A Case of Tracheal Stenosis as an Isolated Form of Immunoproliferative Hyper-IgG4 Disease in a 17-Year-Old Girl
Natalia Gabrovska,
Svetlana Velizarova,
Albena Spasova,
Dimitar Kostadinov,
Nikolay Yanev,
Hristo Shivachev,
Edmond Rangelov,
Yanko Pahnev,
Zdravka Antonova,
Nikola Kartulev,
Ivan Terziev,
Kaloyan Gabrovski
Affiliations
Natalia Gabrovska
Department of Pulmonary Diseases, Multiprofile Hospital for Active Treatment of Pulmonary Diseases “St. Sofia”, Medical University–Sofia, 1431 Sofia, Bulgaria
Svetlana Velizarova
Department of Pulmonary Diseases, Multiprofile Hospital for Active Treatment of Pulmonary Diseases “St. Sofia”, Medical University–Sofia, 1431 Sofia, Bulgaria
Albena Spasova
Department of Pulmonary Diseases, Multiprofile Hospital for Active Treatment of Pulmonary Diseases “St. Sofia”, Medical University–Sofia, 1431 Sofia, Bulgaria
Dimitar Kostadinov
Department of Pulmonary Diseases, Multiprofile Hospital for Active Treatment of Pulmonary Diseases “St. Sofia”, Medical University–Sofia, 1431 Sofia, Bulgaria
Nikolay Yanev
Department of Pulmonary Diseases, Multiprofile Hospital for Active Treatment of Pulmonary Diseases “St. Sofia”, Medical University–Sofia, 1431 Sofia, Bulgaria
Hristo Shivachev
Department of Pediatric Thoracic Surgery, Pediatric Surgery Clinic, University Multiprofile Hospital for Active Treatment and Emergency Medicine “N. I. Pirogov”, 1606 Sofia, Bulgaria
Edmond Rangelov
Department of Pediatric Thoracic Surgery, Pediatric Surgery Clinic, University Multiprofile Hospital for Active Treatment and Emergency Medicine “N. I. Pirogov”, 1606 Sofia, Bulgaria
Yanko Pahnev
Department of Pediatric Thoracic Surgery, Pediatric Surgery Clinic, University Multiprofile Hospital for Active Treatment and Emergency Medicine “N. I. Pirogov”, 1606 Sofia, Bulgaria
Zdravka Antonova
Department of Pediatric Thoracic Surgery, Pediatric Surgery Clinic, University Multiprofile Hospital for Active Treatment and Emergency Medicine “N. I. Pirogov”, 1606 Sofia, Bulgaria
Nikola Kartulev
Department of Pediatric Thoracic Surgery, Pediatric Surgery Clinic, University Multiprofile Hospital for Active Treatment and Emergency Medicine “N. I. Pirogov”, 1606 Sofia, Bulgaria
Ivan Terziev
Department of Pathology, University Hospital ‘’Tsaritsa Uoanna–ISUL’’, 1527 Sofia, Bulgaria
Kaloyan Gabrovski
Department of Neurosurgery, University Hospital “St. Ivan Rilski”, Medical University–Sofia, 1431 Sofia, Bulgaria
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.
