C3 Glomerulonephritis
   HOME

TheInfoList



OR:

Glomerulonephritis (GN) is a term used to refer to several
kidney In humans, the kidneys are two reddish-brown bean-shaped blood-filtering organ (anatomy), organs that are a multilobar, multipapillary form of mammalian kidneys, usually without signs of external lobulation. They are located on the left and rig ...
diseases (usually affecting both kidneys). Many of the diseases are characterised by
inflammation Inflammation (from ) is part of the biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. The five cardinal signs are heat, pain, redness, swelling, and loss of function (Latin ''calor'', '' ...
either of the glomeruli or of the small blood vessels in the kidneys, hence the name, but not all diseases necessarily have an inflammatory component. As it is not strictly a single disease, its presentation depends on the specific disease entity: it may present with isolated
hematuria Hematuria or haematuria is defined as the presence of blood or red blood cells in the urine. "Gross hematuria" occurs when urine appears red, brown, or tea-colored due to the presence of blood. Hematuria may also be subtle and only detectable with ...
and/or
proteinuria Proteinuria is the presence of excess proteins in the urine. In healthy persons, urine contains very little protein, less than 150 mg/day; an excess is suggestive of illness. Excess protein in the urine often causes the urine to become fo ...
(blood or protein in the
urine Urine is a liquid by-product of metabolism in humans and many other animals. In placental mammals, urine flows from the Kidney (vertebrates), kidneys through the ureters to the urinary bladder and exits the urethra through the penile meatus (mal ...
); or as a
nephrotic syndrome Nephrotic syndrome is a collection of symptoms due to kidney damage. This includes proteinuria, protein in the urine, hypoalbuminemia, low blood albumin levels, hyperlipidemia, high blood lipids, and significant edema, swelling. Other symptoms ...
, a
nephritic syndrome Nephritic syndrome is a syndrome comprising medical sign, signs of nephritis, which is kidney disease involving inflammation. It often occurs in the Glomerulus (kidney), glomerulus, where it is called glomerulonephritis. Glomerulonephritis is ch ...
,
acute kidney injury Acute kidney injury (AKI), previously called acute renal failure (ARF), is a sudden decrease in renal function, kidney function that develops within seven days, as shown by an increase in serum creatinine or a decrease in urine output, or both. ...
, or
chronic kidney disease Chronic kidney disease (CKD) is a type of long-term kidney disease, defined by the sustained presence of abnormal kidney function and/or abnormal kidney structure. To meet criteria for CKD, the abnormalities must be present for at least three mo ...
. They are categorized into several different pathological patterns, which are broadly grouped into non-proliferative or proliferative types. Diagnosing the pattern of GN is important because the outcome and treatment differ in different types. Primary causes are intrinsic to the kidney. Secondary causes are associated with certain infections (bacterial, viral or parasitic pathogens), drugs, systemic disorders ( SLE, vasculitis), or diabetes.


Signs and symptoms

Glomerulonephritis refers to an inflammation of the
glomerulus ''Glomerulus'' (; : glomeruli) is a common term used in anatomy to describe globular structures of entwined vessels, fibers, or neurons. ''Glomerulus'' is the diminutive of the Latin ''glomus'', meaning "ball of yarn". ''Glomerulus'' may refer to ...
, which is the unit involved in filtration in the kidney. This inflammation typically results in one or both of the nephrotic or
nephritic syndrome Nephritic syndrome is a syndrome comprising medical sign, signs of nephritis, which is kidney disease involving inflammation. It often occurs in the Glomerulus (kidney), glomerulus, where it is called glomerulonephritis. Glomerulonephritis is ch ...
s.


