Nephritic Syndrome in Children

Nephritic syndrome is a renal condition with signs and symptoms produced by inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body's defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation of the glomeruli (glomerulonephritis) and increased permeability of the glomerular barriers. Defining features include hematuria, proteinuria (but below nephrotic range), RBC casts with dysmorphic RBCs on urine microscopy, and increased serum creatinine. Causes can be genetic, autoimmune, idiopathic, or post-infectious. The most common cause is acute post-streptococcal glomerulonephritis. General clinical findings include edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema, hypertension Hypertension Hypertension, or high blood pressure, is a common disease that manifests as elevated systemic arterial pressures. Hypertension is most often asymptomatic and is found incidentally as part of a routine physical examination or during triage for an unrelated medical encounter. Hypertension, and oliguria. Diagnosis is made based on history, physical exam, and laboratory data. A renal biopsy is sometimes necessary to establish the underlying cause. There can be a combined nephritic-nephrotic picture, especially in the chronic presentation. Treatment and prognosis depend on cause and severity.

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Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

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Overview

Definition

Nephritic syndrome is defined as renal disease caused by immune-mediated inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body's defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation and injury of the glomeruli with classic features of:

  • Hematuria
  • Proteinuria (subnephrotic range)
  • Hypertension
  • RBC casts in the urine
  • Edema

Epidemiology

  • Acute post-streptococcal glomerulonephritis ( PSGN PSGN Post-streptococcal glomerulonephritis (PSGN) is a type of nephritis that is caused by a prior infection with group A beta-hemolytic Streptococcus (GAS). The clinical presentation of PSGN can range from asymptomatic, microscopic hematuria to full-blown acute nephritic syndrome, which is characterized by red-to-brown urine, proteinuria, edema, and acute kidney injury. Poststreptococcal Glomerulonephritis):
    • The most common glomerulonephritis (GN) in children
    • Worldwide:
      • > 400,000 cases per year
      • 5,000 deaths annually
      • More common in developing countries
    • Most common between the ages of 2 and 15 years
  • Affected demographics depends on the cause:
    • Henoch-Schönlein purpura Henoch-Schönlein Purpura Henoch-Schönlein purpura (HSP), also known as immunoglobulin A vasculitis, is an autoimmune small-vessel vasculitis that typically presents as a tetrad of abdominal pain, arthralgia, hematuria, and purpuric rash. Henoch-Schönlein Purpura (HSP) nephritis:
      • Usually affects children < 10 years of age
      • Slightly higher prevalence in boys
    • Lupus nephritis is more common in girls.
    • Alport syndrome Alport Syndrome Alport syndrome, also called hereditary nephritis, is a genetic disorder caused by a mutation in the genes encoding for the alpha chains of type IV collagen, resulting in the production of abnormal type IV collagen strands. Patients present with glomerulonephritis, hypertension, edema, hematuria, and proteinuria, as well as with ocular and auditory findings. Alport Syndrome predominantly affects boys.

Etiology

Primary (renal) causes of nephritic syndrome in children:

  • IgA nephropathy IgA nephropathy IgA nephropathy (Berger's disease) is a renal disease characterized by IgA deposition in the mesangium. It is the most common cause of primary glomerulonephritis in most developed countries. Patients frequently present in the second and third decades of life and, historically, with a preceding upper respiratory or GI infection. IgA Nephropathy (Berger’s disease)
  • Membranoproliferative glomerulonephritis Membranoproliferative Glomerulonephritis Membranoproliferative glomerulonephritis (MPGN) is also known as mesangiocapillary glomerulonephritis. Membranoproliferative glomerulonephritis is a pattern of glomerular injury characterized by mesangial hypercellularity, endocapillary proliferation, and thickening of the glomerular basement membrane (double contour formation). Membranoproliferative Glomerulonephritis ( MPGN MPGN Membranoproliferative glomerulonephritis (MPGN) is also known as mesangiocapillary glomerulonephritis. Membranoproliferative glomerulonephritis is a pattern of glomerular injury characterized by mesangial hypercellularity, endocapillary proliferation, and thickening of the glomerular basement membrane (double contour formation). Membranoproliferative Glomerulonephritis) ( nephrotic syndrome Nephrotic syndrome Nephrotic syndrome is characterized by severe proteinuria, hypoalbuminemia, and peripheral edema. In contrast, the nephritic syndromes present with hematuria, variable loss of renal function, and hypertension, although there is sometimes overlap of > 1 glomerular disease in the same individual. Nephrotic Syndrome often also present) 
  • Idiopathic crescent GN (rapidly progressive GN)
  • Anti-glomerular basement membrane (GBM) or Goodpasture’s disease (rare in children)

