Pediatric gastrointestinal sarcoidosis: Successful treatment with infliximab

Abstract

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.

Erken Yaşta Malabsorpsiyon ile Başvuran Gastrointestinal Sarkoidoz

“…The presence of non-caseating granuloma consisting of multinucleate giant cells is the gold standard for the diagnosis of sarcoidosis ( 7 ). Moreover, confirmation of granulomas in extra-intestinal organs strongly supports the diagnosis of sarcoidosis ( 4 ). Elevated LFTs and the detection of granulomas in the liver biopsy later supported the diagnosis of sarcoidosis in this patient.…”

“…Sarcoidosis of the colon is also very rare and may mimic CD, thus the differentiation of the two diseases is particularly difficult. Histopathologic and clinical findings, which may favor the diagnosis of sarcoidosis rather than CD, include mucosal involvement instead of transmural inflammation, absence of perianal disease or fistulas, and elevated serum ACE levels ( 4 ). The presence of non-caseating granuloma consisting of multinucleate giant cells is the gold standard for the diagnosis of sarcoidosis ( 7 ).…”

“…The current therapy of choice for childhood sarcoidosis is corticosteroids, usually initiated at 1 to 2 mg/kg/day for 4 to 8 weeks as induction treatment ( 2 , 4 ). The disease activity is monitored clinically, radiographically, and through serum ACE levels ( 2 , 7 ).…”

“…The prominent gastrointestinal symptoms at initial presentation are also rare in the pediatric age group, and there are scarce case reports. Furthermore, clinically prominent gastrointestinal sarcoidosis is exceptionally rare before the age of 5 years ( 3 , 4 ).…”

Biologic Therapies in Sarcoidosis and Uveitis: A Review

“…Studies by Aguiar et al and Hostettler et al included patients with multi-system involvement, and infliximab therapy was more effective in achieving disease remission in patients with multi-organ involvement compared to patients with only pulmonary sarcoidosis [ 9 , 36 ]. Cases of sarcoidosis in the gastrointestinal tract and a renal transplant were managed successfully with infliximab [ 15 , 27 ]. Infliximab was ineffective in articular sarcoidosis [ 37 ].…”