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Published on: July 31, 2016
Clinical outcome in pediatric refractory gastrointestinal Henoch-Schönlein purpura treated with mycophenolate mofetil
Haiyan Wang1, Bihong Zhang1, Sha Li1
1Department of Pediatrics, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, Guangdong, 510120, People's Republic of China.
Abstract:
The aim of the present study was to investigate the clinical outcome of mycophenolate mofetil in pediatric refractory gastrointestinal (GI) Henoch-Schönlein purpura (HSP). Most of the HSP patients with GI symptoms may benefit from early introduction of glucocorticoid; however, a number of patients still do not achieve remission following the administration of steroids. Therefore, the present study was to investigate the clinical features and the clinical outcome of mycophenolate mofetil in refractory GI HSP. A total of 110 HSP patients with a median onset age of 6.3 years were included. Sixty-one (55.5%) exhibited GI involvement, and 18 (18/61, 29.5%) presented with refractory GI involvement, with a median onset age of 6.3 years. Intractable abdominal pain, GI hemorrhage, intussusception, and chronic ulcers were common presentations of GI involvement. Of those refractory ones, Arthralgia was observed in 9 cases and renal involvement was observed in 13 cases. Glucocorticoids were administered in all 18 patients, but remission was not achieved. However, complete remission of abdominal pain was achieved in all patients within a median time of 3 days (1-14 days) after mycophenolate mofetil therapy. The infection rate of Epstein-Barr virus and cytomegalovirus in the refractory group was significantly higher compared with that in non-refractory group.Conclusion: GI symptoms in HSP patients with refractory GI involvement were more severe compared with non-refractory cases. Epstein-Barr virus and cytomegalovirus infection may be risk factors for refractory GI HSP. The efficacy of mycophenolate mofetil treatment was evident in these patients. What is Known: • Abdominal pain, gastrointestinal hemorrhage, intussusceptions, and intestinal perforation were the main presentations of gastrointestinal involvement in Henoch-Schönlein purpura. What is New: • Epstein-Barr virus and Cytomegalovirus infection may be the high risk factor of refractory GI. Refractory gastrointestinal Henoch-Schönlein purpura was associated with renal involvement. • Mycophenolate mofetil treatment was effective for refractory gastrointestinal Henoch-Schönlein purpura.
Insights
Mycophenolate mofetil effectively treated refractory pediatric gastrointestinal Henoch-Schönlein purpura (HSP) when steroids failed. This study also identified Epstein-Barr virus and cytomegalovirus as potential risk factors for severe GI HSP.
Area of Science:
- Pediatric Rheumatology
- Gastroenterology
- Infectious Diseases
Background:
- Henoch-Schönlein purpura (HSP) commonly presents with gastrointestinal (GI) symptoms, which can be severe.
- While glucocorticoids are a primary treatment, some pediatric patients develop refractory GI involvement.
- Identifying risk factors and effective treatments for refractory GI HSP is crucial.
Purpose of the Study:
- To investigate the clinical features and outcomes of mycophenolate mofetil in pediatric refractory GI HSP.
- To explore potential risk factors, including viral infections, associated with refractory GI HSP.
- To evaluate the efficacy of mycophenolate mofetil in achieving remission in these challenging cases.
Main Methods:
- Retrospective analysis of 110 pediatric HSP patients, focusing on 18 with refractory GI involvement.
- Clinical data collection including symptoms, treatments (glucocorticoids, mycophenolate mofetil), and outcomes.
- Comparison of infection rates (Epstein-Barr virus, cytomegalovirus) between refractory and non-refractory groups.
Main Results:
- Refractory GI HSP patients experienced severe symptoms like intractable abdominal pain and GI hemorrhage.
- All 18 refractory patients failed to achieve remission with glucocorticoids.
- Mycophenolate mofetil led to complete remission of abdominal pain in all patients within a median of 3 days.
- Higher rates of Epstein-Barr virus and cytomegalovirus infections were observed in the refractory group.
- Refractory GI HSP was associated with arthralgia and renal involvement.
Conclusions:
- Refractory GI HSP in children is characterized by severe gastrointestinal manifestations and may be linked to Epstein-Barr virus and cytomegalovirus infections.
- Mycophenolate mofetil demonstrates significant efficacy in treating refractory GI HSP, offering a valuable therapeutic option.
- Early identification of risk factors and prompt treatment are essential for managing severe pediatric GI HSP.
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