Tubular atrophic lesions
Introduction
Introduction Axillary atrophy: Focal segmental glomerulosclerosis (FSGS) is a common primary glomerular disease common in children and adults with nephrotic syndrome (NS). Histopathological features are glomerular segmental scars with or without glomerular capillary cell formation and adhesion. Focality means that only part of the glomerulus is involved (affected glomerulus <50%); segmental means that part of the glomerulus is involved; spherical sclerosis refers to the entire glomerular phase of the glass Change or scar formation. Pathological features are often associated with tubular atrophy and renal interstitial fibrosis.
Cause
Cause
Pathological features:
1 segmental: one or several glomerular sclerosis, normal renal glomeruli;
2 focal: a partial glomerular sclerosis;
More than 3 with tubular atrophy, renal interstitial fibrosis;
4 can be merged with MCD;
5 can be merged with MsPGN. According to domestic statistics, mesangial proliferative nephritis (membrane proliferative glomerulonephritis) is the most common, followed by focal segmental glomerulosclerosis, membrane proliferative nephritis, membranous nephropathy.
Examine
an examination
Related inspection
Adrenal CT examination of kidney CT
The disease occurs mostly in children and adolescents, with slightly more men than women. A small number of patients have upper respiratory tract infections or allergic reactions before onset. The most common clinical first symptom is nephrotic syndrome. About 2/3 of the patients have a large amount of proteinuria and severe edema. The amount of urine protein can be 1~30g/d. About 50% of patients have hematuria. The microscopic hematuria is more common. Occasionally, gross hematuria. 30% to 50% of patients with mild persistent hypertension or chronic nephritis syndrome, 24h urine protein <3.5g / d, edema is not obvious, often hematuria, hypertension and renal insufficiency, and 50 More than % can be manifested as a kidney complex, with obvious clinical manifestations of "three highs and one low". A small number of patients have no obvious symptoms, and occasionally proteinuria is found during routine urine tests. This type of asymptomatic proteinuria lasts for a long time and has a good prognosis. A small number of patients of this type can also gradually develop into end-stage renal failure. Most proteinuria is non-selective, but can be highly or moderately selective in the early stages. Serum C3 concentration was normal and IgG levels were decreased. There is often a manifestation of impaired proximal tubular function. Upper respiratory tract infections or allergies can exacerbate these symptoms.
The clinical manifestations of different pathological types of this disease are slightly different. Typical FSGS with glomerular hypertrophy, less urine protein; cell type FSGS often has a large amount of proteinuria (>10g / d), and is prone to renal insufficiency. It has been reported that 60% of patients with cell type FSGS have serum creatinine >2 mg/dl, while only 10% of patients with typical FSGS have elevated serum creatinine. Collapsed FSGS also has significant proteinuria, often >10g/d, and renal insufficiency is more severe than other types, while hypertension is relatively rare. This type of onset is acute and progresses rapidly. It usually enters end-stage renal failure (ESRF) 1 to 2 years after onset.
The clinical manifestations of children are similar to those of adults, mostly nephrotic syndrome, and the incidence of hypertension and renal insufficiency is lower than that of adults. Most (40% to 60%) FSGS showed chronic progressive progression, eventually leading to renal failure, a small number of patients (10% to 15%) progressed faster, and kidney failure occurred earlier.
There is no reliable indicator for the diagnosis of this disease. In the diagnosis of FSGS, renal biopsy should be relied upon and all possible secondary factors such as HIV infection and drug use should be excluded. Careful examination of medical history, physical examination and laboratory tests can help to differentiate the diagnosis. For example, patients with nephrotic syndrome or simple proteinuria are associated with proximal tubular dysfunction; persistent nephrotic syndrome with hypertension, microscopic hematuria, non-selective proteinuria; patients who are not sensitive to hormones should Suspected FSGS. Renal biopsy is helpful in diagnosis. Typical focal segmental glomerulosclerosis (FSGS) is characterized by focal lesions affecting a small number of glomeruli (focal) and glomerular parts (segments). It begins in the proximal medullary glomerulus, and the light involves only a few capillary sacral areas. The severe ones affect most glomeruli. The lesions are uniform and have no cell- or cell-free hyaline degeneration. Foam cells, transparent drops), severe cases of balloon adhesion, visceral epithelial cell proliferation to form a "hat-like" structure, and even "umbilical" lesions.
