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Published on: September 12, 2016
APDS patients with immune-complex vasculitis and resolution with leniolisib.
Mohammad-Ali Doroudchi1, Alexis V Stephens1, Ziwei Wang2
1Division of Immunology, Allergy, and Rheumatology, Department of Pediatrics, University of California Los Angeles, Los Angeles, CA, USA.
Activated phosphoinositide 3-kinase delta syndrome (APDS) can cause immune dysregulation. A case series shows leniolisib effectively treated IgA vasculitis in APDS patients, suggesting APDS screening for such cases.
Area of Science:
- Immunology
- Genetics
- Rheumatology
Background:
- Activated phosphoinositide 3-kinase delta syndrome (APDS) is a primary immunodeficiency.
- Clinical features include infections, autoimmunity, and lymphoproliferation.
- Immune complex-mediated vasculitis has not been previously reported in APDS.
Purpose of the Study:
- To describe immune complex-mediated vasculitis in APDS patients.
- To evaluate the efficacy of leniolisib in treating refractory IgA vasculitis in APDS.
Main Methods:
- Case series of three APDS patients with refractory IgA vasculitis.
- Treatment with leniolisib, a PI3K p110δ inhibitor.
Main Results:
- All three patients presented with refractory IgA vasculitis (Henoch-Schönlein purpura).
- IgA vasculitis resolved in all patients following treatment with leniolisib.
- Leniolisib demonstrated efficacy in managing this vasculitic manifestation.
Conclusions:
- Immune complex-mediated vasculitis, specifically IgA vasculitis, can occur in APDS.
- Leniolisib is an effective treatment for IgA vasculitis in APDS patients.
- Screening for APDS should be considered in patients with immune dysregulation and IgA vasculitis.
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