Table 1.
Total (n = 311) | S-LAM (n = 272) n (%) | TSC-LAM (n = 39) n (%) | P value | |
---|---|---|---|---|
Age at presentation (y) Mean ± SD |
39.1 ± 9.4 | 39.2 ± 9.2 | 38.3 ± 10.3 | P = 0.481 |
Median (range) | 38 (19 – 71) | 38 (19 – 71) | 36 (21 – 66) | |
Cyst appearance | ||||
Common appearance* alone | 254 (81.7%) | 223 (82.0%) | 31 (79.5%) | P = 0.706 |
Common appearance + large cysts† |
52 (16.7%) | 44 (16.2%) | 8 (20.5%) | P = 0.497 |
Common appearance + cysts with irregularly thickened walls | 2 (0.6%) | 2 (0.7%) | 0 | |
Multiple thin-walled cysts, mostly large | 2 (0.6%) | 2 (0.7%) | 0 | |
Multiple thin-walled cysts, mostly irregularly shaped | 1 (0.3%) | 1 (0.4%) | 0 | |
Findings in addition to cysts | ||||
Lymphatic congestion | 24 (7.7%) | 20 (7.4%) | 4 (10.3%) | |
Lobar area | 16 (5.1%) | 12 (4.4%) | 4 (10.3%) | |
Limited area | 6 (1.9%) | 6 (2.2%) | 0 | |
Mediastinal area | 2 (0.6%) | 2 (0.7%) | 0 | |
Diffuse noncalcified nodules¶ | 6 (1.9%) | 6 (2.2%) | 0 | |
with small cavitary changes | 4 (1.3%) | 4 (1.5%) | 0 | |
with ground-glass attenuation | 3 (1.0%) | 3 (1.1%) | 0 | |
TSC-related findings | ||||
Rounded ground-glass opacities suggestive of MMPH |
15 (4.8%) | 1 (0.4%) | 14 (35.9%) | P < 0.001 |
Myocardial fatty foci | 26 (8.4%) | 8 (2.9%) | 18 (46.1%) | P < 0.001 |
Bone nodules | 60 (19.3%) | 28 (10.3%) | 32 (82.1%) | P < 0.001 |
HRCT high resolution computed tomography, LAM lymphangioleiomyomatosis, MMPH multifocal micronodular pneumocyte hyperplasia, SD standard deviation, S-LAM sporadic LAM, TSC-LAM tuberous sclerosis complex-associated LAM, y years of age.
*Common appearance is multiple thin-walled oval-shaped pulmonary cysts distributed evenly throughout both lung fields.
†Large cysts are those > 2 cm.
¶One patient had diffuse nodules with both small cavitary changes and ground-glass opacities.