An autopsy-proven case of normotensive scleroderma renal crisis with refractory pleuroperitoneal effusion

Scleroderma renal crisis (SRC) is a serious complication of systemic sclerosis that typically presents with hypertension and acute kidney injury (AKI). However, about 10% of cases are normotensive, while refractory pleural or ascitic effusion preceding SRC is rare in clinical practice. Here, we present a case of normotensive SRC preceded by refractory pleural and ascitic effusions.

The patient was a 66-year-old woman with a history of rheumatoid arthritis who was managed as an outpatient. Three months prior to admission, she developed sudden, generalized refractory pleural and ascitic effusions; however, the cause remained unknown and there was no improvement. Subsequently, she developed AKI and was transferred to our hospital. Hemodialysis was initiated, but despite efforts to reduce dry weight, transudative pleural effusion persisted. The patient was positive for anti-SCL-70 antibodies, but had no hypertension, and a kidney biopsy could not be performed. She later developed pneumonia and ultimately died of respiratory failure caused by pneumonia and pleural effusion. An autopsy revealed widespread fibrosis in multiple organs and marked intimal thickening and stenosis of the vasculature, leading to a diagnosis of normotensive SRC.

It is important to consider normotensive SRC as a potential cause of refractory transudative pleural and ascitic effusions resistant to treatment with hemodialysis associated with AKI.