Management of Hypertension in Scleroderma Patients
For patients with scleroderma and hypertension, ACE inhibitors should be the cornerstone of antihypertensive therapy, as they are life-saving in preventing and treating scleroderma renal crisis, which is a medical emergency with historically fatal outcomes. 1, 2
Primary Treatment: ACE Inhibitors
ACE inhibitors (or ARBs if ACE inhibitors are not tolerated) must be used as first-line therapy in all scleroderma patients with hypertension, regardless of whether scleroderma renal crisis is present, as these agents specifically block the renin-angiotensin system that drives the pathophysiology of renal crisis. 1, 2
The introduction of ACE inhibitors has dramatically reduced mortality from scleroderma renal crisis, transforming a previously uniformly fatal complication (with survival of only 1-3 months before 1971) into a potentially reversible condition when diagnosed and treated promptly. 2, 3
Captopril or enalapril are appropriate choices, with typical dosing starting at captopril 6.25-12.5 mg three times daily and titrating up to 50 mg three times daily, or enalapril 2.5-5 mg daily titrating to 10-40 mg daily. 4, 5
Additional Antihypertensive Agents
Once ACE inhibitor therapy is optimized, additional agents should be added in a stepwise fashion if blood pressure remains uncontrolled:
Add a dihydropyridine calcium channel blocker (such as amlodipine 5-10 mg daily) as the second agent, which also has the added benefit of treating Raynaud's phenomenon commonly seen in scleroderma. 1, 6
Add a thiazide or thiazide-like diuretic as the third agent if blood pressure remains above target on ACE inhibitor plus calcium channel blocker. 6, 7
Target blood pressure should be <130/80 mmHg for most patients with scleroderma and hypertension. 6, 7
Critical Monitoring and Warning Signs
Monitor closely for scleroderma renal crisis, which presents with abrupt onset or worsening of hypertension (often severe, >150/90 mmHg), Grade III-IV retinopathy, markedly elevated plasma renin activity (at least twice upper limit of normal), and rapid deterioration of renal function within less than one month. 2, 3
Scleroderma renal crisis can occur even in patients without skin involvement, so maintain high clinical suspicion in any scleroderma patient with new or worsening hypertension. 8
Check renal function (serum creatinine) within 1-2 weeks of starting or adjusting ACE inhibitor therapy, as some patients may experience initial decline in renal function that does not necessarily indicate treatment failure. 4, 9
Important Caveats
Do not use cyclosporine in scleroderma patients, as experts note it may decrease renal function and induce hypertension, potentially precipitating renal crisis. 1
While ACE inhibitors are highly effective at controlling blood pressure in scleroderma renal crisis, rare cases have been reported where renal function continued to deteriorate despite blood pressure normalization, ultimately requiring dialysis. 9 However, this should not deter aggressive ACE inhibitor use, as the overall benefit is well-established. 2
If hypertension remains uncontrolled on three medications (ACE inhibitor + calcium channel blocker + diuretic), add spironolactone 25 mg daily as the fourth agent, monitoring potassium and renal function within 1-2 weeks. 6, 7
Losartan (an ARB) may be specifically beneficial for treating Raynaud's phenomenon in scleroderma patients, reducing frequency and severity of attacks. 1