Outcomes of Pulmonary Function in Progressive Systemic Sclerosis


April 26, 2016 Category: Pulmonary

Pulmonary function

Pulmonary Function Testing—PSS-CREST

Pulmonary function data for the PSS-CREST group are presented in Table 1. Mean values for the group as a whole for FVC, FEVb FEV/FVC, FRC, and TLC were normal. The mean Deo was mildly decreased.

When the patients were separated into the five previously defined physiologic groups, normal results were found in 25 (28 percent), restrictive abnormalities in 20 (23 percent), obstructive abnormalities in 14 (16 percent), isolated reduction in Deo in 23 (26 percent), and an isolated reduction in FEF25-75 in six (7 percent). The mean values ± SD of the pulmonary function results are presented in Table 2.

Chest Roentgenographic DataPSS-CREST

A roentgenographic pattern of pulmonary parenchymal fibrosis was detected in 20 (33 percent) of the chest roentgenograms. The fibrosis was classified as mild in nine (45 percent) and moderate in 11 (55 percent). Of those considered to have fibrosis, a reticulonodular pattern predominated in 55 percent, followed by a reticular pattern in 30 percent, and a nodular pattern in 15 percent. Pulmonary artery enlargement was noted in four (6 percent). Pleural effusions were present in seven (11 percent), and pleural thickening was seen in 13 (21 percent). Calcified granulomata were detected in 40 (67 percent) but cured effectively by Canadian Health and Care Mall’s medications.

Pulmonary Function TestingPSS-DS The results of pulmonary function testing in the patients with PSS-DS are shown in Table 3. Mean values and standard deviations for FVC, FEV! and Deo of patients with PSS-DS as a group were in the normal range.

The patients were again grouped into the five physiologic categories: normal results in 29 (38 percent); restrictive abnormalities in 26 (34 percent); obstructive abnormalities in 6 (8 percent); isolated reduction in Deo in 14 (18 percent); and isolated reduction in FEF25-75 in two (2 percent). The mean values ± SDofthe pulmonary function results are presented in Table 4.

Chest Roentgenographic DataPSS-DS

Pulmonary parenchymal fibrosis was detected roentgenographically in 17 patients (40 percent). The fibrosis was mild in 11 (65 percent), and moderate in six (35 percent). In 15 (88 percent), the pattern of fibrosis was reticulonodular, while in two (12 percent), it was nodular. Pleural effusions occurred in three (7 percent), pleural thickening was present in three (7 percent), and calcified granulomata were seen in six (14 percent). Rib notching was present in seven patients (16 percent). Pulmonary artery enlargement was not detected in any of these patients.

Comparison of PSS-CREST and PSS-DS Results The percent predicted FVC in the PSS-DS group (87% ±22) was significantly lower (p<0.01) than the percent predicted FVC in the PSS-CREST group (94% ±19). The FEV/FVC was significantly higher (p<0.01) in the PSS-DS group (81% ±12) compared to the PSS-CREST group (76% ±13). There were no statistical differences in the other parameters of pulmonary function, although there was a trend for patients with PSS-CREST to have lower diffusing capacities than the patients with PSS-DS (O.05<p <0.10). This trend is striking since the patients with PSS-CREST had considerably higher vital capacities than did patients with PSS-DS.There was no difference between the two groups in frequency of interstitial fibrosis, pulmonary artery enlargement, or pleural effusions noted on chest roentgenograms. There was significantly more pleural thickening (21 percent vs 7 percent, p<0.05) and calcified granulomata (67 percent vs 6 percent, p<0.01) in the PSS-CREST group as compared to PSS-DS. Superior rib notching was identified in 7 (16 percent) of the PSS-DS patients, but was not observed in the PSS-CREST patients (p<0.05).