Secondary causes of nephritic syndrome in children:

  • Post-infectious GN:
    • Post-streptococcal GN
    • May occur with other bacterial (e.g., Staphylococcus Staphylococcus Staphylococcus is a medically important genera of Gram-positive, aerobic cocci. These bacteria form clusters resembling grapes on culture plates. Staphylococci are ubiquitous for humans, and many strains compose the normal skin flora. Staphylococcus aureus), viral (e.g., rubella Rubella Rubella (also known as German measles or three-day measles) is caused by a single-stranded, positive-sense RNA virus of the Togaviridae family. Rubella only infects humans and spreads prenatally via vertical transmission or postnatally via droplet contact. Congenital rubella is associated with a classic triad of symptoms: cataracts, cardiac defects, and deafness. Infection in children and adults may be mild and present with constitutional symptoms along with a viral exanthem. Rubella Virus), and parasitic (e.g., Toxoplasma Toxoplasma Toxoplasmosis is an infectious disease caused by Toxoplasma gondii, an obligate intracellular protozoan parasite. Felines are the definitive host, but transmission to humans can occur through contact with cat feces or the consumption of contaminated foods. The clinical presentation and complications depend on the host's immune status. Toxoplasma/Toxoplasmosis gondii) infections 
  • IgA vasculitis (HSP)
  • Alport syndrome Alport Syndrome Alport syndrome, also called hereditary nephritis, is a genetic disorder caused by a mutation in the genes encoding for the alpha chains of type IV collagen, resulting in the production of abnormal type IV collagen strands. Patients present with glomerulonephritis, hypertension, edema, hematuria, and proteinuria, as well as with ocular and auditory findings. Alport Syndrome
  • Lupus nephritis
  • Granulomatosis with polyangiitis Granulomatosis with Polyangiitis Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease of unknown etiology. It leads to a necrotizing granulomatous inflammation of small and medium-sized blood vessels of the nose, sinuses, throat, lungs, and kidneys. Granulomatosis with Polyangiitis
  • Nephritis associated with infective endocarditis Endocarditis Endocarditis is an inflammatory disease involving the inner lining (endometrium) of the heart, most commonly affecting the cardiac valves. Both infectious and noninfectious etiologies lead to vegetations on the valve leaflets. Patients may present with nonspecific symptoms such as fever and fatigue. Endocarditis

Pathophysiology

  • Immune-mediated inflammatory injury to glomeruli leading to basement cell membrane Cell Membrane A cell membrane (also known as the plasma membrane or plasmalemma) is a biological membrane that separates the cell contents from the outside environment. A cell membrane is composed of a phospholipid bilayer and proteins that function to protect cellular DNA and mediate the exchange of ions and molecules. The Cell: Cell Membrane damage by either:
    • Immunoglobulin directly binding to specific antigens within the glomeruli (e.g., Goodpasture’s disease: glomerular basement antigen) 
    • Immune complexes (antigen-antibody) deposited in the glomeruli (e.g., post-infectious/streptococcal GN)
  • Cascade of immune-mediated inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body's defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation is triggered, leading to:
    • Complement system activation                        
    • Leukocyte activation                          
  • Immune-mediated inflammatory effects:
    • Basement cell membrane Cell Membrane A cell membrane (also known as the plasma membrane or plasmalemma) is a biological membrane that separates the cell contents from the outside environment. A cell membrane is composed of a phospholipid bilayer and proteins that function to protect cellular DNA and mediate the exchange of ions and molecules. The Cell: Cell Membrane damage: impaired glomerular barriers
    • Hematuria: from leakage of RBCs, RBC casts
    • Proteinuria: from leakage of proteins 
    • Hypertension: from salt and water retention, with renin production
    • Edema: from the proteinuria, with salt and water retention
    • Oliguria: decline in glomerular filtration Glomerular filtration The kidneys are primarily in charge of the maintenance of water and solute homeostasis through the processes of filtration, reabsorption, secretion, and excretion. Glomerular filtration is the process of converting the systemic blood supply into a filtrate, which will ultimately become the urine. Glomerular Filtration 
  • Genetic modifiers play a role in the pathogenesis of some nephritic syndromes.

