Nature clinical practice. Rheumatology · 2008
Conversion of discoid lupus to antiphospholipid syndrome and SLE.
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Fernando Michelle M A, Isenberg David A
Аннотация
BACKGROUND: A 64-year-old man was admitted to hospital with increasing seizure frequency, lethargy and confusion. He had a history of discoid lupus erythematosus, complex partial seizures, cerebral thromboses associated with antiphospholipid syndrome (APS) and hypertension. After admission to hospital, he developed autoimmune hemolytic anemia, thrombocytopenia, severe hypertension, proteinuria and a fluctuating level of consciousness.
INVESTIGATIONS: Physical examination, biochemical and hematological indices, urine dipstick, electrocardiography and chest radiography on admission, with subsequent electroencephalography, lumbar puncture, septic screen, autoimmune screen, CT of the head, MRI of the brain and renal biopsy.
DIAGNOSIS: Conversion of discoid lupus erythematosus with APS to systemic lupus erythematosus with APS.
MANAGEMENT: Pulsed intravenous methylprednisolone and a single infusion of intravenous cyclophosphamide, followed by oral prednisolone, were initially administered for presumed cerebral lupus. When renal biopsy revealed dual pathology, another dose of intravenous cyclophosphamide was administered with intravenous heparin therapy to treat systemic lupus erythematosus and APS, respectively. Intravenous immunoglobulin was used to treat thrombocytopenia, which was unresponsive to immunosuppression and anticoagulation. Gradual improvement occurred with maximal antihypertensive therapy and supportive treatment. The patient was discharged after 9 months of hospital treatment. His medication at this stage included warfarin, clopidogrel, prednisolone, carbamazepine, ramipril, atenolol, calcium and vitamin D supplements, and alendronate.
INVESTIGATIONS: Physical examination, biochemical and hematological indices, urine dipstick, electrocardiography and chest radiography on admission, with subsequent electroencephalography, lumbar puncture, septic screen, autoimmune screen, CT of the head, MRI of the brain and renal biopsy.
DIAGNOSIS: Conversion of discoid lupus erythematosus with APS to systemic lupus erythematosus with APS.
MANAGEMENT: Pulsed intravenous methylprednisolone and a single infusion of intravenous cyclophosphamide, followed by oral prednisolone, were initially administered for presumed cerebral lupus. When renal biopsy revealed dual pathology, another dose of intravenous cyclophosphamide was administered with intravenous heparin therapy to treat systemic lupus erythematosus and APS, respectively. Intravenous immunoglobulin was used to treat thrombocytopenia, which was unresponsive to immunosuppression and anticoagulation. Gradual improvement occurred with maximal antihypertensive therapy and supportive treatment. The patient was discharged after 9 months of hospital treatment. His medication at this stage included warfarin, clopidogrel, prednisolone, carbamazepine, ramipril, atenolol, calcium and vitamin D supplements, and alendronate.