An erratum to this article can be found at http://dx.doi.org/10.1186/s12890-016-0278-1.
The authors declare that they have no competing interests.
TH and TS conceived of the study and its design, performed research, data analysis and interpretation, and manuscript writing. GT helped with the statistical analysis. HK carried out the immunoassays and helped to draft the manuscript. TK, MY, KM, MW, IH and KM discussed the original idea and facilitated plans for this study. YI and HI reviewed the data, and revised the manuscript critically for intellectual content. All authors have contributed to writing and revising the manuscript with final approval of its contents.
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.
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.
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.
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.
These finding suggest that BAFF may be a useful marker for distinguishing CTD-ILD from CFIP.
Navaratnam V, Ali N, Smith CJP, McKeever T, Fogarty A, Hubbard RB. Does the presence of connective tissue disease modify survival in patients with pulmonary fibrosis? Respir Med. 2011;105(12):1925–30. doi: http://dx.doi.org/10.1016/j.rmed.2011.08.015. CrossRefPubMed
Travis WD, Costabel U, Hansell DM, King Jr TE, Lynch DA, Nicholson AG, et al. An official american thoracic society/european respiratory society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188(6):733–48. CrossRefPubMed
Cheema GS, Roschke V, Hilbert DM, Stohl W. Elevated serum B lymphocyte stimulator levels in patients with systemic immune-based rheumatic diseases. Arthritis Rheum. 2001;44(6):1313–9. doi: 10.1002/1529-0131(200106)44:6<1313::aid-art223>3.0.co;2-s. CrossRefPubMed
Kaneko T, Amano H, Kawano S, Minowa K, Ando S, Watanabe T, et al. Increased serum concentration of BAFF/APRIL and IgA2 subclass in patients with mixed connective tissue disease complicated by interstitial lung disease. Mod rheumatol / the Japan Rheumatism Association. 2013. doi: 10.3109/14397595.2013.843748.
Kasukawa R. Mixed connective tissue disease. Internal medicine (Tokyo, Japan). 1999;38(5):386–93. CrossRef
Kobayashi J, Itoh Y, Kitamura S, Kawai T. Establishment of reference intervals and cut-off value by an enzyme immunoassay for KL-6 antigen, a new marker for interstitial pneumonia. Rinsho byori The Japanese journal of clinical pathology. 1996;44(7):653–8. PubMed
Abe S, Honda Y, Ando M, Saita N, Kida K, Jinno S, et al. Clinical significance of levels of lung surfactant protein A in serum, in various lung diseases. Nihon Kyobu Shikkan Gakkai Zasshi. 1995;33(11):1219–25. PubMed
Honda YTH, Abe S, Yuminaga K, Saita N, Ando M, Kondo A, et al. Clinical evaluation of surfactant protein D kit "Yamasa" in various lung disease. Jpn J Med and Pharm Sci. 1996;36(4):809–15.
Ohnishi H, Yokoyama A, Kondo K, Hamada H, Abe M, Nishimura K, et al. Comparative study of KL-6, surfactant protein-A, surfactant protein-D, and monocyte chemoattractant protein-1 as serum markers for interstitial lung diseases. Am J Respir Crit Care Med. 2002;165(3):378–81. doi: 10.1164/ajrccm.165.3.2107134. CrossRefPubMed
Nicholson AG, Colby TV, du Bois RM, Hansell DM, Wells AU. The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med. 2000;162(6):2213–7. doi: 10.1164/ajrccm.162.6.2003049. CrossRefPubMed
Park IN, Jegal Y, Kim DS, Do KH, Yoo B, Shim TS, et al. Clinical course and lung function change of idiopathic nonspecific interstitial pneumonia. The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology. 2009;33(1):68–76. doi: 10.1183/09031936.00158507. CrossRef
Polverino F, Baraldo S, Bazzan E, Agostini S, Turato G, Lunardi F, et al. A novel insight into adaptive immunity in chronic obstructive pulmonary disease: B cell activating factor belonging to the tumor necrosis factor family. Am J Respir Crit Care Med. 2010;182(8):1011–9. doi: 10.1164/rccm.200911-1700OC. CrossRefPubMed
- Serum B cell–activating factor (BAFF) level in connective tissue disease associated interstitial lung disease
- BioMed Central
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