Rapidly progressive glomerulonephritis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Jogeet Singh Sekhon, M.D. [2] Nazia Fuad M.D.

Rapidly progressive glomerulonephritis (RPGN) is a disease of kidney which occurs following severe damage to the kidneys and it can lead to rapid deterioration of kidney function in a few days. It is characterized by the presence of crescents in the glomeruli and hence is also called crescentic glomerulonephritis. Patients with RPGN present with nephritic syndrome, but some may also have proteinuria. RPGN progresses to end stage renal disease if it is not treated in time. RPGN is classified into three types, all of which involve immune-mediated damage to the glomeruli. In type I RPGN, injury is caused by antibodies directed against the glomerular basement membrane. Type II RPGN is characterized by the deposition of immune complexes in the glomerulus. Type III, or pauci-immune RPGN, features antibodies directed against neutrophils (anti-neutrophil cytoplasmic antibodies, ANCA). Treatment depends on the underlying disease process. For example, plasmapheresis, corticosteroids, and cytotoxic drugs may promote recovery in Goodpasture syndrome, a cause of type I RPGN. Despite even early treatment, however, many patients with RPGN may ultimately require dialysis and possibly renal transplant.

1919 Ernst Goodpasture made case reports about glomerulnophritis and pulmonary haemorrhage. Stanton and Tait from Australia studied these case reports and then named the findings as Goodpasture syndrome in 1958. They gave the anti GBM antibodies classification and discovered RPGN in these cases. In 1960s electron microscopy and immunofluorescence helped to learn RPNG on immunological level.

Rapidly progressive glomerulonephritis can be classified on the basis of cause of glomerular injury.The immunoflourescent microspcopic findings are used in determining the cause of glomerular injury.

Rapidly progressive glomerulonephritis is a disease of the kidney in which the renal function deteriorates in a few days. Atleast 50% reduction in GFR occurs in RPGN in a few days to weeks. RPGN occurs from severe and fast damage to the GBM which results in crescent formation, the main pathological finding in RPGN. Injury can occur by anti GBM antibodies-type I RPGN, Immune complex– type II RPGN or pauci immune RPGN(ANCAs)-type III RPGN. Crescents are present in the Bowmans space. Light, immunofluoresnce and electron microscopy are used to diagnose RPGN.

Rapidly progressive glomerulonephritis can be caused by multiple factors.These include life threatening conditions such as sepsis and other preexisting renal diseases. Infections, drugs and some types of cancer also cause RPGN.

The various types of glomerulonephritides should be differentiated from each other based on associations, presence of pitting edema, hemeturia, hypertension, hemoptysis, oliguria, peri-orbital edema, hyperlipidemia, type of antibodies, light and electron microscopic features.

The incidence of RPGN is 1 per 1 million individuals per year and the incidence is affected by race and age.

Common risk factors in the development of rapidly progressive glomerulonephritis may be occupational, environmental, genetic, and viral.

There is insufficient evidence to recommend routine screening for rapidly progressive glomerulonephritis.

Patients with RPGN present with flu like symptoms initially and then develop nephritic syndrome with proteinuria in some cases as well. In type III RPGN, systemic features of vasculitis are present in some cases.Pulmonary symptoms are also present in Goodpastures syndrome and Churg Strauss syndrome.The prognosis is usually poor due to rapid deterioration of renal function and is dependent on age, presence of pulmonary symptoms, serum creatinine levels and presence of ANCAs.

Determination of ANCAs can aid in the diagnosis, but positivity is not conclusive and negative ANCAs are not sufficient to reject the diagnosis. Cytoplasmic staining ANCAs that react with the enzyme proteinase 3 (cANCA) in neutrophils (a type of white blood cell) are associated with Granulomatosis with polyangiitis. If the patient has renal failure or cutaneous vasculitis, these are the most logical organs to obtain a biopsy from. Rarely, thoracoscopic lung biopsy is required.

