Pediatric Gastrointestinal Sarcoidosis: Successful Treatment with Infliximab

Gastrointestinal sarcoidosis is a rare disease with very limited data in children. Here we report the first pediatric case of successful treatment with infliximab. The first case was an 8-year-old Saudi girl who presented with fever, weight loss, and abdominal pain that was followed in a few months with hematemesis and development of hepatosplenomegaly. The second case was a 9-year-old Sudanese boy who manifested with vomiting, epigastric pain, and weight loss. On upper endoscopy, both cases demonstrated severe erosive nodular gastric mucosa. Gastric and esophageal biopsies had shown noncaseating granulomatous inflammation. The first case had histopathological evidence of granulomatous hepatitis, and both cases demonstrated lung nodularity on computed tomography chest. The boy had elevated angiotensin-converting enzyme level. Given the multisystem involvement with significant chest findings, tissue findings of granulomatous disease, and negative workup for other causes of granulomatous inflammation, both cases were diagnosed with active disseminated sarcoidosis, and treated with corticosteroids. The girl continued to be symptom-free for 4 years after tapering steroid therapy. The boy had relapses off steroids and the disease was brought into remission for 5 years off steroid therapy by infliximab. Pediatric GI sarcoidosis is a rare disease that exhibits heterogeneity in natural course. The chronic relapsing progressive form of the disease might benefit from infliximab therapy.Key Words: Atrophic gastritis, children, gastrointestinal sarcoidosis, infliximab, Vitamin B12 deficiencySarcoidosis is a multiorgan systemic disease characterized by the formation of non-necrotizing epithelioid granulomas in the affected organs, including skin, lungs, heart, nervous system, hilar lymph nodes, liver, eyes, and joints. Sarcoidosis occurs mainly in the 20- to 40-year-old age group. The prevalence is reported to be 1–40 per 100,000 in the United States.[1] Sarcoidosis of the gastrointestinal tract (GIT) is reported to be extremely rare. Several autopsy studies found no GI involvement,[2] while another reported gastrointestinal involvement in 2.5%.[2] In contrast, liver follows lymph nodes and lung in the frequency of involvement. About 50%–79% of livers are involved by biopsy and 67–70% by autopsy.[2] Treatment with corticosteroids results in symptomatic improvement in majority of patients. Other steroid-sparing agents that had been used in steroid-dependent or -resistant cases included chloroquine, azathioprine, methotrexate, and cyclophosphamide. Infliximab has been shown to produce clinical improvement and reduce the requirement for corticosteroids in a very small number of adult patients with sarcoidosis.[3]We report two children with gastrointestinal (GI) sarcoidosis, added to the four cases of GI sarcoidosis already reported in the pediatric literature, and report the first pediatric case of successful treatment with infliximab.