Table 7.
Patient number | Response to initial therapy | Disease onset | Diagnosis | Pathological diagnosis | Initial therapy | Observation period (months) | Period from initial therapy to relapse (months) | Therapy at relapsea | Prognosis | Disease behavior |
---|---|---|---|---|---|---|---|---|---|---|
1 | improved | acute | IIP | c-NSIP | PDN | 29 | - | - | alive | A |
2 | improved | acute | IIP | unclassifiable IP | PDN + CyA | 27 | - | - | alive | A |
3 | improved | acute | IIP | unclassifiable IP | PDN + TAC | 90 | - | - | alive | A |
4 | improved | chronic | PM | f-NSIP | Low-PDN | 72 | - | - | alive | A |
5 | improved | chronic | IIP | c-NSIP | PDN + TAC | 76 | - | - | alive | A |
6SS | improved | chronic | DM | f-NSIP | PDN + TAC | 19 | - | - | alive | A |
7 | relapsed | acute | IIP | f-NSIP | PDN | 94 | 29 | PDN | alive | B |
8 | relapsed | acute | PM | f-NSIP | PDNc | 82 | 61 | PDN | alive | B |
9 | relapsed | acute | DM | c-NSIP | PDNc | 70 | 8 | PDN | alive | B |
10 | relapsed | acute | IIP | c-NSIP | PDNc + CyA | 115 | 46 | PDN + CyA | alive | B |
11 | relapsed | chronic | PM | f-NSIP | PDN + AZA | 104 | 78 | PDN | alive | B |
12 | deteriorated | acute | PMb | unclassifiable IP | PDN + CyA | 17 | - | - | dead | C |
aPatients have received these therapies until ILD relapsed., SS: Sjögren’s syndrome, IIP: idiopathic interstitial pneumonia, PM: polymyositis, DM: dermatomyositis, bsimultaneous onset of PM and interstitial lung disease, c-NSIP: cellular nonspecific interstitial pneumonia, f-NSIP: fibrosing nonspecific interstitial pneumonia, IP: interstitial pneumonia, PDN: 0.5 mg/kg/day of prednisone, CyA: cyclosporine A, TAC: tacrolimus, Low-PDN: 0.2 mg/kg/day of prednisone, cstarted with methylprednisone pulse therapy (500 mg/body), AZA: azathioprine, A: progressive, irreversible disease with potential for stabilization, B: reversible disease with risk of progression, C: progressive, irreversible disease despite therapy