What is IGA nephropathy

IgA nephropathy (IgA nephropathy, IgAN), also known as Berger disease, refers to the glomerular mesangial area with IgA or IgA deposition, with or without other immunoglobulin deposition in the glomerular mesangial Glomerulopathy.IgA nephropathy: caused by the disease of the kidney itself, more common. 2. Secondary IgA nephropathy: caused by diseases other than the kidney, such as: purpura nephritis, HIV infection, serum negative spondylitis, cancer, leprosy, liver disease, familial IgA nephropathy. [1] pathogenesis IgA nephropathy, the exact pathogenesis is not yet fully clear, a variety of factors and the incidence. The more consistent view is that IgAN is an immune complex caused by glomerular disease. 1. Relationship with the immune system: IgA nephropathy is predominantly multimeric IgA (PIgA) in glomerular deposition, suggesting that the IgA immune system leads to the presence of PIgA molecules in the circulatory system and deposition of glomerular mesangial regions The 2. The relationship with the bone marrow: glomerular mesangial deposition of IgA1, and exists in the blood IgA1 similar hinge glycosylation abnormalities, suggesting that IgA nephropathy in the glomerular mesangial area of ​​IgA for bone marrow IgA. 3. Relationship with cytokines: IgA1 in the mesangial area of ​​IgA nephropathy causes mesangial cell secretion of inflammatory factors. [2] [3] pathophysiology IgA nephropathy landmark pathological changes are IgA deposition in the glomerular mesangial area. Most patients at the same time with C3, IgG, IgM deposition. LEE grade: grade Ⅰ: glomerular lesions: the vast majority of normal, occasional mild mesangial widening (segment), with or without cell proliferation; tubulointerstitial changes: normal. Ⅱ grade: glomerular lesions: glomerular mesangial proliferation and sclerosis (50%), rare small crescent; tubule stromal changes: normal. Grade Ⅲ: glomerular lesions: diffuse mesangial proliferation and widening (occasionally focal stage), occasionally small crescent, balloon adhesions; tubulointerstitial changes: focal interstitial edema, occasionally cell infiltration, Rare tubules atrophy. Grade Ⅳ: glomerular lesions: severe diffuse mesangial proliferation, associated with sclerosis, partial or total glomerular sclerosis, visible crescent (<45%); tubulointerstitial changes: tubal atrophy, interstitial infiltration, even See interstitial foam cells. Ⅴ grade: glomerular lesions: lesions similar to grade Ⅳ, but more serious, visible crescent (> 45%); tubule interstitial changes: similar to grade Ⅳ, but more serious. [4] [5] Clinical manifestations 1. Recurrent nausea hematuria (30-40%) 1) Occurs in the upper respiratory tract infection (gastrointestinal or urinary tract infection) after a few hours to 1-2 days; 2) 3) gross hematuria children and adolescents (80-90%) are more common than adults (30-40%); 4) has nothing to do with the severity of the disease; 5) the number of patients with narcolepsy, Renal pathology is generally Lee's grade Ⅱ ~ Ⅲ grade. 2) with or without proteinuria (+); 3) a few have high blood pressure; 4) kidney (2) with or without proteinuria (+); 3) Pathology is generally Lee's grade Ⅱ ~ Ⅲ grade. (2) often high blood pressure; 3) renal function may be decreased; 4) renal pathology is generally Lee's grade Ⅱ ~ 2 ~ Grade IV. A large number of proteinuria or nephrotic syndrome 1) nephrotic syndrome, with or without microscopic hematuria; 2) more than high blood pressure; 3) some patients with nephrotic syndrome, kidney light microscope can be: small Lesions and mild diffuse proliferative glomerulonephritis; 4) renal pathology is generally Lee's grade Ⅰ ~ Ⅳ grade. 5) proteinuria (+ - ++), with or without microscopic hematuria; 3) often associated with renal insufficiency; 4) renal pathology is generally Lee's classification Ⅲ ~ Ⅳ level. (1) renal function progressive deterioration, progressive oliguria; 2) proteinuria (+ - ++), with or without gross hematuria; 3) more than high blood pressure, anemia; 4 ) Renal pathology is generally crescentic nephritis, Lee's grade IV ~ V grade. [5] [1] diagnosis of auxiliary examination 1. Immunological examination: 50% of patients with elevated serum IgA levels. 37 to 75% of patients measured specific IgA-containing circulating immune complexes. 2. Proteinuria: proteinuria quantitative and classification of IgA nephropathy to determine the prognosis is very important. Proteinuria <1g / 24 h are often mild and focal mesangial hyperplasia. Moderate to severe proteinuria mostly diffuse mesangial hyperplasia, often accompanied by crescent and glomerular sclerosis. 