Syndrome nephritic rapidly progressive

Syndrome nephritic rapidly progressive

Rapidly progressive nephritic syndrome is characterized by focal and segmental necrosis with proliferation of epithelial cells of the renal glomeruli in the form of half moons, in the clinical picture – proteinuria, hematuria and erythrocyte casts, the development of renal failure over several weeks or months. Etiology

    • infections
    • Post-streptococcal glomerulonephritis
    • Infective endocarditis
    • Sepsis
    • Systemic diseases
    • SLE
    • Sjoshain-Gönoch disease
    • Vasculitis, incl. Wegener’s granulomatosis
    • Essential cryoglobulinemia
    • Malignant tumors (rare)
    • primary kidney disease
    • Glomerulonephritis with crescents
    • Mesangioproliferative glomerulonephritis
    • Berger disease
    • Membranous glomerulonephritis, complicated by the formation of AT to the basement membrane of the glomeruli (rarely).

Pathogenesis. The disease is caused by the production of autoantibodies and immune complexes

    • Anti-basement membrane-mediated glomerulonephritis (20%)
    • No pulmonary hemorrhage, e.g. glomerulonephritis with anti-basement membrane AT
    • With pulmonary bleeding, such as Goodpasture’s syndrome
    • Immune complex mediated glomerulonephritis (40%)
    • Postinfectious
    • Diffuse connective tissue diseases
    • Other immunocomplex glomerulonephritis (IgA nephropathy, membranoproliferative glomerulonephritis, idiopathic glomerulonephritis)
    • Glomerulonephritis mediated by antineutrophil cytoplasmic autoantibodies (40%)
    • Glomerulonephritis with systemic necrotizing arteritis, such as microscopic polyarteritis
    • Glomerulonephritis with necrotizing granulomas, for example, Wögener’s granulomatosis.


    • Focal proliferation of glomerular epithelial cells and infiltration of leukocytes form crescents in 50-100% of all capsules. Fibrin is found inside the crescents
    • In the glomeruli, the number of cells is reduced, they subside, glomerular necrosis often develops
    • Diffuse interstitial edema with inflammatory infiltration of cells is replaced by fibrosis and a decrease in the number of inflammatory cells
    • Vacuoles, hyaline droplets, erythrocyte and hyaline cylinders are formed in the tubules
    • In the final stages of the disease, the renal tissue atrophies, the basement membrane thickens.
    • Fluorescent microscopy reveals linear deposits of IgG and segmental deposits of the C3 complement component.

Clinical picture

    • Symptoms may be similar to acute glomerulonephritis, but most often develops gradually. History of acute influenza-like illness 4 weeks before onset of symptoms of renal failure
    • Symptoms of CRF develop over several weeks or months: weakness, fatigue, nausea, vomiting, anorexia, joint and abdominal pain
    • Oliguria
    • Proteinuria, hematuria
    • Malignant arterial hypertension, retinopathy with retinal detachment
    • Hemoptysis in Goodpasture’s syndrome.

Research methods

    • Increased concentrations of urea, creatinine in the blood serum
    • General urine test
    • Macrohematuria (not always)
    • In the urine sediment, erythrocyte, leukocyte, granular, waxy casts
    • In blood tests, anemia, leukocytosis
    • Serological studies
    • Increased AT titer to streptococci
    • CEC
    • cryoglobulinemia
    • Hypocomplementemia
    • AT to glomerular basement membrane
    • Antineutrophil cytoplasmic autoantibodies
    • Ultrasound and x-ray examination: at the beginning of the disease – an increase in the size of the kidneys, then their decrease
    • Kidney biopsy.


    • Diet number 7a
    • Immunosuppressive therapy is a must
    • Glucocorticoids
    • Against the background of oral administration of prednisolone 0.5–1 mg/kg, it is recommended to conduct monthly pulse therapy with methylprednisolone 1 g/day IV for 3 days. The dose of prednisolone is reduced on average after 1-2 months gradually by 5 mg / week to ZOmg / day, then by 2.5-1.25 mg / week or simultaneously by 1/2 or 1/3, then by 2.5- 1.25 mg/week
    • Pulse therapy with methylprednisolone: ​​30 mg / kg (up to 3 g) intravenously for 20 minutes on days 1, 3 and 5, then from day 8, 2 mg / kg orally every other day, gradually reducing the dose over 3- 6 months
    • Cytostatics
    • Cyclophosphamide (in combination with glucocorticoids or without them) 1.5-3 mg/kg/day every day (no more than 12 weeks) or in the form of pulse therapy 1 g/day intravenously monthly. When combined with methylprednisolone pulse therapy, cyclophosphamide is prescribed on the first day
    • Cyclosporine is not indicated for severe renal failure, severe arterial hypertension, tubulo-interstitial changes in the kidney biopsy.
    • Antiplatelet agents (chimes, aspirin).
    • Plasmapheresis: 3-5 sessions precede pulse therapy with cyclophosphamide or methylprednisolone.
    • Antihypertensive therapy (see also Arterial Hypertension)
    • Restriction of table salt to 5 g / day
    • ACE inhibitors with or without diuretins
    • Calcium antagonists – with or without diuretics
    • Diuretics (furosemide, ethacrynic acid)
    • With refractory malignant arterial hypertension – a combination of ACE inhibitors, diuretins and B-blockers
    • For relief of hypertensive crisis (verapamil, furosemide, nifedipine, captopril, clonidine)
    • With the ineffectiveness of drug therapy, extracorporeal methods of sodium excretion are of additional importance – ultrafiltration, hemodialysis
    • Correction of doses of products that increase blood pressure (glucocorticoids, erythropoietin, cyclosporine).
    • In the terminal stages of the disease – hemodialysis, kidney transplantation. The risk of disease recurrence in the graft is high.

Course and forecast

    • In post-infectious glomerulonephritis and glomerulonephritis in SLE, Wegener’s granulomatosis, peri-arteritis nodosa, treatment improves kidney function
    • In untreated patients, the disease progresses to a terminal stage within 1–2 years.
    • In elderly patients with crescents in 75% of the glomeruli and more, the prognosis is unfavorable.

Synonym. Rapidly Progressive Glomerulonephritis Berger’s disease, Chronic nephritic syndrome, Acute nephritic syndrome, Lupus nephritis, Systemic lupus erythematosus, Goodpasture’s syndrome, ICD Wegener’s syndrome. N01 Rapidly progressive nephritic syndrome

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