From the Guidelines
For scleroderma crisis management, immediate hospitalization and aggressive treatment with ACE inhibitors are essential to control blood pressure and protect organ function, particularly the kidneys, as recommended by the most recent guidelines 1. The primary focus should be on controlling blood pressure and protecting organ function, particularly the kidneys. Key steps in management:
- Admit to ICU for close monitoring.
- Aggressive blood pressure control:
- Use ACE inhibitors as first-line treatment (e.g., captopril 25mg orally every 8 hours, titrated as needed) as recommended by the 2023 EULAR update 1.
- Add calcium channel blockers if necessary (e.g., nifedipine 30-60mg extended-release daily).
- Intravenous prostacyclin therapy:
- Epoprostenol: Start at 2 ng/kg/min, increase gradually to maximum tolerated dose.
- Corticosteroids for associated inflammatory conditions:
- Prednisone 1 mg/kg/day orally, tapered over several weeks, but with caution as steroids are associated with a higher risk of scleroderma renal crisis 1.
- Supportive care:
- Fluid and electrolyte management
- Dialysis if severe renal involvement
- Oxygen therapy as needed
- Monitor for and treat complications:
- Pulmonary hypertension
- Cardiac involvement
- Gastrointestinal issues The goal is to rapidly control blood pressure to prevent further organ damage. ACE inhibitors are preferred due to their renoprotective effects, as demonstrated in several cohort studies showing improved survival with their use in patients with scleroderma renal crisis 1. Prostacyclin therapy helps improve blood flow and reduces tissue damage. Close monitoring is crucial as the condition can deteriorate rapidly. Early intervention and aggressive management can significantly improve outcomes and prevent long-term organ damage in scleroderma crisis.
From the FDA Drug Label
Chronic Infusion in PAH/Scleroderma Spectrum of Diseases (SSD) Hemodynamic Effects Chronic continuous infusions of epoprostenol in patients with PAH/SSD were studied in a prospective, open, randomized trial of 12 weeks’ duration comparing epoprostenol plus conventional therapy (N = 56) to conventional therapy alone (N = 55) Conventional therapy varied among patients and included some or all of the following: anticoagulants in essentially all patients, supplemental oxygen and diuretics in two thirds of the patients, oral vasodilators in 40% of the patients, and digoxin in a third of the patients
The management of scleroderma crisis may involve conventional therapy including:
- Anticoagulants
- Supplemental oxygen
- Diuretics
- Oral vasodilators
- Digoxin Additionally, epoprostenol may be used in conjunction with conventional therapy to improve hemodynamic effects and exercise capacity in patients with PAH/SSD 2.
From the Research
Management of Scleroderma Renal Crisis
The management of scleroderma renal crisis (SRC) involves aggressive blood pressure control and treatment of renal failure.
- The mainstay of therapy for SRC is the use of angiotensin-converting enzyme (ACE) inhibitors, such as captopril 3 and enalapril 4, to control malignant hypertension.
- Additional antihypertensive drugs, such as calcium-channel blockers, diuretics, and alpha-blockers, may be used if blood pressure control remains suboptimal despite maximum tolerated doses of ACE-inhibitors 5.
- Dialysis is frequently indicated, but can be stopped in about half of patients, mainly those with good blood pressure control 6, 7.
- Renal transplantation is a treatment option for patients who require long-term dialysis, with a 5-year patient survival rate of about 82% 5.
- Recent case-series and case reports have suggested the use of C5-inhibitors and endothelin receptor antagonists in the therapy of SRC, mainly in refractory cases 5.
- Plasma-exchange may give some benefits in patients with SRC and microangiopathy or intolerant to ACE-inhibitors 5.
Treatment Outcomes
The prognosis of SRC has improved with the introduction of ACE inhibitors, but the 5-year survival for systemic sclerosis patients with full SRC remains low (65%) 6.
- Long-term outcomes of SRC can be favorable, with 61% of patients having good outcomes, including no dialysis or temporary dialysis 7.
- Patients who initially require dialysis may be able to discontinue it 3 to 18 months later, and survival of patients in the good outcome group is similar to that of patients with diffuse scleroderma who do not have renal crisis 7.