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[Clinical features of IgG4-related lung disease]
W Zhang1, M Q Zhang1, P H Gong1
1Department of Respiratory and Critical Care Medicine, Peking University People's Hospital, Beijing 100044, China.
Insights
This study reveals that IgG4-related lung disease often presents atypically, with many patients being asymptomatic. Clinical features and imaging outcomes do not significantly differ between symptomatic and asymptomatic individuals.
Area of Science:
- Pulmonology
- Immunology
- Radiology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a multisystemic fibroinflammatory condition.
- IgG4-related lung disease (IgG4-RLD) is an under-recognized manifestation of IgG4-RD.
- Understanding the clinical spectrum of IgG4-RLD is crucial for timely diagnosis and management.
Purpose of the Study:
- To elucidate the diverse clinical manifestations of IgG4-related lung disease.
- To analyze laboratory findings, imaging characteristics, and prognostic factors in IgG4-RLD patients.
- To compare clinical features and outcomes between patients with and without respiratory symptoms.
Main Methods:
- Retrospective analysis of clinical data from 60 patients diagnosed with IgG4-RLD.
- Inclusion criteria: Diagnosis of IgG4-RLD between February 2012 and May 2021.
- Data collected: Clinical presentation, serological markers (IgG4, IgE, complement), imaging findings, pathology, and treatment outcomes.
Main Results:
- Atypical presentations are common, with a high proportion of asymptomatic cases (63.3% nodular changes, 56.7% lymphadenopathy, 51.7% GGOs).
- Elevated serum IgG4 (>1.35 g/L) was observed in 94.6% of patients. Other common findings included elevated IgE (79.2%) and decreased C3/C4 (53.7%).
- Patients with respiratory symptoms showed higher total IgG and eosinophils, but no significant differences in IgG4, IgE, complement, or treatment outcomes compared to asymptomatic patients.
Conclusions:
- IgG4-related lung disease exhibits heterogeneous clinical and imaging features, often presenting asymptomatically.
- Multiple concurrent imaging abnormalities are frequent in IgG4-RLD.
- The presence or absence of respiratory symptoms does not significantly alter the main clinical features or imaging outcomes of IgG4-RLD.
Abstract:
Objective: To explore the clinical features of IgG4-related lung disease. Methods: The clinical data of 60 patients diagnosed with IgG4-related lung disease in Peking University People's Hospital from February 2012 to May 2021 were retrospectively collected. Analysis was made to explore the features of clinical manifestation, laboratory, imaging, prognosis and other characteristics of the disease. Results: A total of 60 patients were included, with 40 males, age of (58.2±12.9) years, an age of onset of (57.1±13.2) years, and 31.7% (19 cases) of the patients had a history of allergic disease. 36.7% (22 cases) of the patients had respiratory symptoms during the disease. 94.6% (53/56) of patients had serum IgG4>1.35 g/L, 24.1% (14/58) of patients had increased eosinophils, 79.2% (38/48) of patients had increased IgE level, and 53.7% (29/54) of patients had decreased C3 or C4. Common imaging findings included nodular changes (38 cases, 63.3%), mediastinal and/or hilar lymphadenopathy (34 cases, 56.7%), and ground glass opacities (31 cases, 51.7%). Fifty-three cases (88.3%) showed two or more imaging changes. The pathological examination of the patient was mainly characterized by lymphoplasmacytic infiltration and fibrosis, with only one case of phlebitis obliterans. Compared with the asymptomatic group (38 cases), patients with respiratory symptoms (22 cases) showed higher level of serum total IgG and eosinophils (43.2 vs 17.8 g/L, 0.30×109/L vs 0.14×109/L, P<0.05), lower proportion of allergic diseases, and higher proportion of consolidation shadows on chest CT (P<0.05). There were no significant differences in serum IgG4, IgE, complement levels, and imaging outcomes after treatment between the two groups (P>0.05). Conclusions: The clinical manifestations of IgG4-related lung disease are atypical, and asymptomatic patients account for a high proportion. The imaging of the disease is highly heterogeneous, and patients are prone to show coexisted multiple imaging changes. The main clinical features and imaging outcomes of patients with and without respiratory symptoms are not significantly different.
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