Medicine
A 33-year-old woman known to have systemic sclerosis with normal kidney function, diagnosed 2 years ago, is…
A 33-year-old woman known to have systemic sclerosis with normal kidney function, diagnosed 2 years ago, is on pantoprazole. She complains of drowsiness and visual changes, denies fever, cough, shortness of breath, chest pain, seizures, abdominal pain, or vomiting. Examination reveals an unwell patient with raised JVP, clear chest, and +3 lower limb edema (see lab result). Which of the following is the most appropriate management?
Explanation
Core Concept: Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis characterized by malignant hypertension, acute kidney injury, and microangiopathic hemolytic anemia.
Clinical Presentation:
- Sudden, severe hypertension leading to hypertensive encephalopathy (drowsiness, visual changes, headache)
- Oliguric acute renal failure and fluid overload (edema, raised JVP)
Diagnosis:
- First-line diagnostic plan: Frequent blood pressure monitoring, serum creatinine, and urinalysis.
- Gold standard: Clinical diagnosis in a patient with systemic sclerosis presenting with new-onset malignant hypertension and acute renal failure.
Management:
- Acute management: Immediate initiation of an ACE inhibitor (e.g., lisinopril or captopril) is the absolute gold standard and life-saving intervention, even if serum creatinine initially worsens.
- Gold standard long-term management: Lifelong ACE inhibitor therapy to maintain blood pressure control and preserve renal function; corticosteroids should be avoided as they can precipitate SRC.