• Tuberculosis and Respiratory Diseases
• /
• v.61 no.5
• /
• pp.447-455
• /
• 2006

Background: MMPs and TIMPs are important factors for abnormal remodeling the pulmonary parenchyme in idiopathic interstitial pneumonia(IIP) This study evaluated the expression of MMPs and TIMPs in the tissue of IPF, NSIP and normal control subjects. Method: The MMP-2 and -9 activity in the lung tissue was studied by gelatin zymography, and the expression of MMP-1, -2, -9, TIMP-1 and -2 in the lung tissue was measured by immunohistochemistry. Thirty five patients, who were diagnosed with IIP (UIP ; 22, NSIP ; 13), were enrolled in the immunohistochemical study. Thirteen patients with IIP (UIP ; 9, NSIP ; 4) and five patients with lung cancer were enrolled in the zymographic assay. Results: (1) The immunohistochemistry for MMP-1,-2,-9, TIMP-1 and-2 ; MMP-1,-9 and TIMP-2 were stained stronger in the UIP subjects than NSIP and the normal control. TIMP-2 was strongly stained in the UIP tissue. particularly the fibroblasts in the fibroblastic foci. (2) Zymography for MMP-2 and MMP-9 revealed MMP-2 to have prominent expression in the UIP tissue than in the NSIP tissue. Conclusions: These results suggest that the overexpression of the TIMPs and gelatinases in UIP might be important factors in the irreversible fibrosis of the lung parenchyme.

• Tuberculosis and Respiratory Diseases
• /
• v.50 no.6
• /
• pp.732-739
• /
• 2001

Systemic lupus erythematosus frequently has thoracic involvement among connective tissue diseases. One of the pleuropulmonary manifestations is diffuse interstitial lung disease including nonspecific interstitial pneumonia(NSIP). NSIP is a newly classified disease among interstitial lung diseases. Systemic lupus erythematosus has a better prognosis than usual interstitial peumonia(UIP) and responds well to steroids. In this report, a 34 year-old woman who complained of a dry cough, and exertional dyspnea for 2 months is described. The chest X-ray showed fine reticular opacities and a mild honeycomb appearance in both basal lungs. High resolution computed tomography(HRCT) showed bilateral patchy areas of ground-glass attenuation and a mild honeycomb appearance in the subpleural of both the lower and the middle portion of the lung fields. An open lung biopsy showed prominent lymphocytic interstitial inflammation and fibrosis with small areas with a honeycomb appearance. This case was diagnosed as NSIP associated with systemic lupus erythematosus and was managed with oral steroids. Here we report a case of nonspecific interstitial pneumonia associated with systemic lupus erythematosus confirmed by HRCT and an open lung biopsy with a review of the relevant literature.

• Tuberculosis and Respiratory Diseases
• /
• v.55 no.6
• /
• pp.631-635
• /
• 2003

Idiopathic interstitial pneumonia is a group of lung disease classified by clinical, radiological and pathologic findings. Steroid is described in many reports as an effective treatment for the idiopathic interstitial pneumonitis. For the treatment of steroid non-responders, immuno-suppressive or cytotoxic agents are used as second line drug of choice. We experienced a case of nonspecific interstitial pneumonitis who was treated initially with glucocorticoid and antibiotics without effects, but later did respond to cyclosporin treatment. This 63 year-old patient was admitted due to dyspnea, cough, and sputum production. He was started on glucocorticoid and antibiotics after he was diagnosed as having nonspecific interstitial pneumonitis by open lung biopsy. The initial steroid treatment was not effective, however, after cyclosprin treatment, the patient showed clinical and radiologic improvements.

• Clinical and Experimental Pediatrics
• /
• v.45 no.4
• /
• pp.529-534
• /
• 2002

Interstitial pneumonia is a heterogenous group of inflammatory and fibrosing lesions that manifest themselves as infiltrative lung disease. Of these, nonspecific interstitial pneumonia is characterized as a variable degree of interstitial inflammation with or without fibrosis and is distinguished from usual interstitial pneumonia and desquamative interstitial pneumonia, histologically. The influx of inflammatory cells and the responses of immune effector cells injury to the alveolar wall and these initial injuries results in alveolitis and fibrosis. Consequently, the gas exchange throughout the alveolar wall is impaired and the patients suffer from lung diseases of a restrictive pattern. The chief complaints represented are dyspnea and dry cough. We experienced a case of nonspecific interstitial pneumonia in a 10-year old girl. The patient had been healthy and had not been exposed to organic dusts or other toxic materials. The pathology of lung biopsy tissue showed that the alveoli were thickened by a mixture of chronic inflammatory cells and collagen type fibrosis. High resolution computed tomography(HRCT) found the patchy areas of ground-glass opacity with patchy consolidation and irregular reticular opacity, and diffuse distribution without zonal predominance. The forced vital capicity(FVC) was 31%, forced expiratory volume in one second ($FEV_1$) 29% and $FEV_1/FVC$ 90%, so a restrictive pulmonary insufficiency was found.

