Rapidly Progressive Glomerulonephritis

Rapidly progressive glomerulonephritis (RPGN) is a syndrome of severe glomerular disease with progressive loss of kidney function within weeks to months. Rapidly progressive glomerulonephritis is associated with 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 and is a manifestation of different diseases. Histologically, crescents (the proliferation of epithelial cells and the infiltration of monocytes/macrophages in the Bowman space) are found in the glomeruli and arise from immunologic injury. The major mechanisms of immunologic injury are classified into anti-glomerular basement membrane (anti-GBM) disease, pauci-immune crescentic glomerulonephritis, and immune complex-mediated injury. Rapidly progressive glomerulonephritis can manifest with hematuria, proteinuria, and varying degrees of 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 and 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. Diagnosis is by presentation, laboratory tests, imaging, and renal biopsy. Prompt treatment is essential because RPGN can develop into end-stage renal disease within a short period of time. Modalities include corticosteroids, cyclophosphamide or other immunosuppressants Immunosuppressants Immunosuppressants are a class of drugs widely used in the management of autoimmune conditions and organ transplant rejection. The general effect is dampening of the immune response. Immunosuppressants, and plasmapheresis (depending on the underlying disease).

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Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

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Overview

Definition

Rapidly progressive glomerulonephritis (RPGN) is a syndrome of severe glomerular disease with progressive loss of kidney function within weeks to months.

  • Associated with nephritic syndrome Nephritic syndrome Nephritic syndrome is a broad category of glomerular diseases characterized by glomerular hematuria, variable loss of renal function, and hypertension. These features are in contrast to those of nephrotic syndrome, which includes glomerular diseases characterized by severe proteinuria, although there is sometimes overlap of > 1 glomerular disease in the same individual. Nephritic Syndrome
  • A manifestation of various diseases, not an etiology
  • Histologically characterized by the presence of crescents:
    • Proliferated cells (predominantly epithelial), with infiltration of monocytes and macrophages in the Bowman space
    • Also called crescentic glomerulonephritis
  • Generally, biopsies of < 10% crescents are not included in the category.

Classification

Most cases are from immunological injury of the glomeruli. The mechanisms and findings are classified as:

  • Anti-glomerular basement membrane (anti-GBM) antibody disease:
    • A rare disease characterized by vasculitis of the small vessels
    • Can affect glomerular and pulmonary capillaries Capillaries Capillaries are the primary structures in the circulatory system that allow the exchange of gas, nutrients, and other materials between the blood and the extracellular fluid (ECF). Capillaries are the smallest of the blood vessels. Because a capillary diameter is so small, only 1 RBC may pass through at a time. Capillaries 
  • Immune-complex mediated injury: immune complex deposition in the glomeruli
  • Pauci-immune necrotizing and crescentic glomerulonephritis:
    • Glomerulonephritis with minimal or no detectable 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 (anti-GBM) or immune complex deposits on immunofluorescence or electron microscopy
    • Most cases are ANCA-positive.
  • Double antibody-positive disease (very rare): features of both ANCA-positive RPGN and anti-GBM antibody disease
  • Idiopathic, including:
    • An immune complex disease not belonging to the identifiable categories
    • ANCA-negative pauci-immune disease

Epidemiology

  • Annual incidence: 7 cases per 1 million individuals
  • White individuals affected > Black individuals
  • Rare in the pediatric population
  • Peak incidence: middle of the 6th decade

Etiology

  • Anti-GBM antibody-mediated disease (≤ 10% of cases):
    • Anti-GBM glomerulonephritis (without lung hemorrhage)
    • Goodpasture syndrome Goodpasture Syndrome Goodpasture syndrome, also known as anti-glomerular basement membrane (GBM) disease, is an autoimmune disease characterized by circulating antibodies directed against glomerular and alveolar basement membranes. Affected individuals present with symptoms of rapidly progressive glomerulonephritis and alveolar hemorrhage. Goodpasture Syndrome with lung hemorrhage
  • Immune complex-mediated injury (≤ 40% of cases):
    • Postinfectious causes:
      • Poststreptococcal glomerulonephritis Poststreptococcal Glomerulonephritis 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
      • 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
      • Vascular prosthetic nephritis
      • Viral 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
    • Others:
      • Lupus nephritis 
      • IgA-associated vasculitis glomerulonephritis
      • Cryoglobulinemic glomerulonephritis
      • 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
      • 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
      • Idiopathic crescentic glomerulonephritis (rare)
  • Pauci-immune disease (up to 50% of cases):
    • GPA
    • Microscopic polyangiitis