Nephrotic syndrome

The nephrotic syndrome is characterised by the finding of
edema Edema (American English), also spelled oedema (British English), and also known as fluid retention, swelling, dropsy and hydropsy, is the build-up of fluid in the body's tissue (biology), tissue. Most commonly, the legs or arms are affected. S ...
in a person with increased protein in the urine and decreased protein in the blood, with increased fat in the blood. Inflammation that affects the cells surrounding the glomerulus,
podocyte Podocytes are cells in Bowman's capsule in the kidneys that wrap around capillaries of the glomerulus. Podocytes make up the epithelial lining of Bowman's capsule, the third layer through which filtration of blood takes place. Bowman's capsule ...
s, increases the permeability to
protein Proteins are large biomolecules and macromolecules that comprise one or more long chains of amino acid residue (biochemistry), residues. Proteins perform a vast array of functions within organisms, including Enzyme catalysis, catalysing metab ...
s, resulting in an increase in excreted proteins. When the amount of proteins excreted in the urine exceeds the liver's ability to compensate, fewer proteins are detected in the blood – in particular
albumin Albumin is a family of globular proteins, the most common of which are the serum albumins. All of the proteins of the albumin family are water- soluble, moderately soluble in concentrated salt solutions, and experience heat denaturation. Alb ...
, which makes up the majority of circulating proteins. With decreased proteins in the blood, there is a decrease in the
oncotic pressure Oncotic pressure, or colloid osmotic-pressure, is a type of osmotic pressure induced by the plasma proteins, notably albumin, in a blood vessel's plasma (or any other body fluid such as blood and lymph) that causes a pull on fluid back into ...
of the blood. This results in edema, as the oncotic pressure in tissue remains the same. Although decreased intravascular oncotic (i.e. osmotic) pressure partially explains the patient's edema, more recent studies have shown that extensive sodium retention in the distal nephron (collecting duct) is the predominant cause of water retention and edema in the nephrotic syndrome. This is worsened by the secretion of the hormone
aldosterone Aldosterone is the main mineralocorticoid steroid hormone produced by the zona glomerulosa of the adrenal cortex in the adrenal gland. It is essential for sodium conservation in the kidney, salivary glands, sweat glands, and colon. It plays ...
by the adrenal gland, which is secreted in response to the decrease in circulating blood and causes
sodium Sodium is a chemical element; it has Symbol (chemistry), symbol Na (from Neo-Latin ) and atomic number 11. It is a soft, silvery-white, highly reactive metal. Sodium is an alkali metal, being in group 1 element, group 1 of the peri ...
and
water Water is an inorganic compound with the chemical formula . It is a transparent, tasteless, odorless, and Color of water, nearly colorless chemical substance. It is the main constituent of Earth's hydrosphere and the fluids of all known liv ...
retention.
Hyperlipidemia Hyperlipidemia is abnormally high levels of any or all lipids (e.g. fats, triglycerides, cholesterol, phospholipids) or lipoproteins in the blood. citing: and The term ''hyperlipidemia'' refers to the laboratory finding itself and is also use ...
is thought to be a result of the increased activity of the liver.


Nephritic syndrome

The nephritic syndrome is characterised by
blood in the urine Hematuria or haematuria is defined as the presence of blood or red blood cells in the urine. "Gross hematuria" occurs when urine appears red, brown, or tea-colored due to the presence of blood. Hematuria may also be subtle and only detectable with ...
(especially Red blood cell casts with dysmorphic red blood cells) and a decrease in the amount of urine in the presence of
hypertension Hypertension, also known as high blood pressure, is a Chronic condition, long-term Disease, medical condition in which the blood pressure in the artery, arteries is persistently elevated. High blood pressure usually does not cause symptoms i ...
. In this syndrome, inflammatory damage to cells lining the glomerulus are thought to result in destruction of the
epithelial Epithelium or epithelial tissue is a thin, continuous, protective layer of cells with little extracellular matrix. An example is the epidermis, the outermost layer of the skin. Epithelial ( mesothelial) tissues line the outer surfaces of man ...
barrier, leading to blood being found in the urine. At the same time, reactive changes, e.g. proliferation of mesangial cells, may result in a decrease in kidney blood flow, resulting in a decrease in the production of urine. The
renin–angiotensin system The renin–angiotensin system (RAS), or renin–angiotensin–aldosterone system (RAAS), is a hormone system that regulates blood pressure, fluid, and electrolyte balance, and systemic vascular resistance. When renal blood flow is reduced, ...
may be subsequently activated, because of the decrease in perfusion of
juxtaglomerular apparatus The juxtaglomerular apparatus (also known as the juxtaglomerular complex) is a structure in the kidney that regulates the function of each nephron, the functional units of the kidney. The juxtaglomerular apparatus is named because it is next to ...
, which may result in hypertension.


