RESUMEN
Lupus myelitis is a rare but serious condition characterized by myelopathy in patients with systemic lupus erythematosus (SLE). Its presentation is usually acute or subacute, and it is often refractory to treatment. We reported a rare presentation of lupus myelitis in a 38-year-old Japanese woman with a 20-year history of SLE. She developed paraparesis and bladder/bowel dysfunction 6 months prior to presentation. Magnetic resonance imaging revealed atrophy of the entire thoracic spinal cord with high intensity on T1-weighted sequence. She was initially treated with intravenous pulse steroid therapy, and prednisolone (20 mg/day) was continued; mizoribine was changed to azathioprine (100 mg/day). In addition, she underwent a rehabilitation program to improve lower-extremity muscle weakness. Moreover, because of the refractory clinical condition, intravenous cyclophosphamide pulse therapy was added. Within 1 month, she could walk with a cane and had a desire to urinate and defecate. In conclusion, early and aggressive treatment improves the permanent damage of lupus myelitis.
Asunto(s)
Ciclofosfamida/administración & dosificación , Lupus Eritematoso Sistémico/tratamiento farmacológico , Metilprednisolona/administración & dosificación , Mielitis/tratamiento farmacológico , Administración Intravenosa , Adulto , Ciclofosfamida/uso terapéutico , Femenino , Humanos , Japón , Lupus Eritematoso Sistémico/complicaciones , Imagen por Resonancia Magnética , Metilprednisolona/uso terapéutico , Mielitis/diagnóstico por imagen , Mielitis/etiología , Resultado del TratamientoRESUMEN
We report a case of a 12-year-old male who initially presented with active systemic lupus erythematosus (SLE) with lupus nephritis and secondary macrophage activation syndrome (MAS). He went on to develop left-sided upper motor neuron (UMN) facial palsy secondary to lupus-related tumefactive demyelination. Tumefactive lesions secondary to demyelination are a very rare manifestation in neuropsychiatric SLE. This child responded to aggressive immunosuppression with steroids and cyclophosphamide.
Asunto(s)
Lupus Eritematoso Sistémico , Síndrome de Activación Macrofágica , Trastornos Mentales , Absceso/etiología , Niño , Ciclofosfamida/uso terapéutico , Humanos , Lupus Eritematoso Sistémico/complicaciones , MasculinoRESUMEN
We report a case of a new diagnosis of systemic lupus erythematosus (SLE) in a patient with HIV who presented to the outpatient department with a fever, headache and lymphadenopathy. Cerebrospinal fluid analysis showed lymphocytic pleocytosis. Initial concerns were for an infectious process, and investigations for systemic and central nervous system infection were negative. Serum testing for ANA, dsDNA, nucleosome, anti-histone and ribosomal-P antibodies was positive. A magnetic brain imaging scan of the brain showed a well-circumscribed lesion in the right cerebellar peduncle on T2/FLAIR. The patient was commenced on prednisolone and rituximab, and had a good clinical response. The cerebellar lesion resolved and has not recurred with sequential imaging. SLE and HIV are both multi-systemic diseases which rarely co-occur. Autoimmune processes should be considered in HIV patients with multi-systemic symptoms and signs.
Asunto(s)
Encéfalo/patología , Infecciones por VIH/inmunología , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/inmunología , Adulto , Anticuerpos Antinucleares/sangre , Encéfalo/efectos de los fármacos , ADN/inmunología , Femenino , Fiebre/etiología , Infecciones por VIH/complicaciones , Cefalea/etiología , Humanos , Lupus Eritematoso Sistémico/tratamiento farmacológico , Linfadenopatía/etiología , Imagen por Resonancia Magnética , Nucleosomas/inmunología , Prednisolona/uso terapéutico , Rituximab/uso terapéuticoRESUMEN
This study analyzed maternal and fetal outcomes of pregnancies of neuropsychiatric systemic lupus erythematosus patients followed in a reference unit. This retrospective cohort study included 26 pregnancies of patients seen between 2011 and 2015 included with history and/or active neuropsychiatric systemic lupus erythematosus among 135 pregnancies. Three patients had active neuropsychiatric systemic lupus erythematosus at conception, but only one remained with neurological activity during gestation, characteristically related to the inadvertent suspension of medications. Twenty six percent of the newborns were small for gestational age and 40% of live births were premature, with no neonatal death or early complications of prematurity. Preeclampsia was diagnosed in nine pregnancies, with two cases of early severe form that resulted in intrauterine fetal death. Patients with neuropsychiatric systemic lupus erythematosus had more prematurity and preeclampsia compared to patients without neuropsychiatric disease. However, when concomitant lupus nephritis was excluded, the gestational results of neuropsychiatric systemic lupus erythematosus patients were more favorable.