Nephroticnephritic syndrome Hrishi Narayanan Learning Outcomes Understand the

Nephrotic/nephritic syndrome Hrishi Narayanan

Learning Outcomes • Understand the key differences between nephrotic and nephritic syndrome (nephritis) • Describe initial investigations and management of nephrotic and nephritic syndrome (nephritis) • Describe the possible complications of nephrotic syndrome

Scenario • 24 year old man • Feeling more tired recently • No systemic symptoms on questioning • Frothy urine, no urinary symptoms, no blood • No past medical history, no medications • Non-smoker, social alcohol use • Examination is unremarkable • Urine dipstick - protein +++

Scenario 1. What are your main differential diagnoses for this gentleman? 2. What are the features of nephrotic syndrome and nephritic syndrome? 3. How would you investigate this gentleman? 4. What would your management plan be for this gentleman? 5. What are the complications of nephrotic syndrome?

Differentials • • • • Emotional stress Exercise Fever UTI Orthostatic Seizures Focal segmental glomerulonephritis Ig. A nephropathy (ie Berger's disease) Ig. M nephropathy Membranoproliferative glomerulonephritis Membranous nephropathy Minimal change disease Haemoglobinuria Multiple myeloma Myoglobinuria Pre-eclampsia/eclampsia • • • Alport's syndrome Amyloidosis Sarcoidosis Drugs (eg non-steroidal antiinflammatory drugs (NSAIDs), penicillamine, gold, angiotensin-converting enzyme (ACE) inhibitors) Anderson-Fabry disease Sickle cell disease Malignancies (eg lymphoma, solid tumours) Infections (eg HIV, syphilis, hepatitis, poststreptococcal infection) Aminoaciduria Drugs (eg NSAIDs, antibiotics) Fanconi's syndrome Heavy metal ingestion

Differentials • Transient – exercise, stress, UTI • Primary glomerular disease – minimal change, FSGS • Secondary glomerular disease – drugs (e. g. NSAIDS), infx (e. g. HIV/hepatitis), sarcoid • Tubular – drugs • Serum excess – multiple myeloma • Other – pre-eclampsia

Nephrotic syndrome • Kidney disease leading to proteinuria, hypoalbuminaemia, oedema & lipiduria • Proteinuria - >3 g per day • Damage to glomerular basement membrane causes increased permeability • This causes proteinuria → hypoalbuminaemia →oedema

Nephrotic syndrome • Kidney disease leading to proteinuria, hypoalbuminaemia, oedema & hyperlipidaemia • Proteinuria - >3 g per day • Damage to glomerular basement membrane causes increased permeability • This causes proteinuria → hypoalbuminaemia →oedema

Causes Primary Secondary • Minimal change disease • Focal glomerulosclerosis • Membranous nephropathy • Diabetes • SLE • Amyloidosis

Symptoms • Oedema • Foamy urine • Complications

Nephritic syndrome • Collection of findings associated with glomerular inflammation and glomerulonephritis • Features: ▫ ▫ ▫ Haematuria & red cell casts Proteinuria Hypertension Uraemia Oliguria

Nephritic syndrome • Collection of findings associated with glomerular inflammation and glomerulonephritis • Features: ▫ ▫ ▫ Haematuria & red cell casts Proteinuria Hypertension Uraemia Oliguria

Causes 1. Post-streptococcal 2. Primary: ▫ ▫ ▫ Membranous glomerulonephritis Rapidly progressive glomerulonephritis Ig. A nephropathy (Berger’s disease) 3. Secondary ▫ ▫ HSP Vasculitis

Symptoms & signs • • • Classically 2 -3 weeks after URTI Oedema Oliguria Haematuria Flank pain General symptoms

Investigations • Urine dip • Urine microscopy • Bloods – FBC, U&E, ESR, complement, autoantibodies, myeloma screen, ASOT • Renal Ultrasound • Renal biopsy

Investigations • Urine dipstick • Urine microscopy • Bloods – FBC, U&E, ESR, complement, autoantibodies, myeloma screen, ASOT • Renal Ultrasound • Renal biopsy

Management Nephrotic syn. Nephritis • Conservative ▫ Salt & fluid restriction • Medical ▫ Diuretics ▫ ACE-inhibitors/ARB ▫ Steroids/Immunosuppressi on • Surgical • Conservative ▫ Salt & fluid restriction • Medical ▫ Diuretics ▫ Steroids/immunosuppressio n • Surgical • Dialysis

Management Nephrotic syn. Nephritis • Conservative ▫ Salt & fluid restriction • Medical ▫ Diuretics ▫ ACE-inhibitors/ARB ▫ Steroids/Immunosuppressi on • Surgical • Conservative ▫ Salt & fluid restriction • Medical ▫ Diuretics ▫ Steroids/immunosuppressio n • Surgical • Dialysis

Complications of nephrotic syndrome: • Infection • Hyperlipidaemia • Hypocalcaemia • Hypercoagulability • Hypovolaemia

Scenario • A 12 year old male with dark "cola coloured" urine for 2 days • Well until 14 days ago - had a sore throat and fever – resolved without medical input • Now facial puffiness and nonspecific abdominal pain. • Dark brown urine, voiding less, normal smell • Reduced appetite, lethargy, back pain • Normally fit & well, no other symptoms • Examination reveals only mild periorbital oedema Urine dip: blood +++, protein + Urine microscopy - RBCs are too numerous to count, RBC casts FBC & U&E normal, ASO titre high, complement C 3 low

Scenario • A 12 year old male with dark "cola coloured" urine for 2 days • Well until 14 days ago - had a sore throat and fever – resolved without medical input • Now facial puffiness and nonspecific abdominal pain. • Dark brown urine, voiding less, normal smell • Reduced appetite, lethargy, back pain • Normally fit & well, no other symptoms • Examination reveals only mild periorbital oedema • Urine dip: blood +++, protein + • Urine microscopy - RBCs are too numerous to count, RBC casts • FBC & U&E normal, ASO titre high, complement C 3 low

Scenario 1. What are your main differential diagnoses for this patient? 2. How would you investigate this patient? 3. What would your management plan be for this patient?

Key points • Nephrotic syndrome – heavy proteinuria, nephritis (nephritic syndrome) – haematuria • Always do a urine dip for patients with oedema • Important complications include infection and hypoercoagulability

Questions?