Neuromyelitis optica spectrum disorder in patient with systemic lupus erythematosus - our experience

Ksenija Bozic, Nenad Komatina, Milan Petronijevic, Bojana Knezevic, Dejan Kostic, Dusan Stefanovic
2018 Vojnosanitetski Pregled  
Introduction. Neuromyelitis optica spectrum disorder (NMOSD) is a rare demyelinating immune-mediated central nervous system disease. It is extremely rare to occur in patients with systemic lupus erythematosus (SLE), and it represents a diagnostic and therapeutic challenge. Case report. A 38-year-old Caucasian woman with medical history of SLE and new onset of flaccid paraparesis, fecal and urinary incontinence, persistent nausea and vomiting was admitted to our hospital. Based on the clinical
more » ... d on the clinical presentation, magnetic resonance imaging findings and positive aquaporin 4 (AQP4) antibodies, a NMOSD with coexisting SLE were diagnosed. Pulse-doses of cyclophosphamide and glucocorticoids were efficient in patient treatment. Conclusion. In a patient with SLE and symptoms of longitudinal extensive transverse myelitis and/or optic neuritis and area postrema syndrome, assessment of AQP4 antibodies is neccessary for diagnosing NMOSD. Accurate diagnosis, and timely and long-term administration of immunosuppressive therapy are crucial for favorable outcome of these two coexisting diseases. Uvod. Bolesti iz spektra optičkog neuromijelitisa (BSON) su retka imunski -posredovana demijelinizaciona oboljenja centralnog nervnog sistema. Kako se veoma retko javljaju kod bolesnika sa sistemskim eritemskim lupusom, predstavljaju veliki dijagnostički i terapijski izazov. Prikaz bolesnika. Bolesnica, stara 38-godina, sa sistemskim eritemskim lupusom i novonastalom flakcidnom paraparezom, inkontinencijom urinarnog i analnog sfinktera, prolongiranom mučninom i povraćanjem, primljena je u našu kliniku. Na osnovu kliničke prezentacije, nalaza nuklearne magnetne rezonance i pozitivnih antitela prema akvaporinu 4 (AQP4) dijagnostikovana je BSON udružena sa sistemskim eritemskim lupusom. Terapija pulsnim dozama ciklofosfamida i glikokortikoida je bila efikasna. Zaključak. Kod bolesnika sa sistemskim eritemskim lupusom i simptomima longitudinalnog ekstenzivnog tranverzalnog mijelitisa i/ili optičkog neuritisa i sindroma area postrema, neophodno je odrediti AQP4 antitela radi postavljanja dijagnoze BSON. Tačna dijagnoza, pravovremena i dugotrajna primena imunosupresivne terapije je od presudnog značaja za povoljan ishod udruženosti ove dve bolesti.
doi:10.2298/vsp160331322b fatcat:64ivlahapbg3lmaql66x4xxcki