In rapidly progressive glomerulonephritis The most common early presentation is flulike symptoms characterized by malaise, fever, arthralgias, myalgias, anorexia, and weight loss. This is seen in more than 90% of patients. Following the initial phase, the other symptoms include abdominal pain, painful cutaneous nodules or ulcerations, and a migratory polyarthropathy. When pulmonary or upper airway involvement is present, patients complain of sinusitis symptoms, cough, and hemoptysis

Common physical examination findings of rapidly progressive glomerulonephritis include, hematuria, hypertension, edema, skin nodules, gastrointestinal bleeding.

Arthralgia and arthritis may be seen. Nervous system involvement is present in 30% of patients with microscopic polyangiitis and 70% of patients with Churg-Strauss diseas.

The most important step in managing rapidly progressive glomerulonephritis is rapid diagnosis. It is essential for organ preservation. Laboratory studies include, complete blood cell count (CBC) with differential, serum electrolytes, BUN, creatinine, lactate dehydrogenase (LDH), creatine phosphokinase (CPK), and liver function tests: The most common abnormality is an increased serum creatinine level. However, the level can be normal at presentation. Tissue enzyme (ie, LDH, CPK) levels may be elevated if there is significant muscle inflammation and myalgias. Urinalysis with microscopy usually show proteinuria but is rarely greater than 2-3 g in 24 hours. Microscopic hematuria may be present and may be the only clue to renal disease at presentation. The presence of red cell casts indicates glomerulonephritis and is a very helpful clue. Erythrocyte sedimentation rate is usually elevated with active disease. C-reactive protein levels are elevated and correspond with disease activity. High antinuclear antibody (ANA) titer rises the suspicion toward systemic lupus erythematosus. More than 80% of patients with microscopic polyangiitis are ANCA-positive. The symptoms of cryoglobulinemia are very similar to those of ANCA-related disease. However, in persons with ANCA-related diseases, the cryoglobulin titer result should be negative. Hepatitis profile should be performed as hepatitis B is associated with polyarteritis nodosa and hepatitis C is associated with mixed cryoglobulinemia. Urine and serum protein electrophoresis is done in any middle-aged or elderly person presenting with rapidly progressive glomerulonephritis to exclude the presence of light-chain disease or noticeable multiple myeloma as a cause of the clinical findings

There are no EKG findings associated with rapidly progressive glomerulonephritis

There are no x-ray findings associated with rapidly progressive glomerulonephritis unless it is associated with anti-GBM antibody disease (Goodpasture syndrome). On chest X-ray, Goodpasture syndrome is characterized by parenchymal consolidations that are most often present in both lungs, perihilar, and bibasilar. When pulmonary hemorrhage is recurrent an interstitial pattern is observed

There are no EKG findings associated with rapidly progressive glomerulonephritis

Kidney ultrasound is usually done during the diagnostic biopsy. Due to its rapid progression, the renal biopsy usually shows normal-sized kidneys. Although the test is not diagnostic, its non-invasive nature and the necessity to rule out other etiologies of renal impairment are both in favor of performing a renal ultrasound

There are no CT scan findings associated with rapidly progressive glomerulonephritis.

There are no MRI findings associated with rapidly progressive glomerulonephritis.

There are no other imaging findings associated with rapidly progressive glomerulonephritis

The sensitivity and the specificity of ANCA testing for pauci-immune glomerulonephritis is only 80-90%, a renal biopsy is crucial for diagnosis of rapidly progressive glomerulonephritis. It helps to determine the severity of the disease. The initiation of therapy should not be delayed for biopsy results. Renal biopsy specimens show a diffuse, proliferation, necrotizing glomerulonephritis with crescent formation. Immunofluorescence microscopy shows finding of linear deposition of immunoglobulin G (IgG) along the glomerular capillaries and occasionally the distal tubules.

Treatment of RPGN depends on the underlying disease process. For example, plasmapheresis, corticosteroids, and cytotoxic drugs may promote recovery in Goodpasture syndrome, a cause of type I RPGN. Despite even early treatment, however, many patients with RPGN may ultimately require dialysis and possibly renal transplant.

Surgery is not the first-line treatment option for patients with rapidly progressive glomerulonephritis. Renal transplantation is usually reserved for patients who present with undetectable circulating anti-glomerular basement antibodies in serum for 12 months and at least 6 months after stopping the use of cytotoxic agents.

