Fig 1.
Rate of FVC % predicted decline over time.
Time course of the % predicted FVC change in slow and rapid decliners obtained using all the FVC measurements performed during follow-up for each patient. Trends have been estimated using a linear model allowing for non-linearity of time trends, estimated via cubic-splines. The longitudinal trends of the slow and rapid declines are significantly different (p <0.001). The % predicted FVC at diagnosis (time 0) is significantly higher in rapid than in slow decliners (see Table 1).
Table 1.
Clinical characteristics of slow and rapid progressors.
Fig 2.
Total inflammatory cells in slow and rapid progressors.
Number of total leukocytes (CD45+/mm2) in the lungs of slow and rapid progressors. Horizontal bars represent median values; bottom and top of each box plot 25th and 75th, brackets 10th and 90th percentiles. Slow: white; rapid: red.
Fig 3.
Differential Inflammatory cells in slow and rapid progressors.
Number of innate inflammatory cells (neutrophils and macrophages) and adaptive inflammatory cells (CD4+, CD8+, and B lymphocyte) in the lungs of slow and rapid progressors. Horizontal bars represent median values, bottom and top of each box plot 25th and 75th, brackets 10th and 90th percentiles. Slow: white; rapid: red.
Fig 4.
Total inflammatory cells in slow and rapid progressors with and without acute exacerbation (AE).
A. Numbers of total leukocytes (CD45+/mm2) in the lungs of slow and rapid progressors; subjects who developed and those who did not develop AE in each group are considered separately. Horizontal bars represent median values, bottom and top of each box plot 25th and 75th, brackets 10th and 90th percentiles. Slow not AE: white; rapid not AE: red; AE: blue. B. Microphotographs showing total leukocyte (CD45+) infiltration (in red) in the lung of a slow decliner, a rapid decliner and an AE patient. Original magnification X10; immunostaining with anti-CD45.
Fig 5.
Differential inflammatory cells in slow and rapid progressors with and without acute exacerbation (AE).
Number of innate inflammatory cells (neutrophils, macrophages) and adaptive inflammatory cells (CD4+, CD8+, and B lymphocytes) in the lungs of slow and rapid progressors; subjects who developed and those who did not develop AE are considered separately. Horizontal bars represent median values, bottom and top of each box plot 25th and 75th, brackets 10th and 90th percentiles. Slow not AE: white; rapid not AE: red; AE: blue. * P<0.001 compared to slow who develop AE and rapid who do not develop AE
Table 2.
Pathological characteristics of slow and rapid progressors with and without AE.