Gastrointestinal Manifestations of Pediatric Lupus Erythematosus

Source: Sönmez HE, Batu ED, İşgüder R, et al. Exploring gastrointestinal manifestations in childhood onset systemic lupus erythematosus — insights from a multicenter study. Lupus. 2024;33(12):1358-1364; doi: 10.1177/09612033241279071.Investigators from Turkey conducted a retrospective study to describe gastrointestinal (GI) manifestations among children with systemic lupus erythematosus (SLE). Study participants were patients with childhood-onset SLE and GI system (GIS) abnormalities seen at 1 of 16 centers in Turkey. Specific criteria were used to confirm the diagnosis of SLE. The medical records of participants were reviewed and demographic and clinical information abstracted. GIS manifestations were categorized as related to SLE, related to drug treatment for SLE, or as comorbidities. GIS manifestations were considered to be related to drug treatment when they developed 1 week to 3 months after treatment initiation and resolved after the medication was discontinued. GIS manifestation was classified as being present at the time of SLE diagnosis or developing subsequently.A total of 123 patients with SLE and GIS manifestations were included in study analyses; 101 (82.1%) were females. The mean age at the time of SLE diagnosis was 12.5 ± 3.5 years, and median follow-up was 44.5 months. GIS manifestations were present at the time of diagnosis in 78 (63.4%) patients and developed a median of 12 months later in 45 (36.6%). Overall, GIS manifestations were related to SLE in 82 (66.7%) study participants, drug treatment in 35 (28.5%), and from a comorbidity in 6 (0.5%). The GIS manifestations resulted in symptoms in 82 (66.7%) children and were discovered through laboratory testing in the remaining patients. The most common GIS manifestations were abdominal pain (occurring in 62.2% of study participants) and elevated liver transaminases (56.9%). Among the 82 patients with SLE-related GIS manifestations, 31 were diagnosed with autoimmune hepatitis, 16 with hepatic steatosis, and 14 with lupus hepatitis. The most common drug adverse events were steroid-related gastritis, occurring in 19 patients, and toxic hepatitis, in 11. In addition to corticosteroids-related gastritis, toxic hepatitis was associated with hydroxychloroquine in 4 patients, methotrexate in 5, and azathioprine in 2.The authors conclude that evaluation of GIS manifestations is warranted in children with SLE. Conversely, a diagnosis of SLE should be considered in a pediatric patient with unexplained GI symptoms.Dr Doolittle has disclosed no financial relationship relevant to this commentary. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.The current study highlights the broad, protean manifestations of SLE. In particular, the investigators note that GIS symptoms and laboratory abnormalities are common among children with SLE. The diagnosis of SLE often is difficult to make among children and may take weeks to months to make because of the fluctuating symptoms.1 Since the underlying pathophysiology is an auto-immune reaction to intracellular DNA, the patient can manifest symptoms in nearly every organ system, as well as global symptoms of fever and fatigue.1 In 2019, new criteria proposed by the European League Against Rheumatism/American College of Rheumatology include a positive antinuclear antibody (ANA) and a weighted score of physical manifestations, which include dermatologic, renal, cardiac, neurologic, and hematologic findings.1SLE among children is rare, with only 5,000 to 10,000 children afflicted in the US and a worldwide prevalence of only 3.3 to 9.7 per 100,000 children.2,3 The results of 1 study revealed that 17% of children exhibited GIS manifestations as the initial presentation of their SLE.4 Other investigators found that GIS manifestations were common among SLE patients—as high as 68.4% to 82%.5,6 Of note, GIS manifestations are not included as diagnostic criteria.1The etiology of GIS manifestations in children with SLE is varied. As noted by the current authors, some cases are due to a primary auto-immune process, such as auto-immune hepatitis; other GIS manifestations are due to side effects of medications, such as steroid-induced gastritis and vasculitis-serositis. While the current investigators propose that clinicians have an awareness of GIS symptoms as a clue to possible SLE, it would be unusual for a patient with SLE to manifest only GIS abnormalities.GIS manifestations are common among children with SLE, with varied etiology, and merit evaluation. Similarly, abdominal discomfort may merit evaluation for SLE.The current study, whose strength lies in its broad patient base, illuminates the spectrum of GIS abnormalities in Turkish children with SLE. However, the applicability of the results to North American pediatric patients with SLE may be limited by ethnic differences. Black Americans and Hispanics are disproportionately diagnosed with SLE and have more severe disease manifestations.7