Pathophysiology

  • Crescents form in response to injury to the glomerular capillary wall, which can be damaged by:
    • Immune complexes
    • 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 (cross-react with the basement membrane of the pulmonary alveoli and lead to pulmonary hemorrhage)
  • Followed by a nephritogenic inflammatory response mediated by Th1
  • Focal gaps (also known as rents) in the glomerular capillary wall are produced, allowing entry of the following into the Bowman space:
    • Coagulation factors (e.g., fibrinogen) → fibrin formation
    • Fibrinolytic inhibitory factors
    • Cellular elements (e.g., macrophages, T cells T cells T cells, also called T lymphocytes, are important components of the adaptive immune system. Production starts from the hematopoietic stem cells in the bone marrow, from which T-cell progenitor cells arise. These cells migrate to the thymus for further maturation. T Cells)
    • Proinflammatory cytokines
  • Crescents can cause obliteration of the urinary space.

Clinical Presentation

  • Manifestations of RPGN:
    • Hematuria with urine RBC casts
    • Proteinuria up to 30% (reaching nephritic range)
    • Variable 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 may or may not be present.
  • Insidious onset
  • Symptoms include:
    • Oliguria
    • Arthralgia
    • Fatigue
    • Fever
    • 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, nausea, and vomiting
    • Anorexia
  • Individuals with anti-GBM disease, 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 (GPA), and microscopic polyangiitis (MPA) may have 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 from pulmonary involvement.

Diagnosis

Laboratory evaluation

  • Serum creatinine: almost always elevated
  • Urinalysis:
    • Hematuria
    • Dysmorphic RBCs
    • Sediment with multiple elements (e.g., WBCs, RBCs, and other casts)
  • CBC may show:
    • Anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview 
    • Leukocytosis
  • Erythrocyte sedimentation rate (ESR): usually elevated
  • Serologic testing (as indicated by clinical presentation) may include:
    • 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 for anti-GBM antibody disease
    • Antistreptolysin O 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 for postinfectious RPGN
    • Anti-dsDNA 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 for lupus-related RPGN
    • Cryoglobulins for cryoglobulinemia 
    • ANCA titer for pauci-immune RPGN
  • Serum complement levels for hypocomplementemia in immune-complex RPGN 
  • Other tests:
    • Hepatitis B (associated with polyarteritis nodosa)
    • Hepatitis C Hepatitis C Hepatitis C is an infection of the liver caused by the hepatitis C virus (HCV). The infection can be transmitted through infectious blood or body fluids and may be transmitted during childbirth or through IV drug use or sexual intercourse. Hepatitis C virus can cause both acute and chronic hepatitis, ranging from a mild to a serious, lifelong illness including liver cirrhosis and hepatocellular carcinoma (HCC). Hepatitis C Virus (associated with cryoglobulinemia)
    • Urine and serum electrophoresis for suspected multiple myeloma Multiple myeloma Multiple myeloma (MM) is a malignant condition of plasma cells (activated B lymphocytes) primarily seen in the elderly. Monoclonal proliferation of plasma cells results in cytokine-driven osteoclastic activity and excessive secretion of IgG antibodies. Multiple Myeloma
  • Imaging: 
    • Renal ultrasound:
      • To exclude obstructive etiology
      • To determine the presence of 2 functional kidneys Kidneys The kidneys are a pair of bean-shaped organs located retroperitoneally against the posterior wall of the abdomen on either side of the spine. As part of the urinary tract, the kidneys are responsible for blood filtration and excretion of water-soluble waste in the urine. Kidneys (needed with biopsy).
    • Chest X-ray or CT: the presentation includes 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 pulmonary symptoms

