Figures
- FIGURE 1
Pulmonary function test results before and after immunosuppressive therapy. a) Received acute steroid to induce remission, b) received mycophenolate mofetil with or without corticosteroids to maintain remission and c) received no treatment other than immunoglobulin replacement therapy. Each line represents an individual patient. FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s; DLCO: diffusing capacity of the lung for carbon monoxide.
Tables
- TABLE 1
Patient characteristics (n=14)
Sex Male 6 (43) Female 8 (57) Age years 52±14 Age at CVID diagnosis years 32±16 Smoking Non-smoker 12 (86) Former smoker 2 (14) Autoimmune manifestations Splenomegaly 13 (93) ITP 6 (43) AIHA 4 (29) Uveitis 3 (21) Neutropenia 2 (14) Other; plaque psoriasis, rheumatoid arthritis 2 (14) Data are presented as n (%) and mean±sd. CVID: common variable immunodeficiency disorder; ITP: immune thrombocytopenic purpura; AIHA: autoimmune haemolytic anaemia.
- TABLE 2
Laboratory characteristics of granulomatous lymphocytic interstitial lung disease patients
Laboratory characteristic Acute steroid Chronic MMF±corticosteroids Immunoglobulin replacement Serum immunoglobulin levels prior to period under study g·L−1 IgG 9.70 (8.30–10.40) 8.00 (6.70–11.15) 10.15 (6.60–14.38) IgA 0.05 (0.05–0.10) 0.05 (0.05–0.50) 0.05 (0.01–0.16) IgM 0.70 (0.50–1.30) 0.70 (0.08–2.70) 0.15 (0.06–0.28) Trough immunoglobulin levels during period under study g·L−1 IgG 9.50 (8.93–11.60) 10.70 (10.20–11.90) 10.55 (10.05–12.70) IgA 0.05 (0.04–0.16) 0.05 (0.05–0.50) 0.05 (0.05–0.31) IgM 0.30 (0.16–0.90) 0.30 (0.05–1.30) 0.05 (0.05–0.24) Lymphocyte subsets count prior to period under study ×109 per L Lymphocyte count 1.09 (0.85–1.21) 1.32 (0.68–1.70) 1.07 (0.72–2.03) CD3 0.96 (0.82–1.01) 1.01 (0.55–1.28) 0.81 (0.55–1.33) CD4 0.59 (0.35–0.73) 0.44 (0.28–0.77) 0.61 (0.42–0.71) CD8 0.25 (0.20–0.42) 0.35 (0.22–0.63) 0.14 (0.09–0.76) CD19 0.04 (0.02–0.09) 0.20 (0.10–0.23) 0.15 (0.10–0.39) CD16+CD56 0.08 (0.03–0.38) 0.08 (0.02–0.13) 0.08 (0.05–0.12) Lymphocyte subsets count during period under study ×109 per L Lymphocyte count 0.78 (0.43–1.17) 1.13 (0.55–1.87) 0.60 (0.28–1.13) CD3 0.66 (0.39–0.98) 0.88 (0.48–1.29) 0.40 (0.19–0.93) CD4 0.32 (0.22–0.65) 0.43 (0.27–0.74) 0.35 (0.18–0.38) CD8 0.24 (0.18–0.29) 0.29 (0.17–0.59) 0.06 (0.03–0.51) CD19 0.03 (0.01–0.06) 0.16 (0.02–0.38) 0.10 (0.03–0.16) CD16+CD56 0.05 (0.02–0.11) 0.06 (0.04–0.18) 0.04 (0.02–0.06) CVID EUROClass smB+21Lo 5 3# smB+21norm 1 1 smB-21Lo 2 3 B- 1 Data are presented as median (interquartile range). MMF: mycophenolate mofetil; CVID: common variable immunodeficiency disorder. #: two patients were also in the acute steroid group.
- TABLE 3
Medications used for treatment of granulomatous lymphocytic interstitial lung disease and lung function results before and after treatment
Group Time months PFT Before treatment After treatment p-value Acute corticosteroid 0.5–1 mg·kg−1 daily 3.8 FVC% 77 (50–95) 74 (54–110) 0.09 FEV1% 77 (42–96) 73 (48–96) 0.92 DLCO% 59 (43–69) 66 (57–86) 0.04 Mycophenolate mofetil 500–1000 mg twice a day 46 FVC% 79 (71–102) 89 (72–102) 0.35 FEV1% 74 (66–100) 81 (73–102) 0.08 DLCO% 63 (55–75) 72 (67–79) 0.04 Immunoglobulin replacement only
400–800 mg·kg−1 3-weekly94 FVC% 92 (80–95) 72 (65–89) # FEV1% 82 (53–85) 57 (46–83) # DLCO% 77 (69–90) 64 (54–68) # Data are presented as median (interquartile range), unless otherwise stated. PFT: pulmonary function test; FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s; DLCO: diffusing capacity of the lung for carbon monoxide. #: no significant difference compared with pre-treatment period due to small group numbers.
