Question

    Acute Glomerulonephritis (Acute Glomerulonephritis Syndrome)

    Post-infectious glomerulonephritis - very high mag[1]

    Acute glomerulonephritis (AGN) is an immune-mediated inflammation and destruction of the glomeruli and peri-glomerular tissues including the basement membrane, mesangium, and the capillary endothelium.

    Acute Glomerulonephritis is related to this process: auto-immune.

    Presentation

    AGN is asymptomatic in approximately 50% of patients. In those who present with symptoms, the initial presentation is peripheral edema, low urine volume, and dark-colored urine due to hematuria.The reduction in GFR coupled with the salt and water retention in the distal tubule results in an increased intravascular volume, edema, and systemic hypertension. Edema appears as facial and periorbital swelling initially, before becoming obvious in the lower extremities. Elevated blood pressure progresses with the kidney damage. Non-specific symptoms are also common and include nausea, malaise, and dizziness.

    Fever and fatigue are the most frequent presentations of rapidly progressive glomerulonephritis. Other symptoms include nausea, anorexia, vomiting, arthralgia, and abdominal pain. Furthermore in these cases, hypertension is usually absent or mild.

    The symptoms of acute post-streptococcal glomerulonephritis include passage of coke-colored or dark brown urine, hypertension, facial puffiness and pedal edema, and proteinuria on urinalysis [11]. Occasionally, the complications of hypertension such as seizures and persistent headaches may be the only presentations of the patient.

