Table 1– Patient profiles, laboratory data, chest computed tomography (CT) findings, pulmonary function tests (PFTs) and bronchoscopic data
Case123456
Age yrs635050556078
SexFMMMMF
Smoking pack-yrs0191426.200
Prior medical history--DM-DMDM
Respiratory symptomCoughCoughCoughCoughCoughCough
Serological tests (normal range)
 LDH IU·mL−1 (114–220)161168150215149214
 CRP mg·dL−1 (<0.1)0.030.040.130.090.030.01
 ACE U·L−1 (8.3–21.4)5.37.817.319.514.619.0
 IL-6 pg·mL−1 (<2.41)1.270.302.021.520.540.38
 IgG4 U·mL−1 (<108)16601610773297019101980
 IgG mg·dL−1 (870–1700)288927412001554537673118
 IgG4/IgG %57.458.738.653.550.763.5
Chest CT findings
 BHL++++++
 Bronchial wall and BVB thickening++++++
 Small nodular shadow-+-+++
 Pleural irregularity-+-+++
 Interlobular septal thickening-----+
 Consolidation------
 Emphysematous change---+--
PFTs
 VC L3.115.104.963.734.272.01
 VC % pred129.0138.2130.5106.0100.782.4
 FEV1 L2.173.603.523.082.671.56
 FEV1 % pred106.7104.79796.677.690.2
 FEV1/FVC %67.869.969.677.660.574.3
 PEF L·s−13.167.938.097.555.232.79
 PEF % pred56.293.391.992.160.151.8
BALF
 Total cells ×104 cells·mL−14.05.45.51.44.01.9
 Macrophages %60.669.354.424.479.154.8
 Lymphocytes %39.025.140.631.019.936.7
 Neutrophils %0.40.40.90.40.81.8
 Eosinophils %05.24.144.20.26.7
 CD4/CD8 ratio4.482.401.531.711.271.70
Immunohistochemical positive cells in bronchi
 IgG4-positive cells per HPF80.080.654.371.643.036.6
 IgG-positive cells per HPF126.011164.3107.686.051.6
 IgG4-positive/IgG-positive cells %63.472.684.434.550.070.9
  • 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.