Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an uncommon inflammatory CNS disease classically involving the pons and adjacent structures. The disease is thought to occur de novo; however, due to its rarity it is uncertain whether it can be a secondary manifestation of autoimmune diseases.

A 48-years-old woman with pertinent history of lupus on low dose prednisone for 30 years and epilepsy was admitted for breakthrough seizures. During the hospitalization, her brain MRI showed numerous infratentorial (primarily pontine and cerebellar) and supratentorial contrast enhancing lesions in a speckled pattern. Cerebrospinal fluid, CT scans of the chest, abdomen and pelvis, as well as infectious and paraneoplastic workup were negative. A brain biopsy of a right frontal lesion did not show evidence of vasculitis, lymphoma, granulomatous infiltration, or primary brain tumor.  Flow cytometry analysis showed T-cells and rare B-cells.  There was an increased microvascular proliferation and microglial/histiocytic infiltration in small areas of the sample.  Given the appearance of the lesions on the brain MRI and the histopathological findings, CLIPPERS is the most compatible diagnosis. The patient was discharged after her seizure medications were optimized.  For CLIPPERS, the oral dose of prednisone was increased and IV cyclophosphamide was added for management of lupus with newly discovered cardiac involvement.

CLIPPERS usually presents with cranial nerves dysfunction, ataxia, and cognitive symptoms in conjunction with striking speckled enhancing lesions in the pons and adjacent structures. Our case demonstrates a speckled pattern of lesions that extends supratentorially. Of relevance, our patient has a long history of systemic lupus.  The histopathological features seen in the brain biopsy were not consistent with CNS lupus, implying an independent co-existence of CLIPPERS. The treatment for this patient included increasing the dose of steroids and adding Cyclophosphamide to address both co-existing diseases.