Aseptic leptomeningitis induced by azathioprine in systemic lupus erythematosus: a rare manifestation

Abstract

Systemic lupus erythematosus (SLE) is an autoimmune systemic disease and these patients can have neurological involvement; however, aseptic leptomeningitis is considered to be a very rare feature, observed in 1.4-2.0% of patients. Here, we described a case of a young male with SLE treated with azathioprine with progressive headache, which revealed diffuse posterior fossa leptomeningitis, relatively sparing the supratentorial compartment, that represent an adverse drug reaction - a rare manifestation of central nervous system involvement in SLE. Treatment with azathioprine was interrupted and methylprednisolone was initiated and the patient has significant improvement of his neurological state in 5 days later, demonstrating total involution of the leptomeningeal enhancement on MRI follow-up.

Figure 1.

(a) Sagittal MRI T ₁-weighted images, (b) axial T2-weighted images, (c) axial T2-FLAIR MRI and axial diffusion-weighted images (DWI), (d) show no abnormal findings. Sagittal (e and f), axial, (g) and coronal, (h) T1-weighted images post-contrast depict diffuse and bilateral leptomeningeal enhancement in posterior fossa between cerebellar folia (arrows) and in occipital lobes, less extensively (arrowhead, e).

Figure 2.

(a) Sagittal MR T1-weighted images, (b) axial T2-weighted images, (c) axial DWI, (d) axial susceptibility-weighted images (SWI) and (e) axial T2-FLAIR MRI continued demonstrating no abnormalities. (f, g and h) Axial T ₁-weighted images post-contrast show complete resolution of the leptomeningeal enhancement 5 days after drug withdrawal and corticosteroid therapy.