Primary pneumococcal peritonitis can be the first presentation of a familial complement factor I deficiency1
| Autor: | Peter J. Lachmann, D. Kavanagh, P Gurugama, Sanja Ugrinovic, Theodore Gouliouris, Effrossyni Gkrania-Klotsas, Dinakantha S. Kumararatne, Helen Baxendale, Helen V. Firth |
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| Rok vydání: | 2020 |
| Předmět: |
0301 basic medicine
Hereditary Complement Deficiency Diseases Adolescent Phagocytosis Immunology Complement factor I Peritonitis medicine.disease_cause Pneumococcal Infections 03 medical and health sciences 0302 clinical medicine Streptococcus pneumoniae medicine Immunodeficiency Humans Immunology and Allergy complement business.industry Original Articles Complement C3 medicine.disease Complement (complexity) Complement system Antibody opsonization 030104 developmental biology Primary immunodeficiency Original Article Female business 030215 immunology |
| Zdroj: | Clinical and Experimental Immunology |
| ISSN: | 1365-2249 0009-9104 |
| Popis: | We describe a case of primary pneumococcal peritonitis which uncovered a family with compound heterozygous deficiency for complement factor I. Primary pneumococcal peritonitis has been described in women but has not been previously linked with complement disorders. We propose that unexplained pneumococcal peritonitis should be considered a “red flag” for primary immunodeficiency. Summary Primary pneumococcal peritonitis is a rare infection that has been described in women but has not been previously linked with immunodeficiency. The complement system plays a central role in immune defence against Streptococcus pneumoniae and, in order to evade complement attack, pneumococci have evolved a large number of mechanisms that limit complement‐mediated opsonization and subsequent phagocytosis. We investigated an apparently immunocompetent woman with primary pneumococcal peritonitis and identified a family with deficiency for complement factor I. Primary pneumococcal peritonitis should be considered a possible primary immunodeficiency presentation. |
| Databáze: | OpenAIRE |
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