    Entire body system
    Congestive Heart Failure
    • heart failure, acute or chronic renal failure, and end-stage renal disease.[medi-info.com]
    • Hypertensive encephalopathy or congestive heart failure Sodium Nitroprusside is the first choice. 31.[slideshare.net]
    • The following conditions can also occur due to GN: acute kidney failure chronic kidney disease electrolyte imbalances, such as high levels of sodium or potassium chronic urinary tract infections congestive heart failure due to retained fluid or fluid[healthline.com]
    • If left untreated, glomerulonephritis can lead to chronic renal failure, end-stage renal disease, high blood pressure, congestive heart failure, pulmonary edema, and an increased risk of other infections, especially urinary tract and kidney infections[chealth.canoe.com]
    Fever
    • Acute glomerulonephritis in dengue hemorrhagic fever: A rare case report.[atmph.org]
    • Crescentic type Similar to PSGN, but the onset is more insidious and weakness, fatigue, and fever are the predominant symptoms IgA neuropathy Similar to PSGN, but the onset usually occurs 1 to 2 days after upper respiratory infection or enteral illness[medi-info.com]
    • (MJA 2011; 195: 150-152) A 10-year-old boy from a remote village in Western Province, Papua New Guinea (PNG), presented to Saibai Island Primary Health Centre in the northern Torres Strait, Queensland, with a 4-year history of intermittent malaise, fevers[mja.com.au]
    • Among the signs and symptoms of acute proliferative glomerulonephritis are the following: Hematuria: Oliguria Edema Hypertension Fever, headache, malaise, anorexia, nausea.[en.wikipedia.org]
    • In fact, fever is sometimes the only sign. learn more hematuria (blood in urine) Common causes of blood in the urine include urinary tract infections (UTIs), kidney stones, irritation of the urethra, injuries, and vigorous exercise.[childrensdayton.org]
    Malaise
    • (MJA 2011; 195: 150-152) A 10-year-old boy from a remote village in Western Province, Papua New Guinea (PNG), presented to Saibai Island Primary Health Centre in the northern Torres Strait, Queensland, with a 4-year history of intermittent malaise, fevers[mja.com.au]
    • Among the signs and symptoms of acute proliferative glomerulonephritis are the following: Hematuria: Oliguria Edema Hypertension Fever, headache, malaise, anorexia, nausea.[en.wikipedia.org]
    • […] that may occur include: Blurred vision , usually from burst blood vessels in the retina of the eye Cough containing mucus or pink, frothy material from fluid buildup in the lungs Shortness of breath, from fluid buildup in the lungs General ill feeling (malaise[nlm.nih.gov]
    • Nonspecific symptoms include malaise, lethargy, abdominal or flank pain, and fever. 15.[slideshare.net]
    Pallor
    • Signs and Symptoms The signs and symptoms of this disorder is fever, lethargy, irritability, fatigue (from anemia), hematuria ( blood in the urine), proteinuria (protein in the urine), scanty urine output, headache, high blood pressure, pallor, abdominal[yourkidshealth.net]
    • Other signs Pharyngitis Impetigo Respiratory infection Pulmonary hemorrhage Heart murmur may indicate endocarditis Scarlet fever Weight gain Abdominal pain Anorexia Back pain Skin pallor Palpable purpura in patients with Henoch-Schönlein purpura Oral[sites.google.com]
    Rapidly Progressive Glomerulonephritis
    • Rapidly progressive glomerulonephritis For rapidly progressive glomerulonephritis, drugs to suppress the immune system are started promptly.[merckmanuals.com]
    • Fever and fatigue are the most frequent presentations of rapidly progressive glomerulonephritis.[symptoma.com]
    • People with rapidly progressive glomerulonephritis may present with a nephritic syndrome .[en.wikipedia.org]
    • This is called rapidly progressive glomerulonephritis.[mountsinai.org]
    • “Pure” mesangial proliferative GN [2] Idiopathic rapidly progressive glomerulonephritis - This form of GN is characterized by the presence of glomerular crescents.[emedicine.medscape.com]
    Weight Gain
    • Symptoms PSGN Onset of symptoms occurs 1 to 6 weeks after infection; symptoms include hypertension, headache, edema, oliguria, dark urine, reduced urine output, flank pain, weight gain, fever, chills, nausea, and vomiting; about half of cases are asymptomatic[medi-info.com]
    • Despite corticosteroids being effective, in many patients high doses for prolonged periods can have significant side affects including: weight gain, hyperglycemia, destruction of bones, easy bruising, and thinning of the skin.[nephrocor.com]
    • During your physical examination, your doctor will measure your blood pressure, weigh you to check for weight gain resulting from water retention, and check for swelling in your legs or elsewhere.[drugs.com]
    • Although effective, in many patients, at high doses, for long periods of time, these agents have significant side affects including : weight gain, hyperglycemia, and agitation, destruction of bones, easy bruising, and thinning of the skin.[hopkinsmedicine.org]
    • Other signs Pharyngitis Impetigo Respiratory infection Pulmonary hemorrhage Heart murmur may indicate endocarditis Scarlet fever Weight gain Abdominal pain Anorexia Back pain Skin pallor Palpable purpura in patients with Henoch-Schönlein purpura Oral[sites.google.com]
  • more...
  • Face, Head & Neck
    Facial Edema
    • Facial edema, or swelling, is common which tends to be more prominent in the morning.[yourkidshealth.net]
    • Most often, the patient is a boy, aged 2-14 years, who suddenly develops puffiness of the eyelids and facial edema in the setting of a poststreptococcal infection.[sites.google.com]
  • more...
  • neurologic
    Headache
    • […] or other symptoms of high blood pressure In severe cases, symptoms of kidney failure, including fatigue; seizures; nausea and vomiting; loss of appetite; overall itching; headache; easy bruising; frequent hiccups or bleeding; and impaired vision Increased[healthcommunities.com]
    • Among the signs and symptoms of acute proliferative glomerulonephritis are the following: Hematuria: Oliguria Edema Hypertension Fever, headache, malaise, anorexia, nausea.[en.wikipedia.org]
    • If severely elevated blood pressure develops, some people will have headaches (although most people with high blood pressure have no symptoms and most headaches are unrelated to blood pressure).[drugs.com]
    • […] burst blood vessels in the retina of the eye Cough containing mucus or pink, frothy material from fluid buildup in the lungs Shortness of breath, from fluid buildup in the lungs General ill feeling (malaise), drowsiness , confusion , aches and pains, headache[nlm.nih.gov]
    Papilledema
    • Other signs and symptoms were fever 19%, arthralgia 4%, epistaxis 6% and bilateral papilledema 1% (Figure 3).[jpma.org.pk]
  • more...
  • gastrointestinal
    Abdominal Pain
    • pain Chronic Glomerulonephritis Prevention There is no known way to prevent chronic glomerulonephritis.[healthcommunities.com]
    • Symptoms include: Swollen feet and legs Puffy face and eyes Decrease in urine production Bubbly, cloudy or bloody urine Nausea Shortness of breath Weakness Fatigue Fever Hypertension Loss of appetite Abdominal pain Headaches Visual disturbances Seizures[davita.com]
