A Case of Tracheal Stenosis as an Isolated Form of Immunoproliferative Hyper-IgG4 Disease in a 17-Year-Old Girl

Children

Kaloyan Gabrovski¹  Ivan Terziev²  Yanko Pahnev³  Edmond Rangelov³  Nikola Kartulev³  Hristo Shivachev³  Zdravka Antonova³  Dimitar Kostadinov⁴  Nikolay Yanev⁴  Natalia Gabrovska⁴  Svetlana Velizarova⁴  Albena Spasova⁴ 

[1] Department of Neurosurgery, University Hospital “St. Ivan Rilski”, Medical University–Sofia, 1431 Sofia, Bulgaria;Department of Pathology, University Hospital ‘’Tsaritsa Uoanna–ISUL’’, 1527 Sofia, Bulgaria;Department of Pediatric Thoracic Surgery, Pediatric Surgery Clinic, University Multiprofile Hospital for Active Treatment and Emergency Medicine “N. I. Pirogov”, 1606 Sofia, Bulgaria;Department of Pulmonary Diseases, Multiprofile Hospital for Active Treatment of Pulmonary Diseases “St. Sofia”, Medical University–Sofia, 1431 Sofia, Bulgaria;

关键词: tracheal stenosis;    children;    IgG4 immunoproliferative disease;   

DOI  :  10.3390/children8070589

来源: DOAJ

【 摘 要 】

Immunoglobulin G4-related disease (IgG4-RD) is a lymphoproliferative disease which is described almost exclusively in adults. There are only a few pediatric patients who have been observed with this disorder. Here, we describe a rare case of IgG4-RD in a 17-year-old girl with a single manifestation—tracheal stenosis without previous intubation or other inciting event. She had mixed dyspnea and noisy and weakened breathing. Immunoproliferative hyper-IgG4 disease was diagnosed, based on elevated serum IgG4 and histological findings. Until now we have chosen to treat the girl only with corticosteroids with a good response so far. The general condition as well as the respiratory function are regularly monitored. The tracheal involvement of IgG4-RD is uncommon. Nonetheless, it is a manifestation that should be included in the differential diagnosis of tracheal stenosis.

【 授权许可】

Unknown