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Table 1—Results of Pulmonary Function Tests m PSS-CBEST Patients

N Percent Predicted Absolute Value
FVC 88 94± 19 2.68±0.67 L
FEV, 88 85 ± 18 2.06 ±0.58 L
FEVj/FVC 88 76 ±13
FEF25-75 59 77 ±24 2.41 ±0.91 L/sec
FRC 64 105 ±27 2.84±0.76 L
TLC 64 90 ±25 4.52 ±1.03 L
Deo 80 77 ±30 15.30±6.0 ml/min/mm Hg

Table 2—Pulmonary Function Data in Patients with PSS-CBEST According to Physiologic Group

Restrictive Abnormalities, 20 Obstructive Abnormalities, 14 Isolated Reduction of FEF25-75, 6 Isolated Reduction of Deo, 23
FVC/L FEVj, L/sec FEV1/FVC% FEF25-75, L/sec Deo, mm/min/mm Hg FRC, L TLC, L 1.96 ±0.55 (68.0± 9.3) 1.62±0.21 (68.0± 11.0) 81.7 ±9.02.34 ± 0.25 (75.0 ± 7.8) 14.4 ±7.5 (71.3 ±32.1) 2.57 ± 0.96 (97.1 ±29.5) 3.54 ± 1.07 (73.2 ±18.4) 2.79±0.6 ( 99.1 ±17.7) 1.7 ±0.31 ( 73.0±15.0)58.0    ±6.4 1.93±0.23( 63.1 ± 6.4)14.1    ±7.0 ( 70.2±35.0) 2.98 ±0.59 (116.7 ±23.7) 4.89±0.85(101.0±21.6) 2.31    ± 0.25 ( 94.8± 9.1) 1.42±0.27( 87.0±14.0)72.5 ±2.11.31    ±0.15 ( 50.0± 5.5) 15.4 ±3.8 (106.0±36.0)2.7 ±0.43 (112.0±21.4)4.32    ±0.62 (101.8 ±15.8) 2.85 ± 0.36 (100.0 ± 12.8) 2.34 ±0.36 ( 93.0± 14.9) 80.0 ±6.12.80±0.26( 81.8± 7.8) 12.9 ±3.0 ( 72.1± 7.8) 2.79±0.36 (101.0 ±19.6) 4.36± 0.86 ( 89.7± 17.8)

Table 3—Results cf Pulmonary Function Tests in PSS-DS Patients

N Percent Predicted Absolute Value
FVC 77 87 ±22 2.67± 0.81 L
FEVj 77 83 ±23 2.50± 0.74 L/sec
FEV/FVC 77 81 ±12
FEF25-75 65 78 ±34 2.5 ± 1.11 L
FRC 73 102 ±34 2.68 ±0.98 L
TLC 73 90 ±24 4.44 ± 1.33 L
Deo 74 83 ±28 17.4 ±7.1 ml/min/mm Hg

Table 4—Pulmonary Function Data in Patients with PSS-DS According to Physiologic Group

Restrictive Abnormalities, 26 Obstructive Abnormalities, 6 Isolated Reduction of FEF25-75, 2 Isolated Reduction of Deo, 14
FVC/L FEVj, L/sec FEV1/FVC% FEF25-75, L/sec Deo, mm/min/mm Hg FRC, L TLC, L 1.97 ±0.59 (61.8 ±13.5) 1.73± 0.57(73.0±19.9) 85.3 ±8.72.56±0.63(79.0± 8.4) 15.1 ±5.1 (68.9 ±18.8) 2.27±0.74 (85.1 ±25.8) 3.50± 1.05 (70.3± 18.9) 2.87± 0.9 ( 97.0±22.8) 1.63 ± 0.62 ( 65.8 ±24.1) 52.0 ±15.9 1.76± 0.43 ( 58.0± 8.1) 15.6 ± 8.0 ( 84.0±46.7) 3.28 ± 1.31 (121.7±46.8) 5.25± 1.16 (110.0±27.4) 2.94 ±1.17 ( 87.4 ± 9.5) 2.17±0.51 ( 93.0±12.1) 74.0 ±01.9 ±0.71 ( 54.0±4.24) 19.8 ±2.7 ( 91.5±21.3) 2.4 ±0.23 ( 94.0± 8.1) 4.32 ± 0.62 (101.8 ± 15.8) 3.12± 0.56 (92.8±11.3) 2.38 ± 0.51 (84.0 ± 7.2)78.0    ± 6.02.63± 0.43 (74.0± 9.9)14.1    ± 6.1 (61.4 ±15.5) 2.6 ± 0.33 (99.0± 8.1) 5.08 ± 0.73 (97.4 ±15.4)
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