Clinical Types of IgA Nephropathy

Many patients with IgA Nephropathy may doubt how many types of this disease there are and which type they belong to. This article mainly focuses on solving this problem for the patients.
Type I: Gross hematuria
This type usually occurs secondary to mucomembranous diseases like upper respiratory tract infection, cold, overstrain, prophylactic immunization, pneumonia or enteritis. The urine is usually brown or seems like water where flesh is washed and blood clot is rarely seen. The immune latent period is the shortest, and it will remit spontaneously after lasting for several hours to one week. Those with repeated gross hematuria have a good prognosis while those with one isolated gross hematuria have a poor prognosis because they are usually accompanied with Nephrotic Syndrome and Hypertnesion and even acute oliguria or Acute Renal Failure which is probably related to blocking of the renal tubules by erythrocyte cast or necrosis of tubules.
Type II: Abnormal urinalysis
Onset of this type is latent. It can be divided into two types according to whether it is combined with hematuria.
A. Only persistent microscopic hematuria without proteinuria or hypertension. The cases are mainly mesangial proliferative changes, and renal interstitium and vascular lesions are not obvious.
B. Persistent microscopic hematuria with slight proteinuria. This type is more common and protein quantitation is less than 1.0g/d, without hypertension or hypofunction of the intrinsic cells. Pathological changes of this type vary greatly from mesangial proliferative changes to sclerosis of glomerular intrinsic cells, as well as interstitial lesions.
Type III: Massive proteinuria (Nephrotic Syndrome)
Protein quantitation is more than 2.0g/d and it is one of the factors that lead to a poor prognosis of IgA Nephropathy. This type often occurs in cases with severe glomerular changes and it usually shows focal segmental glomerulosclerosis with foot cell damage and fairly wide tubule-interstitial damage or crescents formation. If it is presented as sudden persistent proteinuria without gross hematuria and hypertension history, it can be further divided into two types.
A: Typical “three highs and one low” Nephrotic syndrome, with its pathological changes dominated by slight mesangial proliferation and usually without sclerosis of the intrinsic cells or obvious interstitial change.
B: massive proteinuria and unobvious swelling, usually with increased nocturia, which is clinically called “dry nephrosis”. The pathological changes are: extensive sclerosis of the glomerular intrinsic cells in renal tissues, interstitial fibrosis, protracted course of disease and poor prognosis.
Type IV: Acute Renal Failure
The features are acute onset, rapid progress and poor prognosis. It can be further divided into two types:
A: large amount of crescents formation and vasculitis-like changes ANCA (+).
B: during gross hematuria, large amounts of erythrocyte cast block the renal tubules, making it easy for the patients who are receiving anticoagulant treatment to have large amount of erythrocyte cast.
Type V: Chronic Renal Insufficiency (End-stage)
At the early stage of this type, renal tissues are often at the stage of “inflammatory reaction period” of the fibrosis. Functions of the renal intrinsic cells especially the “filtratin function” is in a high filtration and compensative state and there is no decrease of GFR. Along with development of the renal fibrosis, mesnagial cells gradually appear apoptosis and necrosis and are rapidly replaced by ECM (Extracellular Matrixes). There is also progressive decline and rapid deterioration of the cellular functions, which is followed by a series of Uremia symptoms. SCR (serum creatinine) >442, GFR<15ml/min. B-ultrasonic test shows atropy of both kidneys and thinning of cortex which becomes more sensitive to light. Gradually the disease enters scar formation period.
Certain period after damage of the mesangial cells, Acute Renal Failure will occur in 5% of patients, chronic renal damage in 10% of patients and 50% to 70% of the patients will develop Uremia after 20 years.
Type VI: Hypertension
Pathological features of this type: pathological changes will occur when endothelial cells are involved by mesangial cells lesions, manifested as functional disorder of endothelial cells which will cause more release of vasoconstrictive agents. Besides hematuria, clinical manifestations may also include hypertension especially in the elderly. This is an important sign of deteriorating progression of mesaangial cell diseases, which must be treated with hypotensive drugs to control the symptoms.
Pathological changes show much discarding changes like focal or global sclerosis of the glomeruli and extensive interstitial fibrosis.