Nephritic Syndrome Flashcards
Clinical presentation of nephritic syndrome.
AKI (GFR drops)
Haematuria (sometimes visible)
Mild to moderate oedema
Proteinuria < 3.5g
Hypertension
Give examples of nephritic syndromes.
Post-streptococcal GN
IgA nephropathy
Small vessel vasculitis (ANCA)
Anti-GBM
Thin Basement Membrane
Alport Syndrome
Lupus Nephritis
Investigation of nephritic syndrome.
Assess damage and potential cause
Bloods - FBC, U&Es, LFT, CRP, Immunoglobulins, Electrophoresis, COmplement, Autoantibodies, blood cultures, ASOT, Hepatitis serology
Urine - MC&S, Bence Jones proteins, RBC casts
Imaging - CXR and renal ultrasound
Renal biopsy
What is post-streptococcal GN caused by?
It occurs 1-2 weeks after a tonsillitis/pharyngitis infection or 3-4 weeks after impetigo/cellulitis.
It is a group A beta-haemolytic streptococcal infection.
Epidemiology of PS G.
Usually affects children ages 3-12 years
Can lead to RPGN
Presentation of PS GN.
Haematuria to acute nephritis with haematuria, oedema, hypertension and oliguria.
Investigations of PS GN.
Bloods
Biopsy
Investigation findings in PS GN.
+ve anti-streptococcal antibodies (ASO titre)
Low serum C3
anti-DNAse
Evidence of streptococcal infection
Immune complex deposition - IgG, IgM and C3 in the mesangium.
Treatment of PS GN.
Usually self-limiting
Treat infection
ACEi/ARB to treat hypertension and proteinuria.
Low sodium diet.
What is the most common GN worldwide?
IgA Nephropathy
Presentation of IgA nephropathy.
Episodic gross haematuria during or directly after URTI or GI infection, or strenous exercise.
Increased BP
Slow and indolent disease that causes renal failure within 30 years of diagnosis in 25-50% of cases.
Epidemiology of IgA Nephropathy.
Males > Females
2nd to 3rd decade of life usually
In workbook it says 25-30% of patients progress to ESRF within 20-25 years.
Worse prognosis in IgA Nephropathy.
Male
High BP
High crea
Proteinuria
Investigation findings in IgA nephropathy.
Asymptomatic microhaematuria with intermittent visible haematuria.
Increased serum IgA
Normal C3 and C4
On biopsy - IgA depositions in the mesangium
Treatment of IgA nephropathy.
Supportive therapy
ACEi/ARBs for proteinuria and hypertension
Corticosteroids might be considered if persistent proteinuria > 1g
Give examples of small vessel vasculites (ANCA +ve)
Granulomatosis with polyangiitis (Wegener’s)
Microscopic polyangiitis
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
Features of Granulomatosis with polyangiitis.
Renal disease leading to rapidly progressive GN with crescent formation, haematuria or proteinuria.
Pulmonary involvment with possible cough, haemoptysis or pleuritis.
Nasal obstruction
Ulcers
Epistaxis
Destruction of nasal septum -> Saddle-nose
Sinusitis
Skin purpura, nodules, peripheral neuropathy, mononeuritis multiplex, arthritis, eyes etc…
Investigation findings in Granulomatosis with polyangiitis.
cANCA (PR3) positive
Urinalysis with possible haematuria and proteinuria.
Biopsy - segmetal necrotising GN
CXR may show nodules and fluffy infiltrates of pulmonary haemorrhage
CT may show diffuse alveolar haemorrhage
Treatment of Granulomatosis with polyangiitis.
Severe disease - corticosteroids and cyclophosphamide or rituximab (this is to induce remission)
As maintenance methotrexate or azathioprine can be used.
Plasma exchange might be needed.
Co-trimoxazole as prophylaxis vs Pneumocystis jirovecii or Staphylococcal.
What is microscopic polyangiitis?
Necrotising vasculitis affecting small and medium sized vessels.
Symptoms of microscopic polyangiitis.
