HTN: Scleroderma

Question 1

Scleroderma renal crisis

Answer 1

occurs in 2% with limited disease (skin involvement below elbows and knees only)

Question 2

Scleroderma renal crisis

Answer 2

SRC occurs in ~5% to 10% of diffuse scleroderma (skin involvement below and above elbows and knees)

Question 3

Scleroderma renal crisis

Clincal Manifestations

Answer 3

Typically presents at diagnosis of scleroderma or within 3 to 4 years of disease onset.

Question 4

Scleroderma renal crisis

Clincal Manifestations

Answer 4

Acute onset of moderate to severe “accelerated” HTN and oliguric kidney failure

Question 5

Scleroderma renal crisis

Clincal Manifestations

Answer 5

Accompanying features: hyperrenin, thrombotic microangiopathy, anemia, congestive heart failure, and/or hypertensive encephalopathy and retinopathy

Question 6

Scleroderma renal crisis

Clincal Manifestations

Answer 6

10% of SRC occurs with relative normotension which may reflect low baseline BP or concurrent acute illness with associated fall in BP.

Question 7

Scleroderma renal crisis

Risks

Answer 7

Positive anti-RNA polymerase III antibodies (not anti-Scl70 or anti-U3RNP antibodies)

Question 8

Scleroderma renal crisis

Risks

Answer 8

Early diffuse scleroderma, rapidly progressive skin disease and tendon friction rubs

Question 9

Scleroderma renal crisis

Risks

Answer 9

Corticosteroid exposure

Question 10

Scleroderma renal crisis

Risks

Answer 10

HLA DRB10407, HLA-DRB11304, endothelin B receptor polymorphisms, soluble CD147

Question 11

Scleroderma renal crisis

Factors not associated with SRC

Answer 11

Positive anticentromere antibody
Baseline BP, creatinine, proteinuria, hematuria
Gender

Question 12

Scleroderma renal crisis

Management

Answer 12

ACEI are first-line therapy. ARB are not sufficient as first-line to control BP

Question 13

Scleroderma renal crisis

Management

Answer 13

Goal: reduce SBP/DBP by 20/10 mm Hg per 24 hours.

Question 14

Scleroderma renal crisis

Management

Answer 14

Prophylaxis therapy: None proven effective

Prophylactic ACEI may lead to worse outcome and higher likelihood or dialysis dependency for unclear reasons.

Question 15

Scleroderma renal crisis

Management

Answer 15

ARB are not effective. Reports of patients developing SRC while on ARB

Question 16

Scleroderma renal crisis

Management

Answer 16

Effective and improve survival

Question 17

Scleroderma renal crisis

Management

Answer 17

Patients should be on ≥2 years on dialysis before consideration for transplant due to high recovery rates.

Question 18

Scleroderma renal crisis

Management

Answer 18

Recurrence of SRC < 5% and more common in those with early native kidney loss due to SRC.