Nonproliferative

This is characterised by forms of glomerulonephritis in which the number of cells is not changed. These forms usually result in the
nephrotic syndrome Nephrotic syndrome is a collection of symptoms due to kidney damage. This includes proteinuria, protein in the urine, hypoalbuminemia, low blood albumin levels, hyperlipidemia, high blood lipids, and significant edema, swelling. Other symptoms ...
. Causes include:


Minimal change disease

Minimal change disease Minimal change disease (MCD), also known as lipoid nephrosis or nil disease, among others, is a disease affecting the kidneys which causes nephrotic syndrome. Nephrotic syndrome leads to the loss of significant amounts of protein to the urine ( ...
is characterised as a cause of
nephrotic syndrome Nephrotic syndrome is a collection of symptoms due to kidney damage. This includes proteinuria, protein in the urine, hypoalbuminemia, low blood albumin levels, hyperlipidemia, high blood lipids, and significant edema, swelling. Other symptoms ...
without visible changes in the glomerulus on microscopy. Minimal change disease typically presents with
edema Edema (American English), also spelled oedema (British English), and also known as fluid retention, swelling, dropsy and hydropsy, is the build-up of fluid in the body's tissue (biology), tissue. Most commonly, the legs or arms are affected. S ...
, an increase in proteins passed from urine and decrease in blood protein levels, and an increase in circulating
lipid Lipids are a broad group of organic compounds which include fats, waxes, sterols, fat-soluble vitamins (such as vitamins A, D, E and K), monoglycerides, diglycerides, phospholipids, and others. The functions of lipids include storing ...
s (i.e.,
nephrotic syndrome Nephrotic syndrome is a collection of symptoms due to kidney damage. This includes proteinuria, protein in the urine, hypoalbuminemia, low blood albumin levels, hyperlipidemia, high blood lipids, and significant edema, swelling. Other symptoms ...
) and is the most common cause of the nephrotic syndrome in children. Although no changes may be visible by light microscopy, changes on
electron microscopy An electron microscope is a microscope that uses a beam of electrons as a source of illumination. It uses electron optics that are analogous to the glass lenses of an optical light microscope to control the electron beam, for instance focusing i ...
within the glomeruli may show a fusion of the
foot processes Podocytes are cells in Bowman's capsule in the kidneys that wrap around capillaries of the glomerulus. Podocytes make up the epithelial lining of Bowman's capsule, the third layer through which filtration of blood takes place. Bowman's capsule ...
of the
podocyte Podocytes are cells in Bowman's capsule in the kidneys that wrap around capillaries of the glomerulus. Podocytes make up the epithelial lining of Bowman's capsule, the third layer through which filtration of blood takes place. Bowman's capsule ...
s lining the basement membrane of the capillaries of glomerulus. It is typically managed with
corticosteroid Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are invo ...
s and does not progress to
chronic kidney disease Chronic kidney disease (CKD) is a type of long-term kidney disease, defined by the sustained presence of abnormal kidney function and/or abnormal kidney structure. To meet criteria for CKD, the abnormalities must be present for at least three mo ...
.


