Granulomatosis with polyangiitis with nasal symptoms as the first clinical manifestation: clinical analysis of 18 cases.
10.3760/cma.j.cn115330-20200714-00587
- Author:
Xue Qing CHENG
1
;
Gui Ling ZHANG
1
;
Wei Yi WANG
1
;
Lei SHI
1
;
Er Peng ZHANG
1
;
Bing LI
1
;
Zhi Yu ZHANG
1
;
Guang Gang SHI
1
Author Information
1. Department of Otorhinolaryngology Head and Neck Surgery, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, China.
- Publication Type:Journal Article
- MeSH:
Antibodies, Antineutrophil Cytoplasmic;
Cyclophosphamide;
Endoscopy;
Female;
Granulomatosis with Polyangiitis/diagnosis*;
Humans;
Male;
Paranasal Sinuses
- From:
Chinese Journal of Otorhinolaryngology Head and Neck Surgery
2021;56(6):613-618
- CountryChina
- Language:Chinese
-
Abstract:
Objective: To analyze and summarize the diagnosis, treatment and prognosis of granulomatosis with polyangiitis (GPA) with nasal symptoms as the first clinical manifestation. Methods: The data of 18 patients of GPA with nasal mucosal symptoms as the first clinical manifestation from the Department of Otorhinolaryngology Head and Neck Surgery, Shandong Provincial Hospital Affiliated to Shandong First Medical University between 2005 and 2019 was collected, including 8 males and 10 females, aged from 5 to 68 years. Nasal endoscopy, imaging examination, laboratory examination, immunological and histopathological examination of nasal mucosa were completed. All patients were treated with glucocorticoid combined with cyclophosphamide and were followed up for 2 to 15 years. Descriptive statistical method was used for analysis. Results: All the 18 patients had the nasal mucosal symptoms as the first clinical manifestation, including nasal obstruction, running nose and epistaxis. Nasal endoscopy showed swelling, erosion, scab and bleeding of nasal mucosa, and 6 cases had nasal septal perforation. Nasal sinus CT scan showed high density shadow of sinus, as well as hyperostosis and osteosclerosis. CT imaging features of pulmonary showed nodular lesion or patchy infiltration in 12 patients and cavitation was found in 6 cases. Laboratory results showed that 13 cases were positive for anti-neutrophil cytoplasmic antibodies (ANCA), and 5 cases were negative. During follow-up period, thirteen patients were symptomatic controlled and survived; two patients died of disease progression; one patient gave up treatment and died; two patients were lost to follow-up. Conclusions: Nasal symptoms are the first clinical manifestation of GPA. Early diagnosis and early treatment with glucocorticoid combined with cyclophosphamide can effectively improve the survival rate.
