neonatal lupus erythematosus treatment

As anti-Ro/SSA antibodies can be detected in one in 200 pregnant women, the risk for an anti-Ro/SSA-positive woman to have an infant with NLE is relatively low [26]. Neonatal lupus erythematosus (NLE) refers to a clinical spectrum of cutaneous, cardiac, and systemic abnormalities observed in newborn infants whose mothers have autoantibodies against Ro/SSA and La/SSB. J Rheumatol. 5(3):139-48. Morgan TA, Watson L, McCann LJ, Beresford MW. Screening of infants with NLE for the presence of these antibodies is strongly recommended [2]. There is no association with paternal autoimmune diseases [12]. Borgyogaszati Venerol Szemle. 1–3 Rarely, it is also associated with hepatic and hematologic abnormalities. CHICAGO— Neonatal lupus is a rare disorder, but its onset can be dramatic, and it can be fatal. Terzoglou AG, Routsias JG, Avrameas S, Moutsopoulos HM, Tzioufas AG. Lymphopenia is a relatively common finding in adults with SLE but is not a characteristic hematologic abnormality of NLE [26]. 153(7):540. It is a clinical spectrum of cutaneous, cardiac, and systemic abnormalities observed in the newborn or infants whose mothers have autoantibodies against Ro/SSA and/or La/SSB. Alisa N Femia, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Society for Investigative Dermatology, Medical Dermatology Society, Rheumatologic Dermatology SocietyDisclosure: Nothing to disclose. 2016 Mar 9. Laser therapy may be considered for residual telangiectasia. Floristan U, Feltes R, González-Beato M, Feito M, Laguna Rde L. Targetoid lesions and neutrophilic dermatosis within neonatal lupus erythematosus: unusual clinical and histologic presentations. Active erythematous lesions after the first year of life should be suspect. Asboth D, Kassay E, Noll J, Szalai Z. Neonatal lupus erythematosus: deep and ulcerating form. 2014 Feb. 40(1):61-85. On the other hand, anti-U1RNP autoantibodies are usually associated with atypical cutaneous lesions without cardiac or systemic abnormalities in a small number of NLE cases and may play a role in the pathogenesis of thrombocytopenia [10]. [33] Therefore, carefully monitor subsequent pregnancies with serial ultrasonography and echocardiography, particularly at 18-24 weeks gestation. In rare cases, skin lesions may persist into childhood. 2006 Feb. 77(2):82-6. The infants may have no skin lesions at birth but develop them during the first weeks of life. Conduction disturbances may also present as irregular heartbeat and prolongation of the QT interval [24]. 2005 Dec. 44(12):1564-8. Patients with NLE with cardiac involvement require regular monitoring to assess cardiac function and the need for a pacemaker. 2005 Dec. 96(10):690-6. If the cardiac involvement is severe, activity may have to be restricted in the young child. Demaestri M, Sciascia S, Kuzenko A, Bergia R, Barberis L, Lanza MG, et al. Hematologic disturbances (e.g., hemolytic anemia, thrombocytopenia, and neutropenia) may occur in the first 2 weeks of life. [Full Text]. Lupus. [Medline]. Anemia, thrombocytopenia, and neutropenia are self-limited. Pediatr Dermatol. Neonatal lupus erythematosus is a disease of the newborn characterized by congenital heart block and cutaneous lupus lesions. Guettrot-Imbert G, Cohen L, Fermont L, Villain E, Francès C, Thiebaugeorges O, et al. 2009 Mar. Chronic cutaneous lupus in childhood: a report of two cases and review of the literature. Borgyogaszati Venerol Szemle. 2011 Feb. 38(2):378-86. 