NEPHRITIC SYNDROME

PYUZA,MD
 Renal anatomy
• Paired retroperitoneal organs

• Length 6cm neonate and 12cm adult

• Weight 24g neonate and 150g adult

• Each kidney has 1 million nephrons

• Nephron formation complete at birth

 Functions of the Kidney
• Filter blood removing toxins and metabolic
wastes
• Maintain the proper balance between water
and salts, acids and bases
• Production of rennin for BP control
• erythropoietin to stimulate RBC production.
• Activation of vitamin D.
In the cortex
Glomeruli, proximal & distal convoluted tubules
and collecting ducts

In the medulla
• Straight portions of tubules, loops of Henle,
vasa recta, terminal collecting ducts
Renal Blood Supply
 The Glomerulus
• Filtering mechanism of kidney

• Made up of a tuft of capillaries and the

Bowman's capsule

• The capillaries are supplied by an afferent

arteriole and drained by the efferent
Learning Objectives
By the end of this session, a student is expected to learn
the following;
– Introduction and definition of Nephritic syndrome
– Explain aetiology/risk factors of Nephritic syndrome
– Outline epidemiology of Nephritic syndrome
– Explain clinical features of Nephritic syndrome
– Establish provisional and differential diagnosis of
Nephritic syndrome
Learning Objectives cont…
– Provide pre-referral treatment of Nephritic
syndrome
– Provide appropriate supportive care for
Nephritic syndrome
– Provide follow-up services Nephritic
syndrome
 Introduction

Glomerulus – capillary loop with basement membrane
which allows passage of specific molecules into the
nephron

Glomerulonephritis – inflammation/damage of the
glomerular basement membrane resulting in altered
function

Is characterised by inflammation and thinning of the
glomerular basement membrane and the occurrence of
small pores in the podocytes of the glomerulus.
•
Can present as nephrotic and/or nephritic syndrome.
 Introduction cont…
• While the nephrotic syndrome is mediated by damage
to the podocyte (an epithelial cell) and the glomerular
basement membrane (GBM), Nephritic syndrome
occurs as a result of inflammatory damage to the renal
endothelium
• The main causes share intraglomerular inflammation
and include vasculitis, antibody-mediated damage,
and immune complex disease.
• Clinical and Biochemical differences is, Nephritic
syndrome presents with glomerular macroscopic
haematuria/red cell casts. While Nephrotic syndrome
presents with massive proteinuria
 Definition of Nephritic Syndrome
• Consists of a combination of some or all of the
following signs:
– Hematuria with acanthocytes
– RBC casts in urine  
– Proteinuria (< 3.5 g/24h)
– Hypertension
– Mild to moderate edema
– Sterile pyuria
– Azotemia (increased blood Urea and Creatinine)
– Oliguria (low urine output 0.5 to 1 ml/kg/hr)
Note!
• Nephritic syndrome typically presents acutely and in
this case may be called an acute
glomerulonephritis

• If renal function (as measured by the glomerular

filtration rate [GFR]) deteriorates over a period of
days to weeks, a rapidly progressive
glomerulonephritis (RPGN) may be present and is
typically associated with crescents on renal biopsy
• Some nephritic syndromes can smolder over months
to years with no change in the renal function and are
thus referred to as a chronic glomerulonephritis
 Epidemiology of Nephritic Syndrome

• Acute nephritic syndrome (ANS) is uncommon

in developed countries due to improved
hygiene and decreased post-streptococcal and
other post-infection glomerulonephritis.
• However, it is still a common presentation in
developing countries.
• Children between 2 and 12 are most commonly
affected, but it may occur at any age.
• Males are more commonly affected, especially
with the more severe cases.
Risk Factors of Nephritic Syndrome
• Age
• Genetic predisposition
• Infected skin lesions and occurs more
often in the summer
• Streptococcal throat infection during the
winter months
• Bacterial virulence
Aetiology of Nephritic Syndrome
• Primary renal diseases:
– Immunoglobulin A nephropathy;
– Membranoproliferative glomerulonephritis;
– Idiopathic rapidly progressive glomerulonephritis
(crescentic glomerulonephritis)
• Secondary renal diseases:
– Subacute bacterial endocarditis;
– Infected ventriculoperitoneal shunt;
– GN with visceral abscess;
– GN with bacterial, viral or parasitic infections
Aetiology of Nephritic Syndrome cont…

