Nephritic Syndrome Flashcards
… syndrome is classically described as the presence of haematuria, variable proteinuria, renal impairment, and hypertension.
Nephritic syndrome is classically described as the presence of haematuria, variable proteinuria, renal impairment, and hypertension.
The nephritic syndrome describes a very classic presentation of glomerular disease that is characterised by
Haematuria
Variable proteinuria (may be nephrotic range)
Renal impairment
Hypertension
The cause of nephritic syndrome is commonly differentiated based on the underlying mechanism.
Glomerular inflammation is the hallmark of nephritic syndrome. This may be due to inflammation of the small vessels within the capillary tuft (i.e. vasculitis), immune complex deposition or formation of anti-glomerular basement membrane (GBM) autoantibodies.
Immune complex deposition
NEPHRITIC SYNDROME
Systemic lupus erythematosus (i.e. lupus nephritis)
Post-streptococcal glomerulonephritis (PSGN)
IgA Nephropathy
Cyroglobulinaemia
ANCA-associated vasculitis is an umbrella term for three conditions:
Microscopic polyangiitis (MPA)
Granulomatosis with polyangiitis (GPA): previously known as Wegener’s granulomatosis
Eosinophilic granulomatosis with polyangiitis (EGPA): previously known as Churg-Strauss syndrome
Small vessel vasculitis
This is characterised by very limited or no immune deposition in the glomeruli. Instead, patients usually have circulating autoantibodies known an anti-neutrophil cytoplasmic antibody (ANCA). These autoantibodies target self-antigens leading to a vasculitis (inflammation of arterioles, capillaries & venules) that may be limited to the kidneys or multisystem.
ANCA-associated vasculitis is an umbrella term for three conditions:
Microscopic polyangiitis (MPA)
Granulomatosis with polyangiitis (GPA): previously known as Wegener’s granulomatosis
Eosinophilic granulomatosis with polyangiitis (EGPA): previously known as Churg-Strauss syndrome
Anti-GBM autoantibodies
Anti-GBM disease is a rare small-vessel vasculitis that results from the formation of GBM antibodies that target type IV collagen within the basement membrane. This results in linear deposition of IgG in the glomerular capillaries. Due to different pathogenesis, this condition is discussed differently to the ANCA-associated vasculitides.
The clinical presentation of glomerulonephritis can be highly variable.
Nephritic syndrome is a distinct clinical syndrome characterised by haematuria, variable proteinuria, renal impairment, and hypertension. However, this represents a single presentation that forms part of a spectrum of severity in patients with glomerulonephritis.
Presentation of glomerulonephritis is highly variable and may include:
Minimal haematuria and proteinuria
Acute self-limiting disease
Acute severe disease: severe renal impairment usually develops without treatment
Rapidly progressive glomerulonephritis: deterioration in renal function over days to weeks to a few months
Chronic kidney disease: slowly progressive deterioration in renal function due to inflammation
glomerulonephritis - symptoms
Lethargy
Recent infection: fever, sore throat, coryzal symptoms. Typical of poststreptococcal glomerulonephritis and IgA nephropathy
Haematuria
Oliguria: reduce urine output
Oedema: peripheral or periorbital
Shortness of breath: due to fluid overload
Haemoptysis: due to pulmonary haemorrhage (e.g. Anti-GBM, ANCA-vasculitis)
Signs -glomerulonephritis
Haematuria Hypertension Oedema: peripheral, periorbital Fluid overload: raised JVP, bibasal crackles on auscultation, peripheral oedema, ascites Reduce urine output
Extra-renal manifestations - glomerulonephritis
This refers to clinical features reflecting involvement of other organs systems. It is typically seen in patients with systemic causes (e.g. vasculitis, systemic lupus erythematosus, anti-GBM disease).
Skin: vasculitic rash (palpable purpura)
ENT: rhinosinusitis, nasal discharge, polychondritis (inflammation of cartilage)
Eyes: red, painful eyes (e.g. conjunctivitis, scleritis, uveitis)
Lungs: haemoptysis, pleuritic pain, wheeze
Heart: chest pain due to pericardial or myocardial involvement
Nervous system: mononeuropathies
The diagnosis of nephritic syndrome is based on identification of…
Look for features supportive of haematuria of glomerular origin:
Dysmorphic red blood cells: red cell structure appears abnormal under the microscope
Red blood cell casts: identification of red bloods cells within urinary casts seen under the microscope. A cast is a microscopic cluster of particles wrapped in uromodulin (Tamm-Horsfall protein). Uromodulin is secreted by epithelial cells lining the loop of Henle, distal tubule & collecting duct.
Look for features supportive of haematuria of glomerular origin:
Dysmorphic red blood cells: red cell structure appears abnormal under the microscope
Red blood cell casts: identification of red bloods cells within urinary casts seen under the microscope. A cast is a microscopic cluster of particles wrapped in uromodulin (Tamm-Horsfall protein). Uromodulin is secreted by epithelial cells lining the loop of Henle, distal tubule & collecting duct.
Any patient with haematuria, proteinuria and declining renal function or evidence of an underlying disorder (e.g. new mononeuropathy or vasculitic rash) requires urgent assessment for suspected … In these patients a full renal screen should be requested and the majority of patients will need to undergo renal biopsy to confirm the underlying diagnosis.
Any patient with haematuria, proteinuria and declining renal function or evidence of an underlying disorder (e.g. new mononeuropathy or vasculitic rash) requires urgent assessment for suspected glomerulonephritis. In these patients a full renal screen should be requested and the majority of patients will need to undergo renal biopsy to confirm the underlying diagnosis.