Clinical Presentation

Signs and symptoms

  • Acute GN:
    • Sudden-onset gross/microscopic hematuria
    • Edema (usually mild lower extremity or periorbital edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema)
    • Oliguria
    • Hypertension
    • Dull abdominal or flank tenderness
    • Fever (about 50% of the time in acute PSGN PSGN Post-streptococcal glomerulonephritis (PSGN) is a type of nephritis that is caused by a prior infection with group A beta-hemolytic Streptococcus (GAS). The clinical presentation of PSGN can range from asymptomatic, microscopic hematuria to full-blown acute nephritic syndrome, which is characterized by red-to-brown urine, proteinuria, edema, and acute kidney injury. Poststreptococcal Glomerulonephritis)
  • Chronic GN:
    • May be asymptomatic and found incidentally on routine urinalysis
    • Hypertension
    • Can have features of nephrotic syndrome Nephrotic syndrome Nephrotic syndrome is characterized by severe proteinuria, hypoalbuminemia, and peripheral edema. In contrast, the nephritic syndromes present with hematuria, variable loss of renal function, and hypertension, although there is sometimes overlap of > 1 glomerular disease in the same individual. Nephrotic Syndrome or mixed nephritic- nephrotic syndrome Nephrotic syndrome Nephrotic syndrome is characterized by severe proteinuria, hypoalbuminemia, and peripheral edema. In contrast, the nephritic syndromes present with hematuria, variable loss of renal function, and hypertension, although there is sometimes overlap of > 1 glomerular disease in the same individual. Nephrotic Syndrome
  • Rapidly progressive GN:
    • Relatively more rapid progression of acute GN over days or weeks
    • Rare in children
  • Recurrent macroscopic hematuria:
    • Can be seen in IgA nephropathy IgA nephropathy IgA nephropathy (Berger's disease) is a renal disease characterized by IgA deposition in the mesangium. It is the most common cause of primary glomerulonephritis in most developed countries. Patients frequently present in the second and third decades of life and, historically, with a preceding upper respiratory or GI infection. IgA Nephropathy 12–72 hours after a mild upper respiratory infection (URI)
    • Can also be seen in Alport syndrome Alport Syndrome Alport syndrome, also called hereditary nephritis, is a genetic disorder caused by a mutation in the genes encoding for the alpha chains of type IV collagen, resulting in the production of abnormal type IV collagen strands. Patients present with glomerulonephritis, hypertension, edema, hematuria, and proteinuria, as well as with ocular and auditory findings. Alport Syndrome
Urinary sample with hematuria

Urinary sample with hematuria: dark or tea-colored urine

Image: “hematuria” by omicsonline.org. License: CC BY 4.0

Physical exam

  • Mild edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema in lower extremities
  • Periorbital edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema
  • Scrotal edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema
  • Abdominal or flank pain Pain Pain has accompanied humans since they first existed, first lamented as the curse of existence and later understood as an adaptive mechanism that ensures survival. Pain is the most common symptomatic complaint and the main reason why people seek medical care. Physiology of Pain
  • Palpable purpuric rash seen in HSP with distribution from lower extremities to buttocks
Henoch-schönlein purpura

Severe case of IgA vasculitis or Henoch-Schönlein purpura Henoch-Schönlein Purpura Henoch-Schönlein purpura (HSP), also known as immunoglobulin A vasculitis, is an autoimmune small-vessel vasculitis that typically presents as a tetrad of abdominal pain, arthralgia, hematuria, and purpuric rash. Henoch-Schönlein Purpura:
Palpable purpura is noted on on the child’s foot, leg Leg The lower leg, or just "leg" in anatomical terms, is the part of the lower limb between the knee and the ankle joint. The bony structure is composed of the tibia and fibula bones, and the muscles of the leg are grouped into the anterior, lateral, and posterior compartments by extensions of fascia. Leg, and arm Arm The arm, or "upper arm" in common usage, is the region of the upper limb that extends from the shoulder to the elbow joint and connects inferiorly to the forearm through the cubital fossa. It is divided into 2 fascial compartments (anterior and posterior). Arm.