• Tuberculosis and Respiratory Diseases
• /
• v.51 no.1
• /
• pp.59-64
• /
• 2001

Nonspecific interstitial pneumonia (NSIP) was first described as a new category of idiopathic interstitial pneumonia in 1994. This is a disease with a more insidious onset and has a chronic course. The histological findings are unusual for other idiopathic interstitial pneumonia cases (usual interstitial pneumonia, diffuse interstitial pneumonia, and acute interstitial pneumonia). In contrast to NSIP, acute interstitial pneumonia (AIP) has an acute onset and a fulminant course with the rapid development of respiratory failure. A pathological examination demonstrated characteristic diffuse interstitial fibrosis, hyaline membranes, thrombi, and architectural derangement. Here we report a 48-year-old woman who was diagnosed pathologically NSIP, but with a rapid progressive course similar to AIP.

• Tuberculosis and Respiratory Diseases
• /
• v.64 no.2
• /
• pp.138-143
• /
• 2008

Pneumatosis intestinalis or spontaneous pneumomediastinum are rarely associated with nonspecific interstitial pneumonia (NSIP). However, the development of both conditions in the same patient simultaneously has not been reported previously. A 56-year-old man with NSIP developed spontaneous pneumomediastinum accompanied by subcutaneous emphysema and pneumatosis intestinalis after the treatment with intravenous high dose steroid. The development of spontaneous pneumomediastinum, subcutaneous emphysema and pneumatosis intestinalis in this patient was possibly due to the factors such as NSIP, high dose steroid therapy and subclinical dermatomyositis. Treatment with corticosteroid and cyclosporin gradually improved his exacerbated NSIP and pneumomediastinum, subcutaneous emphysema, pneumatosis intestinalis.

• Tuberculosis and Respiratory Diseases
• /
• v.48 no.6
• /
• pp.932-943
• /
• 2000

Background : Nonspecific interstitial pneumonitis (NSIP) is most likely to be confused with usual interstitial pneumonitis (UIP). Unlike patients witþ UIP, the majority of patients with NSIP have a good prognosis, with most patients improving after treatment with corticosteroids. Therefore it is clinically important to differentiate NSIP from UIP. Up to now, the only means of differentiating these two diseases was by means of surgical lung biopsy. American Thoracic Society (ATS) proposed a clinical diagnostic criteria for UIP to provide assistance to clinicians in its diagnosis without surgical lung biopsy. This study is aimed to investigate whether there were clinical and radiological differences between NSIP and UIP, and the usefulness of ATS clinical diagnostic criteria for UIP in Korea. Methods : We studied 60 patients with UIP and NSIP confirmed by surgical lung biopsy. Clinical manifestations, pulmonary function test, arterial blood gas analysis, bronchoalveolar lavage (BAL), and high resolution computed tomography (HRCT) were evaluated and analyzed by Chi-square test or t-test. The clinical criteria for UIP proposed by ATS were applied to all patients with idiopathic interstitial pneumonia. Results : Forty-two patients with UIP and 18 with NSIP were pathologically identified. Among the 18 patients with NSIP (M : F=1 : 17), the mean age was 55.2$\pm$8.4 (44~73) yr. Among the 42 patients with UIP (M : F=33 : 9), the mean age was 59.5$\pm$7.1 (45~74) yr (p=0.046). Fever was more frequent in NSIP (39%) (p=0.034), but clubbing was frequently observed in UIP (33%) (p=0.023). BAL lymphocytosis was more frequent (23%) (p=0.0001) and CD4/CD8 ratio was lower in NSIP (p=0.045). On HRCT, UIP frequently showed honeycomb appearance (36 of 42 patients) though not in NSIP (p=0.0001). Six of 42 UIP patients (14.3%) met the ATS clinical criteria for IPF, and 3 of 16 NSIP patients (18.8%) met the diagnostic criteria. Conclusion : Being a relatively young female and having short duration of illness, fever, BAL lymphocytosis, low CD4/CD8 ratio with the absence of clubbing and honeycomb appearance in HRCT increase the likelihood of the illness being NSIP. The usefulness of ATS clinical diagnostic criteria for UIP may be low in Korea.