    • Some symptoms include: blood or excess protein in your urine, which may be microscopic and show up in urine tests high blood pressure swelling in your ankles and face frequent nighttime urination bubbly or foamy urine, from excess protein abdominal pain[healthline.com]
    • pain Blood in the vomit or stools Cough and shortness of breath Diarrhea Excessive urination Fever General ill feeling , fatigue, and loss of appetite Joint or muscle aches Nosebleed The symptoms of chronic kidney disease may develop over time.[mountsinai.org]
    • Nonspecific symptoms include weakness, fever, abdominal pain, and malaise.[sites.google.com]
    Loss of Appetite
    • […] of appetite; overall itching; headache; easy bruising; frequent hiccups or bleeding; and impaired vision Increased pigmentation of the skin Abdominal pain Chronic Glomerulonephritis Prevention There is no known way to prevent chronic glomerulonephritis[healthcommunities.com]
    • Symptoms include: Swollen feet and legs Puffy face and eyes Decrease in urine production Bubbly, cloudy or bloody urine Nausea Shortness of breath Weakness Fatigue Fever Hypertension Loss of appetite Abdominal pain Headaches Visual disturbances Seizures[davita.com]
    • When kidney failure is severe or near end stage, symptoms include: confusion decreased urination fatigue headache high blood pressure itchy skin loss of appetite muscle twitching or cramping nausea and vomiting nosebleeds puffy eyes, hands, and feet ([chealth.canoe.com]
    • […] of appetite and vomiting.[yourkidshealth.net]
    • […] of appetite Joint or muscle aches Nosebleed The symptoms of chronic kidney disease may develop over time.[mountsinai.org]
    Nausea
    • Among the signs and symptoms of acute proliferative glomerulonephritis are the following: Hematuria: Oliguria Edema Hypertension Fever, headache, malaise, anorexia, nausea.[en.wikipedia.org]
    • Loss of appetite, nausea, vomiting, abdominal pain, and joint pain are also common.[merckmanuals.com]
    • Non-specific symptoms are also common and include nausea, malaise, and dizziness.[symptoma.com]
    • […] legs or ankles or other parts of the body, due to fluid accumulation (edema) Shortness of breath during exertion due to anemia Headache or other symptoms of high blood pressure In severe cases, symptoms of kidney failure, including fatigue; seizures; nausea[healthcommunities.com]
    • Symptoms PSGN Onset of symptoms occurs 1 to 6 weeks after infection; symptoms include hypertension, headache, edema, oliguria, dark urine, reduced urine output, flank pain, weight gain, fever, chills, nausea, and vomiting; about half of cases are asymptomatic[medi-info.com]
    Vomiting
    • […] ankles or other parts of the body, due to fluid accumulation (edema) Shortness of breath during exertion due to anemia Headache or other symptoms of high blood pressure In severe cases, symptoms of kidney failure, including fatigue; seizures; nausea and vomiting[healthcommunities.com]
    • Symptoms PSGN Onset of symptoms occurs 1 to 6 weeks after infection; symptoms include hypertension, headache, edema, oliguria, dark urine, reduced urine output, flank pain, weight gain, fever, chills, nausea, and vomiting; about half of cases are asymptomatic[medi-info.com]
    • Glomerulonephritis commonly affects children causing fever, headache, loss of appetite, vomiting and puffiness of the face and body (oedema).[medical-dictionary.thefreedictionary.com]
    • Individuals with more severe cases of the disease may exhibit: fatigue nausea and vomiting shortness of breath disturbed vision high blood pressure swelling, especially noted in the face, hands, feet, and ankles blood and protein in the urine, resulting[encyclopedia.com]
  • more...
  • musculoskeletal
    Back Pain
    • […] pathology Learn about this topic in these articles: description Acute glomerulonephritis is characterized by severe inflammation, renal (kidney) insufficiency, swelling, increased blood pressure, and severe back pain.[britannica.com]
    • pain and hematuria followed by oliguria or anuria.[emedicine.medscape.com]
    • Hippocrates originally described the manifestation of back pain and hematuria, which lead to oliguria or anuria.[sites.google.com]
  • more...
  • cardiovascular
  • more...
  • urogenital
    Dark Urine
    • If symptoms appear, they can include swelling around the feet, ankles, lower legs, and eyes, reduced urination and dark urine (due to the presence of red blood cells in the urine).[drugs.com]
    • Symptoms PSGN Onset of symptoms occurs 1 to 6 weeks after infection; symptoms include hypertension, headache, edema, oliguria, dark urine, reduced urine output, flank pain, weight gain, fever, chills, nausea, and vomiting; about half of cases are asymptomatic[medi-info.com]
    • However, those who come down with symptoms may present with swelling of the face and legs, reduced urine volume, passage of coke-colored or dark urine, urine frothiness and a high blood pressure.[symptoma.com]
    • […] fluid (ECF) Tenderness over the costo-vertebral angle ( kidney punch), due to swelling of kidneys ‡ ‡ ‡ ASSESSMENT AND DIAGNOSTICS ‡ ‡ ‡ ‡ ‡ ‡ ‡ ‡ ‡ ‡ ‡ History taking; 1-3 weeks post-streptococcal infection (1-2 weeks post-pharyngitis) Urinalysis; dark[scribd.com]
    Hematuria
    • Hematuria : Gross hematuria (30-50%), microscopic hematuria are more common.[slideshare.net]
    • One of Cohen and Levitt's cases had no hematuria and essentially no proteinuria.[annals.org]
    • Diagnostic Methods urine examination hematuria , mild to moderate proteinuria, concentrated urine (there is oliguria!)[wikilectures.eu]
    • The gross hematuria of AGN is virtually always painless; dysuria accompanying gross hematuria points to acute hemorrhagic cystitis [ 5 ] rather than renal disease.[hindawi.com]
    • Microscopic hematuria is not the norm, but may occur in up to 25% of patients, and macroscopic hematuria is decidedly rare.[clevelandclinicmeded.com]
    Macroscopic Hematuria
    • Macroscopic hematuria typically has a rusty or tea-color.[slideshare.net]
    • Microscopic hematuria is not the norm, but may occur in up to 25% of patients, and macroscopic hematuria is decidedly rare.[clevelandclinicmeded.com]
    Oliguria
    • Diagnostic Symptoms edema (75% of patients); gross hematuria (65%) - tea colored or cola colored urine; hypertension (50%); acute renal insufficiency with oliguria. consequences of hypertension, oliguria and renal insufficiency can be" heart failure;[wikilectures.eu]
    • Oliguria and anuria : transient oliguria.[slideshare.net]
    • Type: Term Definitions 1. glomerulonephritis that frequently occurs as a late complication of pharyngitis or skin infection, due to a nephritogenic strain of β-hemolytic streptococci, characterized by abrupt onset of hematuria, edema of the face, oliguria[medilexicon.com]
    • Definition Acute glomerulonephritis (AGN) is a clinical complex characterized by the presence of hematuria or red blood cell (RBC) casts accompanied by at least two of the following clinical findings: edema (periorbital in most of the cases), azotemia, oliguria[link.springer.com]
    • Acute postinfectious (poststreptococcal) diffuse proliferative glomerulonephritis is a primary glomerulonephritis, which produces nephritic syndrome (hematuria, oliguria, uremia, hypertension and mild proteinuria).[pathologyatlas.ro]
  • more...
  • Eyes
  • more...
  • Workup