Focal segmental glomerulosclerosis

Focal segmental glomerulosclerosis Focal segmental glomerulosclerosis (FSGS) is a histopathologic finding of scarring (sclerosis) of glomeruli and damage to renal podocytes. This process damages the filtration function of the kidney, resulting in protein presence in the urine du ...
is characterised by a sclerosis of segments of some glomeruli. It is likely to present as a nephrotic syndrome. This form of glomerulonephritis may be associated with conditions such as
HIV The human immunodeficiency viruses (HIV) are two species of '' Lentivirus'' (a subgroup of retrovirus) that infect humans. Over time, they cause acquired immunodeficiency syndrome (AIDS), a condition in which progressive failure of the im ...
and
heroin Heroin, also known as diacetylmorphine and diamorphine among other names, is a morphinan opioid substance synthesized from the Opium, dried latex of the Papaver somniferum, opium poppy; it is mainly used as a recreational drug for its eupho ...
abuse, or inherited as
Alport syndrome Alport syndrome is a genetic disorder affecting around 1 in 5,000–10,000 children, characterized by glomerulonephritis, end-stage kidney disease, and hearing loss. Alport syndrome can also affect the eyes, though the changes do not usually affec ...
. The cause of about 20–30% of focal-segmental glomerulosclerosis is unknown. On microscopy, affected glomeruli may show an increase in hyalin, a pink and homogeneous material,
fat cells Adipocytes, also known as lipocytes and fat cells, are the cell (biology), cells that primarily compose adipose tissue, specialized in storing energy as fat. Adipocytes are derived from mesenchymal stem cells which give rise to adipocytes through ...
, an increase in the mesangial matrix and collagen. Treatment may involve
corticosteroid Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are invo ...
s, but up to half of people with focal segmental glomerulonephritis continue to have progressive deterioration of kidney function, ending in kidney failure.


Membranous glomerulonephritis

Membranous glomerulonephritis Membranous glomerulonephritis (MGN) is a slowly progressive disease of the kidney affecting mostly people between ages of 30 and 50 years, usually white people (i.e., those of European, Middle Eastern, or North African ancestry.). It is the sec ...
may cause either nephrotic or a nephritic picture. About two-thirds are associated with auto-antibodies to phospholipase A2 receptor, but other associations include
cancer Cancer is a group of diseases involving Cell growth#Disorders, abnormal cell growth with the potential to Invasion (cancer), invade or Metastasis, spread to other parts of the body. These contrast with benign tumors, which do not spread. Po ...
s of the lung and bowel, infections such as
hepatitis B Hepatitis B is an infectious disease caused by the '' hepatitis B virus'' (HBV) that affects the liver; it is a type of viral hepatitis. It can cause both acute and chronic infection. Many people have no symptoms during an initial infection. ...
and
malaria Malaria is a Mosquito-borne disease, mosquito-borne infectious disease that affects vertebrates and ''Anopheles'' mosquitoes. Human malaria causes Signs and symptoms, symptoms that typically include fever, Fatigue (medical), fatigue, vomitin ...
, drugs including
penicillamine Penicillamine, sold under the brand name of Cuprimine among others, is a medication primarily used for the treatment of Wilson's disease. It is also used for people with kidney stones who have high urine cystine levels, rheumatoid arthritis, ...
, and connective tissue diseases such as
systemic lupus erythematosus Lupus, formally called systemic lupus erythematosus (SLE), is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue in many parts of the body. Symptoms vary among people and may be mild to severe. Common ...
. Individuals with
cerebral shunt A cerebral shunt is a device permanently implanted inside the head and body to drain excess fluid away from the brain. They are commonly used to treat hydrocephalus, the swelling of the brain due to excess buildup of cerebrospinal fluid (CSF). If ...
s are at risk of developing shunt nephritis, which frequently produces MGN. Microscopically, MGN is characterized by a thickened
glomerular basement membrane The glomerular basement membrane of the kidney is the basal lamina layer of the glomerulus. The glomerular endothelial cells, the glomerular basement membrane, and the filtration slits between the podocytes perform the filtration function of th ...
without a hyperproliferation of the glomerular cells.
Immunofluorescence Immunofluorescence (IF) is a light microscopy-based technique that allows detection and localization of a wide variety of target biomolecules within a cell or tissue at a quantitative level. The technique utilizes the binding specificity of anti ...
demonstrates diffuse granular uptake of IgG. The basement membrane may completely surround the granular deposits, forming a "spike and dome" pattern. Tubules also display the symptoms of a typical Type III hypersensitivity reaction, which causes the endothelial cells to proliferate, which can be seen under a light microscope with a PAS stain. Prognosis follows the rule of thirds: one-third remain with MGN indefinitely, one-third remit, and one-third progress to end-stage kidney failure. As the glomerulonephritis progresses, the tubules of the kidney become infected, leading to atrophy and hyalinisation. The kidney appears to shrink. Treatment with
corticosteroid Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are invo ...
s is attempted if the disease progresses. In extremely rare cases, the disease has been known to run in families, usually passed down through the females. This condition, similarly, is called Familial Membranous Glomerulonephritis. There have only been about nine documented cases in the world.


