrohematuria, increased blood pressure and nephrotic syndrome. In severe cases, disseminated intravascular coagulation (DIC) syndrome may develop.
Lupus nephritis in children
In 1/5 of all patients with systemic lupus erythematosus, the first symptoms of the disease appeared in childhood. In children under 10 years of age, it practically does not occur, but there is a description of cases of SLE in a child one and a half months old.
The development of the disease in babies does not differ from that in adults. The clinical picture can be varied: from an asymptomatic course to a rapidly progressing one. Acute renal failure is rare.
There are several signs of SLE in a child:
- erythema on the face;
- discoid rashes on the body;
- sensitivity to sunshine;
- ulcers on the mucous membranes;
- joint inflammation;
- kidney damage
- violation of the central nervous system;
- increased bleeding;
- immunological disorders;
- the presence of antinuclear antibodies.
If the clinic has at least four signs from this list, then we can confidently say that the child has systemic lupus erythematosus. Kidney damage in childhood rarely comes to the fore. Symptoms typically range from glomerulonephritis to manifestations of antiphospholipid syndrome.
The prognosis of the development of the disease in children is more favorable. Ten years after diagnosis, only 10 percent of patients require dialysis.
Diagnostics
What gives the doctor a reason to suspect lupus nephritis? Diagnosis, as a rule, is based on already existing clinical and laboratory confirmed data on SLE:
- joint pain and inflammation;
- rashes on the skin of the face in the form of a butterfly;
- history of effusion in the cavity (pleurisy, pericarditis);
- rapid weight loss, fever.
In a general blood test, anemia, a decrease in platelets, an increase in the erythrocyte sedimentation rate, a decrease in complement proteins are observed. For diagnosis, it is important to identify antibodies to native DNA.
As a rule, this is enough to diagnose systemic lupus erythematosus and, as a result, lupus nephritis. However, the appearance of protein in the urine may be one to two years late from the manifestation of the disease. In this case, the doctor relies on enzyme-linked immunosorbent assay and antibody detection. If there is no confirmed laboratory data, then you need to continue the diagnostic search, especially for male patients, since for them this nosology is quite rare.
Lupus nephritis is differentiated in SLE with bacterial endocarditis, exacerbations of rheumatoid arthritis, myeloma, chronic hepatitis, amyloidosis and Shenlein-Genoch syndrome.
First stage of treatment
Treatment for lupus nephritis is a long and painstaking process that often lasts the rest of your life. It takes place in two stages. At the first stage, the exacerbation of the disease is stopped. The goal of treatment is to achieve stable remission, or at least reduce clinical manifestations.
Medication should begin as early as possible from the time of diagnosis. Everything happens so fast that even a delay of five to seven days can be a fatal mistake. If the activity of the process is low (antibody titers will show this), then the doctor may restrict the appointment of high-dose corticosteroids for a period of two months, followed by a slow dosage reduction (the drug cannot be abruptly canceled, the adrenal glands may refuse).
If the course of the disease is more rapid, then, in addition to steroids, large doses of cytostatics are administered intravenously. Such pulse therapy is carried out for six months. And only after this period, you can begin to reduce the dose of drugs and transfer the patient to oral medication.
Do not forget that patients with SLE often develop DIC syndrome, so it is recommended to take preventive measures, namely:
- blood transfusion and its components;
- intravenous administration of Trental;
- subcutaneous administration of 2.5 thousand units of Heparin.
Second stage of treatment
Lupus nephritis in SLE in the second stage is also treated with steroid hormones and cytostatics. Only their dose is much smaller. Very slowly, over a period of four to six months, a dose of Prednisolone is titrated up to 10 milligrams per kilogram of weight. Cytostatics are also prescribed in bolus doses once every three months, and if the positive dynamics of the disease persists, then they switch to a single injection in six months.
Such maintenance therapy can last for years. Over time, prevention of side effects from drugs and symptomatic treatment are added to it (if necessary) .
But even with timely treatment, fifteen percent of patients still develop renal failure. In this case, only hemodialysis sessions or kidney transplantation can help. Unfortunately, such treatments are not available to the general public.
Diet for lupus nephritis
In order to maintain kidney function, patients with lupus nephritis must adhere to certain rules:
- Drink plenty of fluids to filter blood well and maintain metabolic rate.
- Food should contain a small amount of potassium, phosphorus and protein, as these elements negatively affect damaged kidneys.
- To refuse from bad habits.
- Engage in light sports.
- Check your blood pressure regularly.
- Limit fatty foods.
- Do not take NSAIDs, as they negatively affect the kidneys.
If the patient follows these recommendations, then the quality of his life improves significantly, and the prognosis of survival becomes more promising.
Forecast
The prognosis for treating membranous lupus nephritis depends on how severely the kidneys are affected and the time of initiation of therapy. The sooner the patient goes to the doctor, the greater the likelihood of a favorable outcome.
Forty years ago, only a few patients with lupus nephritis lived more than a year from the time of diagnosis. Thanks to modern methods of treatment and diagnosis, patients can count on more than five years of life.