(Glomerulonephritis with crescent formation)
The rapidly progressive glomerulonephritis (RPGN) is accompanied within weeks to months, acute nephritic syndrome from a microscopic glomerular crescent formation with progression to renal failure. The diagnosis is based on history, urine findings, serological tests and kidney biopsy. Treatment is with corticosteroids with or without cyclophosphamide, sometimes by plasma exchange.
RPGN, a kind of nephritic syndrome is accompanied by severe glomerular crescent-shaped formations (which show up in the biopsy) that within weeks or months if left untreated can ESRD. It is relatively rare, affects 10-15% of patients with glomerulonephritis (GN) and is mainly used in patients aged 20 to 50 years ago. Forms and causes are divided based on the findings on immunofluorescence microscopy and serological tests for. As anti-GBM antibodies, anti-neutrophil cytoplasmic antibodies [ANCA] -see Table: Classification of rapidly progressive glomerulonephritis based on immunofluorescence microscopy).
The rapidly progressive glomerulonephritis (RPGN) is accompanied within weeks to months, acute nephritic syndrome from a microscopic glomerular crescent formation with progression to renal failure. The diagnosis is based on history, urine findings, serological tests and kidney biopsy. Treatment is with corticosteroids with or without cyclophosphamide, sometimes by plasma exchange. RPGN, a kind of nephritic syndrome is accompanied by severe glomerular crescent-shaped formations (which show up in the biopsy) that within weeks or months if left untreated can ESRD. It is relatively rare, affects 10-15% of patients with glomerulonephritis (GN) and is mainly used in patients aged 20 to 50 years ago. Forms and causes are divided based on the findings on immunofluorescence microscopy and serological tests for. As anti-GBM antibodies, anti-neutrophil cytoplasmic antibodies [ANCA] -see Table: Classification of rapidly progressive glomerulonephritis based on immunofluorescence microscopy). Classification of rapidly progressive glomerulonephritis on the basis of immunofluorescence microscopy type percentage of cases of RPGN causes Type 1: Anti-GBM antibody-mediated ? 10% anti-GBM-GN (without pulmonary hemorrhage *) Goodpasture’s syndrome (pulmonary hemorrhage) Type 2: immune complex ? 40% post-infectious causes: anti-streptococcal antibody (. eg post streptococcal glomerulonephritis) Infectious endocarditis Vask uläre prosthetic nephritis Neonatal Hepatitis B infection visceral abscess or septicemia connective tissue anti-DNA antibodies (eg. . B. Lupus nephritis) IgA immune complexes (for example, IgA-associated vasculitis GN) Mixed IgG IgM cryoglobulins (eg cryoglobulinämische GN) Other glomerulopathies IgA nephropathy Membranoproliferative GN Idiopathic crescentic GN (rare) type. 3: pauci-immune ? 50% eosinophils granulomatosis with polyangiitis Pulmonary necrotizing granulomas limited to kidney disease, Microscopic polyangiitis type 4 (eg granulomatosis with polyangiitis.) (e.g., idiopathic crescentic GN.): Double-antibody positive rare the same as in the types 1 U.N d 3 * If the lungs are also affected, an anti-GBM glomerulonephritis is called Goodpasture’s syndrome. GBM Glomerular basement membrane =; GN = glomerulonephritis; RPGN = rapidly progressive glomerulonephritis. Antiglomerular basement membrane antibody disease, the anti-glomerular basement membrane (GBM) antibodies, glomerulonephritis (RPGN type 1) is an autoimmune GN and makes up to 10% of the RPGN-cases. It can occur when (eg cigarette smoke. B., virus UTI) a respiratory exposure or other stimulus alvelokapilläres collagen is released and triggers the formation of anti-collagen antibodies. The anti-collagen antibodies cross-react with GBM by fix complement and promote cell-associated inflammatory response in kidneys and usually the lungs. The term Goodpasture’s syndrome is the combination of a GN and alveolar hemorrhage in the presence of anti-GBM antibodies. GN without Alveolarblutungen in the presence of anti-GBM antibodies called anti-GBM glomerulonephritis. Immunofluorescence staining of renal biopsy tissue shows linear IgG deposits. Immune complex RPGN The immune complex RPGN (type 2 RPGN) results in numerous infectious diseases and connective tissue complications and also comes in other primary Glomerulopathies ago. Immunofluorescence staining shows nonspecific granular immune complex deposits. The change affects up to 40% of RPGN cases. The pathogenesis is unknown. Pauci-immune RPGN The pauci-immune RPGN (type 3 RPGN) is characterized by the absence of an immune complex or Komplementablagerung in immunofluorescence staining. It accounts for