    No symptom or sign is pathognomonic of AGN. Laboratory tests are, therefore, necessary to confirm a suspected case of AGN.

    Urinalysis is necessary and may reveal red blood cell and white blood cell casts. Red blood cell casts are indicative of glomerular damage while the white cell casts are indicative of infection or inflammation of the nephron. Urinalysis also reveals proteinuria. Blood investigations necessary for diagnosis are tests for creatinine and urea levels.

    Imaging studies such as abdominal plain radiographs, ultrasonography, and computed tomography (CT) scans may be necessary. However, a kidney biopsy is the definitive diagnostic modality to confirm AGN [12].

    Pathology

    Biopsy
  • more...
  • Laboratory

    Serum
  • more...
  • Imaging

    X-ray
  • more...
  • Treatment

    The management of AGN involves treatment of the underlying trigger, supportive care, and conservative management of the complications of the disease.

    Diuretics are necessary to resolve the edema and antihypertensives are equally vital [13]. Furthermore, dietary modifications should consist of low protein diet and salt restriction. Antibiotics are administered in cases of infectious AGN, however, antibiotics may be ineffective since the nephritis occurs 1 to 6 weeks after the inciting infection. Typically, the infection resolves before the onset of the nephritis, however, if the infection still persists, antibiotics can be administered.

    Corticosteroids are administered in cases of AGN triggered by autoimmune disease and in rapidly progressive glomerulonephritis. High dose corticosteroids are administered intravenously for up to 7 days after which they are switched to oral forms. Immunosuppressants may also be beneficial for these patients [14]. Plasmapheresis may also be a beneficial procedure to clear off antibodies from the blood. 

    Generally, prompt diagnosis and treatment prevents progression of the disease to ESRD. Renal transplantation is the definitive treatment for ESRD; however, rapidly progressive glomerulonephritis may reappear in the transplanted kidney.

    Prognosis

    Post-streptococcal glomerulonephritis and IgA nephropathy have a minimal risk of progressing to chronic kidney disease if the underlying pathology is treated well in time. Complete resolution of symptoms is observed in most patients, especially children.

