Nephrotic /nephritic syndrome

1. Nephrotic/nephritic syndrome Hrishi Narayanan

2. 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

3. 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 +++

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

5. Differentials • Emotional stress • Exercise • Fever • UTI • Orthostatic • Seizures • Focal segmental glomerulonephritis • IgA nephropathy (ie Berger's disease) • IgM nephropathy • Membranoproliferative glomerulonephritis • Membranous nephropathy • Minimal change disease • Haemoglobinuria • Multiple myeloma • Myoglobinuria • Pre-eclampsia/eclampsia • Alport's syndrome • Amyloidosis • Sarcoidosis • Drugs (eg non-steroidal anti-inflammatory 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, post-streptococcal infection) • Aminoaciduria • Drugs (eg NSAIDs, antibiotics) • Fanconi's syndrome • Heavy metal ingestion

6. 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

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

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

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

10. Symptoms • Oedema • Foamy urine • Complications

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

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

13. Causes • Post-streptococcal • Primary: • Membranous glomerulonephritis • Rapidly progressive glomerulonephritis • IgA nephropathy (Berger’s disease) • Secondary • HSP • Vasculitis

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

15. Investigations • Urine dip • Urine microscopy • Bloods – FBC, U&E, ESR, complement, auto-antibodies, myeloma screen, ASOT • Renal Ultrasound • Renal biopsy

16. Investigations • Urine dipstick • Urine microscopy • Bloods – FBC, U&E, ESR, complement, auto-antibodies, myeloma screen, ASOT • Renal Ultrasound • Renal biopsy

17. Management Nephrotic syn. Nephritis • Conservative • Salt & fluid restriction • Medical • Diuretics • ACE-inhibitors/ARB • Steroids/Immunosuppression • Surgical • Conservative • Salt & fluid restriction • Medical • Diuretics • Steroids/immunosuppression • Surgical • Dialysis

18. Management Nephrotic syn. Nephritis • Conservative • Salt & fluid restriction • Medical • Diuretics • ACE-inhibitors/ARB • Steroids/Immunosuppression • Surgical • Conservative • Salt & fluid restriction • Medical • Diuretics • Steroids/immunosuppression • Surgical • Dialysis

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

20. 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 C3 low

21. 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 C3 low

22. Scenario • What are your main differential diagnoses for this patient? • How would you investigate this patient? • What would your management plan be for this patient?

23. 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

24. Questions?