The other is focal glomerular sclerosis. The renal tubular epithelial cells of the affected nephron often atrophy, and the surrounding matrix shows cell infiltration and fibrosis. Electron microscopy showed that most of the glomeruli or all glomerular foot processes were fused, epithelial cells and their foot processes were detached from the basement membrane, and electron dense deposits were deposited at the endothelial cells and mesangium. Immunofluorescence showed irregular, lumpy, nodular deposits of IgM and C3 in the hardened area. Non-lesion glomeruli were negative or diffuse IgM, C3 deposition, IgA, IgG is rare. The disease is easily misdiagnosed as minimally pathological nephropathy, so it is necessary to combine clinical manifestations, renal histology, response to hormone therapy, and whether there is spontaneous remission or drug-induced remission. Helps the differential diagnosis of FSGS and MCD. Glomerular focal segmental sclerosis, in addition to FSGS, can also be seen in the chronic development of a variety of kidney diseases, such as obstructive nephropathy, reflux nephropathy, AIDS patients and diacetyl morphine addicts; Seen in obese people. Therefore, a comprehensive analysis is required to make a correct diagnosis.
Diagnosis
Differential diagnosis
Non-collapsed focal segmental glomerulosclerosis
The relationship between CG and FSGS is still controversial. Some scholars believe that CG is an independent disease, and most scholars believe that CG is a serious type of non-collapsed focal segmental glomerulosclerosis (NC-FSGS). The difference between them is that clinically, the number of urinary protein greater than 10g/d in CG patients is significantly higher than that of NC-FSGS; the proportion of renal dysfunction is high at onset, and renal function deteriorates rapidly. The pathological difference is: 1CG is the collapse of glomerular capillaries, the matrix is obviously dilated, and the lesion segment rarely adheres to the glomerular capsule, while NC-FSGS is opposite. 2CG epithelial cells are hypertrophied and have intracellular granules. The lesion segment of 3CG is rarely located in the vascular pole of the small ball, and this change is common in NC-FSGS. 4CG tubulointerstitial inflammation, atrophy and fibrosis were more obvious than NC-FSGS. Immunology confirmed that the glomerular tubules of CG have more hyperplasia markers than NC-FSGS. Despite this, because CG lesions are mostly segmental focal distribution, they are still classified as idiopathic focal segmental glomerulosclerosis, but as a special subtype, its clinical manifestations and morphological changes and special characteristics Sexual FSGS is different.
2 focal and segmental proliferative glomerulonephritis
The late lesions can also be similar to the pathological changes of the disease, this lesion is also more common in IgA nephropathy, focal proliferative lupus nephritis and purpuric nephritis, small vasculitis. The pathological changes were focal segmental endothelial cells and mesangial cell hyperplasia, with focal and segmental distribution of crescent formation. According to its corresponding clinical manifestations and characteristic immunofluorescence, it can be identified.
4. Focal glomerular fibrosis
It is less common in the pathology of this disease. Lesions of the diseased glomeruli were stained with collagen fibers, and negative for silver staining and PAS staining.
5. Minimal lesion nephropathy
Most scholars currently believe that MCD and FSGS are two different types of kidney disease. In the early stage of FSGS, lesions were mostly confined to the junction of the skin and the marrow, so kidney biopsy often confused with MCD because it could not be worn. Therefore, attention should be paid to the identification of the two, such as glucocorticoid-insensitive and older, may be early FSGS, repeated renal biopsy if necessary. Continuous sectioning improves diagnostic rates. There are few morphological changes in the glomeruli under MCD. Double-folded fat droplets were seen in the renal tubular epithelial cells, and vacuolar-like changes were observed in the proximal convoluted tubule epithelial cells. Under electron microscope, the epithelial cells were swollen, the foot processes merged into lamellae, the filter pores were occluded, and the epithelial vacuolar degeneration, microvilli morphology, protein absorption drops and lysosomes increased. Immunofluorescence was mostly negative, and occasionally IgG and/or IgM, IgA, and C3 were deposited.
In addition, in the normal population over 40 years old, the subcapsular cortex may have cirrhotic glomeruli, which should be distinguished from this disease.
