Main Disease Association
* Systemic sclerosis
* Scleroderma renal crisis (SRC)

Category
Antibody

When to Order
* Anyone with systemic sclerosis to assess for risk of scleroderma renal crisis

What It Means If Positive
* Specific for systemic sclerosis
* Higher risk for scleroderma renal crisis

What It Means If Negative
* Doesn’t rule out possibility of developing SRC, but patient at potentially lower risk
* Still would avoid high doses of systemic steroids in patients with diagnosis of systemic scleroderma

Of Note
* Use of high dose systemic steroids is a risk factor for development of scleroderma renal crisis.
* Order rna pol 3 to see if pt has predilection for SRC development.
* If positive, avoid prednisone ≧ 15mg/day, given association with SRC.
* Of note: Avoid empiric start of ACEi. ACEi are the drug of choice for TREATMENT of SRC — their empiric use is not recommended.