A 17-year-old man is referred to the local nephrology unit for investigation. He reports having several episodes of visible haematuria. There is no history of abdominal or loin pain. These typically seem to occur within a day or two of developing an upper respiratory tract infection. Urine dipstick is normal. Blood tests show the following:
What is the most likely diagnosis?
Correct Answer C:
IgA nephropathy:
Basics:
Differentiating between IgA nephropathy and post-streptococcal glomerulonephritis:
Presentations:
Associated conditions:
Management:
Prognosis:
A 54-year-old woman with a history membranous glomerulonephritis secondary to systemic lupus erythematous is admitted to hospital. Her previous stable renal function has deteriorated rapidly. The following blood tests were obtained:
What has likely caused the sudden deterioration in renal function?
Correct Answer B: Nephrotic syndrome predisposes to thrombotic episodes, possibly due to loss of antithrombin III. These commonly occur in the renal veins and may be bilateral. Common symptoms include loin pain and hematuria.
A greater rise in the ESR would be expected if the renal failure was due to an exacerbation of SLE.
Nephrotic syndrome complications:
A 25-year-old man has a renal biopsy due to worsening renal function. This reveals linear IgG deposits along the basement membrane.
Correct Answer E:
Goodpasture's syndrome:
These changes are characteristic of Goodpasture's syndrome.
Goodpasture's syndrome: Goodpasture's syndrome is rare condition associated with both pulmonary haemorrhage and rapidly progressive glomerulonephritis. It is caused by anti-glomerular basement membrane (anti-GBM) antibodies against type IV collagen. Goodpasture's syndrome is more common in men (sex ratio 2:1) and has a bimodal age distribution (peaks in 20-30 and 60-70 age bracket). It is associated with HLA DR2.
Features:
Factors which increase likelihood of pulmonary haemorrhage:
Investigations:
A 65-year-old female with a 20 year history of rheumatoid arthritis is referred to the acute medical unit with bilateral leg oedema. The following results are obtained:
Which investigation is most likely to lead to the correct diagnosis?
Correct Answer D: This rather odd question fooled most candidates when it appeared. The chronic inflammatory process (rheumatoid) predisposes to amyloidosis which in turn can cause nephrotic syndrome. Rectal biopsy is an (infrequent) test done to look for amyloidosis. Rheumatoid drugs such as gold may cause nephrotic syndrome but none of the other options point to this as an answer.
Amyloidosis: types
1- AL amyloid:
2- AA amyloid:
3- Familial ATTR Amyloidosis:
4- Wild-type (senile) ATTR amyloidosis:
5- Beta-2 microglobulin amyloidosis:
Which one of the following types of glomerulonephritis is most characteristically associated with partial lipodystrophy?
Correct Answer C: Type 2 mesangiocapillary glomerulonephritis is associated with partial lipodystrophy. Type 1 is seen in association with hepatitis C and cryoglobulinaemia Mesangiocapillary glomerulonephritis is sometimes referred to as membranoproliferative glomerulonephritis.
Mesangiocapillary glomerulonephritis (membranoproliferative):
Glomerulonephritides: Knowing a few key facts is the best way to approach the difficult subject of glomerulonephritis:
Membranous glomerulonephritis:
IgA nephropathy - aka Berger's disease, mesangioproliferative GN:
Diffuse proliferative glomerulonephritis:
Minimal change disease:
Focal segmental glomerulosclerosis:
Rapidly progressive glomerulonephritis - aka crescentic glomerulonephritis:
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