OBJECTIVES:: Celiac disease (CD) is common in patients with autoimmune liver disease. However, the long-term response to treatment of patients with autoimmune liver diseases coexistent with CD has not been explored in detail. The aim of this study was to analyze the features and the long-term response to immunosuppressive treatment in children with autoimmune hepatitis (AIH) associated with CD. METHODS:: Retrospective and prospective evaluation of patients followed at a single center. RESULTS:: Among 79 patients with AIH, 15 (19 %) had celiac disease (9 type 1; 3 type 2; 3 seronegative). In the group of patients with AIH and CD, female sex was significantly more represented that in the group of patients with AIH alone; also, in the former group diagnosis was made significantly earlier (p < 0.05). All of the 15 patients on gluten free diet achieved a sustained remission when treated with prednisone and azathioprine, or cyclosporine. Mean period of follow-up was 73 months; discontinuation of therapy was attempted in 9 patients while in remission: 4 patients relapsed; 5 (33%) could definitively stop immunosuppressive treatment with a mean period of treatment-free sustained remission of 89 months (range 26-174). In the same period, treatment discontinuation, attempted in 24 of 64 patients with AIH without CD, was successful in 5 (8 %; p < 0.05). CONCLUSIONS:: Patients with AIH co-existing with CD achieve treatment-free sustained remission in a significantly higher proportion, when compared to patients with autoimmune liver disease without celiac disease, suggesting a possible long-term adjuvant effect of gluten free diet.
Celiac Disease-Associated Autoimmune Hepatitis In Childhood: Long Term Response To Treatment
VAJRO, Pietro;
2013-01-01
Abstract
OBJECTIVES:: Celiac disease (CD) is common in patients with autoimmune liver disease. However, the long-term response to treatment of patients with autoimmune liver diseases coexistent with CD has not been explored in detail. The aim of this study was to analyze the features and the long-term response to immunosuppressive treatment in children with autoimmune hepatitis (AIH) associated with CD. METHODS:: Retrospective and prospective evaluation of patients followed at a single center. RESULTS:: Among 79 patients with AIH, 15 (19 %) had celiac disease (9 type 1; 3 type 2; 3 seronegative). In the group of patients with AIH and CD, female sex was significantly more represented that in the group of patients with AIH alone; also, in the former group diagnosis was made significantly earlier (p < 0.05). All of the 15 patients on gluten free diet achieved a sustained remission when treated with prednisone and azathioprine, or cyclosporine. Mean period of follow-up was 73 months; discontinuation of therapy was attempted in 9 patients while in remission: 4 patients relapsed; 5 (33%) could definitively stop immunosuppressive treatment with a mean period of treatment-free sustained remission of 89 months (range 26-174). In the same period, treatment discontinuation, attempted in 24 of 64 patients with AIH without CD, was successful in 5 (8 %; p < 0.05). CONCLUSIONS:: Patients with AIH co-existing with CD achieve treatment-free sustained remission in a significantly higher proportion, when compared to patients with autoimmune liver disease without celiac disease, suggesting a possible long-term adjuvant effect of gluten free diet.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