57(2):261-6. Most patients with NLE affecting liver or blood have transient disease that spontaneously resolves within 4–6 months. Baskin E, Ozen S, Cakar N, Bayrakci US, Demirkaya E, Bakkaloglu A. 2000 Oct. 30(10):2782-90. Increased risk of complete congenital heart block in infants born to women with hypothyroidism and anti-Ro and/or anti-La antibodies. Annular erythematous or polycystic plaques with or without fine scales characterize NLE and appear predominately on the scalp, neck, or face (typically periorbital in distribution), but similar plaques may appear on the trunk or extremities [10, 26]. The transplacental passage of these autoantibodies is necessary but not sufficient to cause the disease. For the 40% of women with systemic lupus erythematosus who have antibodies reactive with SSA/Ro, at least one in 50 will have a child with neonatal lupus (NL). Carreno L, Lopez-Longo FJ, Gonzalez CM, Monteagudo I. Skin biopsy is useful in patients with NLE when the diagnosis is in doubt. The latter site may occasionally be associated with a permanent alopecia. The condition is rare and usually benign and self-limited but sometimes may be associated with serious sequelae. Children with NLE have an excellent long-term outcome when only skin lesions are present [36]. Arthritis Rheum. Strategies aimed at preventing disease before irrevocable scarring ensues are a high priority. 62(4):1138-46. B. Cervini, and A. M. Pierini, “Neonatal lupus erythematosus: a report of four cases,”, C. H. McCuistion and E. P. Schoch Jr., “Possible discoid lupus erythematosus in newborn infant: report of a case with subsequent development of acute systemic lupus erythematosus in mother,”, J. P. Buyon and R. M. Clancy, “Neonatal lupus syndromes,”, K. Ayed, Y. Gorgi, I. Sfar, and M. Khrouf, “Congenital heart block associated with maternal anti SSA/SSB antibodies: a report of four cases,”, L. Li, G. F. Dong, F. Z. Han, Y. Cui, Y. In some infants, solar exposure seems to precipitate the eruption [30]. Multidisciplinary team involvement may also be indicated. Chen CC, Lin KL, Chen CL, Wong AM, Huang JL. Tincani et al. Anti-Ro52/SSA autoantibodies antagonize the serotonin-induced L-type calcium channel activation on human fetal atrial cells and trigger an inflammatory response, leading ultimately to fibrosis and scarring of the atrioventricular node, sinus node, and His bundle [9, 10]. 2002 Actas Dermosifiliogr. A central role of plasmin in cardiac injury initiated by fetal exposure to maternal anti-Ro autoantibodies. In typical cases of NLE and positive autoantibodies, skin biopsy is not mandatory to confirm the diagnosis. These children were found to have a normal intelligence quotient [45]. In addition to its presence in the skin and heart, the Ro antigen is also found in the liver, bowel, lungs, brain, and blood cells—the tissues that are most often affected by NLE [3]. [Medline]. However, only some neonates exposed to these antibodies develop complications. Bullous lesions may be seen with a particular predilection for the soles of the feet [25]. Neonatal lupus erythematosus is a rare disease that can affect different organs, mainly the skin and heart. 1994 Mar. [Medline]. Rivera TL, Izmirly PM, Birnbaum BK, et al. 62(4):1147-52. 1999 Jun. 30(3):510. Cardiac manifestations of neonatal lupus erythematosus: guidelines to management, integrating clues from the bench and bedside. Jump to Content Jump to Main Navigation. Telangiectasia, scarring, and atrophic changes are expected to persist. Asboth D, Kassay E, Noll J, Szalai Z. Neonatal lupus erythematosus: deep and ulcerating form. However, the authors remarked that information about long-term outcome of children exposed to immunosuppressive drugs “in utero” are still lacking, and more efforts are needed in this research area. Neonatal lupus erythematosus in identical twins, showing transient bullous lesions. Janet Fairley, MD Professor and Head, Department of Dermatology, University of Iowa, Roy J and Lucille A Carver College of Medicine, Janet Fairley, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Federation for Medical Research, and Society for Investigative Dermatology, Jack Grzybowski, MD Staff Physician, Department of Pediatrics, University of Medicine and Dentistry of New Jersey-New Jersey Medical School, Jack Grzybowski, MD is a member of the following medical societies: Sigma Xi, William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine, William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology, Barry