    For other glomerular pathologies, the outcome is generally better if the patient presents with asymptomatic hematuria open link and proteinuria and in those with focal instead of diffuse glomerular changes on renal biopsy. Patients with diffuse glomerular involvement usually have a poorer prognosis.

    Generally, poor prognostic factors include severe proteinuria, severe renal dysfunction, unresponsiveness to treatment, and extensive fibrotic changes in the glomeruli such as glomerulosclerosis.

    If patients having membranoproliferative disease remain untreated, 50-60% of them will lead to ESRD in a period of 10-15 years. The situation in case of Wegener granulomatosis is different because about 20-25% are at risk of leading to ESRD [9] [10]. 

    Complications

    Acute Kidney Failure
    • kidney failure or chronic kidney disease may develop.[nlm.nih.gov]
    • The following conditions can also occur due to GN: acute kidney failure chronic kidney disease electrolyte imbalances, such as high levels of sodium or potassium chronic urinary tract infections congestive heart failure due to retained fluid or fluid[healthline.com]
    • A complication that can result from glomerulonephritis is acute kidney failure and chronic kidney disease.[yourkidshealth.net]
    • Possible complications of glomerulonephritis include: Acute kidney failure.[mayoclinic.org]
    Edema
    • […] volume, edema, and systemic hypertension.[symptoma.com]
    • Edema (90%): typically presents in the face and upper extremities.[slideshare.net]
    • These complexes trigger many inflammatory mediators, such as complement, white blood cells, and blood clotting proteins, which cause damage to kidney tissue Actions that cause tissue injury to kidneys damage to cell membranes, local edema, movement of[quizlet.com]
    • Circulatory congestion and pulmonary edema The patient often presents with only mild edema.[sites.google.com]
    • Finally, it leads to proligerative glomerulonephritis with decreased glomelural filtration, higher natrium resorption in tubules ( edema), increased renin secretion ( hypertension).[wikilectures.eu]
    Glomerulonephritis
    • Acute proliferative glomerulonephritis is a disorder of the glomeruli (glomerulonephritis), or small blood vessels in the kidneys.[en.wikipedia.org]
    • This finding links NAPlr with the pathogenesis of post-streptococcal glomerulonephritis.[symptoma.com]
    • See Acute glomerulonephritis , Capsular 'drops. ', Casts, Chronic glomerulonephritis, Heymann glomerulonephritis, Membranoproliferative glomerulonephritis type 1 and 2, Membranous glomerulonephritis, Mesangial proliferative glomerulonephritis, Crescents[medical-dictionary.thefreedictionary.com]
    • (diagnosis) , glomerulonephritis , Nephritis-glomerular , Glomerulonephritis NOS , glomerulonephritis NOS , Glomerulonephritis [Disease/Finding] , glomerulonephritides , Unspecified glomerulonephritis NOS (disorder) , GN - Glomerulonephritis , Glomerulonephritis[fpnotebook.com]
    • Chronic glomerulonephritis Often, chronic glomerulonephritis seems to result from some of the same conditions that cause acute glomerulonephritis, such as IgA nephropathy or membranoproliferative glomerulonephritis.[merckmanuals.com]
    Guillain-Barré Syndrome
    • Miscellaneous noninfectious causes of acute GN include the following: Guillain-Barré syndrome Irradiation of Wilms tumor Diphtheria-pertussis-tetanus (DPT) vaccine Serum sickness Epidermal growth factor receptor activation, [ 7 ] and possibly its inhibition[emedicine.medscape.com]
    • Differential Diagnoses Amyloidosis, Familial Renal Pediatrics, Fever Angioedema Pediatrics, Pharyngitis Ascites Pharyngitis Cirrhosis Renal Failure, Acute Diabetes Mellitus, Type 2 Rheumatic Fever Guillain-Barré Syndrome Scarlet Fever Heart Failure Serum[sites.google.com]
    Hypertension
    • Asympatimatic type With urine changes, but no edema or hypertension .[slideshare.net]
    • Hypertensive encephalopathy Severe hypertension associated with signs of cerebral dysfunction is a hypertensive emergency requiring immediate aggressive treatment.[sites.google.com]
    • Diagnostic Symptoms edema (75% of patients); gross hematuria (65%) - tea colored or cola colored urine; hypertension (50%); acute renal insufficiency with oliguria. consequences of hypertension, oliguria and renal insufficiency can be" heart failure;[wikilectures.eu]
    • A low-sodium diet may be needed when hypertension is present.[en.wikipedia.org]
    Hypertensive Encephalopathy
    • Drug treatments are usually effective, and very few individuals die as a result of pulmonary edema, brain inflammation (hypertensive encephalopathy ), or uncontrolled infection.[mdguidelines.com]
    • Hypertensive encephalopathy and pulmonary congestion are treated with parenteral drugs 8 .[jpma.org.pk]
    • Hypertensive encephalopathy or congestive heart failure Sodium Nitroprusside is the first choice. 31.[slideshare.net]
    • Indeed, hypertensive encephalopathy may be the presenting complaint in some children with AGN [ 1 ].[hindawi.com]
    Hypervolemia
    • Acute renal failure Management of hypertension, hypervolemia, electrolytes disorder and metabolic acidosis.[slideshare.net]
    Malignant Hypertension
    • Diabetes mellitus Malignant hypertension (high blood pressure) Amyloidosis Inherited diseases like Alport’s syndrome.[healthhype.com]
    • Headache may occur secondary to hypertension; confusion secondary to malignant hypertension may be seen in as many as 5% of patients.[sites.google.com]
    Proteinuria
    Renal Vasculitis
    Rheumatic Fever
    • Antibodies to PA-Ag are found in 30 of 31 patients with APSGN but are low or absent in those with uncomplicated streptococcal infection or in patients with rheumatic fever.[emedicine.medscape.com]
    • Differential Diagnoses Amyloidosis, Familial Renal Pediatrics, Fever Angioedema Pediatrics, Pharyngitis Ascites Pharyngitis Cirrhosis Renal Failure, Acute Diabetes Mellitus, Type 2 Rheumatic Fever Guillain-Barré Syndrome Scarlet Fever Heart Failure Serum[sites.google.com]
    Scarlet Fever
    • Scarlet fever, and impetigo 5.[proprofs.com]
    • In children, it is most often associated with an upper respiratory infection, tonsillitis, or scarlet fever.[encyclopedia.com]
    • Sixty-eight chil­dren had clinical evidence of acute pharyngitis, 12 had an ASO titer of 500 Todd units without a history of a “sore throat”, 7 gave a history of Scarlet fever and 3 had impetigo.[jpma.org.pk]
    • In children, it is most often associated with an upper respiratory infection, tonsillitis , or scarlet fever .[medical-dictionary.thefreedictionary.com]
    • Other signs Pharyngitis Impetigo Respiratory infection Pulmonary hemorrhage Heart murmur may indicate endocarditis Scarlet fever Weight gain Abdominal pain Anorexia Back pain Skin pallor Palpable purpura in patients with Henoch-Schönlein purpura Oral[sites.google.com]
    Systemic Amyloidosis
  • more...
  • Etiology

