What is the recommended treatment for hypertension in patients with scleroderma?

From the Guidelines

The recommended treatment for hypertension in patients with scleroderma typically begins with calcium channel blockers (CCBs) or angiotensin-converting enzyme (ACE) inhibitors, with the choice of agent depending on the presence of renal involvement or scleroderma renal crisis. When considering the treatment of hypertension in patients with scleroderma, it's crucial to prioritize agents that not only manage blood pressure but also address other aspects of the disease, such as Raynaud's phenomenon and renal crisis.

• Calcium channel blockers (CCBs) like nifedipine (30-90 mg daily), amlodipine (5-10 mg daily), or diltiazem (120-360 mg daily) are preferred for their ability to manage both hypertension and digital vasculopathy.
• Angiotensin-converting enzyme (ACE) inhibitors, such as enalapril (5-40 mg daily) or lisinopril (10-40 mg daily), are particularly beneficial in patients with concurrent renal involvement, as evidenced by studies showing improved survival rates in patients with scleroderma renal crisis treated with ACE inhibitors 1. For patients with scleroderma renal crisis, ACE inhibitors are the treatment of choice, with captopril (25-100 mg three times daily) often used due to its short half-life, allowing for careful dose titration, as recommended by the update of EULAR recommendations for the treatment of systemic sclerosis 1. Blood pressure goals should generally aim for <130/80 mmHg, though individualization is important, and treatment should be initiated at lower doses and gradually increased to minimize side effects like hypotension. Regular monitoring of renal function, particularly when using ACE inhibitors, is essential, as these patients have a higher risk of renal complications, a consideration highlighted in the EULAR recommendations 1. Combination therapy may be necessary in resistant cases, potentially adding angiotensin receptor blockers or diuretics, though careful monitoring is required due to the increased risk of renal dysfunction.

From the Research

Hypertension Treatment in Scleroderma

• The recommended treatment for hypertension in patients with scleroderma is angiotensin-converting enzyme (ACE) inhibitors 2, 3.
• ACE inhibitors have dramatically improved the survival of patients with scleroderma renal crisis, with a 1-year survival rate of 76% compared to 15% without ACE inhibitors 2.
• However, the use of ACE inhibitors prior to the onset of scleroderma renal crisis may be associated with a higher incidence of renal crisis and poorer prognosis 4, 5.
• Alternative antihypertensive drugs, such as calcium channel blockers (CCB) and angiotensin receptor blockers (ARB), may be considered in certain cases 6, 4.
• The treatment of scleroderma renal crisis relies on aggressive control of blood pressure with ACE inhibitors, and dialysis is frequently indicated 3.
• Patients who need dialysis for more than 2 years may qualify for renal transplantation 3.

Treatment Approach

• First-line therapy for scleroderma renal crisis is an ACE inhibitor 6.
• Second-line therapy may include adding a CCB or ARB, and then an alpha-blocker 6.
• The choice of antihypertensive medication should be individualized based on the patient's specific needs and disease severity 6, 4.

Important Considerations

• Patients with systemic sclerosis who develop hypertension should be treated with an ACE inhibitor, but the use of ACE inhibitors prior to the onset of scleroderma renal crisis should be carefully considered 2, 5.
• The prognosis of scleroderma renal crisis remains low, with a 5-year survival rate of 65% 3.
• Early recognition and treatment of scleroderma renal crisis are crucial to improve patient outcomes 2, 3.