There are no established measures for the primary prevention of Rapidly progressive glomerulonephritis.

There are no established measures for the secondary prevention of Rapidly progressive glomerulonephritis.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Jogeet Singh Sekhon, M.D. [2]

In 1919 Ernst Goodpasture made case reports about glomerulnophritis and pulmonary haemorrhages. Stanton and Tait from Australia studied these case reports and then named the findings as Goodpasture syndrome in 1958. They gave the anti GBM antibodies classification and discovered RPGN in these cases. In 1960s electron microscopy and immunofluorescence helped to learn RPNG on immunological level.

• Ernst Goodpasture made case reports about glomerulnophritis and pulmonary haemorrhages in 1919^[1].
• Stanton and Tait from Australia studied these case reports and then named the findings as Goodpasture syndrome in 1958.
• They gave the anti GBM antibodies classification and discovered RPGN in these cases.
• In 1960s, electron microscopy and immunofluorescence helped to learn RPNG on immunological level.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Jogeet Singh Sekhon, M.D. [2]

Rapidly progressive glomerulonephritis is classified on the basis of cause of glomerular injury.The immunoflourescent microspcopic findings are used in determining the cause of glomerular injury.

RPGN is classified on the basis of the cause of glomerular injury and the findings from light and immunofluorescence microscopy.^[1][2].

• Type I RPGN is characterized by the presence of autoantibodies directed against the glomerular basement membrane (GBM)^[3].
• Type I also known as anti-GBM glomerulonephritis.
• The antibodies formed are known as anticollagen antibodies and react against type IV collagen of GBM.^[4]
• The antibodies can be produced by a stimulus such as viral URTI that exposes alveolar collagen membrane or it can be idiopathic.
• The antibodies formed can act against alveolar membrane and lungs get involved in some cases such as in goodpasture syndrome.

• Type II RPGN is caused by the deposition of immune complexes in the GBM.
• Immune complexes can be formed in certain infections or in connective tissue disorders.
• These immune complexes deposit over the GBM and activate the complement system resulting in crescent formation.
• Examples include:
• Postinfectious (staphylococci/streptococci)
• Connective tissue disorders
• Lupus nephritis
• Henoch-Schönlein purpura
• Immunoglobulin A nephropathy
• Mixed cryoglobulinemia
• Membranoproliferative glomerulonephritis

• Type III RPGN is also known as pauci immune RPGN^[4].
• There are no anti GBM antibodies or no immune complexes involved.
• It is further classified into 2 types:
  • Immunogenic – ANCA positive
  • Non immunogenic- ANCA negative/ Idiopathic
• ANCAs cause the release of lytic enzymes from neutrophils that damage the GBM.
• Systemic vasculitis is present in most of the cases but some occur without systemic involvement and only renal findings maybe present.
• Examples include
  • Granulomatosis with polyangiitis (Wegener granulomatosis)
  • Microscopic polyangiitis (MPA)
  • Renal-limited necrotizing crescentic glomerulonephritis (NCGN)
  • Eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome)
  • Drugs- hydralazine, allopurinol and rifampin.

.Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Jogeet Singh Sekhon, M.D. [2] Syed Ahsan Hussain, M.D.[3]

Rapidly progressive glomerulonephritis is a disease of the kidney in which the renal function deteriorates in a few days. Atleast 50% reduction in GFR occurs in RPGN in a few days to weeks. RPGN occurs from severe and fast damage to the GBM which results in crescent formation, the main pathological finding in RPGN. Injury can occur by anti GBM antibodies-type I RPGN, Immune complex– type II RPGN or pauci immune RPGN(ANCAs)-type III RPGN. Crescents are present in the Bowmans space. Light, immunofluoresnce and electron microscopy are used to diagnose RPGN.

The key for the renal corpuscle figure is: A – Renal corpuscle, B – Proximal tubule, C – Distal convoluted tubule, D – Juxtaglomerular apparatus, 1. Basement membrane (Basal lamina), 2. Bowman’s capsule – parietal layer, 3. Bowman’s capsule – visceral layer, 3a. Pedicels (Foot processes from podocytes), 3b. Podocyte, 4. Bowman’s space (urinary space), 5a. Mesangium – Intraglomerular cell, 5b. Mesangium – Extraglomerular cell, 6. Granular cells (Juxtaglomerular cells), 7. Macula densa, 8. Myocytes (smooth muscle), 9. Afferent arteriole, 10. Glomerulus Capillaries, 11. Efferent arteriole.