    The etiological factors responsible for AGN may be classified into infectious and non-infectious causes. The commonest infections responsible for AGN are streptococcal infections, particularly strep pharyngitis.

    Noninfectious causes of AGN are mostly autoimmune diseases and include systemic lupus erythematosus (SLE), goodpasture's syndrome, amyloidosis, Wegener granulomatosis, and polyarteritis nodosa [3].

    Prolonged and heavy use of nonsteroidal anti-inflammatory drugs (NSAIDs) have also been suggested as a risk factor for AGN.

    Epidemiology

    AGN accounts for 10-15% cases of glomerular diseases in the United States. However, the incidence rates have been variable, partially because of the asymptomatic nature of this condition in more than 50% of patients. Generally, statistics reveal a decline in the incidence of post-streptococcal glomerulonephritis in the United States. This decline is attributable to improved healthcare delivery and socioeconomic conditions.

    In the United States, AGN is responsible for 25-30% of cases of ESRD. In about 25% of cases, the disease progresses to an acute nephritic syndrome. Acute nephritic syndrome, in turn, progresses to end-stage renal failure after several weeks or months.

    Worldwide, the commonest cause of AGN is Berger disease or IgA nephropathy. Post-streptococcal glomerulonephritis open link is most commonly seen in Africa, the Caribbean, India, South America, Malaysia, and Pakistan.

    In Nigeria's Port Harcourt city, the incidence of AGN in children between the ages of 3 to 16 years was 15.5 cases per year. Furthermore, the incidence of the disease was higher among males than females with a ratio of 1.1:1. However, currently the difference in incidence among males and females is not so significant [4].

    Generally, there are more marked variations in geographic and seasonal incidence rates of post-streptococcal glomerulonephritis following pharyngeal infections than those which follow streptococcal skin infections [4] [5].

    Sex distribution
    Age distribution

    Pathophysiology

    AGN presents with both morphological and functional changes in the kidneys. There is cellular proliferation in the glomerular tufts as a result of the excess proliferation of the mesangial, epithelial, and endothelial cells triggered by the immune-complex deposition [6]. The cellular proliferation may be confined to the glomerular capillary, in what's described as endocapillary proliferation. Alternatively, it may occur in the Bowman's capsule beyond the glomerular cells and it is referred to as extra capillary proliferation. Accompanying this cellular proliferation is neutrophilic and monocytic infiltration of the glomerular capillary lumen.

    On light microscopy, the glomerular capillary walls appear thickened. A study using electron microscopy clearly shows the basement membrane as thickened due to the deposits of electron-dense particles on the subendothelial, mesangial, and subepithelial parts of the basement membrane. The structural changes in the kidneys may be focal, segmental, diffuse, or global. Morphologically the kidneys appear grossly enlarged as a result of swelling effect in the glomerular tufts.

    The main feature of AGN is the deposition or formation of immune complexes in the glomerulus. The deposition of complement factors and immunoglobulins is demonstrable by immunofluorescence.

    The triggers for the formation of immune complexes in the glomerulus are largely unclear, except in cases of post-streptococcal glomerulonephritis. In post-streptococcal glomerulonephritis, a streptococcal neuraminidase alters the host immunoglobulin G (IgG) which combines with the host antibodies forming IgG/anti-IgG complexes which subsequently deposit in the glomeruli. Furthermore, the presence of high titers of antistreptolysin O or antihyaluronidase, streptokinase, and DNAase-B strongly suggest a preceding streptococcal infection. Specific antibodies may combine with these antigens to form immune complexes.

    Nephritis-associated plasmin receptor (NAPlr) has been described as a glyceraldehyde-3-phosphate dehydrogenase which functions a plasminogen receptor. NAPlr deposition was demonstrated in the initial stages of post-streptococcal glomerulonephritis in immunofluorescence staining of renal biopsy specimens with anti-NAPlr antibodies. This finding links NAPlr with the pathogenesis of post-streptococcal glomerulonephritis [7].

    A study conducted among adults revealed that the two commonest infectious triggers of AGN were streptococci and staphylococci, occurring in 27.9% and 24.4% of cases, respectively [8]. In infections of groups A, C, and G streptococcus associated with glomerulonephritis, titers of antibodies against nephritis-associated protease (NAPR) are significantly elevated. Anti-NAPR antibodies persist for several years and have been suggested to help prevent further episodes of post-streptococcal glomerulonephritis.

    Prevention

    Prevention of AGN may include routine urinalysis done for patients with autoimmune diseases such as SLE.

    Summary

    Acute glomerulonephritis (AGN) is inflammation of the glomeruli which occurs as a result of autoimmune response to an infection or immune complex depositions occurring in autoimmune pathologies. AGN may also affect the various parts of glomerulus such as mesangium, the capillary endothelium and basement membrane.

    Generally, the causes of AGN may be infectious or autoimmune diseases. AGN often occurs sequel to a streptococcal infection open link, usually strep throat or strep cutaneous infections [1]. This post-streptococcal glomerulonephritis is often commonly seen in children between 2 and 10 years. Noninfectious causes of AGN include autoimmune diseases such as IgA nephropathy, systemic lupus erythematosus, cryoglobulinemia, goodpasture syndrome, and Wegener granulomatosis [2]. AGN due to these autoimmune diseases is most likely associated with rapidly progressive glomerulonephritis.

