Congenital heart block related to maternal autoantibodies: descriptive analysis of a series of 18 cases from a single center.

The objective of this study was to describe the clinical and immunological characteristics of maternal autoimmune-mediated fetal congenital heart block (CHB) in a cohort of pregnant women from an autoimmune disease pregnancy clinic. This is a retrospective observational study of all women presenting with CHB in our autoimmune disease pregnancy clinic from January 1997 to December 2014. In addition, perinatal outcome is also described. Fourteen patients accounting for 18 fetuses with CHB were identified. The median age was 32.5 years (range, 22-40). Seven (50 %) patients had Sjögren's syndrome, and the remaining seven were asymptomatic carriers of autoantibodies. All patients had anti-Ro/SSA antibodies, and 11/13 (85 %) had anti-La/SSB antibodies. The median gestational age at the time of CHB was 22 weeks (range 18-28). Complete third degree CHB was detected in 12 (67 %). Seven cases of CHB were treated with dexamethasone, two with ritodrine, and one with the association of dexamethasone, ritodrine, and terbutaline. In 9 (50 %) cases that presented with, or developed, very poor prognosis factors, such as a ventricular rate below 50-55 bpm and/or the presence of fetal hydrops, parents opted for the termination of pregnancy, after dedicated counseling. Finally, there were nine newborns (seven males [78 %]) with median age at delivery of 37 weeks (range, 32-39). A definitive epicardial pacemaker was placed in six newborns, four of them within 2 weeks of life. CHB is a severe complication related to maternal anti-Ro/SSA and anti-La/SSB antibodies. Our results confirm previous data showing that therapy is ineffective, and most of the surviving patients will require neonatal pacemaker.

Dermatomyositis presenting with severe subcutaneous edema: five additional cases and review of the literature.

OBJECTIVES: Dermatomyositis (DM) constitutes a subset of idiopathic inflammatory myopathies clinically characterized by proximal muscle weakness and skin involvement. Some of the dermatologic manifestations are highly prevalent and characteristic, but others such as generalized or limb edema are truly rare. The aim of the present study was to describe five cases of edematous DM diagnosed at our institution and to perform a review of the literature, as well as identify clinical, laboratory, or pathological data associated with this manifestation. METHODS: We performed a retrospective clinical, laboratory, and pathological evaluation of five cases of this edematous presentation out of 86 DM cases diagnosed at our hospital from 2004 to 2013. Moreover, we undertook a medical literature search using inflammatory myopathy, dermatomyositis, and edema as key words, limited to articles published in MEDLINE, EMBASE, and LILACS database in English and Spanish from 1987 to 2013. RESULTS: A total of 19 patients were identified, five diagnosed at our hospital and 14 cases from the literature. Overall, the median time from disease onset to diagnosis was 2 months, and most of the patients (16/84 patients, 21%) required more aggressive therapy, including immunosuppressive agents and intravenous immunoglobulin (12/63 patients, 15%). Microinfarction was present 2.3 times more frequently in DM patients with edema compared with those without edema. CONCLUSIONS: The presence of edema in DM is uncommon but seems to be a sign of severe disease, requiring early and aggressive treatment. Microischemia-producing microinfarction may play an important pathophysiological role and determine the degree of disease severity.