• Multi-system disease:
– Systemic lupus erythematosus (SLE);
– Granulomatosis with polyangiitis (formerly
known as Wegener’s granulomatosis);
– Goodpasture’s syndrome;
– Microscopic polyarteritis;
– Mixed cryoglobulinaemia;
– Henoch-Schonlein purpura;
– Haemolytic uraemic syndrome.
• Allergy:
– Acute allergic tubulointerstitial nephritis
Aetiology of Nephritic Syndrome cont…

• Poststreptococcal glomerulonephritis
– Occurs weeks after group A β-
hemolytic streptococci infections
• Pharyngitis/tonsillitis (commonly 1–2 wks)
• Skin infections: 3–4 wks
– Usually affects  ages group of 3–12 years  
– Can lead to RPGN in some cases
• Hemolytic uremic syndrome (HUS)
• Henoch–Schönlein purpura (HSP)
• Sub Acute Bacterial Infective endocarditis
 Aetiology of Nephritic Syndrome cont…

• IgA nephropathy (Berger's disease)

– commonest idiopathic glomerulonephritis worldwid
e
– episodic gross hematuria during or directly after
upper respiratory tract (URT), gastrointestinal (GI)
infections, or strenuous exercise
– occurrence: ♂ > ♀
– peak incidence: 2nd to 3rd decade of life
– ∼ 25-30% of the patients progress to end-
stage renal disease (ESRD) within 20–25 years
Aetiology of Nephritic Syndrome cont…

• Rapidly progressive glomerulonephritis

(RPGN)
• Thin basement membrane disease
– Hereditary disorder
– Abnormalities of type IV collagen
– Good prognosis
• Lupus nephritis
• Complication of systemic lupus erythematosus
• Can be nephritic or nephrotic
• Can lead to RPGN in some cases
Aetiology of Nephritic Syndrome cont…

• Granulomatosis with polyangiitis (Wegener's)

– Pulmonary and nasopharyngeal involvement is
common → hemoptysis, nasal ulcers

• Microscopic polyangiitis
– Usually only mild respiratory symptoms

• Churg-Strauss syndrome
– Patients present with asthma, allergic rhinitis,
purpura and peripheral neuropathy
Aetiology of Nephritic Syndrome cont…

• Alport syndrome
– X-linked (usually affects males)
– Mutation in gene for type IV collagen
– Often leads to ESRD

• Other infections
– Malaria
– Hepatitis B
– Diphtheria
– Pertussis
Aetiology of Nephritic Syndrome cont…

• Medications – e.g.
– cotrimoxazole,
– NSAIDS,
– thiazide diuretics,
– aspirin
– penicillins
• Miscellaneous
– Guillain-Barré syndrome (GBS)
– radiation of Wilm’s tumor
– tetanus vaccine
– serum sickness
Aetiology of Nephritic Syndrome cont…

• Goodpasture syndrome (Anti–

GBM antibody disease)
– Two peaks of occurrence: 3rd decade of
life (♂ > ♀) and ≥ 60 years of age (♀ > ♂)
– Caused by antibodies against type IV
collagen
– Antibodies can cross-react with basement
membrane of pulmonary capillaries and lead
to pulmonary hemorrhage and hemoptysis
– Can lead to RPGN
Pathogenesis of Nephritic Syndrome
• Inflammation → cytokine release
→ glomerular capillary damage Porous glomerular
basement membrane → leakage
of proteins and RBCs → nephritic sediment (all blood components
are detectable on urinalysis)
– Proteinuria (< 3.5 g/24h): leakage of proteins
– Hematuria: leakage of RBCs, which stick together and form red
blood cell casts in the renal tubules
• Oliguria: inflammatory infiltrates reduce fluid movement across
the membrane (↓ GFR)
• Azotemia: inflammation prevents sufficient filtering and excretion
of urea
• Salt retention  → intravascular volume expansion
→ hypertension and edema
Clinical Features of Nephritic Syndrome
– Flank pain
– General systemic symptoms – fever,
vomiting, weakness/malaise, anorexia
– Glomerular Hematuria (dark /cola/cofee/tea
coloured urine)
– Sterile  pyuria and sometimes WBC casts
– Proteinuria < 3.5g/d (protein in the urine)
Clinical Features of Nephritic Syndrome
cont…
– Hypertension
– Blurred vision
– Azotemia (increased blood Urea and
Creatinine)
– Oliguria (low urine output 0.5 to 1 ml/kg/hr)
– Mild to moderate oedema (puffiness of the
face/periorbital swelling that is more marked
on waking up)
– Post-infectious = 2-3 weeks after strep-
throat/URTI
Complications of Nephritic Syndrome

– Acute renal failure/injury

– Nephrotic syndrome
– Secondary hypertension
– Haemorrhagic anaemia
– Hypovolemic shock
– Cardiac failure
– Pericardial & Pleural effusion
– Chronic kidney disease
– Uraemic encephalopathy (presenting with
convulsions)
 Differential Diagnosis of Nephritic
Syndrome