Thin basement membrane disease

Thin basement membrane disease is an
autosomal An autosome is any chromosome that is not a sex chromosome. The members of an autosome pair in a diploid cell have the same morphology, unlike those in allosomal (sex chromosome) pairs, which may have different structures. The DNA in autosome ...
dominant inherited disease characterized by thin glomerular basement membranes on electron microscopy. It is a benign condition that causes persistent microscopic
hematuria Hematuria or haematuria is defined as the presence of blood or red blood cells in the urine. "Gross hematuria" occurs when urine appears red, brown, or tea-colored due to the presence of blood. Hematuria may also be subtle and only detectable with ...
. This also may cause proteinuria which is usually mild and overall prognosis is excellent.


Fibronectin glomerulopathy

Fibronectin glomerulopathy is a rare form of glomerulopathy characterised by enlarged glomeruli with deposits in the mesangium and subendothelial space. The deposits have been shown to be fibronectin. This condition is inherited in an autosomal dominant fashion. About 40% of cases are due to mutations in the fibronectin ( FN1) gene located on
chromosome 2 Chromosome 2 is one of the twenty-three pairs of chromosomes in humans. People normally have two copies of this chromosome. Chromosome 2 is the second-largest human chromosome, spanning more than 242 million base pairs and representing almost ei ...
(2q34).


Proliferative

Proliferative glomerulonephritis is characterised by an increased number of cells in the glomerulus. These forms usually present with a triad of
blood in the urine Hematuria or haematuria is defined as the presence of blood or red blood cells in the urine. "Gross hematuria" occurs when urine appears red, brown, or tea-colored due to the presence of blood. Hematuria may also be subtle and only detectable with ...
, decreased urine production, and
hypertension Hypertension, also known as high blood pressure, is a Chronic condition, long-term Disease, medical condition in which the blood pressure in the artery, arteries is persistently elevated. High blood pressure usually does not cause symptoms i ...
, the
nephritic syndrome Nephritic syndrome is a syndrome comprising medical sign, signs of nephritis, which is kidney disease involving inflammation. It often occurs in the Glomerulus (kidney), glomerulus, where it is called glomerulonephritis. Glomerulonephritis is ch ...
. These forms usually progress to end-stage kidney failure (ESKF) over weeks to years (depending on type).


