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The complement system is a group of approximately 20 plasma proteins that strengthen the body's defenses against infections through opsonization, inflammation, and cell lysis. Opsonization involves coating pathogens with complement proteins, making them more recognizable and facilitating phagocyte engulfment. Certain complement proteins induce inflammation that attracts immune cells to the site of infection. Cell lysis involves the destruction of pathogens through the formation of a...
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The lymphatic system plays a crucial role in bolstering our immune system. It consists of a network of lymphoid organs, lymph, and lymphatic vessels that provide structural and functional support in safeguarding the body against pathogens such as viruses and bacteria.
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Isolation of Human Lymphatic Endothelial Cells by Multi-parameter Fluorescence-activated Cell Sorting
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Complement dysregulation at lymphatics.

Ahmet Ozen1, Salim Can1, Asena Pinar Sefer2

  • 1Division of Allergy and Immunology, Department of Pediatrics, Marmara University School of Medicine, Istanbul, Turkey; Isil Berat Barlan Center for Translational Medicine, Istanbul, Turkey; Istanbul Jeffrey Modell Diagnostic Center for Primary Immunodeficiency Diseases, Istanbul, Turkey.

The Journal of Allergy and Clinical Immunology
|April 29, 2025
PubMed
Summary

Complement hyperactivation causes lymphatic dysfunction in rare CHAPLE disease. C5-blocking antibodies like pozelimab effectively treat this condition, normalizing albumin and reducing hospitalizations.

Keywords:
CD55CHAPLE diseasecomplement dysregulationcomplement systemeculizumabintestinal lymphangiectasiapozelimabprotein-losing enteropathy

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Area of Science:

  • Immunology
  • Vascular Biology
  • Gastroenterology

Background:

  • The complement system is crucial for innate immunity but its dysregulation causes diseases like paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome.
  • Complement hyperactivation is increasingly linked to lymphatic dysfunction, notably in CHAPLE disease.
  • CHAPLE disease stems from CD55 mutations, leading to uncontrolled complement and coagulation activation, causing intestinal lymphangiectasia and protein-losing enteropathy.

Purpose of the Study:

  • To investigate the role of complement hyperactivation in lymphatic dysfunction and its treatment in CHAPLE disease.
  • To evaluate the efficacy of C5-blocking antibodies in managing CHAPLE disease.
  • To explore the broader implications of complement-driven protein-losing enteropathy.

Main Methods:

  • Analysis of complement and coagulation pathways in CHAPLE disease.
  • Clinical evaluation of C5-blocking antibodies (pozelimab, eculizumab) in a phase 2/3 study.
  • Review of emerging evidence on complement-mediated protein-losing enteropathy.

Main Results:

  • Impaired regulation of C3 and C5 convertases leads to severe intestinal lymphangiectasia and protein-losing enteropathy in CHAPLE disease.
  • Patients present with hypoalbuminemia, edema, gastrointestinal issues, growth retardation, and thromboembolic events.
  • Pozelimab treatment normalized serum albumin, reduced hospitalizations, and transfusion needs, leading to FDA approval.

Conclusions:

  • C5-blocking antibodies have transformed CHAPLE disease management.
  • Complement dysregulation is a significant driver of protein-losing enteropathy, with potential implications beyond CHAPLE disease.
  • Further research into complement-mediated lymphatic disorders may yield new diagnostic and therapeutic strategies.