Case Report: Real NPSLE? A patient with systemic sclerosis overlapping systemic lupus erythematosus presenting as epilepsy

Summary: Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc) with a high mortality rate. Although angiotensin-converting enzyme inhibitor therapy has improved renal outcomes, there are currently no preventative measures and patients may rapidly decline. Studies have identified specific phenotypes at higher risk of developing SRC and novel urinary biomarkers may aid in early identification and treatment. Clear guidelines for management have been established to standardize care and promote collaboration between rheumatology and renal physicians. While outcomes following renal transplant have improved, further research is needed to understand the mechanisms and develop new therapies for SRC.

Summary: Posterior Reversible Encephalopathy Syndrome (PRES) is an acute neurological syndrome characterized by seizures, altered mental status, headache, and visual disturbances. It is often associated with Systemic Lupus Erythematosus (SLE), with SLE causing alterations in pathways implicated in the development of PRES. This suggests that PRES in the setting of SLE may be considered a distinct neuropsychiatric syndrome.