Table1.
Demographic, clinical, and serological features of 27 patients with DM-ILD.
| Features | Values |
|---|---|
| Age, years | 57 (50–67) |
| Female, n (%) | 24 (88.9) |
| Disease type, n (%) | |
| CADM | 14 (51.9) |
| A/SIP, n (%) | 13 (48.1) |
| Symptoms, % | |
| Mechanic’s hands | 14 (51.9) |
| Raynaud’s phenomenon | 4 (14.8) |
| Gottron's sign | 15 (55.6) |
| Palmar papules | 6 (22.2) |
| Cutaneous ulcerations | 3 (11.1) |
| Arthritis | 6 (22.2) |
| Disease duration, weeks | 13.1 (2–100)a |
| Positive anti-MDA5Ab, n (%) | 10 (37.0) |
| Positive anti-ARS Ab, n (%) | 17 (63.0) |
| Laboratory findings | |
| Alb, mg/dl | 3.7 (3.1–4) |
| CK, IU/l | 105 (56–203) |
| ALD, IU/l | 5.9 (3.7–8.4)b |
| LDH, IU/l | 233 (190–414) |
| CRP, mg/dl | 0.12 (0.04–1.48) |
| KL-6, U/ml | 772 (480–1,153) |
| SP-D, ng/mL | 112 (36.5–222.5)c |
| Ferritin, ng/ml | 159.4 (90.8–634.7)d |
| AaDO2, mmHg | 16.9 (5.2–40.1)e |
| PFTs | |
| %FVC, % | 87.9 (72.5–93.8)f |
| %Dlco, % | 56.5 (46.6–66.6)g |
| Chest HRCT scores | |
| Right middle lobe GGO score | 1.3 (1–2) |
| Total GGO score | 7.3 (5–11.3) |
| Total fibrosis score | 3.3 (2–5) |
| Treatments | |
| PDN (n = 22), mg/day | 45 (30–56) |
| CSA (n = 8), mg/day | 200 (131–250) |
| TAC (n = 11), mg/day | 4 (3–4) |
| MPDN, n (%) | 5 (18.5) |
| Total IVCY (n = 12), mg | 4,750 (3,000–6,500) |
| IVIg, n (%) | 8 (29.6) |
| Prognosis, n (%) | |
| Alive | 24 (88.9) |
| Dead | 3 (11.1) |
DM, dermatomyositis; ILD, interstitial lung disease; CADM, clinical amyopathic dermatomyositis; A/SIP, acute/subacute interstitial pneumonia; MDA5, anti-melanoma differentiation-associated gene 5; Ab, antibody; ARS, aminoacyl-tRNA synthetase; Alb, albumin; CK, creatine kinase; ALD, aldorase; LDH, lactate dehydrogenase; KL-6, Krebs von den Lungen-6; SP-D, surfactant protein-D; AaDO2, alveolar-arterial oxygen difference; FVC, forced vital capacity; DLco, diffusion capacity of the lung for carbon monoxide; GGO, ground-glass opacity; PDN, prednisolone; CSA, cyclosporine; TAC, tacrolimus; MPDN, methylprednisolone pulse therapy; IVCY, intravenous pulse cyclophosphamide; IVIg, intravenous immunoglobulin; Dead, dead due to ILD. The laboratory markers are presented as the median (interquartile range).
aNumber of subject, n = 23.
bNumber of subject, n = 24.
cNumber of subject, n = 25.
dNumber of subject, n = 24.
eNumber of subject, n = 21.
fNumber of subject, n = 9.
gNumber of subject, n = 8.