HK contributed to envisioning the initial concept and design of this project. the middle or lower lung zone. Nine patients presented pathologically nonspecific interstitial pneumonia with organizing pneumonia, alveolar epithelial injury and prominent interstitial cellular infiltrations whereas the other three patients were diagnosed with unclassifiable interstitial pneumonia. Nateglinide (Starlix) Although all patients but one improved with the initial immunosuppressive therapy, five patients relapsed. When ILD relapsed, four of the five patients were treated with corticosteroid monotherapy. Four of the six patients without relapse have been continuously treated with combination therapy of corticosteroid and immunosuppressant. Conclusions Patients with EJ-ILD often had acute onset of ILD with lower lung-predominant shadows and pathologically nonspecific interstitial pneumonia or unclassifiable interstitial pneumonia with acute inflammatory findings. Although the disease responded well to the initial treatment, relapse was frequent. Because of the diversity of the clinical courses, combination therapy of corticosteroid and immunosuppressant should be on the list Nateglinide (Starlix) of options to prevent relapse of EJ-ILD. of (b). Staining: (a) Hematoxylin Nateglinide (Starlix) and eosin stain, (b, c) Elastica van Gieson stein, Magnification: (a, b) 4, (c) 10 Organizing pneumonia and alveolar epithelial injury with moderate or severe grade were observed not only in patients with acute onset but also in those with chronic onset (Table?6). Table 6 Number of patients of each pathological finding with moderate or severe grade thead th rowspan=”1″ colspan=”1″ Findings /th th rowspan=”1″ colspan=”1″ acute onset ( em n /em ?=?8) /th th rowspan=”1″ colspan=”1″ chronic onset ( em n /em ?=?4) /th /thead Organizing pneumonia83Alveolar epithelial injury64Interstitial cellular infiltration62Pleural fibrosis53Collapse34Alveolar wall fibrosis23Pleuritis01Lymphoid follicles with germinal centers00Fibroblastic focus02Microscopic honeycombing00 Open in a separate window Clinical course and treatment response Details on the clinical courses and therapy of the 12 patients are shown in Table?7. Seven patients were treated with combination therapy of corticosteroid and immunosuppressant as the initial treatment while five were given corticosteroid monotherapy. The initial dose of corticosteroid was 0.5?mg/kg/day of prednisone in 11 patients. The median time of prednisone dose tapering to 10?mg/day was 91?days (range, 60C132 days). Table 7 The clinical courses and therapy of the 12 patients thead th rowspan=”1″ colspan=”1″ Patient number /th th rowspan=”1″ colspan=”1″ Response to initial therapy /th th rowspan=”1″ colspan=”1″ Disease onset /th th rowspan=”1″ colspan=”1″ Diagnosis /th th rowspan=”1″ colspan=”1″ Pathological diagnosis /th th rowspan=”1″ colspan=”1″ Initial therapy /th th rowspan=”1″ colspan=”1″ Observation period (months) /th th rowspan=”1″ colspan=”1″ Period from initial therapy to relapse (months) /th th rowspan=”1″ colspan=”1″ Therapy at relapsea /th th rowspan=”1″ colspan=”1″ Prognosis /th th rowspan=”1″ colspan=”1″ Disease behavior /th /thead 1improvedacuteIIPc-NSIPPDN29–aliveA2improvedacuteIIPunclassifiable IPPDN?+?CyA27–aliveA3improvedacuteIIPunclassifiable IPPDN?+?TAC90–aliveA4improvedchronicPMf-NSIPLow-PDN72–aliveA5improvedchronicIIPc-NSIPPDN?+?TAC76–aliveA6SS improvedchronicDMf-NSIPPDN?+?TAC19–aliveA7relapsedacuteIIPf-NSIPPDN9429PDNaliveB8relapsedacutePMf-NSIPPDNc 8261PDNaliveB9relapsedacuteDMc-NSIPPDNc 708PDNaliveB10relapsedacuteIIPc-NSIPPDNc?+?CyA11546PDN?+?CyAaliveB11relapsedchronicPMf-NSIPPDN?+?AZA10478PDNaliveB12deterioratedacutePMb unclassifiable IPPDN?+?CyA17–deadC Open in a separate window a?Patients have received these therapies until Nateglinide (Starlix) ILD relapsed., em SS /em : Sj?grens syndrome, em IIP /em : idiopathic interstitial pneumonia, em PM /em : polymyositis, em DM /em : dermatomyositis, bsimultaneous onset of PM and interstitial lung disease, em c-NSIP /em : cellular nonspecific interstitial pneumonia, em f-NSIP /em : fibrosing nonspecific interstitial pneumonia, em IP /em : interstitial pneumonia, em PDN /em : 0.5?mg/kg/day of prednisone, em CyA /em : cyclosporine A, em TAC /em : tacrolimus, em Low-PDN /em : 0.2?mg/kg/day of prednisone, c?started with methylprednisone pulse therapy (500 mg/body), em AZA /em : azathioprine, A: progressive, irreversible disease with potential for stabilization, B: reversible disease with risk of progression, C: progressive, irreversible disease despite therapy The initial response was evaluated as improved in 11 of 12 patients. Radiological findings improved in 11 of 12 patients. Representative images of chest radiograph are shown LIMK2 in Fig.?5, indicating the apparent lung volume recovery. Pulmonary function, evaluated in 10 patients, improved or stabilized in nine patients. Forced vital capacity % predicted (%FVC) and/or diffusing capacity of the lung for carbon monoxide % predicted (%DLCO) increased immediately after the initial treatment and remained almost stable between the initial evaluation period (Fig.?6). On disease behavior, the type of reversible disease with risk of progression were six patients, progressive and irreversible disease with potential for stabilization were five patients, and progressive and irreversible disease despite therapy were one patient. Patient #12 was the only patient who did not respond to the treatment and died during the follow-up period. Open in a separate window Fig. 5 Changes of chest radiographs of patient #11. a The initial chest radiograph before treatment showed infiltration and volume loss of lower lung field. b After four months from the initiation of treatment, consolidation and volume loss improved. c However they arose again at the time of relapse. d At the end of follow-up, treatment improved infiltration and volume Open in a separate window Fig. 6 Changes of pulmonary functions of 10 patients during initial evaluation period. Pulmonary function improved or was stable in nine patients. a Line graph of forced vital capacity % predicted. b Line graph of diffusing capacity of the lung for carbon monoxide % predicted The median follow-up time was 74?months (range, 17C115 months). ILD relapsed in five of the 11 improved.
HK contributed to envisioning the initial concept and design of this project