Image: “More severe case of HSP on child’s foot” by Okwikikim. License: Public Domain

Complications

Patients can also present with manifestations of severe disease:

  • Overlap with or progression to nephrotic syndrome Nephrotic syndrome Nephrotic syndrome is characterized by severe proteinuria, hypoalbuminemia, and peripheral edema. In contrast, the nephritic syndromes present with hematuria, variable loss of renal function, and hypertension, although there is sometimes overlap of > 1 glomerular disease in the same individual. Nephrotic Syndrome
  • Hypertensive encephalopathy
  • End-stage renal disease (ESRD)
  • CKD CKD Chronic kidney disease (CKD) is kidney impairment that lasts for ≥ 3 months, implying that it is irreversible. Hypertension and diabetes are the most common causes; however, there are a multitude of other etiologies. In the early to moderate stages, CKD is usually asymptomatic and is primarily diagnosed by laboratory abnormalities. Chronic Kidney Disease

Diagnosis

Diagnostic approach

Clinical findings are specific to the underlying causes.

Laboratory data:

  • Urine studies:
    • Urinalysis and urine microscopy: hematuria with dysmorphic RBCs and/or RBC casts
    • Proteinuria > 150 mg/day but < 3.5 mg/day in older children and teens
    • Proteinuria defined as between 4 and 40 mg/m²/hr in babies and younger children
    • Sterile pyuria also seen
  • Renal function:
    • Elevated BUN
    • Elevated serum creatinine
    • BUN/creatinine ratio > 15
  • Complement levels: dependent on underlying cause of nephritis

Imaging (renal ultrasound):

  • Detects other causes of hematuria (e.g., stones)
  • Shows renal changes, especially of long-standing disease

Disease-specific tests:

  • Acute PSGN PSGN Post-streptococcal glomerulonephritis (PSGN) is a type of nephritis that is caused by a prior infection with group A beta-hemolytic Streptococcus (GAS). The clinical presentation of PSGN can range from asymptomatic, microscopic hematuria to full-blown acute nephritic syndrome, which is characterized by red-to-brown urine, proteinuria, edema, and acute kidney injury. Poststreptococcal Glomerulonephritis:
    • Elevated antistreptococcal-O titers
    • Elevated anti-DNase B antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins
    • Decrease in serum complement C3 due to consumption
  • Systemic lupus erythematosus Systemic lupus erythematosus Systemic lupus erythematosus (SLE) is a chronic autoimmune, inflammatory condition that causes immune-complex deposition in organs, resulting in systemic manifestations. Women, particularly those of African American descent, are more commonly affected. Systemic Lupus Erythematosus:
    • ANA
    • Anti-double-stranded DNA DNA The molecule DNA is the repository of heritable genetic information. In humans, DNA is contained in 23 chromosome pairs within the nucleus. The molecule provides the basic template for replication of genetic information, RNA transcription, and protein biosynthesis to promote cellular function and survival. DNA Types and Structure
  • Granulomatosis polyangiitis: 
    • Antineutrophil cytoplasmic autoantibody (ANCA)
    • Pulmonary and sinus investigation with CT 
  • Anti-GBM (Goodpasture’s) disease:
    • ANCA
    • Anti-GBM antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins
    • Pulmonary workup for symptoms of hemoptysis Hemoptysis Hemoptysis is defined as the expectoration of blood originating in the lower respiratory tract. Hemoptysis is a consequence of another disease process and can be classified as either life threatening or non-life threatening. Hemoptysis can result in significant morbidity and mortality due to both drowning (reduced gas exchange as the lungs fill with blood) and hemorrhagic shock. Hemoptysis or dyspnea Dyspnea Dyspnea is the subjective sensation of breathing discomfort. Dyspnea is a normal manifestation of heavy physical or psychological exertion, but also may be caused by underlying conditions (both pulmonary and extrapulmonary). Dyspnea
  • MPGN MPGN Membranoproliferative glomerulonephritis (MPGN) is also known as mesangiocapillary glomerulonephritis. Membranoproliferative glomerulonephritis is a pattern of glomerular injury characterized by mesangial hypercellularity, endocapillary proliferation, and thickening of the glomerular basement membrane (double contour formation). Membranoproliferative Glomerulonephritis: As this is a pathologic diagnosis with multiple possible etiologies, workup depends on presentation and includes HIV and hepatitis B Hepatitis B Hepatitis B virus (HBV) is a partially double-stranded DNA virus, which belongs to the Orthohepadnavirus genus and the Hepadnaviridae family. Most individuals with acute HBV infection are asymptomatic or have mild, self-limiting symptoms. Chronic infection can be asymptomatic or create hepatic inflammation, leading to liver cirrhosis and hepatocellular carcinoma (HCC). Hepatitis B Virus and C.

