Celiac Disease With Autoimmune Hemolytic Anemia and Autoimmune Hepatitis in a Young Child: Case Report and Literature Review

Abstract

Celiac disease (CD) is a chronic autoimmune condition with intestinal and extra-intestinal features. Extra intestinal features including hematological, neurological, and endocrine symptoms are seen more frequently in elder children. A 4 years 7 months old male child presented in clinic with history of abdominal pain and diarrhea on and off for 1 year. On examination, he was hemodynamically stable, pale, and malnourished with distended abdomen. He was investigated for CD, Anti TTG IgA <0.1 (positive >10), Anti TTG IgG 13 (positive >10). To confirm celiac disease, Esophagogastroduodenoscopy (EGD) was done which was consistent with diagnosis of Celiac disease (MARSH Type 3a). Gluten free diet was advised. Later, after 12 days he again presented with jaundice, fever, anorexia, and dark colored urine and irritability. He was admitted for fulminant hepatic failure, his workup revealed direct hyperbilirubenemia, ANA +ve, and hyper IgG. Liver biopsy confirmed autoimmune hepatitis. Further workup for anemia showed reticulocyte count 7.1, LDH 423, direct and indirect coombs test was positive confirming autoimmune hemolytic anemia. Child responded well to Azathioprine and prednisolone with clinical improvement. We report a rare presentation of celiac disease with polyautoimmunity in a young child. Case reports of autoimmune hepatitis with CD patients have been reported in adult patients. Association of celiac disease with autoimmune hemolytic anemia and autoimmune hepatitis is a distinct and rare condition.

Spectrum of Pediatric Autoimmune Hepatitis in a Cohort of Pakistani Children

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Celiac disease - a pluripathological model in pediatric practice

“…Broadly speaking, specialized literature correlates CD with endocrinopathies such as diabetes, Hashimoto’s thyroiditis, Graves’ disease, Addison’s disease (adrenal insufficiency), secondary hyperparathyroidism, ovarian insufficiency or pituitary disorders (resulting in hypogonadism, prolactin level imbalances) ( 194 , 195 ). Autoimmune hemolytic anemia or autoimmune hepatitis can also be added to this list ( 196 ). By studying a group of 228 patients, Varol Fİ.…”

Genetic link between celiac disease and autoimmune liver diseases: A two-sample Mendelian randomization study

“…CeD and AILD have a common genetic basis and the human leukocyte antigen (HLA) risk alleles play an essential role in the susceptibility of them, suggesting that there may be some association between CeD and AILD (9). Otherwise, it has been estimated that the prevalence of CeD is several times higher in AILD case than in the healthy population (approximately 3-6% in AIH, 3-7% in PBC and 5.1%-11.8% in PSC) and pediatric patients with AIH have higher prevalence from 11.5-46%(8, 15,22). Furthermore, patients with CeD seemed to be more vulnerable to AILD and have demonstrated poorer prognoses (20,26,30).…”