Neonataler Lupus erythematodes - Eine Übersicht

Zusammenfassung

Es handelt sich beim neonatalen Lupus erythematodes um ein seltenes Krankheitsbild des Feten, Neugeborenen und/oder des jungen Säuglings, verursacht durch spezifische mütterliche Antikörper. Er manifestiert sich vorherrschend mit kardialen und/oder kutanen Symptomen. Die Prognose ist abhängig vom Vorhandensein eines kompletten AV-Blocks. Risikogruppen wie anti-Ro/anti-La und U1-RNP-positive Schwangere sowie Feten mit Bradykardien müssen in Richtung neonataler Lupus erythematodes untersucht und ggf. entsprechend überwacht werden.

Abstract

Neonatal lupus erythematosus is a rare disease of fetus, newborn or young infant caused by specific maternal antibodies. It is characterized by dermatitis and complete congenital heart block, less common by thrombocytopenia, and rarely by cholestatic hepatitis. Prognosis depends on the presence of complete congenital heart block. Pregnant women carrying antibodies to Ro/La or U1-RNP as well as fetus with bradycardia are at risk for neonatal lupus erythematosus. They have to be monitored carefully.

Literatur

• 1 Bierman F Z, Baxi L, Jaffe I. et al . Fetal hydrops and congenital complete heart block: Response to maternal therapy.  J Pediatr. 1988;  122 646-648
• 2 Boutjdir M, Chen L, Zhang Z H, Tseng C, DiDonato F, Rashbaum W, Morris A, El-Sherif N, Buyon J P. Arrhythmogenicity of IgG and anti-52-kD SSA/Ro affinity - purified antibodies from mothers of children with congenital heart block.  Circ Res. 1997;  80 354-362
• 3 Buyon J P, Swersky S H, Fox H E, Bierman F Z, Winchester R J. Intrauterine therapy for presumptive fetal myocarditis with acquired heart block due to systemic lupus erythematosus.  Arthritis Rheum. 1987;  30 44-49
• 4 Buyon J P, Tseng C H, DiDonato F, Rashbaum W, Morris A, Chan E K. Cardiac expression of 52β, an alternative transcript of the congenital heart block-associated 52-kd SS-A/Ro autoantigen, is maximal during fetal development.  Arthritis Rheum. 1997;  40 655-660
• 5 Buyon J P, Hiebert R, Copel J, Craft J, Friedman D, Katholi M, Lee L A, Provost T T, Reichlin M, Rider L, Rupel A, Saleeb S, Weston W L, Skovron M L. Autoimmune-associated congenital heart block: demographics, mortality, morbidity and recurrence rates obtained from a national neonatal lupus registry.  J Am Coll Cardiol. 1998;  31 1658-1666
• 6 Eronen M, Heikkilä P, Teramo K. Congenital complete heart block in the fetus: hemodynamic features, antenatal treatment and outcome in six cases.  Pediatr Cardiol. 2001;  22 385-392
• 7 Hogg G R. Congenital, acute lupus erythematosus associated with subendocardial fibroelastosis. Report of a case.  Am J Clin Pathol. 1954;  28 648
• 8 Kitridou R C. The neonatal lupus syndrom. In: Wallace DJ et al (Hrsg) Dubois Lupus erythematosus, 5. Auflage. Baltimore; Williams and Wilkins 1997: 1023-1035
• 9 McCuistion C H, Schoch E P Jr. Possible discoid lupus erythematosus in newborn infant. Case report with subsequent development of acute systemic lupus erythematosus in mother.  Arch Dermatol. 1954;  70 782-785
• 10 Provost T T, Watson R, Gammon W R, Radowsky M, Harley J B, Reichlin M. The neonatal lupus syndrome associated with U1RNP (nRNP) antibodies.  N Engl J Med. 1987;  316 1135-1138
• 11 Ross B A. Atrioventricular Block. In: Garson et al (Hrsg) The science and practice of pediatric cardiology, Band III. Philadelphia, London; Lea & Febiger 1990: 1803-1806
• 12 Scott J S, Maddison P J, Taylor P J, Escher E, Scott O, Skinner R P. Connective-tissue disease, antibodies to ribonucleoprotein, and congenital heart block.  N Engl J Med. 1983;  39 209-221
• 13 Silverman E D. Neonatal lupus erythematosus. In: Cassidy § Petty (Hrsg) Textbook of Pediatric Rheumatology, 4. Edition. Philadelphia; W. B. Saunders Company 2001: 450-458
• 14 Tran H B, Macardle P J, Hiscock J, Cavill J, Bradley J, Buyon J, Gordon T P. Anti-La/SSB Antibodies transported across the placenta bind apoptoic cells in fetal organs targeted in neonatal lupus.  Arthritis Rheum. 2002;  46 1572-1579
• 15 Walkinshaw S A, Welch C R, McCormack J, Walsh K. In utero pacing for fetal congenital heart block.  Fetal Diag Ther. 1994;  9 183-185