Glomerular disease differentiation

  • Nephritic and nephrotic syndromes are common presentations of glomerular diseases, which can be a diagnostic challenge.
  • Nephritic syndrome is characterized by glomerular inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body's defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation, while the nephrotic type has deranged glomerular capillary walls resulting in increased permeability.
  • There are overlapping findings including proteinuria and edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema, but the extent differs.
  • Additionally, some diseases have a mixed picture: nephritic- nephrotic syndrome Nephrotic syndrome Nephrotic syndrome is characterized by severe proteinuria, hypoalbuminemia, and peripheral edema. In contrast, the nephritic syndromes present with hematuria, variable loss of renal function, and hypertension, although there is sometimes overlap of > 1 glomerular disease in the same individual. Nephrotic Syndrome
  • Knowledge of the differences aids in arriving at the possible diagnoses.
Table: Differences between nephrotic and nephritic syndrome
Nephrotic syndrome Nephritic syndrome
Edema ++++ ++
Blood pressure Normal/raised Raised
Proteinuria ++++ ++
Hematuria – or microscopic +++
Other features
  • Hypoalbuminemia
  • Hyperlipidemia
  • Hypercoagulability
  • RBC casts
  • Sterile pyuria

Renal biopsy

  • To confirm diagnosis
  • To determine extent of renal injury and help direct therapy
  • Characteristic features can be seen on histopathology using:
    • Light microscopy: may not be specific, with the same morphologic pattern for several diseases
    • Immunofluorescence microscopy: demonstrates immune complex deposition and pattern, which can be disease specific
    • Electron microscopy: may be useful confirmation tool
Table: Summary of laboratory and biopsy results of specific nephritic syndromes
Nephritic syndrome Laboratory and additional tests Renal biopsy results
Acute post-streptococcal GN
  • ASO
  • Anti-DNase B
  • ↓ C3
  • LM: endocapillary proliferation, leukocytic infiltrates
  • IF: C3 and IgG depositions, “starry sky” pattern C3 deposition
  • EM: subepithelial electron dense “humps”
Membranoproliferative GN (a pattern of glomerular injury, not a specific disease; has types I-III) ↓ C3
  • LM: mesangial hypercellularity, endocapillary proliferation, double-contour/”tram track” appearance of GBM
  • IF:
    • I: IgG, C3, C1q, C4
    • II: C3
    • III: C3
  • EM:
    • I: subendothelial deposits
    • II: dense deposits
    • III: subepithelial and subendothelial deposits
Lupus GN
  • ANA
  • Anti-dsDNA
  • ↓ C3
  • IF: IgG, IgA, IgM, C3, C1q can be seen; known as “full house” deposition
  • EM: tubuloreticular inclusions, subendothelial deposits
IgA nephropathy IgA nephropathy IgA nephropathy (Berger's disease) is a renal disease characterized by IgA deposition in the mesangium. It is the most common cause of primary glomerulonephritis in most developed countries. Patients frequently present in the second and third decades of life and, historically, with a preceding upper respiratory or GI infection. IgA Nephropathy Normal C3
  • LM: mesangial hypercellularity
  • IF: mesangial IgA depositions
  • EM: mesangial dense deposits
HSA/IgA vasculitis
  • Normal C3
  • Skin biopsy: shows IgA
  • LM: mesangial proliferation
  • IF: mesangial IgA depositions
  • EM: electron-dense deposits in mesangium
Alport syndrome Alport Syndrome Alport syndrome, also called hereditary nephritis, is a genetic disorder caused by a mutation in the genes encoding for the alpha chains of type IV collagen, resulting in the production of abnormal type IV collagen strands. Patients present with glomerulonephritis, hypertension, edema, hematuria, and proteinuria, as well as with ocular and auditory findings. Alport Syndrome (hereditary GN) Skin biopsy: monoclonal Ab against the alpha-5 (IV) chain (protein is absent) EM: splitting of the GBM, basketweave appearance
Rapidly progressive GN (denotes severe glomerular injury, but has different etiologies) Depends on the underlying cause Most common histology: crescent formation in glomeruli
Ab: antibody
GN: glomerulonephritis
GBM: glomerular basement membrane
Ig: immunoglobulin
LM: light microscopy
IF: immunofluorescence
EM: electron microscopy

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Management

Management depends on the cause and severity.