Cystitis

Severe malaria (Black water fever)

Schistosomiasis/Bilharzia

Haemorrhagic fevers

Wilm’s tumour/Nephroblastoma

Trauma – urenthral/Bladder/Ureter/Kidney
injury

Nephrotic syndrome

Chronic kidney disease
 Investigations for Nephritic Syndrome
– Renal function tests: Blood urea nitrogen,
Serum creatinine and Glomerular Filtration
Rate
– Serum electrolytes: Potassium and Blood pH
– Urinalysis: Dipstick/C/S: RBCs – micro or macro
hematuria, Casts, protein
– Complete blood cell count (CBC)/Full blood
picture – Hb concentration, Blood cells
– Erythrocyte sedimentation rate (just supportive)
– Abdominal ultrasound: Assesses renal size and
Excludes obstruction
 Investigations for Nephritic Syndrome
cont…
– Kidney biopsy for histopathology
– Blood culture and sensitivity
– Antistreptolysin-O titer (ASOT)
– Antinuclear Antibody (ANA) titre,
Antiglomerular basement membrane
antibody, Antineutrophil cytoplasmic antibody
(ANCA), Serum complements - C3 and C4
– Others: HIV test, Hepatitis panel,
Toxicological panel, RBG, mRDT/BS for
MPS, Bleeding incices – PT/INR
Treatment Plans for Nephritic Syndrome


Conservative
 Special diet
 Fluid balance, weight
 Salt and fluid restriction
 Reduction in potassium (K+) and salt,
 Bed rest
 Treat underlying cause
– Eradicate residual streptococcal infection
Penicillin/Erythromycin for 10 days/single
dose of Benzathine penicillin
 Treatment Plans for Nephritic Syndrome
cont…

Medical
– If proteinuria and/or hypertension, give angiotensin-
converting enzyme inhibitors or angiotensin-receptor blockers
– If severe hypertension and/or edema, diuretics should be given
- Furosemide 1 to 2 mg/kg/day, Nifedipine 0.25 mg/kg/day
– Sometimes immunosuppressive therapy  using corticosteroids,
ciclosporin, cyclophosphamide and azathioprine is indicated.
– If severe renal insufficiency or kidney failure: renal replacement
therapy (e.g., Dialysis – hemodialysis or peritoneal dialysis)


Surgical
 Renal transplant
 Follow - up
– Monitor blood pressure and other vital
signs – PR, RR and BT

– Fluid intake and Urine output in 24

hours
– Renal function tests

– Cardiac functions: through ECG, ECHO,

 Prognosis for Nephritic
Syndrome

The prognosis depends on the underlying
cause.

Post-infectious usually self-resolving
(95% recover renal function)

Others are a bit more nasty

Generally, the prognosis of nephritic
syndrome in children is better than it is in
adults.
 Prevention of Nephritic Syndrome
• Prompt systemic antibiotic therapy for streptococcal
throat and skin infections

• Early diagnosis and treatment of nephritic syndrome

• Referral of patients with advanced kidney disease to

the specialized hospital for paediatric nephrologists
review and definitive management
 Key Points
• Nephritic syndrome is characterized by
glomerular damage leading
to hematuria, pyuria, water retention, and
subsequent hypertension and edema.

• It can be caused by a variety of conditions

including autoimmune, hereditary, and
infectious diseases.
 Key Points cont…
• Nephritic diseases can present in varying degrees of
severity, ranging from asymptomatic hematuria to
systemic involvement as in rapidly progressive
glomerulonephritis.

• The urine sediment is typically characterized by red

blood cell (RBC) casts, mild to
moderate proteinuria (< 3.5 g/day), and sterile pyuria.
Diagnosis of the underlying disease is often based on
presentation and laboratory values, although renal
biopsy may be indicated for confirmation.
 Evaluation
1. A child presents with history of passing
brown colored urine for 4days. On
examination was pale, febrile, raised
blood pressure, edema and cost-phrenic
angle tenderness. Describe the
management of this child.
 R
• A Clinic is called to attend a 3-year-old boy
who swallowed 8 paracetamol tablets 2 hours
ago. He now presents with excessive vomiting
and generalized body malaise. On examination
afebrile lethargic, 20 kg body weight, Pulse
rate 82 beats per minute, Respiratory rate 22
breaths per minute and normal systemic
examination findings.
 References

 Nelson Textbook of Paediatrics – 19th Edition

 Text Book of Pathology – 23rd Edition