Discharge Criteria
Mild cases of clinical nephritis in healthy patients with:
- No comorbid illness
- Strict supervision/monitoring of symptoms, diet, urine output, and medication
- Close follow-up with PMD and nephrology referral
Issues for Referral
Nephrology:
FOLLOW-UP RECOMMENDATIONS
- Adherence to antibiotic and antihypertensive therapy, as indicated
- Restrict salt and fluid intake.
PEARLS AND PITFALLS
- Diagnosis is confirmed by biopsy showing characteristic crescent formation within renal glomeruli.
- Must obtain thorough history of ongoing or recent infections as possible etiology of nephritis
- IgA nephropathy is most common cause of nephritis.
- Patients require aggressive management of BP and volume status.
ADDITIONAL READING
- Ikee R, Kobayashi S, Saigusa T, et al. Impact of hypertension and hypertension-related vascular lesions in IgA nephropathy.
Hypertens Res
. 2006;29(1):15–22.
- Kanjanabuch T, Kittikowit W, Eiam-Ong S. An update on acute postinfectious glomerulonephritis worldwide.
Nat Rev Nephrol
. 2009;5:259–269.
- Kunz R, Friedrich C, Wolbers M, et al. Meta-analysis: Effect of monotherapy and combination therapy with inhibitors of the renin angiotensin system on proteinuria in renal disease.
Ann Intern Med
. 2008;148(1):30–48.
See Also (Topic, Algorithm, Electronic Media Element)
- Acute Renal Failure
- Glomerulonephritis
- Nephrotic Syndrome
CODES
ICD9
- 580.0 Acute glomerulonephritis with lesion of proliferative glomerulonephritis
- 580.9 Acute glomerulonephritis with unspecified pathological lesion in kidney
- 583.2 Nephritis and nephropathy, not specified as acute or chronic, with lesion of membranoproliferative glomerulonephritis
ICD10
- N00.9 Acute nephritic syndrome with unsp morphologic changes
- N01.9 Rapidly progr nephritic syndrome w unsp morphologic changes
- N05.9 Unsp nephritic syndrome with unspecified morphologic changes
NEPHROTIC SYNDROME
Maureen L. Joyner
BASICS
DESCRIPTION
- Diseases causing defect in glomerular filtration barrier, producing proteinuria:
- Proteinuria >3 g in 24 hr
- Hypoalbuminemia (serum albumin <3 g/dL)
- Peripheral edema due to hypoalbuminemia
- Hypogammaglobulinemia
- Hyperlipidemia (fasting cholesterol >200 mg/dL)
- Urine fat (oval fat bodies, fatty/waxy casts)
- Glomerular basement membrane altered by:
- Immune complexes
- Nephrotoxic antibodies
- Nonimmune mechanisms
- Result: More permeable glomerular membranes and excretion of albumin and large proteins
PATHOPHYSIOLOGY
- Proteinuria due to increased filtration within renal glomeruli
- Edema due to sodium retention and hypoalbuminemia
- Postural hypotension, syncope, and shock due to severe hypoalbuminemia
- Hyperlipidemia due to hepatic lipoprotein synthesis stimulated by decreased plasma oncotic pressure
- Cumulative thromboembolism risk increased if:
- Hypovolemia
- Low serum albumin
- High protein excretion
- High fibrinogen levels
- Low antithrombin III levels
ETIOLOGY
- Due to primary renal or systemic diseases
- Membranous nephropathy:
- Primary cause of nephrotic syndrome in adults
- Other causes include chronic infection (hepatitis B virus, hepatitis C virus, autoimmune disorders).
- Renal biopsy shows involvement of all glomeruli.
- Women have better prognosis.
- 30% may slowly progress to renal failure.
- Renal vein thrombosis causes sudden loss of renal function.
- Treat with steroids and cytotoxic agents in severe cases.
- Minimal change disease:
- Most common cause (90%) of nephrotic syndrome in children
- Other causes: Idiopathic, NSAIDs, paraneoplastic syndrome associated with malignancy (often Hodgkin lymphoma)
- Best prognosis among all nephrotic syndromes
- Good response to steroids
- Focal segmental glomerulosclerosis (FSGS):
- Young patients (15–30 yr) with nephrotic syndrome
- Presents with high BP, renal insufficiency, proteinuria, microscopic or gross hematuria.