The disease occurs mostly in children and adolescents, with slightly more men than women. A small number of patients have upper respiratory tract infections or allergic reactions before onset. The most common clinical first symptom is nephrotic syndrome. About 2/3 of the patients have a large amount of proteinuria and severe edema. The amount of urine protein can be 1~30g/d. About 50% of patients have hematuria. The microscopic hematuria is more common. Occasionally, gross hematuria. 30% to 50% of patients with mild persistent hypertension or chronic nephritis syndrome, 24h urine protein <3.5g / d, edema is not obvious, often hematuria, hypertension and renal insufficiency, and 50 More than % can be manifested as a kidney complex, with obvious clinical manifestations of "three highs and one low". A small number of patients have no obvious symptoms, and occasionally proteinuria is found during routine urine tests. This type of asymptomatic proteinuria lasts for a long time and has a good prognosis. A small number of patients of this type can also gradually develop into end-stage renal failure. Most proteinuria is non-selective, but can be highly or moderately selective in the early stages. Serum C3 concentration was normal and IgG levels were decreased. There is often a manifestation of impaired proximal tubular function. Upper respiratory tract infections or allergies can exacerbate these symptoms.
The clinical manifestations of different pathological types of this disease are slightly different. Typical FSGS with glomerular hypertrophy, less urine protein; cell type FSGS often has a large amount of proteinuria (>10g / d), and is prone to renal insufficiency. It has been reported that 60% of patients with cell type FSGS have serum creatinine >2 mg/dl, while only 10% of patients with typical FSGS have elevated serum creatinine. Collapsed FSGS also has significant proteinuria, often >10g/d, and renal insufficiency is more severe than other types, while hypertension is relatively rare. This type of onset is acute and progresses rapidly. It usually enters end-stage renal failure (ESRF) 1 to 2 years after onset.
The clinical manifestations of children are similar to those of adults, mostly nephrotic syndrome, and the incidence of hypertension and renal insufficiency is lower than that of adults. Most (40% to 60%) FSGS showed chronic progressive progression, eventually leading to renal failure, a small number of patients (10% to 15%) progressed faster, and kidney failure occurred earlier.
There is no reliable indicator for the diagnosis of this disease. In the diagnosis of FSGS, renal biopsy should be relied upon and all possible secondary factors such as HIV infection and drug use should be excluded. Careful examination of medical history, physical examination and laboratory tests can help to differentiate the diagnosis. For example, patients with nephrotic syndrome or simple proteinuria are associated with proximal tubular dysfunction; persistent nephrotic syndrome with hypertension, microscopic hematuria, non-selective proteinuria; patients who are not sensitive to hormones should Suspected FSGS. Renal biopsy is helpful in diagnosis. Typical focal segmental glomerulosclerosis (FSGS) is characterized by focal lesions affecting a small number of glomeruli (focal) and glomerular parts (segments). It begins in the proximal medullary glomerulus, and the light involves only a few capillary sacral areas. The severe ones affect most glomeruli. The lesions are uniform and have no cell- or cell-free hyaline degeneration. Foam cells, transparent drops), severe cases of balloon adhesion, visceral epithelial cell proliferation to form a "hat-like" structure, and even "umbilical" lesions.
The other is focal glomerular sclerosis. The renal tubular epithelial cells of the affected nephron often atrophy, and the surrounding matrix shows cell infiltration and fibrosis. Electron microscopy showed that most of the glomeruli or all glomerular foot processes were fused, epithelial cells and their foot processes were detached from the basement membrane, and electron dense deposits were deposited at the endothelial cells and mesangium. Immunofluorescence showed irregular, lumpy, nodular deposits of IgM and C3 in the hardened area. Non-lesion glomeruli were negative or diffuse IgM, C3 deposition, IgA, IgG is rare. The disease is easily misdiagnosed as minimally pathological nephropathy, so it is necessary to combine clinical manifestations, renal histology, response to hormone therapy, and whether there is spontaneous remission or drug-induced remission. Helps the differential diagnosis of FSGS and MCD. Glomerular focal segmental sclerosis, in addition to FSGS, can also be seen in the chronic development of a variety of kidney diseases, such as obstructive nephropathy, reflux nephropathy, AIDS patients and diacetyl morphine addicts; Seen in obese people. Therefore, a comprehensive analysis is required to make a correct diagnosis.