up to 50% of all RPGN cases. Almost all patients have increased anti-neutrophil cytoplasmic antibodies (ANCA usually antiproteinase 3-ANCA or myeloperoxidase-ANCA) and systemic vasculitis. Double antibody Disease The double antibody disease (type 4 RPGN) has characteristics of types 1 and 3, with the presence of anti-GBM and ANCA antibody. It is rare. Idiopathic RPGN idiopathic cases are rare. These include patients with any of the following: immune complexes (similar Type 2), but no obvious cause such as an infection, connective tissue disorder, or glomerular disorder. Pauci-immune features (similar to type 3), but the absence of ANCA antibodies symptoms and complaints The manifestations are usually insidious, with weakness, fatigue, fever, nausea, vomiting, anorexia, arthralgia and abdominal pain. In some patients, it is similar in patients with Pign, the sudden onset of hematuria. About 50% of patients have edema and a past history of acute influenza-like illness and the start of kidney failure within 4 weeks of the usually followed by a severe oliguria. At 10-30% a nephrotic syndrome is present. Hypertension is uncommon and rarely severe. Patients with anti-GBM antibodies can develop a pulmonary hemorrhage, which can manifest itself with hemoptysis or only by the finding of diffuse alveolar infiltration that is discovered during chest x-ray (pulmorenales syndrome or diffuse alveolar hemorrhagic syndrome). Diagnosis suspected Progressive kidney failure over weeks to months Nephritic Harnsediment Serological tests Serumkomplementspiegel renal biopsy The diagnosis is acute renal failure in patients with hematuria and dysmorphic red blood cells or red blood cell casts. Among the studies include serum creatinine, urinalysis, complete blood count, serological tests and kidney biopsy. The diagnosis is usually made by serology and renal biopsy. Serum creatinine is almost always increased. A urinalysis shows that hematuria is always present, and erythrocyte cylinders are usually available. A “telescopic sediment” (. D. H a sediment having a plurality of elements, including leukocytes, erythrocytes, and dysmorphic leukocyte, erythrocyte, granulocyte, wax and broad cylinder) is common. The blood count usually indicates anemia and leukocytosis is common. When serological testing the anti-GBM antibodies should be determined (anti-GBM antibody-GN) and antistreptolysin O antibody, anti-DNA antibodies or cryoglobulins (immune complex-RPGN), as well as the ANCA titer (Pauci-immune -RPGN). Komplementmessungen can be helpful in cases of suspected immune complex-RPGN because hypocomplementemia is common. The early rNierenbiopsie is crucial. A common feature of all RPGN types are focal proliferation of glomerular epithelial cells (sometimes interspersed with numerous neutrophils) that form crescent-shaped cell aggregates and fill in the epithelium of the capsule glomerularis (Bowman’s capsule) in> 50% of glomeruli. The glomerular balls appear hypocellular and collapsed. Necrosis, which may occur within the glomerular tuft or, together with the half-moons, represents the most striking abnormality. In such patients, the histologic evidence of vasculitis should be sought. Results of immunofluorescence microscopy are different for each type. In the anti-GBM antibody GN (type 1) has a band-like deposition of IgG along the GBM is most striking; it is often associated with a linear or sometimes granular deposition of C3. In immune complex RPGN (type 2), immunofluorescence shows diffuse, irregular mesangial IgG and C3 deposits. When Pauci-immune RPGN (Type 3) no immunostaining and deposits can be detected. However, there is independent of the fluorescence pattern fibrin in the crescents. In double antibody RPGN (type 4) is linear staining of GBM available (similar to type 1). In idiopathic RPGN Some patients have immune complexes (similar to type 2), and others have no immunostaining and deposits (similar to type 3). Rapidly progressive glomerulonephritis (type 1) Figure provided by Agnes Fogo, M.D., and the American Journal of Kidney Disease, Atlas of Renal Pathology (see www.ajkd.org). var model = {thumbnailUrl: ‘/-/media/manual/professional/images/rapidly_progressive_glomerulonephritis_type_1_high_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/-/media/manual/professional/images/rapidly_progressive_glomerulonephritis_type_1_high_de.jpg?la = de & thn = 0 ‘, title:’ Rapidly progressive glomerulonephritis (type 1) ‘, description:’ u003Ca id = “v38396901 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “”para “” u003e u003cp u003eAnti glomerulus basement membrane antibody glomerulonephritis (type 1 disease) is characterized by a smooth