3. Renal function: serum creatinine rose to 1.5mg / dl (132.6umol / L) for the progression of the disease. GFR <20ml / min, the pathological changes are more than Ⅲ level. 4. hematuria: urine RBC morphology was pleomorphic, suggesting that the source of hematuria is glomerular origin. Disease diagnosis IgA nephropathy must be diagnosed with renal biopsy pathology, must have immunofluorescence or immunohistochemical results support. The diagnostic features are: light microscopic common diffuse mesangial proliferation or focal segmental proliferative glomerulonephritis; immunofluorescence can be seen in the mesangial area IgA or IgA-based immune complex deposition, which is the diagnosis of IgA nephropathy Logo. Differential diagnosis of the disease should be identified with the following diseases: 1. acute streptococcal glomerulonephritis after infection: 1 to 3 weeks before the onset of streptococcal infection precursor history, hematuria, edema and hypertension as the three main symptoms. Persistent nausea hematuria longer, from a few days to several weeks, this and IgA nephrotic onset hematuria is different. Laboratory tests have complement C3 decline, ASO, erythrocyte sedimentation rate. 2. benign family hematuria: the disease more than a family history, 90% of clinical manifestations of persistent microscopic hematuria, only a small number of intermittent seizures with hematuria. Generally asymptomatic, mostly in physical examination or urine routine examination found. Electron microscopy confirmed that some of them were thin basement membrane (base film thickness of about 1/3 ~ 2/3 of the normal number). Good prognosis. 3. familial hereditary nephritis: mostly persistent microscopic hematuria, male emphasis on women, progressive renal dysfunction, 50% associated with high frequency nerve deafness, 15% of eye abnormalities, high male mortality The 4. Left renal vein pressure syndrome: non-renal hematuria, no clinical manifestations of glomerulonephritis. 5. Idiopathic hypercalciuria: the performance of persistent microscopic hematuria, or associated with nausea hematuria, non-glomerular hematuria, urine Ca> 0.1mmol / kg (4mg / kg / d), urine Uca / Ucr ratio of> 0.21 can be the initial diagnosis of the disease. 6. Purpura nephritis: often manifested as microscopic hematuria. But the patient has skin bleeding, appear in the distal limbs, buttocks and lower abdomen, multi-symmetrical distribution, slightly higher than the skin surface, may have itching, 1 to 2 weeks after the gradual regression, often in batches. [4] [6] disease treatment of the disease without special treatment, according to the clinical manifestations of patients and the course of the disease, the use of different measures, the purpose is to protect renal function, slow the progression of the disease. In accordance with the clinical classification of IgA nephropathy 1. Isolated microscopic hematuria type: no special treatment, regular follow-up 2. Recurrent meniscus hematuria type: lesion removal such as tonsillectomy, according to how much use of proteinuria triple therapy 3. Urine abnormalities : 3) MMF treatment: methylprednisolone intravenous infusion therapy (0.5g / d) for three days, followed by prednisone 0.6 mg / (kg.d), reduced by 5 mg / d to 10 mg / d every 2 weeks, after which the dose was maintained. MMF to 0.5g, 2 / d began to administration, according to the plasma concentration increased to 1.5 ~ 2.0g / d, continuous use of 6 months to 0.75 ~ 1g daily dose maintenance, the total course of 2 years. 2) cyclophosphamide (CTX) treatment: methylprednisolone with MMF treatment. CTX impact 0.5 ~ 1.0g / m2, once a month, a total of 6 months, after every 3 months 1 times. Total dose <8g. CTX treatment after the end of azathioprine maintenance, the total course of 2 years. 5. A large number of proteinuria (with minor lesions): prednisone regular treatment. 6. A large number of proteinuria: Tripterygium glycosides 60mg / d, emodin, ACEI / ARB, low protein diet. 7. Hypertension: control blood pressure 125 / 75mmHg, ACEI / ARB, CCB, diuretics. Proteinuria> 1.5g / 24 cases can be home tripterygium polyglycoside tablets. 