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{{WBRQuestion
{{WBRQuestion
|QuestionAuthor=Mahmoud Sakr M.D.
|QuestionAuthor=Mahmoud Sakr M.D. (Reviewed by  {{YD}})
|ExamType=USMLE Step 1
|ExamType=USMLE Step 1
|MainCategory=Immunology, Pathophysiology
|MainCategory=Immunology, Pathophysiology
|SubCategory=Cardiology, Dermatology
|SubCategory=Musculoskeletal/Rheumatology
|MainCategory=Immunology, Pathophysiology
|MainCategory=Immunology, Pathophysiology
|SubCategory=Cardiology, Dermatology
|SubCategory=Musculoskeletal/Rheumatology
|MainCategory=Immunology, Pathophysiology
|MainCategory=Immunology, Pathophysiology
|SubCategory=Cardiology, Dermatology
|SubCategory=Musculoskeletal/Rheumatology
|MainCategory=Immunology, Pathophysiology
|MainCategory=Immunology, Pathophysiology
|MainCategory=Immunology, Pathophysiology
|MainCategory=Immunology, Pathophysiology
|SubCategory=Cardiology, Dermatology
|MainCategory=Immunology, Pathophysiology
|MainCategory=Immunology, Pathophysiology
|SubCategory=Cardiology, Dermatology
|SubCategory=Musculoskeletal/Rheumatology
|MainCategory=Immunology, Pathophysiology
|MainCategory=Immunology, Pathophysiology
|SubCategory=Cardiology, Dermatology
|SubCategory=Musculoskeletal/Rheumatology
|MainCategory=Immunology, Pathophysiology
|MainCategory=Immunology, Pathophysiology
|SubCategory=Cardiology, Dermatology
|SubCategory=Musculoskeletal/Rheumatology
|MainCategory=Immunology, Pathophysiology
|MainCategory=Immunology, Pathophysiology
|SubCategory=Musculoskeletal/Rheumatology
|MainCategory=Immunology, Pathophysiology
|MainCategory=Immunology, Pathophysiology
|SubCategory=Cardiology, Dermatology
|MainCategory=Immunology, Pathophysiology
|Prompt=A few hours old neonate who’s born to a mother with a history of systemic lupus erythematosus was severely bradycardic and had to be rushed to the operating room for a pacemaker placement. An EKG prior to the procedure revealed complete heart block. The baby is also noted to have several erythematous annular lesions with slight central atrophy and raised active margins that are located primarily on the scalp and periorbital area. Screening of which of the following antibodies may have helped in early treatment and prevention of this emergency situation:
|SubCategory=Musculoskeletal/Rheumatology
|Explanation=Neonatal lupus is responsible for 80 to 95 percent of all cases of congenital complete heart block diagnosed in utero or in the neonatal period. The characteristic skin rash, and maternal history of SLE in addition to congenital heart block makes the diagnosis of neonatal lupus very likely. Neonatal lupus (NL) is presumed to result from transplacental passage of maternal anti-SSA/Ro and/or anti-SSB/La antibodies. Most infants with complete heart block have a mother with anti-SSA/Ro and anti-SSB/La antibodies. Screening for those antibodies would have been appropriate and may have lead initiation of in utero mineralcorticoids which has a potential usefulness in improving outcomes of neonates with NL.
|Prompt=A female neonate with severe bradycardia is rushed to the operating room for pacemaker placement. She is born to a 25-year-old African-American mother who has a medical history significant for an autoimmune disease with chronic joint pains, malar rash, and photosensitivity. Upon further investigation, the mother admits she has not received any prenatal care. Physical examination of the neonate is remarkable for severe bradycardia and presence of erythematous annular lesions with central clearing and raised red borders on the face and the scalp. An ECG prior to the procedure reveals a complete dissociation between the P waves and the QRS complexes. Screening for which auto-antibodies in the mother could have prevented this patient's condition?
|AnswerA=Anti-SSA/Ro and/or anti-SSB/La antibodies
|Explanation=Neonatal lupus is a relatively rare disease of the newborn characterized by cutaneous lesions and third-degree heart block, along with dilated cardiomyopathy, hepatobiliary disease, and hematological abnormalities. Although cutaneous lupus lesions often appear several days later, patients may develop lesions immediately after birth. The lesions are typically described similarly to those described in this patient, showing annular erythema with central clearing and raised red borders. Classically, congenital third-degree (complete) heart block begins in utero and persists antenatally. On ECG, third-degree heart appears as a complete dissociation between the P waves and the QRS complexes. The presence of heart block has been attributed to fibrosis of the AV node region. Patients usually require pacemaker placement, and if left untreated, the majority of patients die.
|AnswerAExp=Neonatal lupus (NL) is presumed to result from transplacental passage of maternal anti-SSA/Ro and/or anti-SSB/La antibodies.
 