Renal biopsy

  • Pathologic findings:
    • The proliferation of glomerular epithelial cells 
    • Crescentic cellular mass fills the Bowman space (may be defined as either 50% of the glomeruli or > 10%).
    • Necrosis in the glomerular tuft 
  • Immunofluorescence microscopy:
    • Anti-GBM antibody disease: linear or ribbon-like deposition of IgG along the GBM 
    • Immune complex RPGN: granular or irregular mesangial IgG and C3 deposits 
    • Pauci-immune RPGN: negative immune staining, no deposits
    • Double antibody RPGN: linear staining of the GBM
    • Idiopathic RPGN: varies (with or without immune complexes)
Table: Microscopic findings in common conditions associated with rapidly progressive glomerulonephritis (RPGN)
Condition Light microscopy Fluorescence microscopy Electron microscopy
Anti-glomerular basement membrane (anti-GBM) disease
  • Extracapillary proliferation of cells (crescents)
  • Fibrin strands seen between layers of crescents
  • Necrosis
Linear IgG and C3
  • No deposits
  • Ruptures in the GBM
Immune complex-mediated injury Granular IgG, other Igs, and complement
  • Immune complexes in various sites
  • Ruptures in the GBM
Pauci-immune crescentic glomerulonephritis No deposits
  • No deposits
  • Ruptures in the GBM
Anti-gbm antibody disease: crescents on light microscopy

Anti-glomerular basement membrane (anti-GBM) antibody disease: crescents on light microscopy

Image: “Crescents on light microscopy” by Mavani G. P., Pommier M., Win S., Michelis M. F., Rosenstock J. License: CC BY 4.0

Management

Treatment approach

  • Because RPGN progresses quickly, treatment should be initiated even while awaiting biopsy interpretation (especially in severe disease).
  • A delay in treatment may cause irreversible renal damage.
  • Initial goals: 
    • Remove circulating 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 the 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.
  • Treatment modalities:
    • Corticosteroids: pulse methylprednisolone followed by prednisone
    • Cyclophosphamide
    • Alternatives to cyclophosphamide:
      • Rituximab
      • Mycophenolate
    • Plasmapheresis:
      • Recommended for anti-GBM antibody disease
      • Allows the removal of free 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, immune complexes, and inflammatory mediators
  • Renal replacement therapy ( dialysis Dialysis Renal replacement therapy refers to dialysis and/or kidney transplantation. Dialysis is a procedure by which toxins and excess water are removed from the circulation. Hemodialysis and peritoneal dialysis (PD) are the two types of dialysis, and their primary difference is the location of the filtration process (external to the body in hemodialysis versus inside the body for PD). Overview and Types of Dialysis or kidney transplantation)

Specific treatments

  • Anti-GBM disease:
    • Plasmapheresis with immunosuppressive therapy
    • May require respiratory support depending on the severity 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 (intubation and mechanical ventilation)
  • ANCA-positive pauci-immune crescentic disease:
    • Induction with corticosteroids combined with rituximab or cyclophosphamide
    • Use plasmapheresis in individuals with concomitant anti-GBM disease.
    • Plasmapheresis is controversial in other cases.
  • Immune complex-mediated crescentic disease:
    • Poststreptococcal glomerulonephritis Poststreptococcal Glomerulonephritis 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: Recovery is typical (in adults, recovery may only be partial).
    • 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:
      • Start corticosteroids in individuals at high risk of progression.
      • For individuals intolerant of corticosteroids, mycophenolate is an option.
    • Lupus nephritis: corticosteroids with cyclophosphamide or mycophenolate
    • Cryoglobulinemia:
      • Treat the underlying disease (antiviral therapy for hepatitis C).
      • Treat RPGN with corticosteroids and rituximab.

Prognosis

  • The likelihood of untreated cases progressing to end-stage renal disease within 6 months is high.
  • Favorable prognostic features:
    • Anti-GBM disease (if treated early)
    • Postinfectious glomerulonephritis
    • 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
    • GPA
    • Microscopic polyangiitis
  • Unfavorable prognostic features:
    • > 60 years of age
    • High serum creatinine level
    • Circumferential crescents in > 75% of glomeruli
    • Oliguric renal failure
    • No response to treatment in pauci-immune RPGN (seen in approximately 30% of individuals)
  • Individuals with double antibody disease have a better renal prognosis than individuals with only anti-GBM antibody disease.