  • Initial approach: Treat the underlying cause.
  • In cases where renal function is preserved (e.g., IgA nephropathy IgA nephropathy IgA nephropathy (Berger's disease) is a renal disease characterized by IgA deposition in the mesangium. It is the most common cause of primary glomerulonephritis in most developed countries. Patients frequently present in the second and third decades of life and, historically, with a preceding upper respiratory or GI infection. IgA Nephropathy) or disease self-resolves (e.g., PSGN PSGN Post-streptococcal glomerulonephritis (PSGN) is a type of nephritis that is caused by a prior infection with group A beta-hemolytic Streptococcus (GAS). The clinical presentation of PSGN can range from asymptomatic, microscopic hematuria to full-blown acute nephritic syndrome, which is characterized by red-to-brown urine, proteinuria, edema, and acute kidney injury. Poststreptococcal Glomerulonephritis), monitoring is done and no further intervention is needed.
  • Treat throat or skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin culture-positive streptococcal infections to eliminate reservoir.
  • Symptomatic treatment:
    • Renal insufficiency: 
      • Supportive management and proper hydration
      • Monitor progression. 
    • Hypertension: 
      • Salt/fluid restriction 
      • Antihypertensives for chronic persistent HTN
    • Edema: salt and fluid restriction +/- diuretics
  • Corticosteroids and/or immunosuppressive medications if indicated (e.g., lupus nephritis)
  • If severe renal insufficiency or kidney failure: renal replacement therapy (e.g., hemodialysis, renal transplantation)

Clinical Relevance

  • Systemic lupus erythematosus Systemic lupus erythematosus Systemic lupus erythematosus (SLE) is a chronic autoimmune, inflammatory condition that causes immune-complex deposition in organs, resulting in systemic manifestations. Women, particularly those of African American descent, are more commonly affected. Systemic Lupus Erythematosus: chronic autoimmune, inflammatory condition that causes immune-complex deposition in organs, resulting in systemic manifestations. Deposition of immune complexes in glomerulus leads to lupus nephritis. This condition is more common in Asian and African American females. Renal injury can be mild to progressive GN. Diagnosis is made with renal biopsy.
  • Post-streptococcal glomerulonephritis: the most common cause of nephritic syndrome in children, and is preceded by an infection from group A beta-hemolytic streptococcus Streptococcus Streptococcus is one of the two medically important genera of gram-positive cocci, the other being Staphylococcus. Streptococci are identified as different species on blood agar on the basis of their hemolytic pattern and sensitivity to optochin and bacitracin. There are many pathogenic species of streptococci, including S. pyogenes, S. agalactiae, S. pneumoniae, and the viridans streptococci. Streptococcus of the throat or skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin. Renal biopsy is usually not needed to establish the diagnosis. The condition is usually self-limited.
  • IgA nephropathy IgA nephropathy IgA nephropathy (Berger's disease) is a renal disease characterized by IgA deposition in the mesangium. It is the most common cause of primary glomerulonephritis in most developed countries. Patients frequently present in the second and third decades of life and, historically, with a preceding upper respiratory or GI infection. IgA Nephropathy: also known as Berger’s disease. Presents as gross or microscopic hematuria shortly after onset of URI symptoms. The disease is characterized by IgA deposition in the mesangium. Berger’s disease is the most common cause of primary glomerulonephritis in most developed countries. 
  • HSP: also known as IgA vasculitis. Clinical features include characteristic rash, arthralgias, abdominal pain Pain Pain has accompanied humans since they first existed, first lamented as the curse of existence and later understood as an adaptive mechanism that ensures survival. Pain is the most common symptomatic complaint and the main reason why people seek medical care. Physiology of Pain, and nephritis. The condition can be triggered by an upper respiratory or GI infection. Usually occurs in children < 10 years old. 
  • Granulomatosis with polyangiitis Granulomatosis with Polyangiitis Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease of unknown etiology. It leads to a necrotizing granulomatous inflammation of small and medium-sized blood vessels of the nose, sinuses, throat, lungs, and kidneys. Granulomatosis with Polyangiitis: This condition is under the antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides Vasculitides Vasculitides are a group of conditions characterized by vasculitis, ischemia, and damage to the organs supplied by the affected vessels. The affected arteries are of different sizes and locations and vary by the type of vasculitis. Vasculitides (AAV) autoimmune disorders, which is characterized by inflammation Inflammation Inflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body's defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation of small vessels and granuloma formation. Granulomatosis with polyangiitis Granulomatosis with Polyangiitis Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease of unknown etiology. It leads to a necrotizing granulomatous inflammation of small and medium-sized blood vessels of the nose, sinuses, throat, lungs, and kidneys. Granulomatosis with Polyangiitis is associated with crescentic, pauci-immune glomerulonephritis. The ear, nose Nose The nose is the human body's primary organ of smell and functions as part of the upper respiratory system. The nose may be best known for inhaling oxygen and exhaling carbon dioxide, but it also contributes to other important functions, such as tasting. The anatomy of the nose can be divided into the external nose and the nasal cavity. Anatomy of the Nose, and throat area, along with the airways, are also involved.
  • Alport syndrome Alport Syndrome Alport syndrome, also called hereditary nephritis, is a genetic disorder caused by a mutation in the genes encoding for the alpha chains of type IV collagen, resulting in the production of abnormal type IV collagen strands. Patients present with glomerulonephritis, hypertension, edema, hematuria, and proteinuria, as well as with ocular and auditory findings. Alport Syndromealso known as hereditary nephritis. Alport syndrome Alport Syndrome Alport syndrome, also called hereditary nephritis, is a genetic disorder caused by a mutation in the genes encoding for the alpha chains of type IV collagen, resulting in the production of abnormal type IV collagen strands. Patients present with glomerulonephritis, hypertension, edema, hematuria, and proteinuria, as well as with ocular and auditory findings. Alport Syndrome is a genetic condition that is commonly X linked and encompasses glomerular disease, sensorineural hearing loss Hearing loss Hearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss, and abnormal ocular findings. Genetic variants affect collagen IV alpha chains found in impacted target organs (basement membrane of kidney, cochlea, and eye).

