대한흉부영상의학회 Korean Society of Thoracic Radiology

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Weekly Chest CasesArchive of Old Cases

Case No : 306 Date 2003-09-06

  • Courtesy of Sang Hyun Paik, M.D., Jai Soung Park, M.D. / Soonchunhyang University Hospital, Bucheon, Korea
  • Age/Sex 24 / F
  • Chief ComplaintDyspnea and Dysphagia
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6

Diagnosis With Brief Discussion

Diagnosis
Non-specific Interstitial Pneumonia (NSIP) assiciasted with Scleroderma
Radiologic Findings
Initial chest radiograph(a) shows poorly defined ground glass opacities(GGO) involving posterior portion of both basal lung zones, not definite. HRCT images(b,c) demonstrate subpleural and basal dominant area of GGO with probable inner fine reticular opacities in both lungs. Follow up chest radiograph(d) shows no interval change in both basal lung zones. Follow up HRCT images after 5 months(e,f) demonstrate slightly increased extent of GGO and more prominent reticular densities in posterior portion of both basal lung zones and mild dilatation of distal esophagus.
Pathologic finding Fig3a: Fibrous thickening of alveolar septum and hyperplasia of alveolar type II pneumocytes(g) (H & E, x100). Fig3b: Small arteries showing medial thickening and medial fibrosis, resulting to nearly obstruction of the vascular lumen(h) (H & E, x400).
Brief Review
Scleroderma is a systemic collagen vascular disease of unknown cause characterized by vascular and connective tissue abnormalities. It has a female predominance of approximately 3:1.
The most common pulmonary manifestations are interstitial fibrosis, which occurs in approximately 80% of patients, and pulmonary hypertesion, which occurs in 50%. At autopsy, the lung are reportedly involved in 100% of cases of scleroderma and that is a major cause of death. HRCT findings of scleroderma are similar to those of IPF including ground-glass opacities, septal lines, traction bronchiectasis and honeycombing. Enlargement of cardiac silhouette and pulmonary artery due to scleroderma induced pulmonary vascular disease also could be seen. The only differences of HRCT findings between scleroderma with interstitial fibrosis and IPF tended to have a fine reticular pattern and lesser upper lung zone involvement for same extent of disease.
Observation of esophageal dilatation may help in narrowing the differential diagnosis in patients who have diffuse interstitial lung disease.
References
1. Arroliga AC, Podell DN, Matthay RA. Pulmonary manifestation of scleroderma. J Thoracic Imaging, 1992, 7(2):30-45.
2. Wechsler RJ, Steiner RM, Spirn PW, et al. The relationship of thoracic lymphadenopathy to pulmonary interstitial disease in diffuse and limited systemic sclerosis: CT findings. AJR, 1996, 167(1):101-4.
3. Bhalla M, Silver, RM, Shepard, JA, McLoud TC, Chest CT in patients with scleroderma: prevalence of asymptomatic esophageal dilatation and mediastinal lymphadenopathy. AJR, 1993, 161(2):269-72.
4. Webb WR, Muller NL, Naidich DP. High-resolution CT of the lung, third edition. Philadelphia: Lippincott Raven Publisher 2001:214-218
Keywords
Lung, Interstitial lung disease, NSIP, Connective tissue diseases, ILD,

No. of Applicants : 26

▶ Correct Answer : 14/26,  53.8%
  • - Asan Medical Center, Korea Eun Jin Chae
  • - Centre d'imagerie Jacques Callot, Nancy, France Lionel Cannard
  • - CHU Nancy-Brabois, France Denis Regent
  • - Chungju Hospital, Konkuk University, Korea Chang Hee Lee
  • - CIM Saint Dizier, France JC Leclerc
  • - Ewha Women's University Hospital, Korea Sung Shine Shim
  • - Geumgang Asan Hospital, Korea Ju Youn Park
  • - Hangang Sacred Heart Hospital, Korea Eil Seong Lee
  • - Hopitaux HIS, Brussels, Belgium Emmanuel Agneessens
  • - Incheon Sarang Hospital, Korea Jung Hee Kim
  • - Kyunghee University Hospital, Korea Kyung Ran Ko
  • - MD anderson cancer center, TX, USA Jeong-Geun Yi
  • - Social Security Hospital, Ankara, Turkey Meric Tuzun
  • - Upasana Hospital, Kollam, India Joy A Thomas
▶ Semi-Correct Answer : 6/26,  23.1%
  • - Annecy Hospital, France Gilles Genin
  • - Annecy Hospital, France Arnaud Gregoire
  • - Annecy Hospital, France Rafik Mahdi
  • - Homs National Hospital, Homs, Syria Rami Abou Zalaf
  • - Kangbuk Samsung Hospital, Korea Semin Chong
  • - Korea University Anam Hospital, Korea Bo Kyung Je
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