J Korean Rheum Assoc.  2007 Sep;14(3):208-218. 10.4078/jkra.2007.14.3.208.

HRCT Findings and Clinical Features in Non-specific and Usual Interstitial Pneumonia with Connective Tissue Diseases

• Ahn JK
• Koh EM
• Lee YS
• Cha HS
• Chung MP
• Han J
• Oh DK
• Lee KS

• KMID: 2202169
• DOI: http://doi.org/10.4078/jkra.2007.14.3.208

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.

Keyword

Usual interstitial pneumonia; Nonspecific interstitial pneumonia; Connective tissue disease; Chest CT; Biopsy

MeSH Terms

Arthritis, Rheumatoid
Biopsy
Bronchoalveolar Lavage
Connective Tissue Diseases*
Connective Tissue*
Diagnosis, Differential
Hematologic Tests
Humans
Idiopathic Pulmonary Fibrosis*
Leukocytosis
Lung Diseases, Interstitial
Macrophages, Alveolar
Mortality
Prognosis
Retrospective Studies
Scleroderma, Systemic
Sjogren's Syndrome
Tertiary Care Centers
Thorax
Tomography, X-Ray Computed

Figure

• 

  Fig. 1. Comparison of survival between NSIP and UIP patients with connective tissue diseases (CTD).

• 

  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).

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