Malignant hypertension, and if it was scleroderma? Lessons from two cases

Inroduction : Scleroderma Renal Crisis (SRC) is characterised by acute hypertension, haemolytic anaemia (HA), and acute kidney injury (AKI). Often presenting as the first manifestation of scleroderma, it is frequently mistaken for malignant hypertension (MHT). Rapid recognition and differentiation of SRC from other hypertensive emergencies are essential for improving patient outcomes.We present two clinical cases that illustrate the diagnostic challenges of SRC in the context of MHT.

Case 1: A 53-year-old man presented with severe hypertension (238/127 mmHg) and AKI (creatinine 390 μmol/L). He was diagnosed MHT due to the presence of grade III hypertensive retinopathy and HA. . However, a urine dipstick test detected haematuria, leading to further immune testing and, a renal biopsy, which confirmed SRC. Treatment with high-dose ramipril led to a sustained recovery of kidney function, 221 μmol/L after five years).

Case 2: A 52-year-old man presented with chest pain, severe hypertension (253/132 mmHg), and AKI (creatinine 183 μmol/L). Initially managed as MHT, his kidney function worsened, prompting further investigation, which revealed haematuria and positive anti-nuclear antibodies. A renal biopsy confirmed SRC. High-dose ramipril was reintroduced, leading to partial kidney function recovery (creatinine 218 μmol/L after five years).

Key findings : These cases underscore the importance of early detection of hematuria and autoimmune markers to expedite diagnosis of SRC in case of MHT. When SRC is suspected, high-dose angiotensin-converting enzyme inhibitors (ACEi) should be initiated immediately, even before biopsy confirmation and continued despite initial kidney function decline. Early intervention is crucial for optimising kidney outcomes and achieving effective blood pressure control.