Cytokine Storm Syndrome Responsive to IL-1 Inhibition in Trisomy 21
Background. Cytokine storm syndromes (CSS) are life-threatening systemic inflammatory disorders caused by immune system dysregulation. They can lead to organ failure and are triggered by various factors, including infections, malignancy, inborn errors of immunity, and autoimmune conditions. Trisomy...
Saved in:
| Main Authors: | , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2024-01-01
|
| Series: | Case Reports in Pediatrics |
| Online Access: | http://dx.doi.org/10.1155/2024/9946401 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1841561543868678144 |
|---|---|
| author | Aimee Magnarelli Julia Shalen Maria J. Gutierrez |
| author_facet | Aimee Magnarelli Julia Shalen Maria J. Gutierrez |
| author_sort | Aimee Magnarelli |
| collection | DOAJ |
| description | Background. Cytokine storm syndromes (CSS) are life-threatening systemic inflammatory disorders caused by immune system dysregulation. They can lead to organ failure and are triggered by various factors, including infections, malignancy, inborn errors of immunity, and autoimmune conditions. Trisomy 21 (TS21), also known as Down syndrome, is a genetic disorder associated with immune dysfunction, increased infection susceptibility, and inflammation. While TS21 has been linked to infectious-triggered hyperinflammation, its role as a primary cause of CSS has not been confirmed. Case Presentation. We present a case of a 16-year-old male with TS21 with fever, rash, joint pain, and abdominal symptoms. Extensive investigations ruled out infections, autoimmune conditions, malignancies, and inborn errors of immunity as triggers for a CSS. The patient’s symptoms improved with treatment using IL-1 inhibition and corticosteroids. Conclusions. This case reinforces that TS21 is an immune dysregulation disorder and highlights the importance of considering CSS in TS21 patients, even when triggers are unclear. The positive response to IL-1 inhibition in this patient suggests that dysregulation of the IL-1 superfamily and the NLRP3 inflammasome may contribute to CSS in TS21. This finding raises the possibility of using IL-1 inhibition as a treatment approach for CSS in TS21 patients. |
| format | Article |
| id | doaj-art-2f2c08c6ad98484288d1447cf4b3dff8 |
| institution | Kabale University |
| issn | 2090-6811 |
| language | English |
| publishDate | 2024-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pediatrics |
| spelling | doaj-art-2f2c08c6ad98484288d1447cf4b3dff82025-01-03T01:30:19ZengWileyCase Reports in Pediatrics2090-68112024-01-01202410.1155/2024/9946401Cytokine Storm Syndrome Responsive to IL-1 Inhibition in Trisomy 21Aimee Magnarelli0Julia Shalen1Maria J. Gutierrez2Division of Pediatric AllergyDivision of Pediatric AllergyDivision of Pediatric AllergyBackground. Cytokine storm syndromes (CSS) are life-threatening systemic inflammatory disorders caused by immune system dysregulation. They can lead to organ failure and are triggered by various factors, including infections, malignancy, inborn errors of immunity, and autoimmune conditions. Trisomy 21 (TS21), also known as Down syndrome, is a genetic disorder associated with immune dysfunction, increased infection susceptibility, and inflammation. While TS21 has been linked to infectious-triggered hyperinflammation, its role as a primary cause of CSS has not been confirmed. Case Presentation. We present a case of a 16-year-old male with TS21 with fever, rash, joint pain, and abdominal symptoms. Extensive investigations ruled out infections, autoimmune conditions, malignancies, and inborn errors of immunity as triggers for a CSS. The patient’s symptoms improved with treatment using IL-1 inhibition and corticosteroids. Conclusions. This case reinforces that TS21 is an immune dysregulation disorder and highlights the importance of considering CSS in TS21 patients, even when triggers are unclear. The positive response to IL-1 inhibition in this patient suggests that dysregulation of the IL-1 superfamily and the NLRP3 inflammasome may contribute to CSS in TS21. This finding raises the possibility of using IL-1 inhibition as a treatment approach for CSS in TS21 patients.http://dx.doi.org/10.1155/2024/9946401 |
| spellingShingle | Aimee Magnarelli Julia Shalen Maria J. Gutierrez Cytokine Storm Syndrome Responsive to IL-1 Inhibition in Trisomy 21 Case Reports in Pediatrics |
| title | Cytokine Storm Syndrome Responsive to IL-1 Inhibition in Trisomy 21 |
| title_full | Cytokine Storm Syndrome Responsive to IL-1 Inhibition in Trisomy 21 |
| title_fullStr | Cytokine Storm Syndrome Responsive to IL-1 Inhibition in Trisomy 21 |
| title_full_unstemmed | Cytokine Storm Syndrome Responsive to IL-1 Inhibition in Trisomy 21 |
| title_short | Cytokine Storm Syndrome Responsive to IL-1 Inhibition in Trisomy 21 |
| title_sort | cytokine storm syndrome responsive to il 1 inhibition in trisomy 21 |
| url | http://dx.doi.org/10.1155/2024/9946401 |
| work_keys_str_mv | AT aimeemagnarelli cytokinestormsyndromeresponsivetoil1inhibitionintrisomy21 AT juliashalen cytokinestormsyndromeresponsivetoil1inhibitionintrisomy21 AT mariajgutierrez cytokinestormsyndromeresponsivetoil1inhibitionintrisomy21 |