• Rapidly progressive glomerulonephritis is a disease of the kidney in which the renal function deteriorates in a few days^[1][2].

• Atleast 50% reduction in GFR occurs in RPGN in a few days to weeks.

• RPGN occurs from severe and fast damage to the GBM which results in crescent formation, the main pathological finding in RPGN.
• The injury to GBM can be caused by multiple factors.
• Crescent formation is the major pathological finding.
• In some cases crescents might be absent.

• Crescents are defined as 2 or more layers of proliferating cells in the Bowman’s space.^[2][3][4]
• The crescents are made up of epithelial cells and macrophages which undergo fibrosis^[5][6].
• Crescents are formed after a severe injury to the glomerulus.
• Injury to the glomerulus causes leakage of cells(epithelial, macrophages, coagulation proteins and fibroblasts) and cytokines(IL-12, TNF-alpha) into the Bowmans space.
• The presence of cytokines and coagulation proteins initiates fibrosis around the epithelial cells.
• The fibrosis blocks the glomerulus and filteration is hindered.
• This results in renal failure.

• Injury to the glomerulus is the initiating factor for crescent formation.
• Injury can occur by the following.

1. Anti GBM antibodies-Type I RPGN

• These are autoantibodies that cross react with type IV collagen of the GBM.
• These can be produced due to genetic causes such as in Goodpasture syndrome or they can be produced after viral URTI or cigarette smoking.
• These autoantibodies react with the GBM resulting in IgG deposition over the GBM.
• The IgG activates helper T cells that attract the inflammatory mediators to the GBM damaging the glomeruli^[7].
• This damage causes leakage of cells and inflammatory mediators resulting in crescent formation.
• The anti GBM antibodies can affect the lungs as well as in Goodpasture syndrome resulting in glomerular necrosis and pulmonary haemorrhages.

2. Immune complex– Type II RPGN

• Immune complexes are formed in certain infections, connective tissue diseases, side effects of some drugs and in some myeloproliferative disorders^[8].
• These immune complexes are deposited over the GBM.
• The immune complexes activate the complement system which sets off the inflammatory process.
• The complement cascade is activated, attracting inflammatory cells and mediators to the GBM.
• The serum levels of c3 and c4 fall down and is an indicator of immune complex mediated glomerular injury.

• This damages the glomeruli and causes leakage of cells and inflammatory mediators resulting in crescent formation.
  • Examples include:
  • Postinfectious (staphylococci/streptococci)
  • Connective tissue disorders
  • Lupus nephritis
  • Henoch-Schönlein purpural)
  • Immunoglobulin A nephropathy^[9]
  • Mixed cryoglobulinemia
  • Membranoproliferative glomerulonephritis

3. Pauci immune RPGN-Type III RPGN

• No circulating immune complexes or antibodies.
• Glomerular damage is caused by circulating ANCAs(anti nuclear cytoplasmic antibodies) or it can be idiopathic(non ANCA).
• ANCAs cause glomerular damage by releasing lytic enzymes from white blood cells such as neutrophils^{[10][11][12][13]}.
• These lytic enzymes damage the GBM and cause leakage of circulating cells and initiate crescent formationin the Bowmans space.
• ANCAs are associated with systemic vasculitis.^[14]
• Examples include
  • Granulomatosis with polyangiitis (Wegener granulomatosis)
  • Microscopic polyangiitis (MPA)
  • Renal-limited necrotizing crescentic glomerulonephritis (NCGN)
  • Eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome)
  • Drugs- hydralazine, allopurinol and rifampin.

Consitions associated with membranous glomerulonephritis include:^[15]

• Hepatitis B
• Hepatitis C
• Congenital Syphilis
• Systemic Lupus Erythematosis
• Malignancy
  • Lung
  • Breast
  • Colon
  • Stomach
  • Kidney
  • Leukemia
  • Lymphomas (Hodgkin’s and non-Hodgkin’s)

• The kidneys appear to be having having haemorrhages and necrosed tissue.
• Pulmonary haemorrhages may also be present in Goodpasture syndrome and type III RPGN.
• Type III RPGN may present with petechiae, rashes and purpuras.

