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

被引：2

作者：

Gabrovska, Natalia ^{[1
]}

Velizarova, Svetlana ^{[1
]}

Spasova, Albena ^{[1
]}

Kostadinov, Dimitar ^{[1
]}

Yanev, Nikolay ^{[1
]}

Shivachev, Hristo ^{[2
]}

Rangelov, Edmond ^{[2
]}

Pahnev, Yanko ^{[2
]}

Antonova, Zdravka ^{[2
]}

Kartulev, Nikola ^{[2
]}

Terziev, Ivan ^{[3
]}

Gabrovski, Kaloyan ^{[4
]}

机构：

[1] Med Univ Sofia, Multiprofile Hosp Act Treatment Pulm Dis St Sofia, Dept Pulm Dis, Sofia 1431, Bulgaria

[2] Univ Multiprofile Hosp Act Treatment & Emergency, Dept Pediat Thorac Surg, Pediat Surg Clin, Sofia 1606, Bulgaria

[3] Univ Hosp Tsaritsa Uoanna ISUL, Dept Pathol, Sofia 1527, Bulgaria

[4] Med Univ Sofia, Univ Hosp St Ivan Rilski, Dept Neurosurg, Sofia 1431, Bulgaria

来源：

CHILDREN-BASEL | 2021年 / 8卷 / 07期

关键词：

tracheal stenosis; children; IgG4 immunoproliferative disease; IGG4-RELATED DISEASE; RITUXIMAB THERAPY; IGG4;

D O I：

10.3390/children8070589

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

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.

引用

页数：5

共 26 条

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