References

  1. Chang, A., Laszik, Z. (2020). The Kidney. In Kumar, V., Abbas, A., Aster, J. & Robbins, S. Robbins and Cotran. Pathologic Basis of Disease (10th Ed., pp. 895–930). Elsevier, Inc.
  2. Floege, J., & Feehally, J. (2019). Introduction to glomerular disease: Clinical presentations. In Feehally, John, DM, FRCP, Floege, Jürgen, MD, FERA, Tonelli, Marcello, MD, SM, MSc, FRCPC & R. J. Johnson MD (Eds.), Comprehensive clinical nephrology (pp. 18-198). https://www.clinicalkey.es/#!/content/3-s2.0-B9780323479097000159
  3. Flores, F. X. (2020). Isolated glomerular diseases associated with recurrent gross hematuria. In R. M. Kliegman MD et al. (Eds.), Nelson textbook of pediatrics (pp. 272-2728.e1). https://www.clinicalkey.es/#!/content/3-s2.0-B978032352950100537X
  4. Landry, D. W., & Bazari, H. (2017). Aproximación al paciente con enfermedad renal. In L. Goldman MD, & A. I. Schafer MD (Eds.), Goldman-Cecil. tratado de medicina interna (pp. 728-736). https://www.clinicalkey.es/#!/content/3-s2.0-B9788491130338001142
  5. Lewis, J. B., & Neilson, E. G. (2018). Glomerular diseases. In J. L. Jameson, A. S. Fauci, D. L. Kasper, S. L. Hauser, D. L. Longo & J. Loscalzo (Eds.), Harrison’s principles of internal medicine, 20e. New York, NY: McGraw-Hill Education. accessmedicine.mhmedical.com/content.aspx?aid=1156520317
  6. Kehr, K. Acute Glomerular Diseases in Children. openurologyandnephrologyjournal.com/VOLUME/8/PAGE/104/FULLTEXT/
  7. Niaudet, P. Overview of the pathogenesis and causes of glomerulonephritis in children. Retrieved February 16, 2021, from https://www.uptodate.com/contents/overview-of-the-pathogenesis-and-causes-of-glomerulonephritis-in-children
  8. O’Brien, D. Overview of nephritic syndrome. Retrieved February 25, 2021, from https://www.merckmanuals.com/professional/genitourinary-disorders/glomerular-disorders/overview-of-nephritic-syndrome

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