• Glomerular inflammation with signs of necrosis are present^[16][17].
• .Glomerular caplillary wall rupture and damage to GBM.
• Crescents are present in the Bowmans space.
• Crescents are formed by proliferating epithelial cells and monocytes
• Fibroblasts migrate to the Bowman’s space and synthesize collagen.
• When cellular components are mixed with collagen the lesion is called fibroepithelial crescent.
• Renal vessels can show transmural vasculitis, with necrosis and lymphocyte infiltrates.
• Tubular necrosis may also be present.
• Interstitial granulomas in the glomeruli indicate Wegener’s granulomatosis.

• In type I RPGN- diffuse and linear deposition of IgG along the GBM.
• In ttype II RPGN- diffuse and irregular deposition of IgG and C3 in the mesangial matrix.
• In type III RPGN- no finding.

• In type I and type III, no electron dense deposits are seen.
• In type II RPGN, subepithelial electron dense deposits indiacting the presence of immune complexes are seen.

People with HLA DP1,DQ and DRB4 are more susceptible to develop RPGN^[19]. {{#ev:youtube|CqSyj4cVZPE}}

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Jogeet Singh Sekhon, M.D. [2]

Rapidly progressive glomerulonephritis can be caused by multiple factors.These include life threatening conditions such as sepsis and other pre existing renal diseases.Infections, drugs and some types of cancer also cause RPGN.

• Infective endocarditis
• Sepsis

Common causes of rapidly progressive glomerulonephritis may include^[1][2][3]

• Goodpasture syndrome^[4]
• Post streptococcal glomerulonephritis^[5]
• Lupus nephritis
• Henoch-Schönlein purpural)
• Immunoglobulin A nephropathy
• Mixed cryoglobulinemia
• Membranoproliferative glomerulonephritis
• Granulomatosis with polyangiitis (Wegener granulomatosis)>^[6].
• Microscopic polyangiitis (MPA)^[7]

• Renal-limited necrotizing crescentic glomerulonephritis (NCGN)
• Eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome

Less common causes of rapidly progressive glomerulonephritis include:

• Cryoglobulinemia
• Systemic necrotizng vasculitis
• Colon cancer
• Lung cancer
• Lymphoma
• Allopurinol
• Hydralazine
• Rifampin
• Behcet’s disease
• Renal-limited necrotizing crescentic glomerulonephritis (NCGN)

• Rapidly progressive glomerulonephritis is more common in people who have HLA DP1, DQ and DRB4s.

Cardiovascular	Infective endocarditis
Chemical/Poisoning	No underlying causes
Dental	No underlying causes
Dermatologic	No underlying causes
Drug Side Effect	Hydralazine,Allopurinol,Rifampin
Ear Nose Throat	No underlying causes
Endocrine	No underlying causes
Environmental	No underlying causes
Gastroenterologic	Colon cancer
Genetic	HLA DP1, HLA DQ, HLA DRB4s
Hematologic	Lymphoma, Henoch-Schönlein purpural)
Iatrogenic	No underlying causes
Infectious Disease	Hepatitis B,Hepatitis C
Musculoskeletal/Orthopedic	No underlying causes
Neurologic	No underlying causes
Nutritional/Metabolic	No underlying causes
Obstetric/Gynecologic	No underlying causes
Oncologic	Colon cancer, Pulmonary cancer, Lymphoma
Ophthalmologic	No underlying causes
Overdose/Toxicity	No underlying causes
Psychiatric	No underlying causes
Pulmonary	Lung cancer
Renal/Electrolyte	Postinfectious glomerulonephritis,Lupus nephritis,Immunoglobulin A nephropathy ,Membranoproliferative glomerulonephritis, Renal-limited necrotizing crescentic glomerulonephritis (NCGN)
Rheumatology/Immunology/Allergy	Goodpasture syndrome, SLE, Behcet’s disease, Granulomatosis with polyangiitis (Wegener granulomatosis), Microscopic polyangiitis, Eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome),
Sexual	No underlying causes
Trauma	No underlying causes
Urologic	No underlying causes
Miscellaneous	Sepsis

List the causes of the disease in alphabetical order:

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

The various types of glomerulonephritides should be differentiated from each other based on associations, presence of pitting edema, hemeturia, hypertension, hemoptysis, oliguria, peri-orbital edema, hyperlipidemia, type of antibodies, light and electron microscopic features.

