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Background Several studies have evaluated mortality and shortterm morbidity in neonatal lupus (NL), however there is minimaldata on long term outcomes in children exposed to maternalanti-Ro antibodies in utero. A previous pilot study utilising theResearch Registry for Neonatal Lupus (RRNL) raised concernregarding the development of autoimmune disease in childhood, however the numbers evaluated were small and the patientsstudied were young. This study was initiated to ascertain the current prevalence of autoimmune disease in NL children and theirunaffected siblings, and to evaluate whether fetal or maternal factors associated with the development of future autoimmunity.Materials and methods A retrospective cohort of family membersfrom the RRNL were contacted to evaluate for autoimmune disease. Follow-up questionnaires were completed which included35 items describing symptoms and diagnoses associated withautoimmunity in 138 cardiac NL children, 74 cutaneous NL children, and 134 unaffected siblings. Medical records were obtainedand evaluated from the patient's physicians to confirm diagnoses.Maternal diagnosis of systemic lupus and/or Sjogren's syndromeand fetal cardiac disease severity based on a previously describedseverity score were associated with postnatal autoimmune diseases using chi square and Mann-Whitney analyses.Results Seventeen (8.0%) of NL affected children developed anautoimmune disease at the time of follow up (mean age 11.6+/-9.0 years). These included 3 patients with SLE, 1 with JIA, 3with thyroid disease, 5 with psoriasis, 1 with IBD, 1 with uveitis,1 with UAS and 2 with type 1 DM. Six (4.5%) unaffected siblingsdeveloped an autoimmune disease (mean age 10.6+/-7.1 years),which included 1 with JIA, 1 with Sarcoidosis/ITP, 1 with Myasthenia Gravis/Celiac disease, 1 with psoriasis, 1 with UAS and1 with type 1 DM,. There was a significant association ofbetween having an autoimmune disease and having advancedheart block (11.0% vs. 4.2%, p = 0.03) and a trend towards anassociation with cardiac NL disease severity score (4.43+/-4.89 vs.2.58+/-4.24, p = 0.06). Mother's diagnosis of SLE or Sjogren'sdid not associate with the children's development of autoimmunedisease (p = 0.828).Conclusions Fetuses that develop advanced congenital heartblock as a manifestation of NL may be at greater risk for developing autoimmune diseases later in life. This could potentially relateto a genetic component that makes a Ro exposed fetus both moreprone to inflammatory effects of passive immunity and predisposes to future autoimmunity, independent of the mother's rheumatic disease status

OBJECTIVES: Extension of disease beyond the atrioventricular (AV) node is associated with increased mortality in cardiac neonatal lupus (NL). Treatment of isolated heart block with fluorinated steroids to prevent disease progression has been considered but published data are limited and discordant regarding efficacy. This study evaluated whether fluorinated steroids given to manage isolated advanced block prevented development of disease beyond the AV node and conferred a survival benefit. METHODS: In this retrospective study of cases enrolled in the Research Registry for NL, inclusion was restricted to anti-SSA/Ro-exposed cases presenting with isolated advanced heart block in utero who either received fluorinated steroids within 1 week of detection (N=71) or no treatment (N=85). Outcomes evaluated were: development of endocardial fibroelastosis, dilated cardiomyopathy and/or hydrops fetalis; mortality and pacemaker implantation. RESULTS: In Cox proportional hazards regression analyses, fluorinated steroids did not significantly prevent development of disease beyond the AV node (adjusted HR=0.90; 95% CI 0.43 to 1.85; p=0.77), reduce mortality (HR=1.63; 95% CI 0.43 to 6.14; p=0.47) or forestall/prevent pacemaker implantation (HR=0.87; 95% CI 0.57 to 1.33; p=0.53). No risk factors for development of disease beyond the AV node were identified. CONCLUSIONS: These data do not provide evidence to support the use of fluorinated steroids to prevent disease progression or death in cases presenting with isolated heart block.

Both systemic lupus erythematosus (SLE) and its treatments can contribute to increased mortality rates. The focus of this review is recent studies on mortality and comorbidities during the last 5 years from around the world. The authors conducted a literature review, using PUBMED, for articles relating to SLE mortality with a specific focus on literature published within the last 5 years. Our analysis found that while mortality in SLE patients continues to improve, there are differences in survival based on ethnicity, socioeconomic status, age, and gender. The most common cause of mortality is cardiovascular disease, followed closely by infection and severe disease activity. To conclude, while there have been significant advances in the treatment of SLE and its associated comorbidities, increased mortality remains a major concern in patient management.

Autoimmune congenital heart block (ACHB) is an immune-mediated cardiac disease included among the manifestations collectively referred to as neonatal lupus. The placental transference of maternal Ro/La autoantibodies may damage the conduction tissues during fetal development leading to blocking of signal conduction at the atrioventricular (AV) node in an otherwise structurally normal heart. Irreversible complete AV block is the main cardiac manifestation of ACHB, but some babies may develop endocardial fibroelastosis, valvular insufficiency, and/or frank cardiomyopathies with significantly reduced cardiac function requiring transplant. The severity of ACHB is illustrated by a global mortality rate of 20% and pacemaker rates of at least 64%, often within the first year of life. This review analyses the main complex and/or unusual clinical situations associated with ACHB, including unusual maternal immunological profiles, infrequent maternal autoimmune diseases, cardiac damage unrelated to AV block, fetal invasive management, late complications after birth, risk of congenital heart block (CHB) in ovodonation and in vitro fertilization techniques, the role of maternal features other than autoimmunity, the influence of the birth order or the risk of CHB in twins and triplets.

Sjogren's syndrome (SjS) is a systemic autoimmune disease that mainly affects the exocrine glands, leading to generalized mucosal dryness. However, primary SjS may initially present with non-sicca (systemic) manifestations. When these features appear before the onset of an overt sicca syndrome, we may talk of an underlying 'occult' SjS. The European League Against Rheumatism (EULAR) has promoted and supported an international collaborative study group (EULAR-SS Task Force) aimed at developing consensual recommendations to provide a homogeneous approach to the patient with primary SjS presenting with systemic involvement. This review summarizes the key factors that should be taken into account in the diagnostic approach in a patient with suspected SjS according to the main clinical patterns of presentation, and is especially focused on organ-specific systemic disease presentations, including a consensus set of recommendations in order to reach an early diagnosis. Close collaboration with the different specialties involved through a comprehensive multidisciplinary approach is essential in SjS patients presenting with systemic involvements.