Variable response to oral angiotensin‐converting‐enzyme blockade in hypertensive scleroderma patients

Abstract

Twelve scleroderma patients with hypertension, seven of whom had malignant hypertension and renal failure or scleroderma renal crisis, were treated with captopril. The first dose lowered mean pressure in all patients by 21.3 mmHg; in 6 patients it relieved encephalopathy . Blood pressure was controlled in all patients.Two of 7 patients with scleroderma renal crisis had improvement in renal function; the 5 patients who did not have malignant hypertension improved or stabilized. Despite good pressure control, however, renal failure developed in 5 patients with scleroderma renal crisis. The data indicated that captopril is effective antihypertensive therapy in scleroderma and, when given early, may prevent renal failure and death.Scleroderma is a disease in which deposition of collagen in skin and visceral organs is associated with

Successful treatment of the crisis of scleroderma with enalapril maleate (MK-421).

“…In some patients with scleroderma, however, progression of renal failure continued and death occurred despite BP control with captopril (Whitman III et al 1982). Though progression of renal failure has not been detected in our case, long term follow-up and further studies on MK-421 efficacy will be awaited with considerable interest.…”

“…The treatment with a new long-acting angiotensin I converting enzyme inhibitor, enalapril maleate (MK-421), normalized the blood pressure and protected the progress of renal impairment without side effects. scleroderma ; enalapril maleate ; renal function ; hypertension ; angiotensin I converting enzyme inhibitor An angiotensin I converting enzyme inhibitor (CEI), captopril (CP), lowered blood pressure (BP) dramatically and produced rapid symptomatic improvement, especially when the treatment was started early enough in the course of scleroderma crisis (SC) (LopezOvejero et al 1979; Whitman III et al 1982). While CP is no doubt a very effective drug, its introduction to general clinical use is hampered by some potentially hazardous side effects ; such as rash, fever, loss of taste, leukopenia ; probably due to the sulfhydryl group in its structure (Heel et al 1980).…”

Scleroderma renal crisis: New insights and developments

“…More reports of the successful use of captopril for the treatment of SRC followed [36][37][38]. Not only did ACE inhibitor treatment halt progression of renal failure in some patients, but it reversed the process with a return of creatinine to baseline levels in some cases [10,36]. An essential point that must be emphasized is that despite elevations in serum creatinine, which is often seen with institution of ACE inhibitors or even initiation of dialysis, it is important to continue treatment with the ACE inhibitor, because improvement in renal function can be delayed up to 2 years.…”

“…This can further contribute to luminal narrowing of the arteries and formation of microthrombi, leading to microangiopathic hemolytic anemia that can be seen as part of the clinical picture of SRC [9,10]. These structural changes are the primary cause of decreased renal perfusion [1••].…”

“…As previously mentioned, newonset anemia is a predictive risk factor for SRC. One of the contributing causes of anemia is microangiopathic hemolytic anemia [9,10], which is seen in 45% of patients with SRC [16].…”