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Abstract
Objective:
The purpose of this study is to assess the clinical characteristics and the serial changes of high resolution CT (HRCT) findings and to correlate those with the results of clinical parameters in biopsy proven nonspecific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP) with connective tissue diseases (CTD).
Methods:
Retrospective analysis was made of forty patients with CTD diagnosed of NSIP and UIP from a single tertiary hospital between January 1996 and February 2006.
Results:
UIP was common in rheumatoid arthritis, systemic sclerosis and Sjogren's syndrome, while NSIP was frequent in polymyositis/dermatomyositis. No significant difference was found in the clinical characteristics of patients with NSIP and UIP. In initial HRCT findings, extents of honeycombing and reticulation pattern were significantly more in UIP-CTD than in NSIP-CTD. In bronchoalveolar lavage (BAL) results, proportion of alveolar macrophages was significantly higher in NSIP-CTD than in UIP-CTD. In NSIP-CTD, significant increment in the extent of reticulation and honeycombing was noted in the serial HRCT findings despite the aggressive treatment. Significant correlation was found between leukocytosis and honeycombing change in NSIP-CTD. Despite no significant difference of survival between two groups, patients with UIP-CTD seem to have a higher mortality than those with NSIP-CTD.
Conclusion:
It is suggested that chest HRCT and BAL fluid analysis may be helpful in the differential diagnosis of NSIP-and UIP-CTD and leukocytosis in initial blood test might be predictive of honeycombing progression in NSIP-CTD. Further study will be required to compare with the prognosis of NSIP-and UIP-CTD.
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Fig. 2.
Typical HRCT and histologic features of NSIP (A, B) and UIP (C, D) associated with connective tissue diseases (A) Patchy areas of consolidation and wide spread ground-glass attenuation are seen in both subpleural lungs. (B) Histology shows interstitial fibrosis with inflammatory cells infiltration and temporal uniformity without fibrous foci (H&E x200). (C) HRCT scan shows honeycombing change, irregular line and ground glass opacity in the subpleural area of both lungs. (D) Histology shows irregular interstitial fibrosis and inflammation in subpleural area with patch lymphoid follicles containing germinal center, alternating with relatively normal lung parenchyma (arrow)(H&E x 100).
Table 1.
Baseline characteristics of patients with UIP and NSIP with connective tissue diseases
Table 2.
Laboratory findings of patients with biopsy-proven UIP and NSIP in connective tissue disease
Unless otherwise indicated, values are frequency (percentage) or mean±standard deviation (SD). Pts: patients, UIP: usual interstitial pneumonia, NSIP: nonspecific interstitial pneumonia, CTD: connective tissue diseases, WBC: white blood cell, ESR: erythrocyte sedimentation rate, CRP: C-reactive protein, LDH: lactate dehydrogenase, ANA: antinuclear antibody, BAL: bronchoalveolar lavage, FEV1: forced expiratory volume 1, FVC: forced vital capacity
Table 3.
Pathologic diagnosis of NSIP and UIP with connective tissue disease
Table 4.
Extent of individual HRCT pattern on initial HRCT and Inter-scan change in HRCT pattern in patients with CTD-ILD
Table 5.
Relationship between initial PFT and inter-scan change of individual HRCT pattern in patients with CTD-ILD
∗p<0.05, coefficient variable (r) calculated by Spearman's rho correlation. PFT: pulmonary function test, HRCT: high resolution computed tomography, CTD: connective tissue disease, ILD: interstitial lung disease, NSIP: nonspecific interstitial pneumonia, G: interval change of ground glass opacity, C: interval change of consolidation, H: interval change of honeycombing, R: interval change of reticulation, FEV1: forced expiratory volume in 1 sec, FVC: forced vital capacity, WBC: white blood cell, ESR: erythrocyte sedimentation rate, CRP: C-reactive protein
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