8. Other: to avoid colds, fatigue and the use of renal toxicity of Chinese and Western drugs and regular follow-up observation. Attention to the prevention and control of respiratory infections, tonsilitis in patients with recurrent tonsillitis can help reduce the onset of hematuria proteinuria, renal function may have long-term protective effect. [5] [1] disease prognosis and prognosis of IGA nephrotic factors: (1) elderly patients, the older onset of poor prognosis. (2) persistent microscopic hematuria associated with proteinuria, poor prognosis. (3) with renal insufficiency, poor prognosis. (4) IgA nephropathy in patients with high blood pressure, especially difficult to control severe hypertension, poor prognosis. (5) Pathological type above the Lee grade Ⅲ. IgAN prognosis affected by a variety of factors, in the clinical judgment of the prognosis of the disease from all aspects of comprehensive consideration. The prognosis of the disease has a greater heterogeneity, some patients can be completely relieved, poor treatment of patients with 15% to 40% of patients develop chronic renal failure. Disease prevention 1. Appropriate rest, avoid strenuous exercise. But when the condition is stable, proper exercise is necessary. 2. To increase the resistance to disease, to avoid cold, reduce the chance of infection, once the emergence of various infections, should be timely application of strong antibiotics and early control of infection. 3.IgA kidney disease control diet: diet mainly light, should eat more fruits, vegetables and high-quality high-protein foods, ban spicy, Feigan thick taste, and mildew products, pickled food, Ji Jiu. Disease care 1. Psychological care: explain all kinds of questions, explain the disease properly, with successful cases to encourage patients to create a quiet, clean, comfortable treatment environment for patients;IgA nephropathy, it is best to do renal biopsy confirmed. Clear pathology. 3. During the illness to prevent colds and fatigue, so as to avoid recurrence of the disease. 4. to block the invasion of antigen, infectious antigen can be removed through hospital surgery, especially the source of infection, combined with traditional Chinese medicine and anti-infective drugs, often play a multiplier effect. 5. Lee type Ⅲ or early mild IgA nephropathy in patients with general treatment ineffective, should be timely application of immunosuppressive therapy, according to pathological results and the wishes of patients, the choice of individual immunosuppressive therapy. Abandon immunosuppressive therapy, often lead to exacerbations or repeated prolonged healing. 6. have taken hormones, should be based on specific circumstances under the guidance of physicians to reduce the amount of hormone and the number of times. Must not be free to stop. 7. Nephropathy natural course usually takes a year or longer, should adhere to medication and inspection. References 1. People's Liberation Army Kidney Research Institute Academic Committee. Regulation and treatment of IgA nephropathy. Kidney disease and dialysis kidney transplantation, 2004, Volume 13, No. 03 2. Wei Zhenzhong. Clinical, Pathological and Therapeutic Treatment of Adult IgA Nephropathy. Department of Internal Medicine, Vol. 3, No. 04, Yang Xiaoqing, Gao Jin. New progress in the diagnosis and treatment of IgA nephropathy.

2. Life care: to ensure adequate sleep, every day should be 8 hours Above, bed rest to the naked eye hematuria disappeared, given low salt, low fat, low phosphorus, high calcium, high quality low protein diet, such as milk, fish Eat less animal offal and allergic foods; 3. strengthen skin care, keep the skin intact. Zhu Huanzhe often wash the bath for underwear, trim finger (toe) A; to help patients choose no stimulation or irritation of small toiletries; 4. close observation of blood pressure changes, maintaining water and electrolyte balance. Blood pressure 1 time, observe the record 24 hours of urine output, vomiting and the amount of liquid, according to the total amount of liquid volume calculation and the rational use of diuretics; 5. appropriate exercise, such as practicing qigong, tai chi and so on. 6. Actively prevent upper respiratory tract infection, repeated tonsillitis patients with feasible resection therapy. 7. Drug treatment should be carried out under the guidance of a doctor, to avoid the application of drugs that damage the kidney, such as gentamicin, amikacin and so on. Expert opinion 1. Occur in the naked eye hematuria, not chaos medication, first to the hospital after the inspection. 2. suspected

The clinical manifestations: recurrent nausea hematuria or microscopic hematuria, may be associated with varying degrees of proteinuria, some patients can be severe high blood pressure or renal insufficiency. Disease classification 1. Primary