A 55-year-old man with diffuse cutaneous systemic sclerosis presents with sudden onset of malignant hypertension (200/120 mmHg), thrombocytopenia, and acute peripheral smear shows schistocytes. Creatinine is 3.8 mg/dL. What is the most likely diagnosis?
- A Scleroderma renal crisis ✓
- B Thrombotic thrombocytopenic purpura
- C Malignant hypertension due to essential hypertension
- D Antiphospholipid syndrome
Correct answer: A. Scleroderma renal crisis
Explanation
Scleroderma renal crisis presents with malignant hypertension, thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury in patients with diffuse cutaneous systemic sclerosis. It is strongly associated with anti-RNA polymerase III antibodies. ACE inhibitors have dramatically improved outcomes. TTP would show severe ADAMTS13 deficiency and neurological symptoms.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.