Rapidly progressive glomerulonephritis

We thus refer to glomerulonephritis, which rapidly progress to renální insuficience, over the course of weeks or days. They are extremely rare in children, most patients belong to adolescents.

Etiopathogenesis
thumb|right|200px|Histologie u ANCA pozitivní glomerulonefritidy.

Etiologically, RPGN can be primary, i.e. without the presence of another renal or systemic disease, or secondary, i.e. in the course of other primary glomerulopathies or systemic disease.
 * According to the mechanism of formation, we distinguish 3 types of RPGN:
 * Type I = antirenal, with the presence of antibodies against GBM;
 * protilátky quickly destroy the capillary wall glomerulu;
 * genetic factors also play a significant role in pathogenesis, and etiological agents cause RPGN only in predisposed individuals.
 * Type II = immunocomplex, with a high concentration of imunokomplex;
 * immunocomplex etiopathogenesis is the most common.
 * Type III = pauciimmune, activation of immune mechanisms cannot be clearly demonstrated;
 * it is actually a picture of renal vaskulitidy, when no immune deposits are found in the kidneys.


 * According to the presence of autoantibodies and immunofluorescent findings in the biopsy material
 * Type I = ANCA-associated renal vasculitis
 * the finding of antibodies against the cytoplasm of neutrophil leukocytes when the immunofluorescence test is negative or only weakly positive (pauciimmune GN)
 * Type II = GN with a positive finding of anti-GBM antibodies – anti-GBM nephritis
 * Goodpasture syndrome - linear immunofluorescence due to binding of antibodies to GBM
 * Type III = Immunecomplex RPGN
 * IgA IgA nephropathy - with typically granular immunoflorescence

Pathological anatomy
All 3 mechanisms lead to a single morphological form – extracapillary proliferative GN, although they differ from each other in the immunofluorescence picture.Bowmannova prostoru is filled with proliferating epithelial cells (podocytes and Bowmann's capsule cells). Fibrin is always detected in the area of ​​proliferation, which proves the local activation hemokoagulačních mechanisms.

Clinical picture

 * Characterized by its difficult course from the beginning;
 * Characterized by its difficult course from the beginning;
 * the disease itself begins suddenly as an acute nefritický syndrom with macroscopic hematurií, oligoanuria, and hypertension;
 * systemic symptoms (arthralgia Raynaudův fenomén, purpura) are rarer in children.

Laboratory

 * Changes in KO – anémie with schistocytmild manifestations of hemolýzy, trombocytopenie;
 * changes in serum protein electrophoresis – hypergammaglobulinemia, increase in alpha-2-globulins and beta-globulins;
 * the picture of renální insuficience – decrease in GF, increase in urea, creatinine, phosphorus and potassium, hyponatremia, oliguria;
 * urinary findings – erythrocyturia, erythrocytic cylinders, proteinuria (possibly nephrotic in nature);
 * metabolická acidóza.
 * Specific findings:
 * type I is characterized by positivity of anti-GBM antibodies;
 * For type II, a high level of circulating immune complexes, a decrease in C3 and C4 components komplementan increased concentration of cryoglobulins;
 * in type III we can demonstrate antibodies against the cytoplasm of neutrophil leukocytesANCA.


 * c-ANCA are antibodies directed against proteinase-3 in the cytoplasm of neutrophils (we find them in Wegenerovy granulomatózy);
 * p-ANCA are antibodies against myeloperoxidase (found in mikroskopické polyangiitidy).

Diagnosis
It is based on the finding of acute nefritického syndromu and renal insufficiency. It is essential to perform a renal biopsy with findings of extracapillary glomerulonephritis and the corresponding immunofluorescence findings. Linear immunofluorescence of GBM in IgG is typical.

Therapy

 * methylprednisolonu pulses 10–30 mg/kg, max. 1 g/d iv in short infusions, followed by prednison therapy 1–2 mg/kg;
 * imunosupresiva: cyclophosphamide 2–3 mg/kg/d, chlorambucil 0.15 mg/kg/d or cyklosporin A 5–5,5 mg/kg/d;
 * plazmaferéza;
 * is today the therapy of choice ;
 * removes anti-GBM antibodies, high concentrations of immune complexes,komplement, koagulační faktory and trombocyty;
 * antikoagulancia: heparin;
 * antiagregancia: dipyridamole, ticlopidine;
 * transplantace Tx ledvin
 * after kidney Tx, type I recurs in the graft with a frequency of 10-30% - Tx is recommended only after several months of immunosuppressive and hemodialyzační treatment with the disappearance of anti-GBM antibodies.

Prognosis
The prognosis of RPGN is very serious.

Goodpasture syndrom
thumb|right|200px|Histologie u anti-GBM glomerulonefritidy.

Antibodies bind not only to the basement membrane of glomerular capillaries, but also to the basement membranes of pulmonary capillaries. It is assumed that overall toxic factors (especially nicotinism) are involved in the development of the genetically determined terrain. Laboratory findings and diagnosis are identical to type I RPGN, i.e. antirenal type, with the presence of antibodies against GBM. Circulating IgG anti-GBM antibodies can be demonstrated and the titer is an indicator of the severity of nephritis.

Clinic
Hematuria, hemoptýza, cough, dušnost and rapid development of renal insufficiency are characteristic. It very quickly leads to terminal kidney failure. Pulmonary symptoms usually precede renal damage. Pulmonary hemorrhage, hemoptysis, and dyspnea. Bleeding into the alveoli will appear on the X-ray image of the lungs in the form of diffuse cloudy shadows, located bilaterally and converging in the hilums. The patient has a restrictive ventilation disorder.siderofág in the sputum and the disease often results in the picture of idiopathic pulmonary hemosiderózy. Renal lesions are characterized by microscopic hematuria, small proteinuria with rapid progression to renal failure. Oliguria.

Therapy
Plasmapheresis has the greatest application to remove IgG anti-GBM antibodies and stop damage to target organs. The treatment protocol includes treatment with corticosteroids, cyclophosphamide for at least 3 months.

Související články

 * Akutní glomerulonefritida
 * Akutní selhání ledvin

Zdroj

 * HAVRÁNEK, Jiří: Rychle progredující glomerulonefritida.