IgA nephropathy

IgA nephropathy IgA nephropathy (IgAN), also known as Berger's disease () (and variations), or synpharyngitic glomerulonephritis, is a kidney disease, disease of the kidney (or nephropathy) and the immune system; specifically it is a form of glomerulonephritis o ...
, also known as ''Berger's disease'', is the most common type of glomerulonephritis, and generally presents with isolated visible or occult hematuria, occasionally combined with low grade proteinuria, and rarely causes a
nephritic syndrome Nephritic syndrome is a syndrome comprising medical sign, signs of nephritis, which is kidney disease involving inflammation. It often occurs in the Glomerulus (kidney), glomerulus, where it is called glomerulonephritis. Glomerulonephritis is ch ...
characterised by
proteinuria Proteinuria is the presence of excess proteins in the urine. In healthy persons, urine contains very little protein, less than 150 mg/day; an excess is suggestive of illness. Excess protein in the urine often causes the urine to become fo ...
, and visible
blood in the urine Hematuria or haematuria is defined as the presence of blood or red blood cells in the urine. "Gross hematuria" occurs when urine appears red, brown, or tea-colored due to the presence of blood. Hematuria may also be subtle and only detectable with ...
. IgA nephropathy is classically described as a self-resolving form in young adults several days after a respiratory infection. It is characterised by deposits of
IgA IGA or IgA may refer to: Businesses and organizations * IGA (supermarkets) (initially Independent Grocers Alliance), a name used by many independent supermarkets throughout the world ** IGA (Australian supermarket group), the local Australian v ...
in the space between glomerular capillaries.
Henoch–Schönlein purpura IgA vasculitis (HSP), previously known as Henoch–Schönlein purpura, is an autoimmune disease that most commonly affects children. In the skin, the disease causes palpable purpura (small, raised areas of bleeding underneath the skin), often wi ...
refers to a form of IgA nephropathy, typically affecting children, characterised by a rash of small bruises affecting the buttocks and lower legs, with abdominal pain.


Post-infectious

Post-infectious glomerulonephritis can occur after essentially any infection, but classically occurs after infection with the bacteria ''
Streptococcus pyogenes ''Streptococcus pyogenes'' is a species of Gram-positive, aerotolerant bacteria in the genus '' Streptococcus''. These bacteria are extracellular, and made up of non-motile and non-sporing cocci (round cells) that tend to link in chains. They ...
''. It typically occurs 1–4 weeks after a pharyngeal infection with this bacterium, and is likely to present with malaise, a slight
fever Fever or pyrexia in humans is a symptom of an anti-infection defense mechanism that appears with Human body temperature, body temperature exceeding the normal range caused by an increase in the body's temperature Human body temperature#Fever, s ...
,
nausea Nausea is a diffuse sensation of unease and discomfort, sometimes perceived as an urge to vomit. It can be a debilitating symptom if prolonged and has been described as placing discomfort on the chest, abdomen, or back of the throat. Over 30 d ...
and a mild
nephritic syndrome Nephritic syndrome is a syndrome comprising medical sign, signs of nephritis, which is kidney disease involving inflammation. It often occurs in the Glomerulus (kidney), glomerulus, where it is called glomerulonephritis. Glomerulonephritis is ch ...
of moderately increased blood pressure, gross haematuria, and smoky-brown urine. Circulating
immune complex An immune complex, sometimes called an antigen-antibody complex or antigen-bound antibody, is a molecule formed from the binding of multiple antigens to antibodies. The bound antigen and antibody act as a unitary object, effectively an antigen of ...
es that deposit in the glomeruli may lead to an inflammatory reaction. Diagnosis may be made on clinical findings or through antistreptolysin O antibodies found in the blood. A biopsy is seldom done, and the disease is likely to self-resolve in children in 1–4 weeks, with a poorer prognosis if adults are affected or if the affected children are obese.