The various types of glomerulonephritides should be differentiated from each other based on associations, presence of pitting edema, hemeturia, hypertension, hemoptysis, oliguria, peri-orbital edema, hyperlipidemia, type of antibodies, light and electron microscopic features. The following table differentiates between various types of glomerulonephritides:^{[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24]}

Glomerulonephritis	Sub-entity	Causes and associations	History and Symtoms							Laboratory Findings
										Hyperlipidemia and hypercholesterolemia	Nephrotic features	Nephritic features	ANCA	Anti-glomerular basement membrane antibody (Anti-GBM antibody)	Immune complex formation	Light microscope	Electron microscope	Immunoflourescence pattern
			History	Pitting edema	Hemeturia (pre-dominantly microscopic)	Hypertension	Hemoptysis	Oliguria	Peri-orbital edema
Non-proliferative	Minimal change disease	Idiopathic Protein tyrosine phosphatase receptor type O (glomerular epithelial protein 1- GLEPP1)	Young children Recent infection and immunization Atopy Hodgkin lymphoma Thrombosis (due to urinary loss of antithrombin-III)	+	–	–	–	+/-	–	+	+	–	–	–	–	Normal	Fusion of podocytes	–
	Focal segmental glomerulosclerosis	Idiopathic HIV Heroine use Sickle cell disease Interferon Severe obesity Mixed cryoglobulinemia (Hepatitis C)	Adults	+	–	–	–	+/-	–	+	+	–	–	–	–	Focal (some glomeruli) and segmental (only part of glomerulus)	Effacement of podocytes	–
	Membranous glomerulonephritis	Idiopathic Hepatitis B and C Solid tumors Systemic lupus erythematosus Drugs (NSAIDS, pencilamine, gold, captopril)		+	–	–	–	+/-	–	+	+	–	–	–	+	Thick glomerular basement membrane	Sub-epithelial immune complex depositis with ‘spike and dome’ appearance	–
Proliferative	IgA nephropathy	Idiopathic Viral infections	Young children History of mucosal infections (e.g. gastroenteritis) and upper respiratory tract infection 2-3 days after infection (synpharyngitic)	+/-	+	+	–	+	+/-	–	–	+	–	–	+	Crescent formation	Mesangial proliferation	–
	Rapidly progressive glomerulonephritis	Goodpasture syndrome	Young adults	+/-	+	+	+	+	+	–	–	+	–	+	+	Hypercellular and inflamed glomeruli (Crescent formation)	Diffuse thickening of the glomerular basement membrane with absence of sub-epithelial and sub-endothelial deposits	+ (Linear)
		Post infectious glomerulonephritis	Streptococcal skin infections Streptococcal pharyngitis 2-3 weeks after infection	+/-	+	+	+	+	+	–	–	+	–	–	+	Hypercellular and inflamed glomeruli	Sub-epithelial immune complex deposits	+ (Granular)
		Granulomatosis with polyangitis (Wegner’s granulomatosis)	Necrotizing granulomas (Nasopharynx, lungs, kidneys) Conjunctivitis Ulceration of the cornea Episcleritis Peripheral neuropathy	+/-	+	+	+	+	+	–	–	+	+ (C-ANCA)	–	–	Hypercellular and inflamed glomeruli (Crescent formation)	– (pauci-immune)	+/-
		Churg Strauss syndrome	Necrotizing granulomas (Lungs and kidneys) Asthma Peripheral neuropathy	+/-	+	+	+	+	+	–	–	+	+ (C-ANCA)	–	–	Hypercellular and inflamed glomeruli (Crescent formation)	– (pauci-immune)	–
		Microscopic polyangiitis	Necrotizing vasculitis (no granuloma)	+/-	+	+	+	+	+	–	–	+	+ (P-ANCA)	–	–	Hypercellular and inflamed glomeruli (Crescent formation)	– (pauci-immune)	–
	Membranoproliferative glomerulonephritis	Idiopathic Hepatitis B and C (Type 1) C3 nepritic factor (Type2)	Hematuria Oliguria Periorbital edema Hypertension	+/-	+	+	+	+	+	–	+	–	–	–	+	Thick glomerular basement membrane (Tram-track appearance)	Mesangial proliferation and leukocyte infiltration	+ (Granular)

