APDS patients with immune-complex vasculitis and resolution with leniolisib
Copyright © 2024 Elsevier Inc. All rights reserved..
Activated phosphoinositide 3-kinase delta syndrome (APDS) is an inborn error of immunity with heterogeneous clinical manifestations of infections, immune dysregulation, autoimmunity; lymphoproliferation; and malignancy. Immune complex-mediated vasculitides have not yet been described in APDS patients. Here we offer a case series of three patients with APDS who have refractory IgA vasculitis (also called Henoch-Schönlein purpura), a form of immune complex-mediated vasculitis that activates complement and attracts neutrophils, macrophages and eosinophils to cause local tissue injury. Leniolisib is an inhibitor of PI3K p110δ and an FDA-approved treatment for APDS. IgA vasculitis resolved upon treatment with leniolisib. Patients with immune dysregulation including IgA vasculitis should be screened for APDS.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:262 |
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| Enthalten in: |
Clinical immunology (Orlando, Fla.) - 262(2024) vom: 01. Apr., Seite 110176 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Doroudchi, Mohammad-Ali [VerfasserIn] |
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| Links: |
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| Anmerkungen: |
Date Completed 15.04.2024 Date Revised 15.04.2024 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1016/j.clim.2024.110176 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM369518853 |
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| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Copyright © 2024 Elsevier Inc. All rights reserved. | ||
| 520 | |a Activated phosphoinositide 3-kinase delta syndrome (APDS) is an inborn error of immunity with heterogeneous clinical manifestations of infections, immune dysregulation, autoimmunity; lymphoproliferation; and malignancy. Immune complex-mediated vasculitides have not yet been described in APDS patients. Here we offer a case series of three patients with APDS who have refractory IgA vasculitis (also called Henoch-Schönlein purpura), a form of immune complex-mediated vasculitis that activates complement and attracts neutrophils, macrophages and eosinophils to cause local tissue injury. Leniolisib is an inhibitor of PI3K p110δ and an FDA-approved treatment for APDS. IgA vasculitis resolved upon treatment with leniolisib. Patients with immune dysregulation including IgA vasculitis should be screened for APDS | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Activated PI3K Delta syndrome | |
| 650 | 4 | |a IgA vasculitis | |
| 650 | 4 | |a Immune complex | |
| 650 | 4 | |a Leniolisib | |
| 650 | 4 | |a vasculitis | |
| 650 | 7 | |a Antigen-Antibody Complex |2 NLM | |
| 650 | 7 | |a Phosphatidylinositol 3-Kinase |2 NLM | |
| 650 | 7 | |a EC 2.7.1.137 |2 NLM | |
| 650 | 7 | |a leniolisib |2 NLM | |
| 650 | 7 | |a Phosphatidylinositol 3-Kinases |2 NLM | |
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| 650 | 7 | |a Pyridines |2 NLM | |
| 650 | 7 | |a Pyrimidines |2 NLM | |
| 700 | 1 | |a Stephens, Alexis V |e verfasserin |4 aut | |
| 700 | 1 | |a Wang, Ziwei |e verfasserin |4 aut | |
| 700 | 1 | |a Dhami, Jaspreet |e verfasserin |4 aut | |
| 700 | 1 | |a Butte, Manish J |e verfasserin |4 aut | |
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