J Rheum Dis.  2014 Jun;21(3):156-161. 10.4078/jrd.2014.21.3.156.

An Unusual Case of Severe Neonatal Lupus Mimicking Neonatal Sepsis and Literature Review

• Park HM
• Jun HG
• Park JH
• Choi SW
• Oh KW
• Oh JS

• KMID: 2297529
• DOI: http://doi.org/10.4078/jrd.2014.21.3.156

Abstract

Neonatal lupus is an uncommon autoimmune disease that results from transplacental passage of the maternal anti-SSA/Ro and/or anti-SSB/La antibodies. Pancytopenia or severe jaundice is a rare manifestation of neonatal lupus, respectively, and could be misdiagnosed with other neonatal illnesses, such as infection, hematologic disease, or hepatobiliary disease. Here, we report an unusual case of a premature newborn with severe neonatal lupus manifested with skin rash, fever, pancytopenia, and severe jaundice with abnormal liver function tests. His mother had been clinically asymptomatic before delivery; however, she revealed peripheral edema, bilateral pleural effusion, and ascites after delivery and diagnosed with systemic lupus erythematosus based on positive anti-nuclear and anti-cardiolipin antibodies, proteinuria, and serositis. The newborn and his mother had anti-SSA/Ro and anti-SSB/La antibodies. His pancytopenia and jaundice were progressively aggravated, and his illness was confused with neonatal sepsis or biliary obstruction. We decided to treat with high dose of corticosteroid and intravenous immunoglobulin, and he gradually recovered completely with the treatment. His corticosteroid was stopped at 5 months of age without relapse or complication.

Keyword

Neonatal lupus; Severe jaundice; Pancytopenia; Steroid

MeSH Terms

Antibodies
Ascites
Autoimmune Diseases
Edema
Exanthema
Fever
Hematologic Diseases
Humans
Immunoglobulins
Infant, Newborn
Jaundice
Liver Function Tests
Lupus Erythematosus, Systemic
Mothers
Pancytopenia
Pleural Effusion
Proteinuria
Recurrence
Sepsis*
Serositis
Antibodies
Immunoglobulins

Figure

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  Figure 1. Skin rash at admission. Patient showed multiple reddish scaly patches on the face, trunk and extremities at admission.

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  Figure 2. Hepatobiliary scan. In hepatobiliary scan, there was no distinct focal tracer activity in gallbladder and delayed tracer activity in gastrointestinal tract.

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  Figure 3. Changes of laboratory tests before and after treatments. Pancytopenia, hyperbilirubinemia and elevated transaminases improved after use of methylprednisolone. Arrows indicate blood transfusion. WBC, white blood cell; Hb, hemoglobin; PLT, platelet; IVIg, intravenous immunoglobulin; G-CSF, granulocyte colony stimulating factor; AST, aspartate aminotransferase; ALT, alanine aminotransferase.

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