Differential Diagnosis

  • Diffuse proliferative glomerulonephritis Diffuse Proliferative Glomerulonephritis Diffuse proliferative glomerulonephritis (DPGN) is a histopathologic classification of glomerulonephritis (GN) characterized by an increased cellular proliferation affecting > 50% of the glomeruli. Mesangial, endothelial, and epithelial cells are notably increased. Diffuse Proliferative Glomerulonephritis ( DPGN DPGN Diffuse proliferative glomerulonephritis (DPGN) is a histopathologic classification of glomerulonephritis (GN) characterized by an increased cellular proliferation affecting > 50% of the glomeruli. Mesangial, endothelial, and epithelial cells are notably increased. Diffuse Proliferative Glomerulonephritis): a histopathologic classification of glomerulonephritis with increased cellular proliferation (mesangial, endothelial, and epithelial cells) affecting > 50% of the glomeruli. The most common causes are lupus nephritis and 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. Symptoms include fatigue, vomiting, hematuria, proteinuria, 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 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. Microscopic findings show hypercellularity of the aforementioned cells with capillary loop thickening. Early and aggressive therapy is based on the specific etiology. 
  • Poststreptococcal glomerulonephritis Poststreptococcal Glomerulonephritis 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 ( 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): occurs after infection with a group A beta-hemolytic streptococcus. Diagnosis is by history, laboratory tests, urinalysis, and serology testing. Treatment is supportive and the prognosis is excellent. The majority of individuals recover without long-term complications. 
  • 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): histologic renal lesions characterized by glomerular injury with GBM thickening and mesangial proliferation. The pathogenic process can either be immune complex-mediated or complement-mediated. Individuals can present with nephritic syndrome Nephritic syndrome Nephritic syndrome is a broad category of glomerular diseases characterized by glomerular hematuria, variable loss of renal function, and hypertension. These features are in contrast to those of nephrotic syndrome, which includes glomerular diseases characterized by severe proteinuria, although there is sometimes overlap of > 1 glomerular disease in the same individual. Nephritic Syndrome, variable Variable Variables represent information about something that can change. The design of the measurement scales, or of the methods for obtaining information, will determine the data gathered and the characteristics of that data. As a result, a variable can be qualitative or quantitative, and may be further classified into subgroups. Types of Variables proteinuria, and decreased renal function. Renal biopsy shows the pathologic renal lesion. Further investigation (i.e., laboratory tests) will point to the underlying cause (e.g., infection, autoimmune disease). Treatment is based on the etiologic disease. 
  • Lupus nephritis: glomerulonephritis caused by systemic lupus erythematosus. Clinical presentation can include hematuria, 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, and RPGN. Diagnosis is based on renal biopsy, which will help to classify the disease, guide the management, and provide an indication of prognosis. Management includes ACE inhibitors and immunosuppressant drugs.

References

  1. Appel, G.B. et al. (2020). Overview of the classification and treatment of rapidly progressive (crescentic) glomerulonephritis. In Glassock, R.J. et al. (Eds.), UpToDate. Retrieved August 10, 2021, from https://www.uptodate.com/contents/overview-of-the-classification-and-treatment-of-rapidly-progressive-crescentic-glomerulonephritis
  2. Cattran, D., Appel, G., Coppo, R. (2021). IgA nephropathy: Treatment and prognosis. UpToDate. Retrieved Sept 11, 2021, from https://www.uptodate.com/contents/iga-nephropathy-treatment-and-prognosis
  3. Chang, A., Laszik, Z. (2021). The Kidney. In Kumar, V., Abbas, A., Aster, J. (Eds.), Robbins and Cotran Pathologic Basis of Disease, 10th edition. pp. 909–911. Elsevier Inc. 
  4. Falk, R., Dall’Era, M., Appel, G. (2021). Lupus nephritis: Initial and subsequent therapy for focal or diffuse lupus nephritis. UpToDate. Retrieved Sept 11, 2021, from https://www.uptodate.com/contents/lupus-nephritis-initial-and-subsequent-therapy-for-focal-or-diffuse-lupus-nephritis 
  5. Lohr, J.W., et al. (2021). Rapidly progressive glomerulonephritis. In Batuman V. et al. (Eds.), Medscape. Retrieved August 11, 2021, from https://emedicine.medscape.com/article/240457
  6. Kaplan, A., Appel, G., Pusey, C. (2021). Anti-GBM (Goodpasture) disease: Treatment and prognosis. UpToDate. Retrieved Sept 11, 2021, from https://www.uptodate.com/contents/anti-gbm-goodpasture-disease-treatment-and-prognosis 
  7. O’Brien, F. (2021). Rapidly progressive glomerulonephritis (RPGN). MSD Manual Professional Version. Retrieved August 10, 2021, from https://www.msdmanuals.com/professional/genitourinary-disorders/glomerular-disorders/rapidly-progressive-glomerulonephritis-rpgn
  8. Pusey, C.D. (2020). Mechanisms of glomerular crescent formation. In Glassock, R.J. et al. (Eds.), UpToDate. Retrieved August 11, 2021, from https://www.uptodate.com/contents/mechanisms-of-glomerular-crescent-formation

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