क्लिनिकल और सेलुलर इम्यूनोलॉजी जर्नल
खुला एक्सेस

अमूर्त

The Deficiency of Serum Mannose Binding Lectin in Early Onset Idiopathic Pulmonary Fibrosis and Familial Cases

VA Varney, J Evans, H Parnell, B Bajardeen, A Nicholas, A Bansal and N Sumar

Background: Idiopathic pulmonary fibrosis (UIP/IPF) is increasingly recognized that siblings & close blood relatives suggesting a genetic predisposition. Serum mannose binding lectin levels (MBL) forms part of the innate immune system with deficiency produces an opsonisation and phagocytosis defect. Serum levels are genetically determined.

Aims & method: We examined the serum MBL in healthy controls (HC), frequently exacerbating COPD, pulmonary TB & Sarcoidosis along with UIP/IPF patients including those with and without an affected family member.

Results: Mean serum MBL levels were not statistically different in HC, COPD or TB. Sarcoid had statistically higher mean levels. Those with IPF onset at <55 yrs old & those with affected blood relatives (FH) had significantly reduced levels compared with IPF onset >55 yrs and no other affected relative.

Chi squared analysis of these patterns showed no differences for HC, COPD & IPF>55 yrs. TB & Sarcoid had higher frequencies of normal MBL levels compared with HC (p=0.001 & 0.024 respectively). IPF <55 yrs & IPF& FH showed higher frequencies of moderate & severe deficiency compared with HC (p=0.001 & 0.001 respectively).

In early onset IPF and IPF & FH, the odds ratio for severe MBL deficiency is 4.32 (95% CI 1.45, 12.83) p=0.0078, and for moderate or severe deficiency was OR 3.309 (95% CI for OR 1.38, 7.91) p=0.0071.

Conclusion: The data suggests that MBL deficiency is common in early onset disease UIP/IPF and cases with an affected relative. The other groups no not show such a defect and their levels are consistent with published data. The action of MBL is central to much of the described histology changes and this observation needs expanding to further cases to gain a fuller understanding of its likely role in the disease process.