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Gastrointestinal Henoch-Schönlein purpura successfully treated with Mycophenolate Mofetil: Description of 2 case
Maria Francesca Gicchino1, Dario Iafusco1, Maria Maddalena Marrapodi1
1Department of Woman, Child and General and Specialized Surgery.
Rationale:
Henoch-Schönlein Purpura (HSP) is an acute small vessel vasculitis. It is the most common vasculitis in children. In majority of the cases, the disease is self-limited. Relapses can occur, in particular during the first year of the disease. There is no consensus on a specific treatment. The efficacy and safety of steroidal treatment in treating HSP is still controversial. Immunosuppressive treatment of HSP nephritis is used in patients with severe renal involvement (nephrotic range proteinuria and/or progressive renal impairment). The literature on immunosuppressive treatment of severe HSP without kidney involvement is scanty.
Patients Concerns:
We report 2 case reports of 2 adolescents affected from Henoch-Schönlein Purpura and severe gastrointestinal involvement. Both patients presented a poor response to steroids treatment.
Diagnoses:
The diagnosis of HSP was made according to the diagnostic criteria published by European League against Rheumatism and Pediatric Rheumatology European Society in 2006.
Interventions:
In consideration of the recurrence of the Henoch Schönlein Purpura and the gastrointestinal involvement, we decided to start Mycophenolate Mofetil treatment.
Outcomes:
In both patients all clinical manifestations resolved in few days.
Lessons:
In our cases of HSP with gastrointestinal involvement Mycophenolate Mofetil treatment has been very effective. This experience teaches us that immunosuppressive agents may be very useful to induce and maintain remission not only in renal involvement, but in all cases of persistent, recurrent, or complicated Henoch Schönlein Purpura in children.
Insights
Mycophenolate Mofetil effectively treated Henoch-Schönlein Purpura (HSP) with severe gastrointestinal issues in adolescents. This immunosuppressive therapy offers a promising option for persistent or complicated HSP cases beyond kidney involvement.
Area of Science:
- Pediatric Rheumatology
- Vasculitis Research
- Immunosuppressive Therapy
Background:
- Henoch-Schönlein Purpura (HSP) is the most common childhood vasculitis, typically self-limiting but prone to relapse.
- Current treatment strategies for HSP, especially steroidal and immunosuppressive therapies for non-renal involvement, lack established consensus.
- Severe gastrointestinal manifestations in HSP often present challenges in management.
Observation:
- This report details two adolescent cases of HSP with severe gastrointestinal involvement.
- Both patients exhibited a poor response to conventional steroid treatment.
- Recurrence and persistent gastrointestinal symptoms prompted alternative therapeutic considerations.
Findings:
- Mycophenolate Mofetil treatment was initiated for both patients due to recurrent HSP and significant gastrointestinal symptoms.
- Rapid and complete resolution of all clinical manifestations was observed in both cases within days of treatment.
- The diagnosis of HSP was confirmed using the 2006 European League against Rheumatism and Pediatric Rheumatology European Society criteria.
Implications:
- Mycophenolate Mofetil demonstrates significant efficacy in managing severe gastrointestinal involvement in HSP.
- This suggests immunosuppressive agents can be valuable for inducing and maintaining remission in complicated or persistent HSP beyond renal disease.
- Further research into immunosuppressive treatments for severe, non-renal HSP manifestations is warranted.
