Systemic Sclerosis Sine Scleroderma

Abstract

Scleroderma and systemic sclerosis are autoimmune phenomena that can cause progressive and permanent damage to the organs of the human body. Rarely, the only manifestation of systemic sclerosis is severe hypertension with renal failure, a subset known as systemic sclerosis sine scleroderma. In this case report, a 39-year-old Hispanic female with a past medical history of GERD and gestational hypertension had presented for nausea and vomiting. She was a Jehovah's witness. Upon review of admission labs and studies from years prior, her chemistries had shown signs of progressive renal failure and proteinuria, but the cause of such abnormalities was never pursued. On admission, she was immediately transferred to the intensive care unit for severe metabolic acidosis requiring emergent hemodialysis, treatment for hypertensive emergency, severe anemia, and electrolyte derangements. Moreover, due to her religion, she declined blood products, so she required erythropoietin and iron products instead. She remained in the intensive care unit for three days for severe metabolic acidosis with a chronic, non-oliguric nephrotic syndrome pattern. An extensive immune panel was positive for the antinuclear antibody, the Scl-70 antibody, and low C3. A renal biopsy was not attempted because the patient's hemoglobin was too unstable at the time. She was briefly placed on captopril, but due to an ongoing dry cough the patient declined to remain on it. The patient has since been receiving tri-weekly hemodialysis treatments, but her progressive renal failure over the years sequentially led to irreversible, chronic kidney disease. This case report demonstrates a rare incidence of systemic sclerosis sine scleroderma in a patient who was positive for the Scl-70 antibody and had multiple contraindications to appropriate treatments. It also highlights the importance of pursuing abnormalities on routine blood studies because it can ultimately prevent irreversible kidney damage and can aid in anticipating further internal organ involvement.