Rapidly progressive GN with pulmonary haemorrhage in 30%
Usually only mild resp symptoms.
Investigation findings of microscopic polyangiitis.
pANCA (MPO) positive
Segmental necrotising GN
Treatment of microscopic polyangiitis.
Steroids and methotrexate.
For maintenance use methotrexate, rituximab or azathioprine.
What is Churg-Strauss syndrome?
A triad of adult-onset asthma, eosinophilia and vasculitis (+/- vasospasm, MI and DVT)
It affects the lungs, nerves, heart and skin.
You might see allergic rhinitis, purpura and peripheral neuropathy as well.
Investigation findings in Churg-Strauss syndrome.
pANCA positive
Eosinophilia
Focal segmental necrotising GN
Treatment of Churg-Strauss syndrome
Steroids
Rituximab if refractory disease
What is anti-GBM disease?
Auto-antibodies to type IV collagen which is present in glomerular and alveolar basement membranes.
The antibody binds to the basement membrane and activates complement and proteases leading to disruption of filtration.
Epidemiology of anti-GBM.
Two peaks.
3rd decade of life -> male > female
60+ -> female > male
Pulmonary features of anti-GBM.
Reacts with pulmonary basement membrane as well causing pulmonary haemorrhage and haemoptysis.
Presentation of anti-GBM.
Oliguria/anuria, haematuria, AKI, renal failure
Pulmonary haemorrhage, dyspnoea, haemoptysis
Investigation findings of anti-GBM.
Anti GBM antibodies
Pulmonary infilrates on CXR
Biopsy showing linear deposition of IgG along basement membrane.
Treatment of anti-GBM.
Plasma exchange to remove circulating anti-GBM antibodies
Corticosteroids to suppress inflammation
Cyclophosphamide to suppress further antibody synthesis.
What is thin basement membrane disease?
Hereditary autosomal dominant disease.
Abnormalities of Type IV collagen
Good prognosis
Ix findings of thin basement membrane disease.
Persistent microscopic haematuria
Possible intermittent visible haematuria
Diffuse thinning of GBM on biopsy.
Treatment of thin basement membrane disease.
No known
Monitor renal function and supportive treatment.
What is Alport syndrome?
X-linked mutation of COL4A5 gene which is supposed to code for type IV collagen.
Features of Alport Syndrome
Haematuria
Proteinuria
Progressive renal insufficiency
Sensorineural hearing loss
Anterior lenticonus (bulging of lens on slit-lamp examination)
Macular and perimacular flecks
Investigation findings of Alport Syndrome.
Persistent microscopic haematuria with intermittent visible haematuria
Sensorineural hearing loss
Splitting of GB and alternating thickening and thinning of GBM on biopsy.
FH
Treatment of Alport Syndrome.
Supportive treatment
Renal replacement therapy
Renal transplant
Complications of renal transplant in Alport Syndrome.
It can cause anti-GBM disease as the graft type IV Collagen is recognised as foreign.
What is lupus nephritis?
Complication of SLE that can be both nephritic or nephrotic.
SLE is a systemic autoimmune disease with antibodies against nuclear components such as dsDNA.
There is deposition of antibody complexes causing inflammation and tissue damage.
Presentation of lupus nephritis.
Rash
Photosensitivity
Ulcers
Arthritis
Serositis
CNS effects
Cytopenia
Renal disease
Investigation findings in lupus nephritis.
ANA and anti-dsDNA positive
Biopsy can show 6 different classes of lupus nephritis with different presentations and slightly varying tx options.
Treatment of lupus nephritis.
Class I and II -> mild changes and low risk of renal disease. ACEi/ARBs for renal protection and hydroxychloroquine for extra-renal disease.
Class III-V require immunosuppression. Mycophenolate, glucocorticoids, cyclophosphamide, rituximab.
Supportive therapy of GN.
ACEi/ARBs for proteinuria
COntrol BP
Salt and water restriction if fluid overloaded
Diuretics
Consider LMWH if hypoalbuminaemic due to high risk of VTE
Statins for hypercholesterolaemia
