IgA nephropathy new clinical classification and treatment recommendations

Textbooks often will IgA nephropathy (IgA-N) as an independent disease, but in fact, in the pathogenesis and clinical manifestations of change too much, treatment and prognosis is completely different and should not be used as a single disease, but must divide type.

Currently referred to in the literature and the pathological type IgA-N have been too old, such as Lee's, Haans, etc., the fundamental problem is the essence of the disease untouched, at most only a certain prognostic significance.

IgA-N in the pathology must be polymorphic, but so far no performance characteristic; and clinical manifestations of IgA-N has already shown some characteristics of the assembly. Therefore clinical pathological classification based on clinical manifestations may make our understanding of the IgA-N push forward further.

First, the clinical classification basis

1, some clinical manifestations of recognized prognostic significance

Isolated microscopic hematuria; recurrent gross hematuria; proteinuria;

Persistent hypertension

2, some of the pathological features have prognostic significance

Basement membrane damage; pure mesangial lesions

3, some of the pathological features of guiding therapeutic implications

Vasculitis-like lesions (lots of crescent formation)

4, some clinical signs have therapeutic significance

The rapid emergence of recurrent infection gross hematuria

Second, the new clinical classification

(A) simple microscopic hematuria type (I-H): 2.8%

1. urine abnormality: microscopic hematuria, proteinuria no, no gross hematuria.

2. normal renal function, no hypertension.

3. Pathology: pathological changes in light, only mesangial IgA deposition, less hardened ball, no crescent, tubulointerstitial lesions less, vascular disease is not obvious. SEM exclude "thin basement membrane nephropathy."

(B) urinalysis anomaly (U-ab): (42.9%)

1. Onset often hidden, the exact course is not easy to break clear, no obvious clinical symptoms characteristic.

2. urine abnormality: microscopic hematuria or gross hematuria single episode, urine protein <2.0g / 24hr.

3. No hypoproteinemia with normal renal function, no hypertension.

4. Pathology: pathological changes of varying severity, from mild mesangial proliferative disease, FSGS to glomerulosclerosis. Mesangial IgA deposits in addition to outside, often IgG, vascular loop deposition can occur. Mild to moderate interstitial disease. But there is no extensive hardening.

(Iii) recurrent gross hematuria type (R-GH): 13.2%

1. recurrent gross hematuria, can be fresh or stale, frequency ≥2 times. Before the onset of several hours (no longer than 24 hours) prodromal infection (flu on the majority, it may be cholecystitis or diarrhea), during the attack may have backache abdominal pain.

2. The gross hematuria between attacks may have sustained abnormal urine, but urinary protein generally <1.5g / 24h, no more than 2.0g / 24h. No significant hypoproteinemia, renal function normal or mildly abnormal.

3. The age of onset in youth.

4. Pathology: gross hematuria episodes within January, showing segments of cellular crescents (<10%), no loop necrosis. Less glomerulosclerosis, interstitial lesions less, no serious vascular disease. <P = "">

(Iv) crescent shape (Cres IgA-N.): 7.2%

1. sustainable long time, often accompanied by gross hematuria or microscopic hematuria more than 500,000 / ml.

2. Can hypertension, Scr be raised slightly. ANCA may be positive in some patients.

3. Pathology: Often accompanied loop necrosis, crescent> 15%, blood vessels may exhibit fibrinoid degeneration and necrosis, Fibrin staining.

(V) a large number of proteinuria (MP): 10.9%

1. proteinuria and edema as the main performance, usually without gross hematuria. Urinary protein> 3.5g / 24h, hypoproteinemia obvious, Alb <30g / l, hyperlipidemia. There was swollen.

2. normal or mildly elevated blood pressure, kidney function is not normal. Longer course.

3. Pathology: Small ball sclerosis were more common, often based film lesions, tubulointerstitial lesions light - moderate.

(F) hypertensive (HT): 18.9%

Outstanding performance is sustained elevated blood pressure, antihypertensive drugs commonly used to control, may have varying degrees of renal insufficiency, it can also be combined to a certain extent of abnormal urinalysis.

1. isolated hematuria or persistent microscopic hematuria, urinary protein <3.5g / 24h.

2. That is the beginning of the disease have high blood pressure, when the group of blood pressure> 140 / 85mmHg, with or without other target organ damage.

3.Scr normal or elevated, but <5mg / dl.

4. pathology: chronic disease heavier, more global sclerosis, interstitial lesions to severe. Prominent vascular lesions, vascular lesions more transparent.

(Vii) type of end stage renal failure (ESRD)

1. serum creatinine greater than 5.0mg / dl

Common global sclerosis, tubulointerstitial lesions 2. severe pathology.

Third, treatment recommendations for each type of IgA nephropathy

Simple microscopic hematuria immunosuppressant invalid, Chinese medicine treatment, follow-up light

Urine abnormal type of Chinese medicine treatment + tripterygium + ACEI + ARB, you can try Xiao noted (MMF)

Crescent shape MMF + Chinese medicine, hormone + tripterygium

Chinese medicine treatment of recurrent gross hematuria, try tripterygium + tonsil (lesions) debridement

Macroalbuminuria type Chinese medicine treatment, symptomatic and supportive, hormone + immunosuppressants

Hypertensive medicine treatment, symptomatic and supportive, hormone + immunosuppressants


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