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. 2015 Sep 30:15:110.
doi: 10.1186/s12890-015-0105-0.

Serum B cell-activating factor (BAFF) level in connective tissue disease associated interstitial lung disease

Tsutomu Hamada  1 , Takuya Samukawa  2 , Tomohiro Kumamoto  3 , Kazuhito Hatanaka  4 , Go Tsukuya  5 , Masuki Yamamoto  6 , Kentaro Machida  7 , Masaki Watanabe  8 , Keiko Mizuno  9 , Ikkou Higashimoto  10 , Yoshikazu Inoue  11 , Hiromasa Inoue  12
Affiliations

Serum B cell-activating factor (BAFF) level in connective tissue disease associated interstitial lung disease

Tsutomu Hamada et al. BMC Pulm Med. .

Erratum in

Abstract

Background: Interstitial lung diseases (ILDs) are common in patients with connective tissue diseases (CTDs). Although the diagnosis of an underlying CTD in ILD (CTD-ILD) affects both prognosis and treatment, it is sometimes difficult to distinguish CTD-ILD from chronic fibrosing interstitial pneumonia (CFIP). B cell-activating factor belonging to the tumour necrosis factor family (BAFF) plays a crucial role in B cell development, survival, and antibody production.

Methods: We examined serum levels of BAFF, surfactant protein D (SP-D), and Krebs von den Lungen-6 (KL-6) in 33 patients with CTD-ILD, 16 patients with undifferentiated CTD-ILD, 19 patients with CFIP, and 26 healthy volunteers. And we analysed the relationship between serum BAFF levels and pulmonary function, as well as the expression of BAFF in the lung tissue of patients with CTD-ILD.

Results: Serum levels of BAFF were significantly higher in CTD-ILD patients compared to healthy subjects and CFIP patients. However, there were no significant differences in serum levels of SP-D and KL-6. Furthermore, serum BAFF levels in CTD-ILD patients were inversely correlated with pulmonary function. BAFF was strongly expressed in the lungs of CTD-ILD patients, but weakly in normal lungs.

Discussion: This is the first study to demonstrate that serum BAFF levels were significantly higher in CTD-ILD patients compared to healthy subjects and CFIP patients. Furthermore, serum BAFF levels were correlated with pulmonary function. We consider that serum BAFF levels in patients with CTD-ILD reflect the presence of ILDs disease activity and severity.

Conclusion: These finding suggest that BAFF may be a useful marker for distinguishing CTD-ILD from CFIP.

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Figures

Fig. 1
Fig. 1
Distribution of serum a BAFF, (b) SP-D, and (c) KL-6 levels in patients with CTD-ILD (n = 33), undifferentiated CTD-ILD (n = 16), CFIP (n = 19), and healthy control subjects (n = 26). Differences in each variable across groups were first analyzed using the Kruskal-Wallis test, followed by the Bonferroni test. BAFF: B cell–activating factor belonging to the tumour necrosis factor family; KL-6: Krebs von den Lungen-6; SP-D: surfactant protein D; CTD-ILD: interstitial lung disease associated with connective tissue disease; CFIP: chronic fibrosing interstitial lung disease
Fig. 2
Fig. 2
Correlation between serum BAFF, SP-D, and KL-6 levels and pulmonary function testing parameters in patients with CTD-ILD. The correlation between serum BAFF (a), SP-D (b), and KL-6 (c) and FVC % predicted in CTD-ILD patients is shown. BAFF: B cell–activating factor belonging to the tumour necrosis factor family; SP-D: surfactant protein D; KL-6: Krebs von den Lungen-6; FVC: forced vital capacity; CTD-ILD: interstitial lung disease associated with connective tissue disease
Fig. 3
Fig. 3
Representative immunohistochemical findings for BAFF in lung sections. Tissue was obtained from a, b a 23-year-old female non-smoker as a control, (c–g) a 56-year-old female non-smoking patient with CTD-ILD, and (h, i) a 63-year-old male non-smoking patients with CFIP. Weakly positive alveolar macrophages were observed in the a) BAFF b) goat IgG control. Strong cytoplasmic positivity of BAFF was observed in c), h) peripheral airways macrophages and cells in the alveolar walls, d), i) lymphoid follicles, e) peripheral airways, f) vascular endothelial cell and g) fibroblasts. Scale bars = 50 μm. BAFF: B cell–activating factor belonging to the tumour necrosis factor family; CTD-ILD: interstitial lung disease associated with connective tissue disease; CFIP: chronic fibrosing interstitial lung disease
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