Picture this:
A patient presents with a headache, and you find this π on your evaluation.
Why (and how) is this an emergency?
𧡠1/8
A patient presents with a headache, and you find this π on your evaluation.
Why (and how) is this an emergency?
𧡠1/8
To guide our thoughts, here are 2 scleroderma renal crisis (SRC) facts-
1) SRC is a syndrome of kidney injury, elevated blood pressure, +/- hemolysis
2) π Prior to modern therapy, one-year mortality for SRC was 85-100%
𧡠2/8
1) SRC is a syndrome of kidney injury, elevated blood pressure, +/- hemolysis
2) π Prior to modern therapy, one-year mortality for SRC was 85-100%
𧡠2/8
Letβs break down the clinical triad -
1οΈβ£ Kidney injury
- from sclerosis of glomelular arterioles
π decreases renal perfusion
2οΈβ£ Elevated blood pressure
- from decreased perfusion sensed by JGA
π activates RAAS
With 3οΈβ£ hemolysis in mindβ¦
𧡠3/8
1οΈβ£ Kidney injury
- from sclerosis of glomelular arterioles
π decreases renal perfusion
2οΈβ£ Elevated blood pressure
- from decreased perfusion sensed by JGA
π activates RAAS
With 3οΈβ£ hemolysis in mindβ¦
𧡠3/8
π About 50% of SRC cases are complicated by hemolysis due to thrombotic microangiopathy (TMA)
How does scleroderma renal crisis lead to TMA? π€
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How does scleroderma renal crisis lead to TMA? π€
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π Scleroderma (and hypertension) directly causes endothelial disruptionπ
This puts endothelial tissue factor in direct contact with coagulation cascadeβ¦
β¦ leading to TMA π€―
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This puts endothelial tissue factor in direct contact with coagulation cascadeβ¦
β¦ leading to TMA π€―
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For treatment-
π ACE inhibitors increase 5-year overall survival by ~55% π€―
These dilate efferent glomerular arterioles, which then:
- β¬οΈ glomerular pressure
- leading to β¬οΈ RAAS and blood pressure
𧡠6/8
π ACE inhibitors increase 5-year overall survival by ~55% π€―
These dilate efferent glomerular arterioles, which then:
- β¬οΈ glomerular pressure
- leading to β¬οΈ RAAS and blood pressure
𧡠6/8
π Captoprilβs fast onset and short duration allows for faster titration
- Peak effect in 45 minutes
- Duration of action is 6-8 hours
Much faster than other ACE inhibitors π
𧡠7/8
- Peak effect in 45 minutes
- Duration of action is 6-8 hours
Much faster than other ACE inhibitors π
𧡠7/8
Key takeaways:
π€― Prior to ACE inhibitor therapy, one-year mortality for SRC was 85-100%
π ACE inhibitors increase 5-year overall survival by ~55%
π SRC causes hemolysis by endothelial disruption π TMA
𧡠8/end
π€― Prior to ACE inhibitor therapy, one-year mortality for SRC was 85-100%
π ACE inhibitors increase 5-year overall survival by ~55%
π SRC causes hemolysis by endothelial disruption π TMA
𧡠8/end
Thanks for sticking around- I hope we all learned something new!
Thank you to @SophieClaudel and @AshuNephro for the review π
Looking forward to hearing what the experts would add!
@kidney_boy @tony_breu @Bloodman @makhan1390 @waikarss @asupadh @JoshuaPGilman @SSchneiderMD
Thank you to @SophieClaudel and @AshuNephro for the review π
Looking forward to hearing what the experts would add!
@kidney_boy @tony_breu @Bloodman @makhan1390 @waikarss @asupadh @JoshuaPGilman @SSchneiderMD
For a deeper dive, here are the articles below-
pubmed.ncbi.nlm.nih.gov
pubmed.ncbi.nlm.nih.gov
pubmed.ncbi.nlm.nih.gov
pubmed.ncbi.nlm.nih.gov
pubmed.ncbi.nlm.nih.gov
pubmed.ncbi.nlm.nih.gov
and images-
clinicalconnection.hopkinsmedicine.org
imagebank.hematology.org
pubmed.ncbi.nlm.nih.gov
pubmed.ncbi.nlm.nih.gov
pubmed.ncbi.nlm.nih.gov
pubmed.ncbi.nlm.nih.gov
pubmed.ncbi.nlm.nih.gov
pubmed.ncbi.nlm.nih.gov
and images-
clinicalconnection.hopkinsmedicine.org
imagebank.hematology.org
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