A brief case study.
A 39F presents to ED having been sent in by her GP for an abnormal blood test from her GP. She was there for a routine smear test.
She has an eGFR of 11 with no background health conditions.
It turns out she has had intermittent haemoptysis for 6 months and also intermittent haematuria which would occur every few days. She had not been to see her GP about this.
She had no fevers, no dysuria, no cough and no other infective symptoms or other symptoms suggestive of a UTI.
Whilst taking the history she looked very well.
A further look at her bloods shows an Hb of 79, eGFR 11, significant proteinuria and microscopic haematuria on urine dip.
I should mention at this point that it is a Friday afternoon at 4pm which, despite hospitals being a 24 hour service, does complicate matters.
On discussion with the nephrologist on call we have enough information to make an initial treatment decision. This is a young woman with likely rapidly progressing glomerulonephritis who needs further workup but we need to treat her now.
She was given IV methylpred daily over the weekend, a blood transfusion and monitored with daily reviews. In the meantime we ordered some blood tests to aid in diagnosis.
ANA
ENA
ANCA
dsDNA, C3, C4
anti-GBM
and requested renal biopsy for Monday.
She responded well over the weekend with no further worsening of renal function and the tests started to trickle back with results.
What we found of significance was:
p-ANCA positive
MPO antibodies
ANA speckled pattern
dsDNA negative
Renal biopsy showing no immune deposits.
For the eagle eyed amongst you, you will have noticed that the picture at the start of the article in a p-ANCA slide thus giving part of the answer away! Above is also the speckled ANA pattern found on this patients blood tests. Very beautiful images!
Combining all of this information we are able to conclude that this lady had Microscopic Polyangiitis, one of the pauci immune glomerulonephritides.
The next challenge was treatment choice.
For these patients the choice is between rituximab and cyclophosphamide. You have to consent for these drugs and with cyclophosphamide you must inform them about potential infertility. These were taken in conjunction with ongoing prednisolone.
So to summarise rapidly progressive glomerulonephritis requires swift identification and treatment as it is a life threatening condition. If you see someone with this level of acute renal failure alarm bells should ring and specialist involvement should be sought very quickly.
Happy Learning!
@medicine.daily
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