Amyloid-beta-related angiitis (ABRA) is an immune-mediated central nervous system vasculitis characterized by an inflammatory response to amyloid-beta (Aβ) deposition within the walls of the leptomeningeal and cortical arteries. Immunosuppression is the mainstay of treatment. We present a case of ABRA in a patient on immunosuppressive therapy after orthotopic heart transplantation (OHT) for cardiac sarcoidosis.

A 57-year-old man eight months post-OHT developed headaches and dyscognitive seizures during hospitalization for disseminated non-tuberculous mycobacterial infection. Brain MRI revealed bi-hemispheric T2 FLAIR hyperintensities in the cortical and subcortical white matter, predominantly in the right temporo-parietal region, originally ascribed to infectious etiology. Supratentorial and infratentorial microhemorrhages were seen and thought to be sequelae of OHT. Subsequently, he exhibited gait ataxia and confusion. Repeat brain MRI showed more extensive confluent white matter hyperintensities. Right parietal leptomeningeal and cortex biopsy revealed amyloid angiopathy, with perivascular and intramural histiocyte and lymphocyte collections. Mass spectroscopy confirmed Aβ type IV deposition. Notably, the patient was on immunosuppression with daily 5 mg oral prednisone and tacrolimus prior to biopsy. After pulse-dose intravenous methylprednisolone followed by high-dose oral prednisone, he demonstrated significant clinical and radiographic improvement. No relapse was noted despite relatively rapid tapering of the prednisone therapy, as mandated by his systemic infection.