Longitudinal extensive transverse myelitis in systemic lupus erythematosus – a case report and review of literature

Autori:

Marija Šćepović-Ljucović, Miroslav Mayer, Ivan Padjen, Branimir Anić

Sažetak
Longitudinalni ekstenzivni transverzalni mijelitis (LETM) je iznimno rijetka, životno ugrožavajuća komplikacija sistemskog eritemskog lupusa (SLE). Riječ je o upalnoj leziji leđne moždine koja zahvaća najmanje tri susjedna vertebralna segmenta. Klinički se prezentira paraparezom ili tetraparezom, senzornim deficitom i vegetativnom disfunkcijom, a u teškim slučajevima uzrokuje respiratornu insuficijenciju. Prikazana je bolesnica u dobi od 28 godina koja se posljednjih 11 godina prati zbog SLE-a, a kod koje se bolest komplicirala razvojem LETM-a sa zahvaćanjem cervikalne i torakalne medule. LETM je nastupio nakon razdoblja stabilne remisije bolesti, a bio je praćen kliničkom, a prethodno i serološkom aktivacijom SLE-a. U sklopu LETM-a bolesnica je razvila tetraparezu i visoku hipesteziju, a zbog pareze respiratorne muskulature i posljedične respiratorne insuficijencije bila je potrebna mehanička ventilacija. Budući da obradom nije dokazana intratekalna infekcija, provedena je kombinirana imunosupresivna terapija kojom je postignut postupni i djelomični neurološki oporavak i poboljšanje neuroradiološkog nalaza. LETM je rijetka, ali ozbiljna komplikacija SLE-a, koja često ostavlja teške neurološke sekvele ili pak dovodi do letalnog ishoda. Uz prikaz bolesnice u pregledu literature navedena su dosadašnja saznanja o patogenezi, dijagnozi i terapijskom pristupu LETM-u u kontekstu neuropsihijatrijskog lupusa.
Summary

Longitudinal extensive transverse myelitis (LETM) is rather rare life threatening complication of systemic lupus erythematosus (SLE). It is defined as an inflammatory spinal cord lesion that extends over three or more adjacent vertebral segments. Clinical presentation can consist of paraparesis or tetraparesis, sensory disturbances and vegetative dysfunction, and respiratory failure may follow in severe cases. A female patient aged 28 years, diagnosed with SLE 11 years ago, has been herein presented, whose disease was complicated by LETM affecting cervical and thoracic spinal cord. LETM occurred after the period of stable remission, and was followed
by clinical, as well as previously serologically active disease. In the context of LETM the patient developed tetraparesis and high hypoesthesia, while respiratory muscle paralysis and consecutive respiratory failure required mechanical ventilation. After the exclusion of underlying intracranial infection, combined immunosuppressive therapy has been used resulting in a gradual but partial neurological recovery, and an overall neuroradiological improvement. LETM is an uncommon but severe complication of SLE leading to serious neurological sequelae, and often resulting even in fatal otcome. Along with herein presented case report, current available findings on pathogenesis, diagnosis and therapheutic approach in the context of neuropsychiatric lupus have been depicted through the review of the literature.

Volumen: 3-4, 2022

Liječ Vjesn 2022;144:79–86

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