Neonatal lupus is responsible for 80 to 95% of all cases of congenital complete heart block diagnosed in utero or in the neonatal period. The characteristic skin rash, the maternal history consistent with systemic lupus erythematosus (autoimmune disease characterized by joint paints, malar rash, and photosensitivity in an African-American patient), and the congenital heart block makes the diagnosis of neonatal lupus very likely. Neonatal lupus (NL) results from the transplacental passage of maternal anti-SSA/Ro and/or anti-SSB/La antibodies. The presence of anti-Ro-antibodies is considered the most significant risk factor for the development of neonatal lupus. Most infants with complete heart block have a mother with anti-SSA/Ro and anti-SSB/La antibodies, and screening for those antibodies may prevent the patient's condition by administration of systemic corticosteroids, which has demonstrated efficacy in improving outcomes in neonatal lupus.
|AnswerA=Anti-Ro antibodies
|AnswerAExp=Neonatal lupus (NL) results from the transplacental passage of maternal anti-SSA/Ro and/or anti-SSB/La antibodies.
|AnswerB=Anti-RNP antibodies
|AnswerB=Anti-RNP antibodies
 
|AnswerBExp=Anti-RNP antibodies are autoantibodies associated with mixed connective tissue disease and are also detected in nearly 40% of patients with SLE. However, they're not classically associated with NL
 
|AnswerBExp=Anti-RNP antibodies are autoantibodies associated with mixed connective tissue disease and are also detected in nearly 40% of Lupus erythematosus patients. However, they're not the most common type of autoantibodies associated with NL
|AnswerC=Anti-double-stranded DNA (dsDNA) antibodies
|AnswerC=Anti-double-stranded DNA (dsDNA) antibodies
 
|AnswerCExp=Anti-dsDNA antibodies are very specific for SLE and are therefore used in the diagnosis of SLE. However, they are not the most common type of autoantibodies associated with NL
|AnswerCExp=Anti-dsDNA antibodies are very specific for SLE, with studies quoting nearly 100%, and are therefore used in the diagnosis of SLE. Higher titres of anti-dsDNA antibodies are more suggestive of SLE and lower titres can be found in people without the disease. In contrast to the high specificity, estimates of 25-85% have been observed for the sensitivity of anti-dsDNA in SLE. However, they're not the most common type of autoantibodies associated with NL
|AnswerD=Anti-ribosomal P protein antibodies
|AnswerD=Anti-ribosomal P protein antibodies
 
|AnswerDExp=Anti-ribosomal P protein antibodies are autoantibodies present in a minority of patients with SLE. They are highly specific for SLE, not NL
|AnswerDExp=Anti-ribosomal P protein antibodies are autoantibodies directed against three phosphorylated protein (“P protein”) components of ribosomes are present in a minority of patients with systemic lupus erythematosus (SLE) and are highly specific for SLE, not NL
|AnswerE=Antiphospholipid antibodies
|AnswerE=Antiphospholipid antibodies
|AnswerEExp=Antiphospholipid syndrome is an autoimmune, hypercoagulable state caused by antiphospholipid antibodies. APS provokes blood clots (thrombosis) in both arteries and veins as well as pregnancy-related complications such as miscarriage, stillbirth, preterm delivery, or severe preeclampsia.
|AnswerEExp=Antiphospholipid syndrome is an autoimmune disease caused by antiphospholipid autoantibodies. APS is a hypercoagulable state and results in arterial and venous thrombosis and pregnancy-related complications, such as recurrent miscarriages.
|EducationalObjectives=Neonatal lupus (NL) results from the transplacental passage of maternal anti-SSA/Ro and/or anti-SSB/La antibodies. The presence of anti-Ro-antibodies is considered the most significant risk factor for the development of neonatal lupus. Neonatal lupus is a relatively rare disease of the newborn characterized by cutaneous lesions and third-degree heart block, along with dilated cardiomyopathy, hepatobiliary disease, and hematological abnormalities.
|References=Finkelstein Y, Adler Y, Harel L, Nussinovitch M, Youinou P. Anti-Ro (SSA) and anti-La (SSB) antibodies and complete congenital heart block. Ann Med Interne. 1997;148(3):205-8.<br>
Lee LA. Neonatal lupus erythematosus: clinical findings and pathogenesis. J Investig Dermatol Symp Proc. 2004;9:52-6.<br>
Lee LA. Neonatal lupus. clinical features and management. Paediatr Drugs. 2004;6(2):71-8.<br>
First Aid 2014 page 425<br>
|RightAnswer=A
|RightAnswer=A
|WBRKeyword=Lupus, Neonatal lupus, Systemic lupus erythematosus, Autoimmune, Autoantibody, Systemic lupus erythematosus, SLE, Congenital heart block, Third degree heart block, Complete heart block
|Approved=Yes
|Approved=Yes
}}
}}

