Groups ;- numbers of cases in each group MASP-2<100 ng/ml MASP-2 100-200ng/ml MASP-2 Normal>200 ng/ml Chi square analysis* with HC Chi square analysis* All IPF patients Healthy controls n=150 5 (3%) 12 (8%) 133 (89%) - 0.0001 COPD n=34 1 (2%) 0 33 (98%) 1 0.008 TB n=47 3 (6%) 8 (17%) 36 (77%) 0.399 0.0124 Sarcoid n=38 3 (8%) 6 (16%) 29 (76%) 0.2042 0.0402 IPF>55yrs n=94 19 (20%) 22 (24%) 53 (56%) 0.0001 0.5297 IPF<55yrs n=30 10 (33%) 5 (17%) 15 (50%) 0.0001 0.2207 IPF & FH n=21 4 (19%) 4 (19%) 13 (62%) 0.0143 0.7023 All fibrosis n=145 33 (23%) 31 (21%) 81 (56%) 0.0001 - *number of cases with MASP-2<100 ng/ml versus number of cases with MASP-2>100 ng/ml TB versus sarcoid p=1.0 Significance at p<0.01

Table 3: Pattern of MASP-2 deficiency in the study groups with results of chi-square analysis for frequency of severe MASP-2 deficiency (<100 ng/ml versus >100 ng/ml).