L Myones, MD Associate Professor, Departments of Pediatrics and Immunology, Pediatric Rheumatology Section, Baylor College of Medicine; Director of Research, Pediatric Rheumatology Center, Texas Children's Hospital, Barry L Myones, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Immunologists, American College of Rheumatology, American Heart Association, American Society for Microbiology, Clinical Immunology Society, and Texas Medical Association, Robert A Schwartz, MD, MPH Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School, Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi, Michael J Wells, MD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine, Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association, Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference. 332244-overview 76:263-5. [Medline]. Thrombocytopenia may manifest as petechiae. [34] While neither of 2 recent studies demonstrated benefit in outcome IVIG, [32], The use of hydroxychloroquine for anti-Ro/SSA–positive mothers with systemic lupus erythematosus (SLE) has been associated with a lower rate of NLE during pregnancy. Arthritis Rheum. J Am Acad Derma-tol 1999;40:675–81. 2013 Aug 21. 2002 Sep. 46(9):2377-83. Silverman E, Jaeggi E. Non-cardiac manifestations of neonatal lupus erythematosus. J Rheumatol. [Medline]. Mothers of infants with NLE, particularly infants with congenital heart block, have a 2-fold to 3-fold increased risk of having an affected infant in a subsequent pregnancy. Laboratory investigations may reveal pancytopenia, thrombocytopenia, leukopenia, or elevated transaminase level [34]. Lupus 1997;6:132–8. 2012, Article ID 301274, 6 pages, 2012. https://doi.org/10.1155/2012/301274, 1Department of Pediatrics, The Chinese University of Hong Kong, Prince of Wales Hospital, 6/F, Clinical Sciences Building, Shatin, Hong Kong, 2Department of Pediatrics, Alberta Children’s Hospital, The University of Calgary, Calgary, AB, Canada. NLE is a rare acquired autoimmune disease that occurs in 1 of every 20,000 live births in the USA [5]. Deaths may also occur later in life as a result of the failure of the pacemaker. Therefore, other factors such as titers of maternal antibodies, genetic predisposition, and environmental factors such as viral infection may be involved. Additionally, induction of apoptosis in cultured cardiomyocytes has been demonstrated to result in the expression of Ro/La antigens on the cell surface for recognition by circulating maternal antibodies [15]. Ruth Ann Vleugels, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Rheumatology, American Medical Association, Society for Investigative Dermatology, Medical Dermatology Society, Dermatology FoundationDisclosure: Nothing to disclose. [40] Although the child may not be at increased risk of developing SLE, the development of some form of autoimmune disease in early childhood may be of concern. In some cases, myocarditis and pericarditis can develop which may lead to bradycardia. Protective clothing is highly desirable. Eur J Pediatr. 2011 Feb 8. Department of Pediatrics, The Chinese University of Hong Kong, Prince of Wales Hospital, 6/F, Clinical Sciences Building, Shatin, Hong Kong, Department of Pediatrics, Alberta Children’s Hospital, The University of Calgary, Calgary, AB, Canada, S. Garcia and A. C. Campos-de-Carvalho, “Neonatal lupus syndrome: the heart as a target of the immune system,”, L. K. Hornberger and N. Al Rajaa, “Spectrum of cardiac involvement in neonatal lupus,”, M. F. Perez, M. E. de Torres, M. M. Buján, A. Lanoël, A. One or more systems may be involved. 11(4):257-60. [Medline]. [Medline]. 2006 Jan. 33(1):167-70. Successful preventive treatment of congenital heart block during pregnancy in a woman with systemic lupus erythematosus and anti-Sjögren's syndrome A/Ro antibody. Br J Dermatol. Neonatal lupus erythematosus (NLE) is a disease of the newborn defined by the presence of maternal autoantibodies and characteristic clinical features in the neonatal period.