- Causes include HIV, heroin abuse, obesity, hematologic malignancies.
- Primary FSGS responds to steroids.
- Secondary FSGS treated with ACE inhibitors (ACEI)
- Collapsing FSGS usually seen in HIV patients
- Membranoproliferative glomerulonephritis:
- May present with nephrotic, non-nephrotic, or nephritic sediment
- Complement levels are persistently low
- Supportive care: Steroids may be helpful in children.
- Aspirin and dipyridamole may slow progression.
- Diabetes mellitus/diabetic nephropathy:
- Most common secondary cause of nephrotic range proteinuria in adults
- Microalbuminuria (30–300 mg/24hr) is primary indicator of renal disease.
- Worsening of renal function in 5–7 yr
- Does not cause rapid decline in renal function
- Strict control of blood sugar and ACEI therapy slow progression.
- Monoclonal gammopathies:
- Include amyloidosis, multiple myeloma, and light-chain nephropathy
- Renal manifestations include proteinuria, nephrotic syndrome, nephritic syndrome, and acute renal failure.
- Lab findings include pseudohyponatremia, low anion gap, hypercalcemia, and Bence Jones proteinuria.
- Congo red stain of amyloid shows apple green birefringence in polarized light.
- Supportive care: Steroids and melphalan have some benefit.
- Systemic lupus erythematosus (SLE):
- Can present initially as a nephritic process, with progression to nephrotic syndrome
- HIV-associated nephropathy:
- FSGS is most common nephropathy.
- Collapsing glomerulopathy in seropositive HIV carriers with supernephrotic syndrome results in end-stage renal failure that is rapidly progressive (months).
- Other causes include pre-eclampsia, hepatitis, and drug reactions (culprits include NSAIDs, gold, penicillamine).
DIAGNOSIS
SIGNS AND SYMPTOMS
- Many patients are asymptomatic.
- Proteinuria
- Peripheral edema:
- Mild pitting edema to generalized anasarca with ascites
- Hyperlipidemia
- Lipiduria (urine fatty casts and oval fat bodies)
- Postural hypotension, syncope, shock
- Hypertension
- Hematuria:
- Microscopic or gross hematuria (secondary to renal vein thrombosis)
- Renal insufficiency to acute renal failure in some cases
- Tachypnea, tachycardia, with or without hypotension:
- Acute onset: Suggests pulmonary embolus (PE), secondary to renal or deep venous thrombosis and hypercoagulable state
- Up to 30% occurrence of PE in membranous glomerulonephritis
- Chronic or exertional tachypnea due to:
- Pulmonary edema
- Pleural effusions
- Infection risk due to immunosuppressive treatment and frequent exposure to infections such as Pneumococcus
- Ascites
- Protein malnutrition
History
- Systemic disease such as diabetes, SLE, HIV
- Use of NSAIDS, gold, or penicillamine
- History of unintentional weight gain (due to fluid retention)
- History of “foamy” appearance of urine
Physical-Exam
Varies depending on degree of hypoalbuminemia, hemodynamic status, and etiology of nephrotic syndrome:
- Edema
- Hypotension/hypertension
- Shock
ESSENTIAL WORKUP
Urinalysis:
- Dipstick protein largely positive:
- Urine specific gravity >1.025 lowers the diagnostic significance of proteinuria.
- Microscopic analysis for urinary casts and the presence of cellular elements:
- Oval fat bodies
- Free lipid droplets
DIAGNOSIS TESTS & NTERPRETATION
Lab
- CBC + differential:
- Anemia common
- Leukocytosis: Infection
- Leukopenia: Neoplastic disease or sepsis
- Thrombocytopenia: Liver disease
- PT/PTT, international normalized ratio:
- Coagulation profiles abnormal with concurrent liver disease
- D
-dimer, fibrinogen, antithrombin III
- Suspected thromboembolic event:
- Often patients are asymptomatic with PE or renal vein thrombosis; therefore need high clinical suspicion.
- 24-hr urine protein, total protein to creatinine ratio
- Serum albumin: <3 g/dL
- Serum total protein
- Basic metabolic panel with Ca, Mg, P
- Lipid profile: Elevated total cholesterol, LDL, and VLDL
- Additional lab tests may be necessary for systemic diseases:
- Examples include antinuclear antibody, serum and urine protein electrophoresis, hepatitis profile, syphilis, cryoglobulins, complement levels