    The characteristic features of AGN are the formation and deposition of immune complexes in the glomerular tufts, accompanied by polymorphonuclear cellular infiltrates. Functionally, AGN results in hematuria, proteinuria, facial or pedal edema, and systemic hypertension. Nonspecific symptoms are also common in this disease.

    Disease prognosis becomes poor due to severe proteinuria, hematuria, and renal damage on presentation, unresponsiveness to appropriate therapy, and the presence of extensive renal fibrosis or sclerosis on biopsy. The main complication of AGN is the end-stage renal disease (ESRD).

    Renal biopsy is the main diagnostic tool of choice to confirm AGN. However, other investigations including bloodwork, urinalysis, and imaging studies provide details to corroborate the diagnosis.

    Management of AGN comprises of symptomatic control and treatment of the underlying disorder. Antibiotics and corticosteroids are administered for infectious and autoimmune-associated AGN, respectively. Additionally, diuretics are necessary to relieve the edema. 

    Patient Information

    Overview

    The kidney is an organ of waste excretion in the form of urine. However, the process of waste excretion is very complex involving several small structures within each kidney. The first stage of urine production is the filtration of the waste products from the blood. Some useful products are also filtered, but are later reabsorbed into the blood. The units which filter the blood are called the glomeruli (singular: glomerulus).

    Acute glomerulonephritis (AGN) is an inflammation and consequent destruction of the glomeruli and adjacent tissues. This, in turn, would lead to reduced urine production as well as salt and water retention in the body.

    Etiology

    AGN may be caused by infections or certain noninfectious diseases called autoimmune diseases in which the body's antibodies attack certain normal tissues in the body confusing them for foreign harmful substances. The commonest infectious causes of AGN are infections caused by the bacterium streptococcus, which are mostly are responsible for sore throat and skin infections. The common autoimmune diseases which cause AGN includes systemic lupus erythematosus (SLE), Wegener granulomatosis, and Berger disease.

    Pathophysiology

    AGN develops when antibodies formed in the body against certain components of the bacterium or infectious agents bind with these agents and deposit in the glomeruli causing destruction of the glomeruli and surrounding tissues.

    Presentation

    More than half of the patients with this disease may not show any symptoms. However, those who come down with symptoms may present with swelling of the face and legs, reduced urine volume, passage of coke-colored or dark urine, urine frothiness and a high blood pressure.

    The facial and leg swelling results from the kidneys' inability to dilute the urine with water from the blood and so there is an accumulation of excess salt and water in the certain regions of the body such as the feet and face. This is also responsible for the elevated blood pressure in these patients. The dark color of the urine is due to the presence of the blood in it, and the frothiness of the urine is as a result of leakage of small proteins through the damaged glomerular filter into the urine. 

    There are other non-specific symptoms of this disease which include fever, weakness, dizziness, and abdominal aches.

    Diagnosis

    A diagnosis of AGN is made by way of investigations which include blood tests and urinalysis: which involves examining a sample of the patient's urine and analyzing it's contents. Results of urinalysis in AGN usually show presence of white and red blood cells which indicate infective damage or inflammation of the kidney tubules and damage to the glomeruli, respectively.

    The main diagnostic tool for confirming the diagnosis of AGN is kidney biopsy. This procedure involves using a big needle to take a sample of the kidney through the patient's trunk, then analyzing the sample under a microscope.

    Treatment

    Prompt diagnosis and treatment of AGN is necessary to avoid the major complication which is called end-stage renal disease which indicates that the kidney is too damaged to function at all.

    Treatment of AGN involves treating the predisposing condition and correcting the symptoms. A class of drugs called diuretics are prescribed to remove excess fluids from the sites where they accumulate. Drugs which lower blood pressure are also necessary. If an infection is determined as the trigger of AGN, antibiotics are administered and for autoimmune-related cases, drugs called corticosteroids such as prednisolone are administered.

    In cases of end-stage renal disease, kidney transplant is considered.

    Self-assessment

    References

    1. Tejani A, Ingulli E. Poststreptococcal glomerulonephritis: current clinical and pathological concepts. Nephron. 1990; 55:1-5.
    2. Emancipator SN. IgA nephropathy: morphological expression and pathogenesis. Am J Kidney Dis. 1994; 23:451-462.
    3. Kalluri R, Wilson CV, Weber M, et al. Identification of the α-3 chain of type IV collagen as the common autoantigen in anti-glomerular basement membrane disease and Goodpasture’s syndrome. J Am Soc Nephrol. 1995; 6:1178-1184.
    4. Anochie I, Eke F, Okpere A. Childhood acute glomerulonephritis in Port Harcourt, Rivers State, Nigeria. Niger J Med. 2009; 18(2):162-167.
    5. Wong W, Morris MC, Zwi J. Outcome of severe acute post-streptococcal glomerulonephritis in New Zealand children. Pediatr Nephrol. 2009; 24(5):1021-1026.
    6. Wen YK, Chen ML. The significance of atypical morphology in the changes of spectrum of postinfectious glomerulonephritis. Clin Nephrol. 2010; 73(3):173-179. 
    7. Oda T, Yoshizawa N, Yamakami K, et al. The role of nephritis-associated plasmin receptor (NAPlr) in glomerulonephritis associated with streptococcal infection. J Biomed Biotechnol. 2012; 2012:417675.
    8. Nasr SH, Markowitz GS, Stokes MB, et al. Acute postinfectious glomerulonephritis in the modern era: experience with 86 adults and review of the literature. Medicine (Baltimore). 2008; 87(1):21-32.
    9. Donadio JV Jr, Offord KP. Reassessment of treatment results in membranoproliferative glomerulonephritis, with emphasis on life-table analysis. Am J Kidney Dis. 1989; 14:445-451.
    10. Andrassy K, Erb A, Koderisch J, et al. Wegener's granulomatosis with renal involvement: patient survival and correlations between initial renal function, renal histology, therapy and renal outcome. Clin Nephrol. 1991; 35:139-147.
    11. Sad RA, Said SM. Hypertension in Jordanian children: a retrospective analysis of 70 cases Pediatr. Nephrol. 1990; 4:520-522.
    12. Nebuloni M, Barbiano di Belgiojoso G, Genderini A, et al. Glomerular lesions in HIV-positive patients: a 20-year biopsy experience from Northern Italy. Clin Nephrol. 2009; 72(1):38-45.
    13. Tapaneyaand O. AGN in children: a prospective study. J Med Assoc Thai. 1989; 72(suppl):35-38.
    14. Wong W, Morris MC, Zwi J. Outcome of severe acute post-streptococcal glomerulonephritis in New Zealand children. Pediatr Nephrol. 2009; 24(5):1021-1026.