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ahmed Elsaiey, MBBCH [2] Jogeet Singh Sekhon, M.D. [3]

The incidence of RPGN is 1 per 1 million individuals per year and the incidence is affected by race and age.

• The incidence of rapidly progressive glomerulonephritis is around 1 per million individuals per year.^{[1] [2]}

• The prevalence of rapidly progressive glomerulonephritis increases with age.

• Rapidly progressive glomerulonephritis is more prevalent in the white race more than the black race.

• Men and women are affected equally by rapidly progressive glomerulonephritis.

• It is more common in old individuals.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Jogeet Singh Sekhon, M.D. [2]

Common risk factors in the development of rapidly progressive glomerulonephritis may be occupational, environmental, genetic, and viral.

Common risk factors in the development of rapidly progressive glomerulonephritis may be occupational, environmental, genetic, and viral. ^[1][2]

• Pre existing renal disease.

• Presence of HLADRB1, HLA DP1, DQ and DRB4s^[3]

• Upper respiratory tract infection
• Sepsis
• Tobacco smoking
• Intravenous drug abuse

• Cocaine use
• Exposure to hydrocarbons( formaldehyde)
• HIgh concenteration of FiO₂

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

There is insufficient evidence to recommend routine screening for rapidly progressive glomerulonephritis.

There is insufficient evidence to recommend routine screening for rapidly progressive glomerulonephritis^[1].

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Jogeet Singh Sekhon, M.D. [2]

Patients with RPGN present with flu like symptoms initially and then develop nephritic syndrome with proteinuria in some cases as well. In type III RPGN, systemic features of vasculitis are present in some cases.Pulmonary symptoms are also present in Goodpastures syndrome and Churg Strauss syndrome.The prognosis is usually poor due to rapid deterioration of renal function and is dependent on age, presence of pulmonary symptoms, serum creatinine levels and presence of ANCAs.

• Patients with RPGN present with initial flu like symptoms and then progress to nephritic syndrome in 2-3 months if not treated in time^[1][2].
• Symptoms include malaise, arthralgias, fever, anorexia, weight loss, haematuria, edema, hypertension and abdominal pain.
• Patients with respiratory system involvement as in Goodpasture syndrome and Churg strauss, have symtopms of asthma, atopy and haemoptysis as well.
• RPGN can lead to ARF when renal symptoms develop.
• Increase in serum creatinine and fall in GFR are used to measure the severity of the disease.
• 70-80% Patients with RPGN will progress to end stage renal disease if not treated in time.
• Serum creatinine >4.6 mg/dl and GFR less than 50% denote end stage renal disease and requires dialysis.
• Histopathologically, the presence of fibrous crescents indicate irreversible renal damage and poor prognosis.

• Nephritic syndrome
• Nephrotic syndrome
• Acute kidney injury
• Chronic renal failure

• The prognosis of rapidly progressive glomerulonephritis is poor due to rapid deterioration of kidney function in a few weeks.

• Prognosis depends on^[3][4]
  • Age
  • Serum creatinine
  • Presence of pulmonary symptoms at the onset of disease
  • Race
  • Presence of ANCA
  • Presence of fibrous cresecnts on histopathology.
• Factors that are indicators of poor prognosis :
  • Age >60 years
  • Serum creatinine > 7.5 mg/dl.
  • Oliguric renal failure
  • Pauci immune RPGN
  • White race
  • 75 % fibrous cresecents in the glomerulus
• 70-80% of patients with RPGN develop end stage renal disease and require dialysis for a long time and some may require kidney transplant.

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