Anti-Ro/SS-A and anti-La/SS-B antibodies associated with cardiac involvement in childhood systemic lupus erythematosus

Abstract

Objectives-To determine the frequency and type of cardiac manifestations in children with systemic lupus erythematosus (SLE) and investigate whether cardiac involvement of SLE in children was associated with any autoantibody pattern. Methods-Retrospective analysis of the medical records of all children with SLE (31 patients) seen between January 1984 and January 1994 by the paediatric rheumatology service at Children's Hospital in New Orleans. All patients satisfied the American College of Rheumatology criteria for the diagnosis of SLE. Paediatric SLE patients with cardiac manifestations based on echocardiogram were identified. Autoantibody tests at diagnosis were identified retrospectively by chart review, and the correlation between autoantibodies and cardiac involvement was analysed using the two tailed Fisher's exact test. Results-Thirteen (42%) of 31 SLE patients had cardiac manifestations of SLE. Seven (22%) had pericarditis without myocarditis, five (16%) had pericarditis and myocarditis, and one (3%) had myocarditis without pericarditis. Two patients (6%) with pericarditis had cardiac tamponade. Cardiac manifestations of SLE usually occurred at the time of diagnosis or within six months. Anti-Ro/SS-A antibodies were present in serum samples of nine of 11 (82%) patients with cardiac involvement and in five of 15 (33%) without cardiac involvement (p=0.02). Anti-La/SS-B antibodies were present in serum samples of six of 10 (60%) patients with cardiac involvement and two of 15 (13%) without cardiac involvement (p=0.03). Anti-Sm and anti-RNP antibodies showed no correlation with the presence of cardiac disease. Conclusions-Cardiac involvement in our paediatric SLE population was frequently found and correlated significantly with the presence of anti-Ro/SS-A and anti-La/ SS-B antibodies.

Concentration of antibodies to extractable nuclear antigens and disease activity in systemic lupus erythematosus

“…3,4 Our data suggest that repeated quantitative measurements of anti-ENA antibodies do not in general correlate with disease activity, as assessed by SLEDAI scores. In a minority of patients, either a positive or inverse association was found, but this was not necessarily consistently seen throughout the course of the study.…”

The use of laboratory tests in the diagnosis of SLE

“…75 Anti-Ro52 in isolation is associated with CHB but is not detected by assays containing only Ro-60, 76 such as HEp-2000 cells.…”

Organ involvement other than lupus nephritis in childhood-onset systemic lupus erythematosus

“…4,12,37 Anti-SSA/SSB antibodies have been found to be associated with cardiac involvement in cSLE 37,38 and anti-malarial therapy protects from future SLE-associated cardiac diseases. 39 …”

“…37,39 The incidence of cSLE-associated pericarditis ranges from 10-38%. 37,[39][40][41] The clinical presentation, electrocardiogram (EKG) and echocardiographic (ECHO) findings of pericarditis due to cSLE are indistinguishable from those from other causes. Tachycardia, fever, substernal chest discomfort, positional chest pain, diminished heart sounds and rarely friction rub are the typical presenting symptoms.…”

“…37,41 Depending on the degree of cardiac dysfunction, clinical features range from mild dyspnea, chest pain, arrhythmias, and heart failure, to cardiac shock and death. Endomyocardial biopsy is considered the gold standard for diagnosis of LM, typically showing focal lymphohistiocytes and plasma cell infiltrates associated with myocyte necrosis.…”