Membranoproliferative

Membranoproliferative GN (MPGN), also known as ''mesangiocapillary glomerulonephritis,'' is characterised by an increase in the number of cells in the glomerulus, and alterations in the glomerular
basement membrane The basement membrane, also known as base membrane, is a thin, pliable sheet-like type of extracellular matrix that provides cell and tissue support and acts as a platform for complex signalling. The basement membrane sits between epithelial tis ...
. These forms present with the
nephritic syndrome Nephritic syndrome is a syndrome comprising medical sign, signs of nephritis, which is kidney disease involving inflammation. It often occurs in the Glomerulus (kidney), glomerulus, where it is called glomerulonephritis. Glomerulonephritis is ch ...
, hypocomplementemia, and have a poor prognosis. Three subtypes have been proposed: * Type 1 MPGN is caused by immune complex deposition in the mesangium and subendothelial space, typically secondary to
systemic lupus erythematosus Lupus, formally called systemic lupus erythematosus (SLE), is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue in many parts of the body. Symptoms vary among people and may be mild to severe. Common ...
,
hepatitis B Hepatitis B is an infectious disease caused by the '' hepatitis B virus'' (HBV) that affects the liver; it is a type of viral hepatitis. It can cause both acute and chronic infection. Many people have no symptoms during an initial infection. ...
and C, or other chronic or recurring infections. Circulating immune complexes may activate the
complement system The complement system, also known as complement cascade, is a part of the humoral, innate immune system and enhances (complements) the ability of antibodies and phagocytic cells to clear microbes and damaged cells from an organism, promote inf ...
, leading to inflammation and an influx of inflammatory cells. * Type 2 MPGN, also known as ''Dense Deposit Disease'', is characterised by an excessive activation of the complement system. The C3 Nephritic Factor
autoantibody An autoantibody is an antibody (a type of protein) produced by the immune system that is directed against one or more of the individual's own proteins. Many autoimmune diseases (notably lupus erythematosus) are associated with such antibodies. Pr ...
stabilizes
C3-convertase C3 convertase (''C4bC2b'', formerly ''C4b2a'') belongs to family of serine proteases and is necessary in innate immunity as a part of the complement system which eventuate in opsonisation of particles, release of inflammatory peptides, C5 con ...
, which may lead to an excessive activation of complement. Type 2 MPGN is a subgroup of C3 glomerulopathy, a newly proposed diagnosis which also encompasses C3 Glomerulonephritis (C3GN). * Type 3 MPGN, which is caused by immune complex deposition in the subepithelial space.


Rapidly progressive glomerulonephritis

Rapidly progressive glomerulonephritis, also known as ''crescentic GN'', is characterised by a rapid, progressive deterioration in kidney function. People with rapidly progressive glomerulonephritis may present with a
nephritic syndrome Nephritic syndrome is a syndrome comprising medical sign, signs of nephritis, which is kidney disease involving inflammation. It often occurs in the Glomerulus (kidney), glomerulus, where it is called glomerulonephritis. Glomerulonephritis is ch ...
. In management,
steroid A steroid is an organic compound with four fused compound, fused rings (designated A, B, C, and D) arranged in a specific molecular configuration. Steroids have two principal biological functions: as important components of cell membranes t ...
therapy is sometimes used, although the prognosis remains poor. Three main subtypes are recognised: * Type 1 is
Goodpasture syndrome Goodpasture syndrome (GPS), also known as anti–glomerular basement membrane disease, is a Rare disease, rare autoimmune disease in which antibodies attack the basement membrane in lungs and kidneys, leading to bleeding from the lungs, glomeru ...
, an
autoimmune disease An autoimmune disease is a condition that results from an anomalous response of the adaptive immune system, wherein it mistakenly targets and attacks healthy, functioning parts of the body as if they were foreign organisms. It is estimated tha ...
also affecting the
lung The lungs are the primary Organ (biology), organs of the respiratory system in many animals, including humans. In mammals and most other tetrapods, two lungs are located near the Vertebral column, backbone on either side of the heart. Their ...
. In Goodpasture syndrome, IgG antibodies directed against the glomerular
basement membrane The basement membrane, also known as base membrane, is a thin, pliable sheet-like type of extracellular matrix that provides cell and tissue support and acts as a platform for complex signalling. The basement membrane sits between epithelial tis ...
trigger an inflammatory reaction, causing a
nephritic syndrome Nephritic syndrome is a syndrome comprising medical sign, signs of nephritis, which is kidney disease involving inflammation. It often occurs in the Glomerulus (kidney), glomerulus, where it is called glomerulonephritis. Glomerulonephritis is ch ...
and the coughing up of blood. High dose immunosuppression is required (intravenous
methylprednisolone Methylprednisolone (Depo-Medrol, Medrol, Solu-Medrol) is a synthetic glucocorticoid, primarily prescribed for its anti-inflammatory and immunosuppressive effects. It is either used at low doses for chronic illnesses or used at high doses durin ...
) and cyclophosphamide, plus plasmapheresis. Immunohistochemistry staining of tissue specimens shows linear IgG deposits. * Type 2 is characterised by immune-complex-mediated damage, and may be associated with
systemic lupus erythematosus Lupus, formally called systemic lupus erythematosus (SLE), is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue in many parts of the body. Symptoms vary among people and may be mild to severe. Common ...
, post-infective glomerulonephritis, IgA nephropathy, and IgA vasculitis. * Type 3 rapidly progressive glomerulonephritis, also called ''pauciimmune type'', is associated with causes of vascular inflammation including
granulomatosis with polyangiitis Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), after Nazi German physician Friedrich Wegener, is a rare, long-term, systemic disorder that involves the formation of granulomas and vasculitis, inflammatio ...
(GPA) and microscopic polyangiitis. No immune deposits can be seen on staining, however blood tests may be positive for the ANCA antibody. Histopathologically, the majority of glomeruli present "crescents". Formation of crescents is initiated by passage of fibrin into the Bowman space as a result of increased permeability of glomerular basement membrane. Fibrin stimulates the proliferation of endothelial cells of Bowman capsule, and an influx of monocytes. Rapid growing and fibrosis of crescents compresses the capillary loops and decreases the Bowman space, which leads to kidney failure within weeks or months.


