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* Spontaneous remission is most likely in women, children, those with lesser amounts of proteinuria, and adults under age 50 years with a normal serum creatinine concentration and benign histologic features [1-3,8].
* Spontaneous remission is most likely in women, children, those with lesser amounts of proteinuria, and adults under age 50 years with a normal serum creatinine concentration and benign histologic features [1-3,8].
* Although unproven, the rate of partial remission may be higher with the use of ACE inhibitors or ARBs.
* Although unproven, the rate of partial remission may be higher with the use of ACE inhibitors or ARBs.
* Probability of progression — Based upon a study of 184 patients identified through the Toronto Glomerulonephritis Registry, a semiquantitative algorithm has been developed to predict the probability of progression to chronic kidney disease, which was defined as a creatinine clearance ≤60 mL/min per 1.73 m2 [8]. The probability of progression was assessed for those with 4, 6, and 8 g/day of proteinuria that persisted for 6, 9, 12, or 24 months.  Multiple additional variables (including age, sex, serum creatinine, and creatinine clearance on presentation, serum albumin, presence of hypertension, rate of change of creatinine clearance, and therapy) were also tested to determine whether the predictive value provided by proteinuria could be improved. Of these variables, the initial creatinine clearance and the rate of change of clearance were the most useful predictors.  Based upon this approach, the best fitting logistic model utilized the following clinical characteristics:  ●Persistent proteinuria for over six months  ●Creatinine clearance upon presentation  ●Slope of the decline in creatinine clearance over the assessed proteinuria period  The addition of renal pathology as a variable had no effect on the performance of the model.  This model was tested in two additional populations of patients identified through the Italian Idiopathic Membranous Nephropathy Study group (110 patients) and a database obtained from Helsinki University (84 patients) [9]. The accuracy in predicting the development of chronic renal insufficiency was 85, 87, and 79 percent in populations from Toronto, Finland, and Italy, respectively. This was higher than the predictive value of proteinuria >3.5 g/day at the time of biopsy, which was only 52, 43, and 34 percent, respectively, in the same populations.  Based upon the best fitting logistic model, the following are examples of the risk of progression to a creatinine clearance ≤60 mL/min per 1.73 m2 for patients with specific clinical characteristics over a follow-up period of more than five years:  ●The risk is only 6 percent for the patient with proteinuria <3.5 g/day and stable normal renal function over six months  ●The risk increases to 72 percent in the patient with proteinuria of 12 g/day, and a creatinine clearance on presentation of 96 mL/min, which declines to 78 mL/min by six months  Based upon this model, we define low-, moderate-, and high-risk patient subsets with varying degrees of risk for progression to more advanced kidney insufficiency (defined as a creatinine clearance ≤60 mL/min per 1.73 m2) over five years:  ●Low risk – Proteinuria remains less than 4 g/day and creatinine clearance remains normal for a six-month follow-up period. Such patients have a less than 8 percent risk of developing chronic renal insufficiency over five years.  ●Moderate risk – Proteinuria is between 4 and 8 g/day and persists for more than six months. Creatinine clearance is normal or near normal and remains stable over six months of observation. Chronic renal insufficiency develops over five years in approximately 50 percent of these patients.  ●High risk – Proteinuria is greater than 8 g/day and persists for three months and/or renal function that is either below normal (and considered due to MN) or decreases during the observation period. Approximately 75 percent of such patients are at risk of progression to chronic renal insufficiency over five years.  This risk stratification applies only to the likelihood of progressive renal failure. It does not take into account the risks that may be associated with other complications of the nephrotic syndrome, such as worsening atherosclerosis due to hypercholesterolemia and thromboemboli due to the associated hypercoagulable state. Thus, in addition to preservation of renal function, immunosuppressive therapy may be beneficial by shortening the overall exposure to the nephrotic state, thereby minimizing the risk of other complications. (See 'Lipid lowering' below and 'Anticoagulation' below.)  Acute decline in renal function — Progressive MN typically occurs gradually. Some patients develop an acute decline in renal function and at least three disorders should be excluded in this setting:  ●Acute bilateral renal vein thrombosis which may be associated with flank pain. (See "Renal vein thrombosis and hypercoagulable state in nephrotic syndrome".)  ●Drug-induced acute interstitial nephritis, in which white cell, white cell casts, and possibly eosinophils are typically seen in the urine sediment. (See "Clinical manifestations and diagnosis of acute interstitial nephritis".)  ●Superimposed crescentic glomerulonephritis, in which red cells and cellular casts are found in the urine sediment. (See "Causes and diagnosis of membranous nephropathy".)  Secondary MN — In patients with secondary MN, cessation of the offending drug (eg, penicillamine, gold, or nonsteroidal anti-inflammatory drug) or effective treatment of the underlying disease is usually associated with improvement in the nephrotic syndrome. With penicillamine- or gold-associated disease, protein excretion may continue to rise for the first 1 to 12 months (mean 2 months) after the offending drug has been discontinued [18,19]. The mean time to resolution of proteinuria is 9 to 12 months, although two to three years is required in some cases. These issues are discussed in detail elsewhere. (See "Causes and diagnosis of membranous nephropathy".)


