scholarly journals Fetal Congenital Heart Block Associated With Maternal Primary Systemic Lupus Erythematosus and Sjogren’s Syndrome

Cureus ◽  
2021 ◽  
Author(s):  
Sameera Khan ◽  
Priyanka Anvekar ◽  
Petras Lohana ◽  
Mohammed Sheeraz Alam ◽  
Syed R Ali
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Heart Block ◽  
Congenital Heart ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Congenital Heart Block ◽  
Systemic Lupus ◽  
Sjögren‘S Syndrome

Neonatal lupus erythematosus – practical guidelines

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Julia Marta Derdulska ◽  
Lidia Rudnicka ◽  
Agata Szykut-Badaczewska ◽  
Dorota Mehrholz ◽  
Roman Nowicki ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Heart Block ◽  
Congenital Heart ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Congenital Heart Block ◽  
Neonatal Lupus ◽  
Neonatal Lupus Erythematosus ◽  
Sjögren‘S Syndrome

Abstract Background Neonatal lupus erythematosus is an autoimmune disease acquired during fetal life as a result of transplacental passage of maternal anti-Sjögren’s-syndrome-related antigen A (anti-SSA/Ro), anti-Sjögren’s-syndrome-related antigen B (anti-SSB/La) or anti-U1 ribonucleoprotein (anti-U1-RNP) antinuclear autoantibodies. Contents Clinical manifestations include skin lesions, congenital heart block, hepatobiliary involvement and cytopenias. Most of the disorders disappear spontaneously after clearance of maternal antibodies. Cardiac symptoms, however, are not self-resolving and often pacemaker implantation is required. Diagnosis is based on clinical presentation and the presence of typical antibodies in the mother’s or infant’s serum. Outlook Neonatal lupus erythematosus may develop in children born to anti-SSA/Ro or anti-SSB/La women with various systemic connective tissue diseases. However, in half of the cases, the mother is asymptomatic, which may delay the diagnosis and have negative impact on the child’s prognosis. Testing for antinuclear antibodies should be considered in every pregnant woman since early treatment with hydroxychloroquine or intravenous immunoglobulin (IVIG) has proven to be effective in preventing congenital heart block.

scholarly journals Doença Intersticial Pulmonar Grave e Mania Induzida por Corticosteróides em Doente com Lúpus Eritematoso Sistémico e Síndrome de Sjögren Secundária

2017 ◽  
Vol 30 (3) ◽  
pp. 246 ◽  
Author(s):  
Sofia Silvério Serra ◽  
Teresa Pedrosa ◽  
Sandra Falcão ◽  
Jaime Cunha Branco
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Sjögren’S Syndrome ◽  
Lupus Erythematosus ◽  
Sjögren's Syndrome ◽  
Manic Episode ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Neurological Involvement ◽  
Systemic Lupus ◽  
Sjögren‘S Syndrome

Interstitial lung disease occurs in up to 25% of patients with Sjögren’s syndrome and 2% - 8 % of patients with systemic lupus erythematosus. Corticosteroid therapy remains the main treatment for systemic lupus erythematosus. However, it can be associated with several neuropsychiatric disorders especially with prednisolone at a dose of more than 40 mg/day. We present the case of a 51-year-old patient with systemic lupus erythematosus and secondary Sjögren’s syndrome with severe pulmonary involvement four years after the diagnosis. Chest computed tomography revealed neofibrosis and ground glass appearance pattern. After increasing the dose of prednisolone to 60 mg/day, the patient presented a manic episode. There was need of hospitalization and the situation was considered to be secondary to corticosteroids at high doses. Central neurological involvement by organic disease was excluded.We introduced monthly perfusion of cyclophosphamide for six months and later started mycophenolate mofetil 2 g/day, reducing prednisolone to 10 mg/day and maintaining hydroxychloroquine 400 mg/day, with control of disease activity.

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