Renal disease - one of the first or even the only manifestation of the disease. SLE - diffuse autoimmune connective tissue disease . Sick more often young women , often after sun exposure , pregnancy , vaccination. Renal prognosis is that observed in 50-70 % of patients.
Reasons for the development of SLE - Violations of cellular and humoral immunity, the formation of autoantibodies to cell nuclei and components of immune complexes.
Specific value have a viral infection and genetic factors .
symptoms
The clinical picture of lupus nephritis is extremely diverse - from persistent minimal proteinuria , does not reflect the state of health of patients and does not influence the prognosis of the disease , to the hardest of rapidly progressive nephritis with edema , anasarca , renal failure and hypertension . Renal disease occurs more frequently in acute and subacute disease.
Depending on the severity of the clinical picture , course , prognosis are three variants of lupus nephritis :
- Aggressive type ( subacute ) lupus nephritis with nephrotic syndrome occurs , hypertension , sometimes malignant , early development ( in the first months ) renal failure ;
- Is characterized by rapid and progressive course in the absence of an active complex therapy of patients leads to death within 6-12 months. 20% of all cases of lupus nephritis may be accompanied by carditis , cerebrovasculitises ;
- Active lupus vasculitis occurs with marked proteinuria ( half of the patients - with nephrotic syndrome ) , red blood cell , leukocyturia , often with hypertension.
Feature of lupus nephrotic syndrome is a rare and very high proteinuria giperalfaglobuline Prize , a moderate increase of cholesterol in the blood serum. SLE is 5-10% of the cause of nephrotic syndrome. Expressed eritrotsiturii usually associated with significant proteinuria , but may be isolated hematuric lupus nephritis . Pyuria may be a consequence of active inflammation in the kidneys (high limfotsituriya ) and joining often result urinary tract infection with a predominance of neutrophils in the urine sediment . With timely and effective treatment, the prognosis is favorable , with insufficient active treatment developed renal failure, but at a later date than the first embodiment of the flow. Most unfavorable prognostic sign - hypertension and renal failure ;
subclinical proteinuria . Protein content of the urine to 0.3 g / l in combination with a small leukocyturia eritrotsiturii and blood pressure under normal and reduced renal function. With this version of SLE are the leading joint syndrome , serozity , pneumonitis , myocarditis . Renal disease is not progressing normally . Forecast determined by the severity extrarenal lesions.
The diagnosis is not difficult , if kidney failure occurs on the background of other characteristic symptoms of lupus .
Diagnostically important are the following features of SLE :
- General siptomy - fever (usually without shivering ) , weight loss ;
- Joint damage - migrating polyarthralgia defeat small joints of the hands , rarely large joints , deformation is rare ;
- Dermal , which is based on peripheral vasculitis : erythema of the face and hands, pigmentation , cheilitis (vasculitis around the vermilion border ) , Raynaud's syndrome , lupus erythema face preferentially localized on the cheeks , nose and wings of the nose ( "butterfly") , discoid rash with skin atrophy and scarring ;
- Poliserozita - pleurisy ;
- Lung damage ;
- CNS - seizures , psychosis, epileptiform seizures , sometimes depression , behavioral disorders ;
- Laboratory parameters - increased erythrocyte sedimentation rate , leukopenia with lymphopenia , thrombocytopenia, anemia , hyper- gamma globulinemiya , gipokomplementemiya .
In 90% of blood nablyudny find LE- cells and antinuclear factor . Reliable histological features .
Unlike SLE periarteritis nodosa affects mainly men , proceeds with peripheral polyneuritis , abdominal crises , leukocytosis. Renal disease is characterized by vasculitis and malignant hypertension with moderate urinary syndrome , often with hematuria .
Hemorrhagic vasculitis often occurs in young men after respiratory infections occurs with abdominal crises ; unlike SLE often affects the large joints , with episodes of gross hematuria jade , symmetrical rash ( purpura) on the shins with residual pigmentation.
Rheumatoid arthritis also occurs with jade, joint disease , heart, lungs , but for rheumatoid arthritis is characterized by long-term course of the disease with the development of resistant strains of joints and muscles atrophy intercostals expressed radiological characteristic morphological changes to the identification of amyloidosis , which almost never occur in SLE . Detection of amyloid in renal biopsy , gums or rectal mucosa to distinguish SLE from other cases of amyloidosis - both secondary and primary . The differential diagnosis of amyloidosis is important to limit the immunosuppressive therapy , which may potentiate amyl oidogenez .
In some cases it is necessary to differentiate from lupus nephritis myeloma nephropathy flowing normally in older women with bone pain , a sharp increase in ESR, hyperproteinemia proteinuria , anemia. Can clarify the diagnosis of myeloma using immuno protein fractions of blood serum and urine , sternal puncture , bone X-ray studies , identify hypercalcemia. More important exception infections and tumors that require immediate therapy, may resemble SLE , subacute bacterial endocarditis, flowing with fever, leukopenia , anemia , elevated ESR , cardiac disease , nephritis , sometimes with nephrotic syndrome , in doubtful cases required echography, blood cultures . It is also important exception of tuberculosis, which may join the lupus nephritis after immunosuppressive therapy massive and tumors, especially kidney cancer with frequent paraspetsificheskimi reactions.
treatment
The basis of treatment comprise glucocorticoids cytostatics , and only the highest dose may stop the process in the kidneys. Should be considered contraindications to the use of glucocorticoids ( prednisone ) . In severe forms of lupus nephritis shown heparin treatment in combination with antiplatelet agents . With the rapidly progressiruschem lupus nephritis spend four -component scheme (prednisolone + + + heparin cytostatic chimes ); ultrahigh doses of intravenous prednisolone , plasmapheresis . After arresting acute shown maintenance therapy with prednisolone and cytostatic within 2 - 3 years , further treatment is carried out 4- aminoquinolines , indomethacin . When ESRD may conduct chronic hemodialysis and renal transplantation . In the absence of extrarenal activity of immunosuppressive therapy is not indicated , treatment is justified 4- aminoquinolines , indomethacin Curantylum .
Patients with lupus nephritis should be kept under constant medical supervision inspection 1 once a week for 3 months after hospitalization for acute , further inspection of one every 2-3 months . Upon reaching full remission inspection 1 time in 6 months .
Prevention is the prevention of renal damage in extrarenal SLE and prevention of exacerbations of jade with already developed kidney disease .
The prognosis for lupus nephropathy is not quite favorable , but rational treatment can achieve long-term remission and stabilization process.
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