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Differential diagnosis of hypoalbuminemia in childhood: protein losing enteropathy associated to systemic lupus erythematosus in a young boy.

Lupus-protein losing enteropathy (LUPLE) is an uncommon gastrointestinal manifestation of systemic lupus erythematosus (SLE), and it has been even more rarely described in paediatric age. Hereby, we report the case of an adopted 10-year-old Vietnamese child referred to our Unit for recurrent episodes of hypoalbuminaemia and oedema. His natural family history was unknown. His past medical history revealed two previous episodes of hypoalbuminaemia with oedemas, associated to nonnephrotic proteinuria and successfully treated with a course of oral prednisone. After a prednisone free period of 9 months, the patient was admitted to our Unit presenting the third episode of sudden anasarca. Conspicuous pretibial and periorbital oedema was present. Serum albumin was undetectable. Inflammatory markers were increased. Haemogram, liver and kidney function were normal, and 24-hour urinary protein quantification was negative for proteinuria. An increase in faecal alpha-1 antitrypsin was disclosed and 99mTc-labelled human serum albumin scintigraphy confirmed severe protein loss, especially from the duodenojejunal area. Gastrointestinal endoscopies showed gastric hypertrophic folds with mucosal nodularity and lymphoid nodular hyperplasia at the distal ileum. Biopsies revealed gastric mucosal chronic inflammation with foveolar hyperplasia and lymphangiectasia, in the presence of Helicobacter pylori infection. Lymphangiectasia was present at duodenal mucosa as well and mucosal chronic inflammation was noted at colon. The patient underwent nutritional replacement with high-protein diet and medium-chain triglycerides and eradication therapy of H. pylori, without significant improvement. Immunological investigations showed positivity of antinuclear antibody (ANA), anti-SSA and SSB antibodies and later of anti-double-stranded-DNA antibody (anti-ds-DNA). Complement was normal, while circulating immune complexes were highly present. Thereby, the diagnosis of LUPLE was suspected. Intravenous methylprednisolone pulse therapy was started, followed by daily oral prednisone and azathioprine, with progressive clinical resolution. Almost two years later, the patient developed clinical and serological findings that fulfilled diagnostic criteria for childhood-onset SLE (c-SLE). Only 11 LUPLE patients younger than 18 years are reported in the literature. We reviewed and summarized clinical features of these patients in Table 1 [1–11]. This condition is prevalent in females and in Asiatic children. LUPLE can be the first manifestation of the connective tissue disease or occur later, presenting with or without gastrointestinal symptoms. Nonspecific inflammation involving duodenum and colon and lymphangiectasia are the most frequent histopathological findings. ANA is present in all the patients and positivity of anti-ds-DNA and anti-SSA/B antibodies is frequently reported. Coadministration of steroids and immunosuppressive agents is usually necessary in order to control gastrointestinal disease. In conclusion, although LUPLE is not mentioned in diagnostic criteria for SLE, it could characterize c-SLE at the onset or later; therefore, it should be considered in the differential diagnosis of unexplained hypoalbuminaemia in childhood, even in the absence of gastrointestinal symptoms, especially when a connective tissue disease is present or presumptive.