==References==
==References==

Revision as of 15:26, 20 May 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

OR

Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].

OR

Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.

Natural History, Complications, and Prognosis

Natural History

  • The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.
  • The symptoms of (disease name) typically develop ___ years after exposure to ___.
  • If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

Complications

  • Common complications of [disease name] include:
    • [Complication 1]
    • [Complication 2]
    • [Complication 3]

Prognosis

  • Spontaneous remission is most likely in women, children, those with lesser amounts of proteinuria, and adults under age 50 years with a normal serum creatinine concentration and benign histologic features [1-3,8].
  • Although unproven, the rate of partial remission may be higher with the use of ACE inhibitors or ARBs.
  • Probability of progression — Based upon a study of 184 patients identified through the Toronto Glomerulonephritis Registry, a semiquantitative algorithm has been developed to predict the probability of progression to chronic kidney disease, which was defined as a creatinine clearance ≤60 mL/min per 1.73 m2 [8]. The probability of progression was assessed for those with 4, 6, and 8 g/day of proteinuria that persisted for 6, 9, 12, or 24 months. Multiple additional variables (including age, sex, serum creatinine, and creatinine clearance on presentation, serum albumin, presence of hypertension, rate of change of creatinine clearance, and therapy) were also tested to determine whether the predictive value provided by proteinuria could be improved. Of these variables, the initial creatinine clearance and the rate of change of clearance were the most useful predictors. Based upon this approach, the best fitting logistic model utilized the following clinical characteristics: ●Persistent proteinuria for over six months ●Creatinine clearance upon presentation ●Slope of the decline in creatinine clearance over the assessed proteinuria period The addition of renal pathology as a variable had no effect on the performance of the model. This model was tested in two additional populations of patients identified through the Italian Idiopathic Membranous Nephropathy Study group (110 patients) and a database obtained from Helsinki University (84 patients) [9]. The accuracy in predicting the development of chronic renal insufficiency was 85, 87, and 79 percent in populations from Toronto, Finland, and Italy, respectively. This was higher than the predictive value of proteinuria >3.5 g/day at the time of biopsy, which was only 52, 43, and 34 percent, respectively, in the same populations. Based upon the best fitting logistic model, the following are examples of the risk of progression to a creatinine clearance ≤60 mL/min per 1.73 m2 for patients with specific clinical characteristics over a follow-up period of more than five years: ●The risk is only 6 percent for the patient with proteinuria <3.5 g/day and stable normal renal function over six months ●The risk increases to 72 percent in the patient with proteinuria of 12 g/day, and a creatinine clearance on presentation of 96 mL/min, which declines to 78 mL/min by six months Based upon this model, we define low-, moderate-, and high-risk patient subsets with varying degrees of risk for progression to more advanced kidney insufficiency (defined as a creatinine clearance ≤60 mL/min per 1.73 m2) over five years: ●Low risk – Proteinuria remains less than 4 g/day and creatinine clearance remains normal for a six-month follow-up period. Such patients have a less than 8 percent risk of developing chronic renal insufficiency over five years. ●Moderate risk – Proteinuria is between 4 and 8 g/day and persists for more than six months. Creatinine clearance is normal or near normal and remains stable over six months of observation. Chronic renal insufficiency develops over five years in approximately 50 percent of these patients. ●High risk – Proteinuria is greater than 8 g/day and persists for three months and/or renal function that is either below normal (and considered due to MN) or decreases during the observation period. Approximately 75 percent of such patients are at risk of progression to chronic renal insufficiency over five years. This risk stratification applies only to the likelihood of progressive renal failure. It does not take into account the risks that may be associated with other complications of the nephrotic syndrome, such as worsening atherosclerosis due to hypercholesterolemia and thromboemboli due to the associated hypercoagulable state. Thus, in addition to preservation of renal function, immunosuppressive therapy may be beneficial by shortening the overall exposure to the nephrotic state, thereby minimizing the risk of other complications. (See 'Lipid lowering' below and 'Anticoagulation' below.) Acute decline in renal function — Progressive MN typically occurs gradually. Some patients develop an acute decline in renal function and at least three disorders should be excluded in this setting: ●Acute bilateral renal vein thrombosis which may be associated with flank pain. (See "Renal vein thrombosis and hypercoagulable state in nephrotic syndrome".) ●Drug-induced acute interstitial nephritis, in which white cell, white cell casts, and possibly eosinophils are typically seen in the urine sediment. (See "Clinical manifestations and diagnosis of acute interstitial nephritis".) ●Superimposed crescentic glomerulonephritis, in which red cells and cellular casts are found in the urine sediment. (See "Causes and diagnosis of membranous nephropathy".) Secondary MN — In patients with secondary MN, cessation of the offending drug (eg, penicillamine, gold, or nonsteroidal anti-inflammatory drug) or effective treatment of the underlying disease is usually associated with improvement in the nephrotic syndrome. With penicillamine- or gold-associated disease, protein excretion may continue to rise for the first 1 to 12 months (mean 2 months) after the offending drug has been discontinued [18,19]. The mean time to resolution of proteinuria is 9 to 12 months, although two to three years is required in some cases. These issues are discussed in detail elsewhere. (See "Causes and diagnosis of membranous nephropathy".)

References

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