Hui Huang1, Zuo-jun Xu, Yuan-jue Zhu, Hong-rui Liu. 1. Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.
Abstract
OBJECTIVE: To describe the clinical characteristics of 18 patients with different pathological patterns of nonspecific interstitial pneumonia (NSIP). METHOD: The clinical and radiological data of 18 patients with NSIP admitted into Peking Union Medical College Hospital from January 2001 to August 2005 were retrospectively analyzed. The diagnosis of NSIP was confirmed by surgical lung biopsy. RESULTS: Pathologically the cellular mainly pattern (CM-NSIP) was present in 5 patients, all of whom were female, and aged (37 +/- 10) years. The fibrosing mainly pattern (FM-NSIP) was found in 13 patients, 3 males and 10 females, with a mean age of (46 +/- 13) years. The main manifestations of respiratory system were cough, sputum production, dyspnea and inspiratory crackles. Clubbing fingers were found in only one patient with FM-NSIP. Fatigue and weight loss were the main extrapulmonary symptoms. The positive rate of antinuclear antibodies (ANA) of CM-NSIP and FM-NSIP patients were 4/5 and 5/13, respectively. The positive rate of rheumatoid factor of CM-NSIP and FM-NSIP patients were 0/5 and 3/13, respectively. In both groups, ESR and alpha1-globulin were slightly elevated. Pulmonary function tests showed restrictive ventilatory dysfunction and decrease of D(LCO), while arterial blood gas analysis showed mild hypoxemia. Increase of lymphocytes, neutrophils and eosinophils was demonstrated in bronchoalveolar lavage fluid, and the lymphocytes were predominantly CD8+-positive. Ground glass opacities and diffuse intralobular lines were the main manifestations of high resolution CT, while pleural thickening and mediastinal adenopathy were also found in some patients of both groups. Of the patients with CM-NSIP, one lost contact and the rest 4 showed clinical improvements during follow-up. In the FM-NSIP group, one patient died at 8 months after the initiation of therapy, 5 patients remained stable and 7 patients got clinical improvement. CONCLUSIONS: There are no significant differences between CM-NSIP and FM-NSIP in clinical manifestations, laboratory examinations and CT manifestations. But in this series CM-NSIP is found only in females, and with a higher positive rate of ANA and better outcomes. Weight loss and inspiratory crackles are more common in FM-NSIP. However, these two patterns of NSIP can only be differentiated by pathological examination, which is also valuable for prognosis.
OBJECTIVE: To describe the clinical characteristics of 18 patients with different pathological patterns of nonspecific interstitial pneumonia (NSIP). METHOD: The clinical and radiological data of 18 patients with NSIP admitted into Peking Union Medical College Hospital from January 2001 to August 2005 were retrospectively analyzed. The diagnosis of NSIP was confirmed by surgical lung biopsy. RESULTS: Pathologically the cellular mainly pattern (CM-NSIP) was present in 5 patients, all of whom were female, and aged (37 +/- 10) years. The fibrosing mainly pattern (FM-NSIP) was found in 13 patients, 3 males and 10 females, with a mean age of (46 +/- 13) years. The main manifestations of respiratory system were cough, sputum production, dyspnea and inspiratory crackles. Clubbing fingers were found in only one patient with FM-NSIP. Fatigue and weight loss were the main extrapulmonary symptoms. The positive rate of antinuclear antibodies (ANA) of CM-NSIP and FM-NSIPpatients were 4/5 and 5/13, respectively. The positive rate of rheumatoid factor of CM-NSIP and FM-NSIPpatients were 0/5 and 3/13, respectively. In both groups, ESR and alpha1-globulin were slightly elevated. Pulmonary function tests showed restrictive ventilatory dysfunction and decrease of D(LCO), while arterial blood gas analysis showed mild hypoxemia. Increase of lymphocytes, neutrophils and eosinophils was demonstrated in bronchoalveolar lavage fluid, and the lymphocytes were predominantly CD8+-positive. Ground glass opacities and diffuse intralobular lines were the main manifestations of high resolution CT, while pleural thickening and mediastinal adenopathy were also found in some patients of both groups. Of the patients with CM-NSIP, one lost contact and the rest 4 showed clinical improvements during follow-up. In the FM-NSIP group, one patient died at 8 months after the initiation of therapy, 5 patients remained stable and 7 patients got clinical improvement. CONCLUSIONS: There are no significant differences between CM-NSIP and FM-NSIP in clinical manifestations, laboratory examinations and CT manifestations. But in this series CM-NSIP is found only in females, and with a higher positive rate of ANA and better outcomes. Weight loss and inspiratory crackles are more common in FM-NSIP. However, these two patterns of NSIP can only be differentiated by pathological examination, which is also valuable for prognosis.