Late neonatal lupus erythematosus onset in a child born of a mother with primary Sjogren's syndrome

Background: The neonatal lupus syndrome can be present as congenital heart block (CHB) or as neonatal lupus erythematosus (NLE), both seldom passively acquired autoimmune diseases. CHB starts around week 20 of pregnancy and is a lifelong event, whereas NLE is self limiting and usually starts at the 6th week of the child's age - the maximum recorded up to week 20. Case report: An asymptomatic mother with primary Sjogren's syndrome and anti-SSA/Ro52, anti-SSA/Ro60, and anti-SSB/La autoantibodies is described who, at gestational week 23 during her first pregnancy, was diagnosed as having a male fetus with CHB due to third degree atrioventricular block. The boy from the second pregnancy developed skin eruptions which clinically and by biopsy were compatible with NLE at week 20+1 post partum. Conclusions: Our case of NLE, starting at week 20+1 of age, seems to be the latest reported clinical case of NLE. Development of CHB and NLE in two consecutive boy pregnancies is unusual

Mortality and causes of death in primary Sjögren's syndrome: a prospective cohort study.

Objective This study was undertaken to analyze standardized mortality ratios (SMRs) and causes and predictors of death in primary Sjögren's syndrome (SS) diagnosed according to 3 different classification criteria sets (the Copenhagen criteria, the European criteria, and the American-European consensus criteria (AECC). Methods A linked registry study using information from the Malmö Primary SS Registry combined with the Swedish Cause-of-Death Registry was performed, and SMRs were calculated. Kaplan-Meier survival curves and log rank tests were used to compare survival probability between subgroups of patients with primary SS. Cox regression analysis was used to study the predictive value of various laboratory findings at the time of diagnosis. Results Four hundred eighty-four patients with a median followup of 7 years (range 1 month to 17 years 11 months) were included. The SMR for those fulfilling the AECC (n = 265) was 1.17 (95% confidence interval [95% CI] 0.81-1.63). Thirty-four deaths occurred in this group of patients. Excess mortality was found only for lymphoproliferative malignancy (cause-specific SMR 7.89 [95% CI 2.89-17.18]), corresponding to 2.53 excess deaths per 1,000 person-years at risk. In those not fulfilling the AECC (n = 219), 14 deaths occurred, the SMR was 0.71 (95% CI 0.39-1.20), and no excess mortality due to any specific cause was found. Hypocomplementemia, defined as C3 and/or C4 values in the lowest quartile of the SS patients' values at the time of diagnosis, was a significant predictor of death, mainly due to lymphoproliferative malignancy. Conclusion No increased all-cause mortality could be detected for patients with primary SS compared with the general population. When subgroups of primary SS were compared, excess mortality due to lymphoproliferative malignancy was found in patients fulfilling the AECC, the strongest predictor for unfavorable outcome being low C3 and/or C4 levels at the time of diagnosis

Recent findings shed light on the aetiopathogenesis of Sjogren's syndrome

The systemic clinical picture of SS patients can be traced back to a disposition of many cell types to over-react when confronted with stress stimuli. This will influence basic cellular processes such as apoptosis and acetylcholine receptor signaling. This tissue hyper-reactivity may also explain the observed autoimmune features, and may constitute a major aetiopathogenetic determinant in SS

Situational patterns in coping with primary Sjögren’s syndrome

The complexities involved in primary Sjögren’s syndrome (primary SS) were examined by designing and testing an instrument for evaluating the occurrence and severity of various biopsychosocial stressors as perceived by primary SS patients (63 women, 6 men) and at examining the situational patterns involved in appraising and coping with stressful episodes. The results indicated symptoms of fatigue, eye discomfort, dryness of the mouth and sleep disturbances to be particularly stressful. Women were found to be more likely than men—and patients with less education more likely than those with higher education—to report a stressor as occurring and experiencing it as stressful