Surge of Anti-SS-A Antibody
Surge of Anti-SS-A Antibody
Thrombotic thrombocytopenic purpura (TTP) is an uncommon clinical syndrome and is rarely associated with systemic lupus erythematosus (SLE). Diagnosis of TTP in patients with SLE, especially those who are pregnant, is challenging. We report the case of a pregnant woman with a high level of anti-SS-A antibody (162,143 U/mL) and fulminant TTP. The patient responded to plasma exchange treatment. Recent studies indicate that patients with SLE and another serologic abnormality, such as the presence of antiphospholipid antibody, may be at high risk for TTP. We explore the possible pathogenesis of acute TTP in patients with SLE and summarize the risk factors for acute TTP in patients with SLE and the current treatments for SLE-associated TTP.
Thrombotic thrombocytopenic purpura (TTP) is a rare clinical scenario. The diagnosis of TTP can be made by recognizing the clinical characteristic pentad of thrombocytopenic purpura, microangiopathic hemolytic anemia, renal failure, neurologic abnormalities, and fever. However, the diagnosis of TTP in pregnant women is sometimes difficult, especially in pregnant women with systemic lupus erythematosus (SLE), because TTP, pregnancy-associated microangiopathies such as HELLP (hemolysis with elevated liver enzymes and low platelet count) syndrome, and lupus-associated microangiopathy share many features. The outcome for patients with lupus-associated TTP is usually fatal if this clinical scenario is not recognized and treated in time. We successfully treated a pregnant patient who had fulminant TTP at 10 weeks' gestation. An extremely high level of anti-SS-A antibody was seen at the beginning of fulminant TTP. She responded to the treatment of plasma exchange. The fetus did not have any sign of complete heart block, one of the lethal complications in neonatal lupus syndrome.
Thrombotic thrombocytopenic purpura (TTP) is an uncommon clinical syndrome and is rarely associated with systemic lupus erythematosus (SLE). Diagnosis of TTP in patients with SLE, especially those who are pregnant, is challenging. We report the case of a pregnant woman with a high level of anti-SS-A antibody (162,143 U/mL) and fulminant TTP. The patient responded to plasma exchange treatment. Recent studies indicate that patients with SLE and another serologic abnormality, such as the presence of antiphospholipid antibody, may be at high risk for TTP. We explore the possible pathogenesis of acute TTP in patients with SLE and summarize the risk factors for acute TTP in patients with SLE and the current treatments for SLE-associated TTP.
Thrombotic thrombocytopenic purpura (TTP) is a rare clinical scenario. The diagnosis of TTP can be made by recognizing the clinical characteristic pentad of thrombocytopenic purpura, microangiopathic hemolytic anemia, renal failure, neurologic abnormalities, and fever. However, the diagnosis of TTP in pregnant women is sometimes difficult, especially in pregnant women with systemic lupus erythematosus (SLE), because TTP, pregnancy-associated microangiopathies such as HELLP (hemolysis with elevated liver enzymes and low platelet count) syndrome, and lupus-associated microangiopathy share many features. The outcome for patients with lupus-associated TTP is usually fatal if this clinical scenario is not recognized and treated in time. We successfully treated a pregnant patient who had fulminant TTP at 10 weeks' gestation. An extremely high level of anti-SS-A antibody was seen at the beginning of fulminant TTP. She responded to the treatment of plasma exchange. The fetus did not have any sign of complete heart block, one of the lethal complications in neonatal lupus syndrome.