Arterial Thrombosis as a Presentation of Systemic Lupus Erythematosus in a Young Child

Introduction: Systemic lupus erythematosus (SLE) is a rare diagnosis in children and can present with nonspecific symptoms. Similarly, arterial thromboses in children without risk factors is also rare. Together, they present a diagnostic challenge which may lead to a delay in proper management. Our objective is to emphasize the importance of having high suspicion for SLE in children, especially when presenting with arterial thrombosis.

Case Description: Our patient is a 6-year-old female who presented with a 3-week history of severe right foot pain with a 1-week history of discoloration in the same foot. She sought care multiple times prior to presentation. She initially received the diagnosis of “COVID toes” despite a negative COVID PCR. Prior to presentation, she developed fevers, worsening foot pain, increasing discoloration, decreased oral intake, weight loss, and fatigue. Further lab work showed acute kidney injury, elevated inflammatory markers, and coagulopathy. She was also found to have elevated troponins and QTc prolongation. Additionally, a lower extremity Doppler demonstrated an acute partially occluding thrombus in her right popliteal artery.

She was transferred to the pediatric intensive care unit (PICU) and started on a heparin infusion. An Echocardiogram with bubble study showed no interatrial shunting. Thrombolysis was considered but held due to concerns regarding thrombus chronicity. COVID-19 PCR and antibodies were negative, so Infectious Disease team determined this was unlikely related to an acute or remote COVID-19 infection or multisystem inflammatory syndrome in children (MIS-C). Her hemoglobin and platelets began to downtrend and Coombs was positive, raising concern for autoimmune hemolytic anemia. Autoimmune and coagulopathy work-up was significant for positive ANA titer, low complement levels and elevated anticardiolipin, anti-dsDNA, anti-Smith, anti-chromatin, and anti-RNP antibodies. She also had a chest X-ray that showed small non-infectious pleural and pericardial effusions suggestive of serositis. The constellation of these findings eventually led to the diagnosis of SLE.

Discussion: There have been several cases of thrombotic or thromboembolic events reported in pediatric patients with SLE, with a majority being venous related events. Only two reports involving cerebral arteries have been published. For several of these patients, thrombosis was the presenting symptoms of their disease. In our literature review, we did not find any cases of arterial thrombosis outside the central nervous system related to SLE.

Conclusion: Arterial thrombosis can be a presenting symptom for SLE in children. Despite being a rare presentation, rheumatologic diseases such as SLE should always be considered to prevent a delay in diagnosis and management. In our case, our patient experienced a notable delay in care due to a misdiagnosis related to the COVID-19 pandemic. While it is extremely important to consider sequelae of COVID-19, we would like to emphasize the importance of ensuring consideration of other diagnoses as well.