Chiara Rubino1, Costanza Monacelli2, Edoardo Marrani3, Monica Paci4, Giuseppe Indolfi1,5, Gabriele Simonini3,5, Sandra Trapani6,7. 1. Pediatric Unit, Department of Health Sciences, Meyer Children's University Hospital, Viale Pieraccini 24, 50139, Florence, Italy. 2. Department of Health Sciences, University of Florence, Viale Pieraccini 24, 50139, Florence, Italy. 3. Rheumatology Unit, Meyer Children's University Hospital, Viale Pieraccini 24, 50139, Florence, Italy. 4. Gastroenterology Unit, Meyer Children's University Hospital, Viale Pieraccini 24, 50139, Florence, Italy. 5. NEUROFARBA Department, University of Florence, Viale Pieraccini 24, 50139, Florence, Italy. 6. Pediatric Unit, Department of Health Sciences, Meyer Children's University Hospital, Viale Pieraccini 24, 50139, Florence, Italy. sandra.trapani@unifi.it. 7. Department of Health Sciences, University of Florence, Viale Pieraccini 24, 50139, Florence, Italy. sandra.trapani@unifi.it.
Abstract
INTRODUCTION AND OBJECTIVES: Immunoglobulin A vasculitis (IgAV), the most common childhood vasculitis, is associated with gastrointestinal (GI) involvement in 50-75% of cases. The aim of this study was to describe the characteristics of GI involvement in a cohort of hospitalized children with IgAV. METHODS: We retrospectively evaluated patients hospitalized for IgAV at Meyer Children's University Hospital, from January 2010 to December 2020. The children's families were interviewed by phone and asked about disease relapses. RESULTS: In the study period, 118 children had GI involvement, corresponding to 75% of children hospitalized for IgAV. Their median age was 7 years (interquartile range 6-9). The most frequent GI manifestations were abdominal pain (96%), bleeding (71%, mostly occult), vomiting (58%), and diarrhea (17%). GI complications, observed in 18%, were intussusception (14%), appendicitis (3%), gallbladder hydrops (2%), and ileal perforation (1%). Abdomen ultrasound, performed in all cases, was abnormal in 68%. Abdomen X-ray, performed in 19 patients, showed pathologic findings in 84% of them. Selected children with severe manifestations also underwent abdomen computed tomography (2/118; 2%) and GI endoscopy (5/118; 4%). Steroids were used in 80 (67.8%) cases. The use of second- and third-line therapies was necessary in three cases. Relapses, investigated in 80 cases (68%), occurred in 21% of them. Key Points • The present retrospective study describes a cohort of 118 patients hospitalized for gastrointestinal (GI) involvement in immunoglobulin A vasculitis (IgAV) at a tertiary care children's hospital from January 2010 to December 2020. • The most frequent GI manifestations were abdominal pain (96%), vomiting (58%), and diarrhea (17%). GI bleeding was reported in 71% of children, and it was occult in most cases. • GI complications, occurring in 18% of cases, were intussusception (14%), appendicitis (3%), gallbladder hydrops (2%), and ileal perforation (1%). • Steroid treatment has been successfully used in severe GI manifestations; however, our data do not support its association with low relapse risk. CONCLUSIONS: The present study describes a large pediatric cohort of GI involvement in IgAV. Steroid treatment should be used when GI manifestations are severe. The association of steroid use with relapse risk is not currently established.
INTRODUCTION AND OBJECTIVES:Immunoglobulin A vasculitis (IgAV), the most common childhood vasculitis, is associated with gastrointestinal (GI) involvement in 50-75% of cases. The aim of this study was to describe the characteristics of GI involvement in a cohort of hospitalized children with IgAV. METHODS: We retrospectively evaluated patients hospitalized for IgAV at Meyer Children's University Hospital, from January 2010 to December 2020. The children's families were interviewed by phone and asked about disease relapses. RESULTS: In the study period, 118 children had GI involvement, corresponding to 75% of children hospitalized for IgAV. Their median age was 7 years (interquartile range 6-9). The most frequent GI manifestations were abdominal pain (96%), bleeding (71%, mostly occult), vomiting (58%), and diarrhea (17%). GI complications, observed in 18%, were intussusception (14%), appendicitis (3%), gallbladder hydrops (2%), and ileal perforation (1%). Abdomen ultrasound, performed in all cases, was abnormal in 68%. Abdomen X-ray, performed in 19 patients, showed pathologic findings in 84% of them. Selected children with severe manifestations also underwent abdomen computed tomography (2/118; 2%) and GI endoscopy (5/118; 4%). Steroids were used in 80 (67.8%) cases. The use of second- and third-line therapies was necessary in three cases. Relapses, investigated in 80 cases (68%), occurred in 21% of them. Key Points • The present retrospective study describes a cohort of 118 patients hospitalized for gastrointestinal (GI) involvement in immunoglobulin A vasculitis (IgAV) at a tertiary care children's hospital from January 2010 to December 2020. • The most frequent GI manifestations were abdominal pain (96%), vomiting (58%), and diarrhea (17%). GI bleeding was reported in 71% of children, and it was occult in most cases. • GI complications, occurring in 18% of cases, were intussusception (14%), appendicitis (3%), gallbladder hydrops (2%), and ileal perforation (1%). • Steroid treatment has been successfully used in severe GI manifestations; however, our data do not support its association with low relapse risk. CONCLUSIONS: The present study describes a large pediatric cohort of GI involvement in IgAV. Steroid treatment should be used when GI manifestations are severe. The association of steroid use with relapse risk is not currently established.
Authors: Seza Ozen; Stephen D Marks; Paul Brogan; Noortje Groot; Nienke de Graeff; Tadej Avcin; Brigitte Bader-Meunier; Pavla Dolezalova; Brian M Feldman; Isabelle Kone-Paut; Pekka Lahdenne; Liza McCann; Clarissa Pilkington; Angelo Ravelli; Annet van Royen; Yosef Uziel; Bas Vastert; Nico Wulffraat; Sylvia Kamphuis; Michael W Beresford Journal: Rheumatology (Oxford) Date: 2019-09-01 Impact factor: 7.580