Latest revision as of 23:41, 27 October 2020

 
Author [[PageAuthor::Mahmoud Sakr M.D. (Reviewed by Yazan Daaboul, M.D.)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Immunology, MainCategory::Pathophysiology
Sub Category SubCategory::Musculoskeletal/Rheumatology
Prompt [[Prompt::A female neonate with severe bradycardia is rushed to the operating room for pacemaker placement. She is born to a 25-year-old African-American mother who has a medical history significant for an autoimmune disease with chronic joint pains, malar rash, and photosensitivity. Upon further investigation, the mother admits she has not received any prenatal care. Physical examination of the neonate is remarkable for severe bradycardia and presence of erythematous annular lesions with central clearing and raised red borders on the face and the scalp. An ECG prior to the procedure reveals a complete dissociation between the P waves and the QRS complexes. Screening for which auto-antibodies in the mother could have prevented this patient's condition?]]
Answer A AnswerA::Anti-Ro antibodies
Answer A Explanation AnswerAExp::Neonatal lupus (NL) results from the transplacental passage of maternal anti-SSA/Ro and/or anti-SSB/La antibodies.
Answer B AnswerB::Anti-RNP antibodies
Answer B Explanation AnswerBExp::Anti-RNP antibodies are autoantibodies associated with mixed connective tissue disease and are also detected in nearly 40% of patients with SLE. However, they're not classically associated with NL
Answer C AnswerC::Anti-double-stranded DNA (dsDNA) antibodies
Answer C Explanation AnswerCExp::Anti-dsDNA antibodies are very specific for SLE and are therefore used in the diagnosis of SLE. However, they are not the most common type of autoantibodies associated with NL
Answer D AnswerD::Anti-ribosomal P protein antibodies
Answer D Explanation AnswerDExp::Anti-ribosomal P protein antibodies are autoantibodies present in a minority of patients with SLE. They are highly specific for SLE, not NL
Answer E AnswerE::Antiphospholipid antibodies
Answer E Explanation AnswerEExp::Antiphospholipid syndrome is an autoimmune disease caused by antiphospholipid autoantibodies. APS is a hypercoagulable state and results in arterial and venous thrombosis and pregnancy-related complications, such as recurrent miscarriages.
Right Answer RightAnswer::A
Explanation [[Explanation::Neonatal lupus is a relatively rare disease of the newborn characterized by cutaneous lesions and third-degree heart block, along with dilated cardiomyopathy, hepatobiliary disease, and hematological abnormalities. Although cutaneous lupus lesions often appear several days later, patients may develop lesions immediately after birth. The lesions are typically described similarly to those described in this patient, showing annular erythema with central clearing and raised red borders. Classically, congenital third-degree (complete) heart block begins in utero and persists antenatally. On ECG, third-degree heart appears as a complete dissociation between the P waves and the QRS complexes. The presence of heart block has been attributed to fibrosis of the AV node region. Patients usually require pacemaker placement, and if left untreated, the majority of patients die.

Neonatal lupus is responsible for 80 to 95% of all cases of congenital complete heart block diagnosed in utero or in the neonatal period. The characteristic skin rash, the maternal history consistent with systemic lupus erythematosus (autoimmune disease characterized by joint paints, malar rash, and photosensitivity in an African-American patient), and the congenital heart block makes the diagnosis of neonatal lupus very likely. Neonatal lupus (NL) results from the transplacental passage of maternal anti-SSA/Ro and/or anti-SSB/La antibodies. The presence of anti-Ro-antibodies is considered the most significant risk factor for the development of neonatal lupus. Most infants with complete heart block have a mother with anti-SSA/Ro and anti-SSB/La antibodies, and screening for those antibodies may prevent the patient's condition by administration of systemic corticosteroids, which has demonstrated efficacy in improving outcomes in neonatal lupus.
Educational Objective: Neonatal lupus (NL) results from the transplacental passage of maternal anti-SSA/Ro and/or anti-SSB/La antibodies. The presence of anti-Ro-antibodies is considered the most significant risk factor for the development of neonatal lupus. Neonatal lupus is a relatively rare disease of the newborn characterized by cutaneous lesions and third-degree heart block, along with dilated cardiomyopathy, hepatobiliary disease, and hematological abnormalities.
References: Finkelstein Y, Adler Y, Harel L, Nussinovitch M, Youinou P. Anti-Ro (SSA) and anti-La (SSB) antibodies and complete congenital heart block. Ann Med Interne. 1997;148(3):205-8.
Lee LA. Neonatal lupus erythematosus: clinical findings and pathogenesis. J Investig Dermatol Symp Proc. 2004;9:52-6.
Lee LA. Neonatal lupus. clinical features and management. Paediatr Drugs. 2004;6(2):71-8.
First Aid 2014 page 425
]]

Approved Approved::Yes
Keyword WBRKeyword::Lupus, WBRKeyword::Neonatal lupus, WBRKeyword::Systemic lupus erythematosus, WBRKeyword::Autoimmune, WBRKeyword::Autoantibody, WBRKeyword::Systemic lupus erythematosus, WBRKeyword::SLE, WBRKeyword::Congenital heart block, WBRKeyword::Third degree heart block, WBRKeyword::Complete heart block
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