The Diagnosing Challenge of a Positive ANCA Vasculitis in the Paediatric Age
ANCA-positive systemic vasculitides, rare in paediatric age, present multiorganic involvement. A female teenager presented with a history of subglottic stenosis diagnosed at the age of 12. From the investigation carried out, we highlight hematoproteinuria and negative ANCAs. At 15 years old, she was...
Saved in:
| Main Authors: | , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2017-01-01
|
| Series: | Case Reports in Pediatrics |
| Online Access: | http://dx.doi.org/10.1155/2017/2962794 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832566995147554816 |
|---|---|
| author | Clara Preto Armandina Silva Sandra Alves Margarida Guedes Paula Matos Conceição Mota Paula Rocha Paula C. Fernandes |
| author_facet | Clara Preto Armandina Silva Sandra Alves Margarida Guedes Paula Matos Conceição Mota Paula Rocha Paula C. Fernandes |
| author_sort | Clara Preto |
| collection | DOAJ |
| description | ANCA-positive systemic vasculitides, rare in paediatric age, present multiorganic involvement. A female teenager presented with a history of subglottic stenosis diagnosed at the age of 12. From the investigation carried out, we highlight hematoproteinuria and negative ANCAs. At 15 years old, she was admitted for gastrointestinal symptoms and respiratory distress. She presented poor peripheral perfusion, pulmonary haemorrhage, respiratory failure, and severe renal insufficiency. She was started mechanical ventilation and emergency haemodialysis. The immunological study revealed ANCA MPO positive. A presumptive diagnosis of ANCA-positive vasculitis was made, and she was started corticotherapy, cyclophosphamide, and plasmapheresis. A renal biopsy, performed later, showed crescentic glomerulonephritis with chronicity signs. Positive ANCA vasculitis may progress slowly or suddenly. The diagnosis was confirmed by a biopsy; however, we can make a presumptive diagnosis based on clinical findings and in a positive ANCA test in order to start an early treatment and decrease the associated morbimortality. |
| format | Article |
| id | doaj-art-d5e42908488741a8bfbd2db2c4893cac |
| institution | Kabale University |
| issn | 2090-6803 2090-6811 |
| language | English |
| publishDate | 2017-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pediatrics |
| spelling | doaj-art-d5e42908488741a8bfbd2db2c4893cac2025-02-03T01:02:41ZengWileyCase Reports in Pediatrics2090-68032090-68112017-01-01201710.1155/2017/29627942962794The Diagnosing Challenge of a Positive ANCA Vasculitis in the Paediatric AgeClara Preto0Armandina Silva1Sandra Alves2Margarida Guedes3Paula Matos4Conceição Mota5Paula Rocha6Paula C. Fernandes7Paediatric and Neonatal Intensive Care Service, Centro Materno Infantil do Norte, Centro Hospitalar do Porto, Porto, PortugalPaediatric Service, Hospital da Senhora da Oliveira, Guimarães, PortugalOtorhinolaryngology Service, Centro Hospitalar de Vila Nova de Gaia/Espinho, Vila Nova de Gaia, PortugalPaediatric Service, Centro Materno Infantil do Norte, Centro Hospitalar do Porto, Porto, PortugalDepartment of Paediatric Nephrology, Paediatric Service, Centro Materno Infantil do Norte, Centro Hospitalar do Porto, Porto, PortugalDepartment of Paediatric Nephrology, Paediatric Service, Centro Materno Infantil do Norte, Centro Hospitalar do Porto, Porto, PortugalPaediatric and Neonatal Intensive Care Service, Centro Materno Infantil do Norte, Centro Hospitalar do Porto, Porto, PortugalPaediatric and Neonatal Intensive Care Service, Centro Materno Infantil do Norte, Centro Hospitalar do Porto, Porto, PortugalANCA-positive systemic vasculitides, rare in paediatric age, present multiorganic involvement. A female teenager presented with a history of subglottic stenosis diagnosed at the age of 12. From the investigation carried out, we highlight hematoproteinuria and negative ANCAs. At 15 years old, she was admitted for gastrointestinal symptoms and respiratory distress. She presented poor peripheral perfusion, pulmonary haemorrhage, respiratory failure, and severe renal insufficiency. She was started mechanical ventilation and emergency haemodialysis. The immunological study revealed ANCA MPO positive. A presumptive diagnosis of ANCA-positive vasculitis was made, and she was started corticotherapy, cyclophosphamide, and plasmapheresis. A renal biopsy, performed later, showed crescentic glomerulonephritis with chronicity signs. Positive ANCA vasculitis may progress slowly or suddenly. The diagnosis was confirmed by a biopsy; however, we can make a presumptive diagnosis based on clinical findings and in a positive ANCA test in order to start an early treatment and decrease the associated morbimortality.http://dx.doi.org/10.1155/2017/2962794 |
| spellingShingle | Clara Preto Armandina Silva Sandra Alves Margarida Guedes Paula Matos Conceição Mota Paula Rocha Paula C. Fernandes The Diagnosing Challenge of a Positive ANCA Vasculitis in the Paediatric Age Case Reports in Pediatrics |
| title | The Diagnosing Challenge of a Positive ANCA Vasculitis in the Paediatric Age |
| title_full | The Diagnosing Challenge of a Positive ANCA Vasculitis in the Paediatric Age |
| title_fullStr | The Diagnosing Challenge of a Positive ANCA Vasculitis in the Paediatric Age |
| title_full_unstemmed | The Diagnosing Challenge of a Positive ANCA Vasculitis in the Paediatric Age |
| title_short | The Diagnosing Challenge of a Positive ANCA Vasculitis in the Paediatric Age |
| title_sort | diagnosing challenge of a positive anca vasculitis in the paediatric age |
| url | http://dx.doi.org/10.1155/2017/2962794 |
| work_keys_str_mv | AT clarapreto thediagnosingchallengeofapositiveancavasculitisinthepaediatricage AT armandinasilva thediagnosingchallengeofapositiveancavasculitisinthepaediatricage AT sandraalves thediagnosingchallengeofapositiveancavasculitisinthepaediatricage AT margaridaguedes thediagnosingchallengeofapositiveancavasculitisinthepaediatricage AT paulamatos thediagnosingchallengeofapositiveancavasculitisinthepaediatricage AT conceicaomota thediagnosingchallengeofapositiveancavasculitisinthepaediatricage AT paularocha thediagnosingchallengeofapositiveancavasculitisinthepaediatricage AT paulacfernandes thediagnosingchallengeofapositiveancavasculitisinthepaediatricage AT clarapreto diagnosingchallengeofapositiveancavasculitisinthepaediatricage AT armandinasilva diagnosingchallengeofapositiveancavasculitisinthepaediatricage AT sandraalves diagnosingchallengeofapositiveancavasculitisinthepaediatricage AT margaridaguedes diagnosingchallengeofapositiveancavasculitisinthepaediatricage AT paulamatos diagnosingchallengeofapositiveancavasculitisinthepaediatricage AT conceicaomota diagnosingchallengeofapositiveancavasculitisinthepaediatricage AT paularocha diagnosingchallengeofapositiveancavasculitisinthepaediatricage AT paulacfernandes diagnosingchallengeofapositiveancavasculitisinthepaediatricage |