• Tuberculosis and Respiratory Diseases
• /
• v.55 no.2
• /
• pp.175-187
• /
• 2003

Background : Although corticosteroid and cytotoxic agent such as cyclophosphamide have been used for the treatment idiopathic interstitial pneumonia (IIP), efficacy of these toxic drugs are unclear because previous reports included the patients who did not undergo surgical lung biopsy and none evaluated the response according to histopathologic entities of IIP. To answer this, we retrospectively analyzed the treatment response and side effects of corticosteroids and cyclophosphamide therapy in patients with idiopathic UIP and NSIP. Methods : Among 61 patients with UIP and 26 patients with NSIP diagnosed by surgical lung biopsy at Samsung Medical Center from July 1996 to June 2002, those who received corticosteroid or cyclophosphamide therapy for at least 6 months and were followed for at least one year after the initiation of treatment were enrolled (32 UIP, 23 NSIP). Treatment response of 55 patients was assessed by ATS response criteria (clinical symptoms, pulmonary function test and radiological findings). Adverse reactions to either agent (42 cases of cyclophosphamide${\pm}$low-dose prednisolone, 49 cases of prednisolone alone) were also analyzed. Results : Irrespective of treatment regimen, NSIP showed more favorable response than UIP (6 months: 78.3% vs. 9.4%, 12 months: 69.6% vs. 9.4%, p<0.001). Cyclophosphamide showed comparable response to corticosteroid in NSIP while its efficacy was as poor as those of corticosteroid therapy in UIP. Significant adverse reaction to drug more frequently occurred in corticosteroid group (35.7%) than cyclophosphamide group (14.3%) (p=0.017). Conclusion : Cyclophosphamide is effective and more tolerable than corticosteroids in the treatment of idiopathic nonspecific interstitial pneumonia.

• Tuberculosis and Respiratory Diseases
• /
• v.48 no.4
• /
• pp.530-542
• /
• 2000

Background : Nonspecific interstitial pneumonia (NSIP) has been reported recently to have shown much better response to medical treatment and better prognosis compared with idiopathic UIP. However, clinical characteristics of idiopathic NSIP discriminating it from UIP have not been clearly defined. Method : Among 120 patients with biopsy-proven diffuse interstitial lung diseases admitted to the Samsung Medical Center between July 1996 and March 2000, 18 patients with idiopathic NSIP were included in this study. Retrospective chart review and radiographic analysis were performed. Results : 1) At diagnosis, 17 patients were female and the average age was $55.2{\pm}8.4$ years (44~73 years). The average duration from development of respiratory symptom to surgical lung biopsy was $9.9{\pm}17.1$ months. Increase in bronchoalveolar lavage fluid lymphocytes ($23.0{\pm}13.1%$) was noted. On HRCT, ground glass and irregular linear opacity were observed, but honeycombing was absent in all patients. 2) Corticosteroids were initially given to 13 patients, but the medication was stopped in 3 patients due to severe side effects. Further medical therapy was not possible in 1 patient who experienced streroid-induced psychosis. Herpes zoster (n=3), tuberculosis (n=1), avascular necrosis of the hip (n=1), cataract (n=2) and diabetes mellitus (n=1) developed during prolonged corticosteroid administration. Of the 7 patients receiving oral cyclophosphamide therapy, hemorrhagic cystitis hindered one patient from continuing with the medication. 3) After medical treatment, 14 of 17 patients improved, and 3 patients remained stable (mean follow-up ; $24.1{\pm}11.2$ months). FVC increased by $20.2{\pm}11.2%$ of predicted value and the extent of ground glass opacity on HRCT decreased significantly ($15.7{\pm}14.7%$). 4) Of the 14 patients who had stopped medication, 5 showed recurrence of NSIP and 2 became aggravated during steroid tapering. All patients with recurrence showed deterioration within one year after completion of initial treatment. Conclusion : Since idiopathic NSIP has unique clinical profiles and shows good prognosis, diagnosis different from UIP, and aggressive medical treatment are needed.