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

Area of Science:

• Nephrology
• Rheumatology
• Internal Medicine

Background:

• Scleroderma Renal Crisis (SRC) presents with acute hypertension, hemolytic anemia (HA), and acute kidney injury (AKI).
• SRC is often the initial manifestation of scleroderma and can be misdiagnosed as malignant hypertension (MHT).
• Timely differentiation of SRC from other hypertensive emergencies is critical for patient prognosis.

Purpose of the Study:

• To highlight the diagnostic challenges in distinguishing Scleroderma Renal Crisis (SRC) from malignant hypertension (MHT).
• To present two clinical cases illustrating the complexities in diagnosing SRC.
• To emphasize the importance of early diagnostic markers and prompt treatment for SRC.

Main Methods:

• Presentation of two distinct clinical cases of patients with severe hypertension and acute kidney injury.
• Diagnostic workup included urine dipstick tests for hematuria, autoimmune marker screening (e.g., anti-nuclear antibodies), and renal biopsy.
• Treatment involved high-dose angiotensin-converting enzyme inhibitors (ACEi), such as ramipril.

Main Results:

• Case 1: Initial MHT diagnosis revised to SRC upon detecting hematuria, leading to successful treatment with ramipril and sustained kidney function recovery.
• Case 2: SRC confirmed after initial MHT management failure, with subsequent partial kidney function recovery following ramipril reintroduction.
• Both cases demonstrate that early identification of hematuria and autoimmune markers aids in expediting SRC diagnosis amidst MHT suspicion.

Conclusions:

• Early detection of hematuria and autoimmune markers is crucial for differentiating SRC from MHT.
• Immediate initiation of high-dose ACE inhibitors (ACEi) is recommended upon suspicion of SRC, even before biopsy confirmation.
• Prompt intervention with ACEi is essential for optimizing kidney outcomes and achieving effective blood pressure control in SRC patients.