A case report and literature review of McCune–Albright syndrome with SLE

Abstract

Abstract Backgroud: Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple systems with various clinical manifestations. Renal involvement is common, neurological complications can also occur in lupus, such as cerebral hemorrhage, cerebral infarction, cerebral embolism, etc.But McCune–Albright syndrome with SLE is rare.We report a case to Improve the understanding of diseases and accumulate clinical experience. Case presentation: A 9.5-year-old girl was admitted to the hospital due to sudden slurred speech and inability to move her left limb for 6 hours. Emergency head CT showed that a round high-density shadow was seen in the right basal ganglia, the largest layer was about 2.2cmx2.4cm. Blood routine: hemoglobin 67g/L, platelets 2×109/L, surgery cannot be performed, and conservative treatment can only be performed. ANA series: particle type 1:320 positive, anti-SSA-52/Ro52+; Urine occult blood 3+, urine protein 3+; anti-β2-glycoprotein I antibody 42RU/ml. Abdominal CT: a small amount of fluid in the abdominal cavity. Lung CT: scattered inflammation in the left lung and right lower lobe. A small amount of pleural effusion bilaterally.One year ago, the child was admitted to our hospital due to intermittent skin bruising for 2 months. The blood routine showed that the platelets were 5×109/L. During the year, the patient's platelets were in single digits,her parents did not give treatment.The patient was diagnosed with "McCune–Albright syndrome" in the Department of Endocrinology and Metabolism in our hospital 8 years ago, and was treated with oral "tamoxifen", but the drug has been discontinued for 2 years. MRV: The left transverse sinus is not shown, and the left internal jugular vein and sigmoid sinus are slender. After admission, patients were treated with Respiratory support,hormone shock, immunosuppressant, dehydration and intracranial pressure reduction, platelet transfusion, rehabilitation, etc.After the rehabilitation treatment, the child was conscious and could communicate easily, but the lower limbs could not be straightened. Conclusion: SLE can involve multiple systems and various complications. McCune–Albright syndrome with SLE is rare. Early diagnosis and treatment are key to improving the prognosis of children.