Congenital heart block secondary to neonatal lupus

@#Neonatal lupus is a passively acquired autoimmune disease that occurs in children of mothers with anti-Ro/SSA and/or anti-La/SSB antibodies. [1-4] The most serious complication in the neonate is complete heart block. [3-8] This is a case report of a newborn female presenting with persistent bradycardia detected in utero. The diagnosis was confirmed by maternal anti-Ro/SSA and/or anti-La/SSB antibodies and in utero detection of fetal heart block on echocardiogram. Therapeutic management involved placement of a permanent pacemaker.

Advances in fetal immune mediated atrioventricular block / 临床儿科杂志

Fetal atrioventricular block (AVB) is a type of fetal bradyarrhythmias. The reported incidence of fetal complete atrioventricular block (CAVB) and mortality of perinatal fetuses and neonates are signiifcantly higher in pregnancies of anti-SSA/Ro-positive mothers than that of anti-SSA/Ro-negative mothers. The auto-antibodies in maternal serum that can be transported into fetal circulation through placenta may damage fetal cardiac conductive system and eventually result in fetal AVB. There are evidences that early diagnosis and proper treatment can improve the prognosis and survival rate of affected fetuses. In this article, the pathogenesis, risk factors, prenatal diagnosis, treatment and prognosis of fetal immune mediated AVB is reviewed.

Prevalence and significance of anti-SSA60 antibodies and anti-SSB antibodies in systemic lupus erythematosus patients / 中华风湿病学杂志

Objective To investigate the detection rate of anti-SSA60 and SSB antibodies in sera of patients with systemic lupus erythematosus (SLE).The correlation of anti-SSA and SSB antibodies with SLE clinical outcome was also investigated.Methods This study included 251 cases of SLE diagnosed in our hospital between 2007 and 2010.ELISA and double immunodiffusion method was used to detect the sera antiSSA60 and SSB antibodies.The patients were closely monitored for three years in terms of clinical and laboratory parameters and the presence of associated Sj(o)gren' s syndrome (SS).Statistical analysis were performed using student t test or x2 test.Results ① The detection rate of anti-SSA60 antibody in serum of patients with SLE was 65.3％.The detection rate of anti-SSB antibody in serum of patients with SLE was 28.3％; ② During the three-year follow up,patients with anti-SSA60 (29.3％,48 cases) or SSB antibodies (35.2％,25 cases) were more likely to have dry mouth and eyes and later developed SS (P＜0.05); ③ Patients with anti-SSA60 antibody were more likely to develop serositis (20.7％ vs 8.0％),neuropsychiatric lupus erythematosus (NPLE)(18.9％ vs 8.0％),and hematuria (35.4％ vs 21.8％)(P＜0.05).Patients with negative anti-SSB antibody were more likely to have fever (43.7％ vs 57.8％,x2=4.082,P＜0.05); ④ Patients positive for anti-SSB antibody were also positive for anti-Sm antibody (50.7％ vs 32.8％,x2=6.956,P＜0.05);⑤ Younger patients were more likely to have anti-SSA60 and SSB antibodies in their sera (P＜0.05); ⑥Patients positive for anti-SSA60 antibody had higher SLE disease activity index (SLEDAI) than patients with negative anti-SSA60 antibody [(17±9) vs (15±7),t=2.389,P＜0.05].Patients positive for anti-SSB antibody had higher level of IgG [(18±7) vs (16±6) g/L,t=2.304,P=0.023],and lower level of CRP than patients negative for anti-SSB antibody [(14±20) vs (21±33) mg/L,t=-2.173,P=0.031].Conclusion Patients positive for anti-SSA60 antibody have higher SLEDAI and more severe clinical outcomes.Patients with antiSSA or anti-SSB antibody are more likely to develop dry mouth and eyes which eventually leads to SS.

The lung CT findings in patients with primary Sj(o)gren's syndrome and clinical significance / 中华风湿病学杂志

Objective To investigate the lung CT findings in patients with primary Sj(o)gren's syndrome (pSS) and clinical significance.Methods Images of lung CT scans of 68 patients with pSS were retrospectively observed.The distribution characteristics of lung lesions were observed.CT visual score were used to quantitatively analyze lung lesions.Binary classification unconditional logistic regression analysis was used to investigate the relationship between pulmonary arterial hypertension (PAH) and anti-SSB seropositivity in Patients with pSS.Results The 68 cases of patients with pSS,PAH in 13 (19％),anti-SSB seropositivity in 25(37％),CT shows normal in 13 (19％),interlobular septal thickening,ground-glass opacity and intralobular interstitial thickening were frequent findings.The lesion distribution was in the peripheral and lower lung fields.Interlobular septal thickening and cysts were associated with PAH in Patients with pSS (OR=1.780,2.513,P＜0.05),cysts was significantly associated with anti-SSB seropositivity (OR =10.530,P＜0.05).Conclusion Lung CT can quantitatively analyze the degree of pSS lung damage,and provide helps for clinical treatment and prognosis.

A case of neonatal lupus syndrome with congenital complete heart block / 대한산부인과학회잡지

The neonatal lupus syndrome is characterized by skin lesions, hepatic and hematologic abnormalities and congenital heart block. Congenital heart block which is believed to be caused by transplacental passage of the anti-Ro (SSA)/La (SSB) antibodies from mother to infant, is known to occur in 1 in 20,000 live births. In contrast to other manifestation of neonatal lupus syndrome, which usually subside within 6 months after birth, congenital heart block is a permanent and potentially fatal complication. We experienced a case of neonatal lupus syndorme with congenital complete heart block in a newborn of asyptomatic mother with anti-Ro (SSA)/ La (SSB) antibodies.