Recurrent lupus enteritis in a child—chase the calm before the storm

Abstract

Systemic lupus erythematosus (SLE) is a complex multisystemic autoimmune disease. Lupus enteritis (LE), one of the less commonly described manifestations of childhood SLE, presents with relatively nonspecific clinical and laboratory features. In addition, recurrent episodes of LE occurring in temporal proximity are rare in children. Presence of disease activity at other sites (which may not be seen universally) supports the diagnosis of LE in an appropriate setting. Because of its potential role to cause ischemic complications, early recognition and prompt treatment are necessary for a good outcome. Herein, we describe a child with recurrent LE with an interval of about 3 months between the first and the second episode. The first episode correlated with systemic disease activity and bowel thickening was noted on abdominal ultrasonography. This episode was successfully managed with intravenous methylprednisolone pulse therapy. Conversely, the second episode was not associated with significant clinical and laboratory evidence of disease activity at other sites and the initial abdominal ultrasonography was non-contributory. Diagnostic and therapeutic delays, hence, led to the development of fatal complications. We highlight that a high index of suspicion of LE and a timely aggressive treatment is imperative for optimal outcomes even in rare pediatric cases of recurrent LE that may have normal imaging findings initially and may not be associated with systemic lupus erythematosus disease activity index (SLEDAI).

Gastrointestinal Involvement in Children with Systemic Lupus Erythematosus

“…LMV can present with different signs and symptoms ranging from mild abdominal pain to nausea, vomiting, diarrhea, and up to severe acute abdominal pain associated with gastrointestinal bleeding, intestinal necrosis, and perforation. Jejunum and ileum are the most commonly affected as LMV often involves the superior mesenteric artery [75,76]. The oral cavity can also be involved, mostly with painless ulcerations that are usually located in the hard palate; hyperkeratotic lesions and lichenoid infiltrates in the shape of oral macules and papules have also been described [77].…”

“…LMV is usually associated with an active involvement of other organs, although it is rarely an isolated manifestation; in 65% of cases it is concomitant with lupus nephritis. As a consequence of the combined involvement, patients with LMV often present increased disease activity scores, for example on the SLE disease activity index (SLEDAI) or on the British Isles Lupus Assessment Group's (BILAG) disease activity index [72,76,77,81,82].…”