ABSTRACT
ObjectiveTo explore the pathogenesis, clinical manifestations, diagnosis, and treatment of autoanti-body-associated congenital heart block.MethodsThe clinical data of one child with autoantibody-associated congenital heart block was retrospectively analyzed.ResultsIn 24 week gestation, fetal bradycardia had been found by routine fetal echocar-diography. After birth, the anti-SSA/Ro antibodies and anti-SSB/La antibodies were positive in both infant and her mother. The diagnosis of autoantibody-associated congenital heart block was conifrmed. Intravenous immunoglobulin at 1 g/kg was adminis-trated. At 6 months follow-up, the electrocardiogram suggested type I second degree atrioventricular block.ConclusionIn the fetus or neonates found to have bradycardia and excluded the cardiac structural abnormalities, the autoimmune antibody should been tested and early intervention should been promoted.
ABSTRACT
Neonatal lupus erythematosus (NLE) is an autoimmune disease that is associated with transplacental passage of maternal autoantibodies that are reactive to SSA/Ro and SSB/La antigens. Cardiac involvement, hematologic abnormality and hepatic disease may occur in the infants suffering with NLE, in addition to the characteristic skin lesions. We report here on a case of NLE in a 4-week-old female infant who was born to an asymptomatic mother, and the baby displayed the characteristic clinical and histological features of cutaneous NLE with transient anemia and hepatitis. Both the infant and mother were positive for anti-SSA/Ro and anti-SSB/La. There have been 18 case reports of NLE in the Korean literature, including 7 case reports in the dermatological field. We describe herein another case of NLE that showed transient anemia and hepatitis, and we also review the case reports of NLE in the Korean literature.
Subject(s)
Female , Humans , Infant , Anemia , Autoantibodies , Autoimmune Diseases , Hepatitis , Lupus Erythematosus, Systemic , Mothers , Skin , Stress, PsychologicalABSTRACT
PURPOSE: Neonatal lupus is characterized by congenital complete heart block(CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with systemic lupus erythematosus(SLE). This study aims to examine the incidence of CCHB and clinical outcome in neonates born to mothers with SLE. METHODS: The study group consisted of 49 neonates, born from 57 pregnancies of 55 women with SLE, diagnosed at Hanyang University Hospital for the period between January 1997 and January 2005. Clinical and laboratory data were retrospectively identified from medical record. RESULTS: There were 5(8.8 percent) spontaneous abortions and one(1.8 percent) still births among 57 pregnancies of 55 mothers. Of 49 live births, 15(26.3 percent) were premature and eight(12.3 percent) were small for their gestational age. There was one(1.8 percent) CCHB suspected during pregnancy on fetal echocardiograpy in a fetus of mother with systemic lupus erythematosus and the fetus was not born by artificial abortion because of mother. There was no CCHB among EKG findings of 49 newborns. Laboratory testing showed hematologic abnormalities among 25.6 percent(10/39) of the babies. 5.1 percent(2/39) and 7.7 percent(3/39) of them were diagnosed as neutropenia, and thrombocytopenia was seen respectively. Anti-SSA(Ro) and antiphospholipid antibodies were predictive factors for prematurity(P=0.003, P=0.049). Anticardiolipin antibodies were predictive factors for ventilatory care(P=0.018). CONCLUSION: The incidence of CCHB among neonates born to mothers with SLE, which was measured in this study, was lower than that in earlier studies. A high incidence of hematologic abnormalities was found in our study. It is suggested that careful examination should be made of skin for the diagnosis of neonatal lupus.
Subject(s)
Female , Humans , Infant , Infant, Newborn , Pregnancy , Abortion, Spontaneous , Antibodies, Anticardiolipin , Antibodies, Antiphospholipid , Diagnosis , Electrocardiography , Exanthema , Fetus , Gestational Age , Heart , Incidence , Live Birth , Lupus Erythematosus, Systemic , Medical Records , Mothers , Neutropenia , Parturition , Retrospective Studies , Skin , ThrombocytopeniaABSTRACT
Chilblain lupus erythematosus (CLE) is a subtype of lupus erythematosus. It is characterized by cutaneous lesions located on the fingers, toes, nose, ears, elbows, heels and knees and is induced or aggravated by exposure to a cold or damp climate. Various laboratory alterations including antinuclear antibody (ANA), anti-dsDNA antibody, anti-SSA/SSB antibody, rheumatoid factor, and cryoglobulin have been reported in CLE patients. Especially, SSA/Ro antibodies may be especially associated with CLE. Approximately 20% of patients presenting with CLE later develop systemic lupus erythematosus (SLE). A 28-year-old man diagnosed with SLE presented with a 2-year history of pruritic erythematous plaques on the ears and dorsa of his hands and feet. The lesions developed or were aggravated the cold weather. In the summer, they were reported to improve, but they did not heal. ANA anti- SSA/SSB antibodies, and anti-dsDNA antibodies were found to be present. He was treated with a topical steroid and advised to avoid the cold.
Subject(s)
Adult , Humans , Antibodies , Antibodies, Antinuclear , Chilblains , Climate , Ear , Elbow , Fingers , Foot , Hand , Heel , Knee , Lupus Erythematosus, Systemic , Nose , Rheumatoid Factor , Toes , WeatherABSTRACT
Neonatal lupus erythematosus(NLE) is a rare disease characterized by the transplacental passage from the mother to the fetus of autoantibodies, particularly autoantibodies of Ro family. The patient with NLE exhibits one or more of the following findings: congenital heart block, cutaneous lupus lesions, hepatobiliary disease and hematologic disorders(thrombocytopenia, anemia). We report a case of NLE in a 2-week-old male infant, born of a clinically asymptomatic mother, presenting multiple, round, target-like lesions which have not been reported in the English and Korean literature. Both infant and mother were positive for anti-SSA/Ro and anti-SSB/La.
Subject(s)
Humans , Infant , Male , Autoantibodies , Fetus , Heart Block , Mothers , Rare Diseases , SkinABSTRACT
Neonatal lupus erythematosus (NLE) is a transplacentally acquired autoimmune disorder, which is characterized by cutaneous lesions and/or congenital heart block and less commonly hepatic and hematologic abnormalities. Affected infants acquire anti-SSA/Ro antibody, anti-SSB/La antibody or less commonly anti-U1RNP antibody transplacentally from maternal circulation and it is generally thought that these antibodies are pathogenic. We report a case of NLE in a 40-day-old neonate who had erythematous annular patches on his face and extremities. Serological studies were reactive for antinuclear antibody of the speckled pattern and positive for anti-SSA/Ro antibodies and anti-SSB/La antibodies in both mother and infant. Addition to these findings, his mother had complained photosensitivity and arthralgia and showed hematological abnormalities including anemia and leukopenia, so we diagnosed his mother as systemic lupus erythematosus.