It may be helpful to keep in mind that mean hgb in TTP is ~7, and mean platelets ~10,000, in further modulating degree of suspicion.
4/
We got some additional teaching from @mjventuramd about systemic sclerosis and scleroderma renal crisis.
First, the dichotomous phenotypes limited (historically CREST) vs. diffuse disease.
5/
There are multiple autoantibodies associated with different phenotypes of scleroderma. And even all of them combined are not sufficiently sensitive to rule out the disease.
6/
Scleroderma renal crisis is a feared complication of systemic sclerosis.
It is critical to consider in the ddx of thrombotic microangiopathy (thrombocytopenia, schistocytosis, renal failure) even without known SCL diagnosis.
Hypertension is not always severe or present.
7/7
ACE inhibitors are critical in the treatment of scleroderma renal crisis, and the reason why mortality from SRC has fallen over decades.
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