Severe gastrointestinal manifestations in childhood-onset lupus: A single-center cohort and review of the literature

Abstract

ObjectiveSystemic lupus erythematosus (SLE) is a complex autoimmune condition with diverse manifestations. Childhood-onset SLE (cSLE) presents more severely than adult-onset disease with a higher incidence of severe neurologic or renal manifestations and lower estimated survival rates. There is limited literature on the range of severe gastrointestinal manifestations at presentation in cSLE, and this lack of information results in underappreciation of these potentially life-threatening complications and little guidance regarding management of these patients.MethodsWe reviewed cases of patients with cSLE and severe GI manifestations at presentation diagnosed at our institution and additionally provide a comprehensive review of existing cases in the literature to discuss presenting symptoms, other clinical features, longitudinal course, treatment, and long-term outcomes.ResultsWe identified six cases of cSLE with primary, severe GI symptoms at time of diagnosis at our institution and an additional 25 patients who presented similarly in the literature. Severe GI manifestations included protein-losing enteropathy, thoracoabdominal aortitis, severe pancreatitis, lupus enteritis or mesenteric vasculitis, and intestinal pseudo-obstruction. Delays in diagnosis affected several patients, and 26% of patients required surgical intervention. Many patients required intensive immunomodulatory treatment in addition to prolonged bowel rest and parenteral nutrition. Long-term outcomes of GI manifestations varied overall, however, most patients at our institution achieved clinical remission.ConclusionsUnderstanding these rare cases of severe GI manifestations in cSLE can aid clinicians in prompt diagnosis and collaborative management, improving clinical outcomes for this vulnerable population.

Keywords: Protein-losing enteropathy; enteritis; intestinal pseudo-obstruction; pancreatitis; thoracoabdominal aortitis.

Figure 1.

CT abdomen and pelvis. (A) Axial and (B) coronal views demonstrating large pancreatic pseudocyst (20.6 × 5.4 × 8.4 cm).

Figure 2.

CT abdomen and pelvis. (A) Axial and (B) coronal views demonstrating extensive bowel wall thickening and edema primarily involving the proximal small bowel.

Figure 3.

CT of Abdomen and Pelvis. (A) Axial and (B) sagittal views demonstrating severe bowel wall thickening throughout entirety of jejunum and ileum with a transition to decompressed right lower quadrant small bowel, demonstrative of mechanical small bowel obstruction with bowel wall thickening suggestive of ischemia.

Figure 4.

Stark contrast in ischemic versus perfused bowel during initial exploratory laparotomy where 33 cm of jejunum was resected.