Introduction:
Systemic sclerosis (scleroderma) is a rare autoimmune disorder characterized by skin thickening and internal organ fibrosis. Scleroderma renal crisis (SRC) is an acute and severe complication leading to malignant hypertension and renal failure. This case report highlights the rare presentation of SRC in a middle-aged male patient.
Methods:
CASE REPORT
Results:
A 54-year-old male, a government employee, presented with generalized fatigue, weight loss, and decreased appetite over the past month. He reported frothy urine and nocturia but denied hematuria, oliguria, recurrent urinary tract infections, or history of stone disease. He had systemic hypertension for past 2 years but was not on regular medication. Physical examination revealed pallor, mild pitting pedal edema, and elevated blood pressure (220/110 mmHg). All peripheral pulsations were intact with no renal bruit.
Investigations showed mild anemia (Hb 10.1 g/dL), renal failure (RFT 196/13.3), and hypokalemia (3.2 mmol/L). Urine microscopy for active sediments was unremarkable, with a urine protein-to-creatinine ratio of 1.82.
Ultrasound of the kidneys displayed normal-sized kidneys with partially maintained corticomedullary differentiation. Dermatological examination identified distinctive skin pigmentation around the neck and lower back, indicative of systemic sclerosis with strong positivity for anti-ScI-70 antibodies.
Renal biopsy revealed features of thrombotic microangiopathy. The patient was initiated on hemodialysis and antihypertensive treatment, including an ACE inhibitor. Currently, he is on maintenance hemodialysis twice weekly and is being evaluated for renal transplantation.
Conclusions:
This case exemplifies the presentation of scleroderma renal crisis(SRC) with malignant hypertension and renal failure.
Prompt recognition and management of SRC are crucial to improve patient outcomes. The association with thrombotic microangiopathy underscores the complexity of SRC and highlights the need for multidisciplinary care in such rare, life- threatening conditions.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.