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

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.

Pneumatosis Cystoides Intestinalis with Pneumoperitoneum Complicating Systemic Sclerosis: A Case Report / 대한내과학회지

The gastrointestinal tract is commonly involved in patients with systemic sclerosis. The manifestations include motility disorder, pseudo-obstruction, malabsorption, bacterial overgrowth, diverticuli, and, less commonly, pneumatosis cystoides intestinalis (PCI). PCI is characterized by the presence of air in the submucosal or subserosal layer of the bowel wall and is often accompanied by pneumoperitoneum. Although PCI is a benign condition that often responds to conservative management, it is a poor prognostic factor of systemic sclerosis. We report a case of PCI in a patient with systemic sclerosis. The chest and abdominal radiographic findings comprised pneumoperitoneum, marked dilation of the bowels, and intramural air, compatible with PCI. The patient's symptoms improved spontaneously. It is important to recognize PCI as a gastrointestinal manifestation of systemic sclerosis, and physicians should differentiate it from serious complications-such as intestinal perforation-based on clinical manifestations.