A Race to the Finish

This Clinical Problem-Solving case follows a 58-year-old woman whose constellation of symptoms pleuritic chest pain, progressive neurologic deficits, and autoimmune markers revealed a complex diagnosis involving Sjögren’s syndrome, neuromyelitis optica spectrum disorder, and ultimately a rare entity known as RAS-associated autoimmune leukoproliferative disorder. Initial presentations included myopericarditis and lymphoid interstitial pneumonia, with imaging and serology supporting Sjögren’s syndrome. Neurologic manifestations were consistent with aquaporin-4 IgG-positive neuromyelitis optica. Later bone marrow analysis unveiled a somatic NRAS mutation, linking her autoimmune symptoms to hematopoietic clonality. Treatment combined immunosuppression, plasma exchange, and eculizumab.

This Clinical Problem-Solving case follows a 58-year-old woman whose constellation of symptoms pleuritic chest pain, progressive neurologic deficits, and autoimmune markers revealed a complex diagnosis involving Sjögren’s syndrome, neuromyelitis optica spectrum disorder, and ultimately a rare entity known as RAS-associated autoimmune leukoproliferative disorder. Initial presentations included myopericarditis and lymphoid interstitial pneumonia, with imaging and serology supporting Sjögren’s syndrome. Neurologic manifestations were consistent with aquaporin-4 IgG-positive neuromyelitis optica. Later bone marrow analysis unveiled a somatic NRAS mutation, linking her autoimmune symptoms to hematopoietic clonality. Treatment combined immunosuppression, plasma exchange, and eculizumab.