Table 3.
Clinical Characteristics of pSS-PPF and pSS-Non-PPF [Cases (%)]
| pSS-PPF (n=29) | pSS-non-PPF (n=37) | χ²/χ²c | P value | |
|---|---|---|---|---|
| ILD-related symptoms | 26 (89.7) | 33 (89.2) | 0 a | 1 |
| Dry cough | 10 (34.5) | 16 (43.2) | 0.523 b | 0.47 |
| Sputum | 14 (48.3) | 6 (16.2) | 7.912 b | 0.005 |
| Dyspnea | 20 (69) | 25 (67.6) | 0.015 b | 0.904 |
| ILD-related signs | 13 (44.8) | 7 (18.9) | 5.167 b | 0.023 |
| Cyanosis | 2 (6.9) | 0 (0) | 0.189 c | |
| Digital clubbing | 1 (3.4) | 2 (5.4) | 0 a | 1 |
| Crackles | 12 (41.4) | 5 (13.5) | 6.601 b | 0.01 |
| System involvement | ||||
| No sicca | 7 (24.1) | 1 (2.7) | 5.145 a | 0.023 |
| Xerostomia | 21 (72.4) | 32 (86.5) | 2.036 b | 0.154 |
| Xerophthalmia | 21 (72.4) | 24 (64.9) | 0.427 b | 0.513 |
| Purpura rash | 1 (3.4) | 1 (2.7) | 0 a | 1 |
| Parotid enlargement | 1 (3.4) | 12 (32.4) | 8.635 b | 0.003 |
| Arthritis | 2 (6.9) | 10 (27) | 4.429 b | 0.035 |
| Myositis | 0 (0) | 1 (2.7) | 1 c | |
| Renal injury | 1 (3.4) | 0 (0) | 0.439 c | |
| PAH | 4 (13.8) | 1 (2.7) | 1.492 a | 0.222 |
| Pulmonary bullae | 5 (17.2) | 6 (16.2) | 0.012 b | 0.912 |
| PBC | 0 (0) | 1 (2.7) | 1 c | |
| PN | 0 (0) | 3 (8.1) | 0.25 c | |
| Leukocytopenia | 2 (6.9) | 4 (10.8) | 0.014 a | 0.906 |
| Thrombocytopenia | 0 (0) | 1 (2.7) | 1 c |
Note: aχ²c value (Continuity-corrected Chi-Square test), bχ² value (Chi-Square test), cFisher’s exact probability method.
Abbreviations: ILD, Interstitial Lung Disease; PAH, Primary Pulmonary Hypertension; PBC, Primary Biliary Cirrhosis; PN, peripheral neuropathy; pSS, Primary Sjogren’s syndrome, PPF, progressive pulmonary fibrosis.