Case | 1 | 2 | 3 | 4 | 5 | 6 | |
Age yrs | 63 | 50 | 50 | 55 | 60 | 78 | |
Sex | F | M | M | M | M | F | |
Smoking pack-yrs | 0 | 19 | 14 | 26.2 | 0 | 0 | |
Prior medical history | - | - | DM | - | DM | DM | |
Respiratory symptom | Cough | Cough | Cough | Cough | Cough | Cough | |
Serological tests (normal range) | |||||||
LDH IU·mL−1 (114–220) | 161 | 168 | 150 | 215 | 149 | 214 | |
CRP mg·dL−1 (<0.1) | 0.03 | 0.04 | 0.13 | 0.09 | 0.03 | 0.01 | |
ACE U·L−1 (8.3–21.4) | 5.3 | 7.8 | 17.3 | 19.5 | 14.6 | 19.0 | |
IL-6 pg·mL−1 (<2.41) | 1.27 | 0.30 | 2.02 | 1.52 | 0.54 | 0.38 | |
IgG4 U·mL−1 (<108) | 1660 | 1610 | 773 | 2970 | 1910 | 1980 | |
IgG mg·dL−1 (870–1700) | 2889 | 2741 | 2001 | 5545 | 3767 | 3118 | |
IgG4/IgG % | 57.4 | 58.7 | 38.6 | 53.5 | 50.7 | 63.5 | |
Chest CT findings | |||||||
BHL | + | + | + | + | + | + | |
Bronchial wall and BVB thickening | + | + | + | + | + | + | |
Small nodular shadow | - | + | - | + | + | + | |
Pleural irregularity | - | + | - | + | + | + | |
Interlobular septal thickening | - | - | - | - | - | + | |
Consolidation | - | - | - | - | - | - | |
Emphysematous change | - | - | - | + | - | - | |
PFTs | |||||||
VC L | 3.11 | 5.10 | 4.96 | 3.73 | 4.27 | 2.01 | |
VC % pred | 129.0 | 138.2 | 130.5 | 106.0 | 100.7 | 82.4 | |
FEV1 L | 2.17 | 3.60 | 3.52 | 3.08 | 2.67 | 1.56 | |
FEV1 % pred | 106.7 | 104.7 | 97 | 96.6 | 77.6 | 90.2 | |
FEV1/FVC % | 67.8 | 69.9 | 69.6 | 77.6 | 60.5 | 74.3 | |
PEF L·s−1 | 3.16 | 7.93 | 8.09 | 7.55 | 5.23 | 2.79 | |
PEF % pred | 56.2 | 93.3 | 91.9 | 92.1 | 60.1 | 51.8 | |
BALF | |||||||
Total cells ×104 cells·mL−1 | 4.0 | 5.4 | 5.5 | 1.4 | 4.0 | 1.9 | |
Macrophages % | 60.6 | 69.3 | 54.4 | 24.4 | 79.1 | 54.8 | |
Lymphocytes % | 39.0 | 25.1 | 40.6 | 31.0 | 19.9 | 36.7 | |
Neutrophils % | 0.4 | 0.4 | 0.9 | 0.4 | 0.8 | 1.8 | |
Eosinophils % | 0 | 5.2 | 4.1 | 44.2 | 0.2 | 6.7 | |
CD4/CD8 ratio | 4.48 | 2.40 | 1.53 | 1.71 | 1.27 | 1.70 | |
Immunohistochemical positive cells in bronchi | |||||||
IgG4-positive cells per HPF | 80.0 | 80.6 | 54.3 | 71.6 | 43.0 | 36.6 | |
IgG-positive cells per HPF | 126.0 | 111 | 64.3 | 107.6 | 86.0 | 51.6 | |
IgG4-positive/IgG-positive cells % | 63.4 | 72.6 | 84.4 | 34.5 | 50.0 | 70.9 |
LDH: lactate dehydrogenase; CRP: C-reactive protein; ACE: angiotensin-converting enzyme; IL: interleukin; Ig: immunoglobulin; BHL: bilateral hilar lymphadenopathy; BVB: bronchovascular bundle; VC: vital capacity; % pred: % predicted; FEV1: forced expiratory volume in 1 s; FVC: forced VC; PEF: peak expiratory flow; BALF: bronchoalveolar lavage fluid; HPF: high-powered field; F: female; M: male; -: negative; +: positive; DM: diabetes mellitus.