    • Acute eosinophilic interstitial nephritis and renal failure with bone marrow-lymph node granulomas and anterior uveitis: a new syndrome - RS Dobrin, RL Vernier, AJ Fish - The American journal of medicine, 1975 - Elsevier
    • A case of fever and unexplained acute renal failure - V Sakhuja, R Agarwal, N Kalra - Indian journal of nephrology, 2008 - ncbi.nlm.nih.gov
    • Acute glomerulonephritis associated with ECHO virus type 9 infection - AM Yuceoglu, S Berkovich, S Minkowitz - The Journal of pediatrics, 1966 - Elsevier
    • -NUCLEASE B AND DIPHOSPHOPYRIDINE NUCLEOTIDASE ANTIBODY TESTS IN ACUTE RHEUMATIC FEVER AND ACUTE GLOMERULONEPHRITIS - EM Ayoub, LW Wannamaker - Pediatrics, 1962 - Am Acad Pediatrics
    • Abnormal Urinalysis: Hematuria and Proteinuria - AE Bobrowski - childrensmemorial.org
    • Acute glomerulonephritis after human parvovirus B19 infection - T Nakazawa, N Tomosugi, K Sakamoto - American Journal of , 2000 - Elsevier
    • Energy metabolism in acute and chronic renal failure. - B Schneeweiss, W Graninger - The American journal , 1990 - Am Soc Nutrition
    • The Presence of Vascular Hypertension: Report submitted to the American Ophthalmological Society by the committee on Classification of Hypertensive Disease of the - HP Wagener, GE Clay, JF Gipner - Transactions of the American , 1947 - ncbi.nlm.nih.gov
    • Acquired antithrombin III deficiency and thrombosis in the nephrotic syndrome - RH Kauffmann, JJ Veltkamp, NH Van Tilburg - The American journal of , 1978 - Elsevier
    • And molecular characterization of two highly pathogenic clones of Streptococcus pyogenes expressing allelic variants of pyrogenic exotoxin A (scarlet fever toxin) - JM Musser, V Kapur, S Kanjilal, U Shah - Journal of Infectious , 1993 - jid.oxfordjournals.org
    • Acute glomerulonephritis with few urinary abnormalities - JA Cohen, MF Levitt - New England Journal of Medicine, 1963 - Mass Medical Soc
    • Acute glomerulonephritis in infancy - TN Fison - Archives of disease in childhood, 1956 - adc.bmj.com
    • Acute treatment of hypercalcemia with furosemide - WN Suki, JJ Yium, M Von Minden - England Journal of , 1970 - Mass Medical Soc
    • ANTIBODY LOCALIZATION OF ANTIGEN-ANTIBODY COMPLEXES IN GLOMERULAR STRUCTURES OF PATIENTS WITH ACUTE GLOMERULONEPHRITIS - GA Andres, L Accinni, KC Hsu, JB Zabriskie - The Journal of , 1966 - jem.rupress.org
    • Active observation in management of acute abdominal pain in childhood. - PF Jones - British medical journal, 1976 - bmj.com
    • ACUTE LUPUS ERYTHEMATOSUS DISSEMINATUS: A REPORT OF A CASE IN A MALE WITH ASSOCIATED ATYPICAL VERRUCOUS ENDOCARDITIS (LIBMAN- - SG BLOUNT JR, JT BARRETT - Annals of Internal Medicine, 1945 - Am Coll Physicians
    • Acute non-calculous cholecystitis in children: Report of a case in a seventeen day old infant - LA Arnspiger, JG Martin, HO Krempin - The American Journal of Surgery, 1960 - Elsevier
    • Acute renal failure and tubular necrosis associated with hematuria due to glomerulonephritis - P Kincaid-Smith, WM Bennett, JP Dowling, GB Ryan - Clin Nephrol, 1983 - ukpmc.ac.uk
    • Acute interstitial nephritis and anterior uveitis - KJ Acker - Pediatric Nephrology, 1994 - Springer
    • ANTIBODY LOCALIZATION OF ANTIGEN-ANTIBODY COMPLEXES IN GLOMERULAR STRUCTURES OF PATIENTS WITH ACUTE GLOMERULONEPHRITIS - GA Andres, L Accinni, KC Hsu, JB Zabriskie - The Journal of , 1966 - jem.rupress.org
    • Patients with hemorrhagic nephritis. II. Observations on patients suffering from streptococcal infections, rheumatic fever and acute and chronic hemorrhagic nephritis - WT Longcope - Journal of Clinical Investigation, 1936 - ncbi.nlm.nih.gov
    • An immunopathologic study of rapidly progressive glomerulonephritis in the adult - EJ Lewis, T Cavallo, JT Harrington, RS Cotran - Human pathology, 1971 - Elsevier
    • Acute poststreptococcal glomerulonephritis presenting as hypertensive encephalopathy with minimal urinary abnormalities - JR Hoyer, AF Michael, AJ Fish, RA Good - Pediatrics, 1967 - Am Acad Pediatrics
    • ANTIBODY LOCALIZATION OF ANTIGEN-ANTIBODY COMPLEXES IN GLOMERULAR STRUCTURES OF PATIENTS WITH ACUTE GLOMERULONEPHRITIS - GA Andres, L Accinni, KC Hsu, JB Zabriskie - The Journal of , 1966 - jem.rupress.org
    • -NUCLEASE B AND DIPHOSPHOPYRIDINE NUCLEOTIDASE ANTIBODY TESTS IN ACUTE RHEUMATIC FEVER AND ACUTE GLOMERULONEPHRITIS - EM Ayoub, LW Wannamaker - Pediatrics, 1962 - Am Acad Pediatrics
    • ANTIBODY LOCALIZATION OF ANTIGEN-ANTIBODY COMPLEXES IN GLOMERULAR STRUCTURES OF PATIENTS WITH ACUTE GLOMERULONEPHRITIS - GA Andres, L Accinni, KC Hsu, JB Zabriskie - The Journal of , 1966 - jem.rupress.org
    • Cardiac complications of acute hemorrhagic nephritis - MI RUBIN, M Rapoport - Archives of Pediatrics & Adolescent , 1938 - Am Med Assoc
    • Acute glomerulonephritis in middle-aged and elderly patients. - HA Lee, G Stirling, P Sharpstone - British medical journal, 1966 - bmj.com
    • Decision analysis as the basis for computer-aided management of acute renal failure - GA Gorry, JP Kassirer, A Essig, WB Schwartz - The American journal of , 1973 - Elsevier
    • Acute and subacute glomerulonephritis modified by adrenocorticotropin. - EB Farnsworth - Proceedings of the Society for Experimental , 1950 - ebm.rsmjournals.com
    • Glomerular deposition of properdin in acute and chronic glomerulonephritis with hypocomplementemia - NG Westberg, GB Naff, JT Boyer - Journal of Clinical , 1971 - ncbi.nlm.nih.gov
    • In Vivo: Mac-1 Deficiency Abrogates Sustained Fcγ Receptor–dependent Neutrophil Adhesion and Complement-dependent Proteinuria in Acute Glomerulonephritis - T Tang, A Rosenkranz, KJM Assmann - The Journal of , 1997 - jem.rupress.org
    • Acute glomerulonephritis without abnormality of the urine - MS Albert, JM Leeming, PR Scaglione - The Journal of Pediatrics, 1966 - Elsevier
    • Acute anuric glomerulonephritis - GM Berlyne, SBC Baker - QJM, 1964 - Oxford Univ Press
    • Acute diffuse interstitial nephritis: review of the literature and case report - ML Simenhoff, WR Guild, GJ Dammin - The American journal of medicine, 1968 - Elsevier
    • Acute oliguric renal failure due to accelerated (malignant) hypertension - LH Sevitt, DJ Evans, OM Wrong - QJM, 1971 - Oxford Univ Press
    • A case of systemic lupus erythematosus presenting as Guillain-Barré syndrome - KRAY CHAUDHURI, IK Taylor, RM Niven - , 1989 - Br Soc Rheumatology
    • Adult hemolytic-uremic syndrome - L Morel-Maroger - Kidney int, 1980 - nature.com
    • Analytical Pathology: III. New Observations on the Pathogenesis of Glomerulonephritis, Lipid Nephrosis, Periarteritis Nodosa, and Secondary Amyloidosis in Man* - RC Mellors, LG Ortega - The American journal of pathology, 1956 - ncbi.nlm.nih.gov
    • Strains of rats: Brown Norway rats treated with d-penicillamine develop autoantibodies, circulating immune complexes, and disseminated intravascular coagulation - AJ Donker, RC Venuto, AO Vladutiu - Clinical immunology and , 1984 - Elsevier
    • Acute Syphilitic Nephrosis: Case Report - CJ McDONALD, AW BARILE - Archives of Internal Medicine, 1963 - Am Med Assoc
    • -NUCLEASE B AND DIPHOSPHOPYRIDINE NUCLEOTIDASE ANTIBODY TESTS IN ACUTE RHEUMATIC FEVER AND ACUTE GLOMERULONEPHRITIS - EM Ayoub, LW Wannamaker - Pediatrics, 1962 - Am Acad Pediatrics
    • C3b receptor in normal kidney and in seventy-five cases of renal diseases: loss of C3b receptor antigen in focal hyalinosis and in proliferative nephritis of systemic - MD Kazatchkine, DT Fearon, MD Appay - Journal of Clinical , 1982 - ncbi.nlm.nih.gov
    • Acquired antithrombin III deficiency and thrombosis in the nephrotic syndrome - RH Kauffmann, JJ Veltkamp, NH Van Tilburg - The American journal of , 1978 - Elsevier
    • -NUCLEASE B AND DIPHOSPHOPYRIDINE NUCLEOTIDASE ANTIBODY TESTS IN ACUTE RHEUMATIC FEVER AND ACUTE GLOMERULONEPHRITIS - EM Ayoub, LW Wannamaker - Pediatrics, 1962 - Am Acad Pediatrics
    • ANTIBODY LOCALIZATION OF ANTIGEN-ANTIBODY COMPLEXES IN GLOMERULAR STRUCTURES OF PATIENTS WITH ACUTE GLOMERULONEPHRITIS - GA Andres, L Accinni, KC Hsu, JB Zabriskie - The Journal of , 1966 - jem.rupress.org
    • ANTIBODY LOCALIZATION OF ANTIGEN-ANTIBODY COMPLEXES IN GLOMERULAR STRUCTURES OF PATIENTS WITH ACUTE GLOMERULONEPHRITIS - GA Andres, L Accinni, KC Hsu, JB Zabriskie - The Journal of , 1966 - jem.rupress.org
    • -NUCLEASE B AND DIPHOSPHOPYRIDINE NUCLEOTIDASE ANTIBODY TESTS IN ACUTE RHEUMATIC FEVER AND ACUTE GLOMERULONEPHRITIS - EM Ayoub, LW Wannamaker - Pediatrics, 1962 - Am Acad Pediatrics
    • Acute glomerulonephritis - JA James, CW Fink - Clinical Pediatrics, 1964 - cpj.sagepub.com

    Media References

    1. Post-infectious glomerulonephritis - very high mag, CC BY-SA 3.0

    Languages

    Self-assessment