Normotensive scleroderma renal crisis as the presenting symptom of systemic sclerosis sine scleroderma: A case report

J Scleroderma Relat Disord. 2023 Feb;8(1):NP1-NP5. doi: 10.1177/23971983221101296. Epub 2022 Jun 7.

Abstract

Scleroderma renal crisis is a rare but serious complication of systemic sclerosis. It is usually associated with marked hypertension and carries significant risk for morbidity and mortality. Its occurrence prior to the development of skin sclerosis is exceedingly rare. We report a case of a patient who presented with recurrent pericardial effusion and later tested positive for anti-nuclear and anti-topoisomerase antibodies. He later developed normotensive renal crisis as confirmed by kidney biopsy despite complete absence of skin involvement. To our knowledge, this is the first published case of a patient presenting with normotensive renal crisis without any skin involvement.

Keywords: Sine systemic sclerosis; anti-topoisomerase; kidney biopsy; normotension; pericardial effusion; scleroderma renal crisis.

Publication types

  • Case Reports