Diagnosis

Some forms of glomerulonephritis are diagnosed clinically, based on findings on history and examination. Other tests may include: * Urine examination * Blood tests investigating the cause, including FBC, inflammatory markers, and special tests (including ASLO, ANCA, Anti-GBM,
Complement Complement may refer to: The arts * Complement (music), an interval that, when added to another, spans an octave ** Aggregate complementation, the separation of pitch-class collections into complementary sets * Complementary color, in the visu ...
levels,
Anti-nuclear antibodies Antinuclear antibodies (ANAs, also known as antinuclear factor or ANF) are autoantibodies that bind to contents of the cell nucleus. In normal individuals, the immune system produces antibodies to foreign proteins (antigens) but not to human pro ...
) *
Biopsy A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, an interventional radiologist, or an interventional cardiology, interventional cardiologist. The process involves the extraction of sampling (medicine), sample ...
of the kidney *
Renal ultrasonography Renal ultrasonography (Renal US) is the examination of one or both kidneys using medical ultrasound. Ultrasonography of the kidneys is essential in the diagnosis and management of kidney-related diseases. The kidneys are easily examined, and most ...
is useful for prognostic purposes in finding signs of
chronic kidney disease Chronic kidney disease (CKD) is a type of long-term kidney disease, defined by the sustained presence of abnormal kidney function and/or abnormal kidney structure. To meet criteria for CKD, the abnormalities must be present for at least three mo ...
, which however may be caused by many other diseases than glomerulonephritis.


Treatment

Antibiotic therapy to prevent streptococcal infection (prophylaxis). Steroids to suppress inflammation. Provide balanced diet in terms of calories, sodium and potassium. A low-protein diet is often recommended in chronic kidney disease, but benefits may be limited in case of glomerular disorders, the response in patients with chronic glomerulonephritis correlated closely with the effect of diet on proteinuria in these patients. Monitor for sign of kidney failure, heart failure, and hypertensive encephalopathy.


See also

*
Nephritic syndrome Nephritic syndrome is a syndrome comprising medical sign, signs of nephritis, which is kidney disease involving inflammation. It often occurs in the Glomerulus (kidney), glomerulus, where it is called glomerulonephritis. Glomerulonephritis is ch ...
*
Nephritis Nephritis is inflammation of the kidneys and may involve the glomeruli, tubules, or interstitial tissue surrounding the glomeruli and tubules. It is one of several different types of nephropathy. Types * Glomerulonephritis is inflammation ...
*
Lupus nephritis Lupus nephritis is an inflammation of the kidneys caused by systemic lupus erythematosus (SLE) and childhood-onset systemic lupus erythematosus which is a more severe form of SLE that develops in children up to 18 years old; both are autoimmune d ...
* Rapidly progressive glomerulonephritis


References


External links

{{Authority control Inflammations Glomerular diseases Kidney diseases