대한흉부영상의학회 Korean Society of Thoracic Radiology

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Weekly Chest CasesArchive of Old Cases

Case No : 313 Date 2003-10-25

  • Courtesy of Mi-Young Kim, M.D. / Inje University Ilsan Paik Hospital, Seoul, Korea
  • Age/Sex 64 / F
  • Chief ComplaintNo respiratory symptom. Bilateral Submandibular gland swelling for one year, dry mouth & upper eyelid swelling
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Diagnosis With Brief Discussion

Diagnosis
Lymphocytic interstitial pneumonia a/w Sjogren's syndrome
Radiologic Findings
Chest radiograph shows vague interstitial reticular opacities to nodulear opacities in the both lungs.
HRCT show diffuse poorly defined centrilobular nodules, subpleural nodules and thickening of the bronchovascular bundles.
Follow up chest radiograph and HRCT after corticosteroid therapy show almost recovery.

Pathologic finding:
Open lung biopsy shows diffuse lymphoid infiltrate with several lymphoid follicles extending through the pulmonary interstitium (H&E, x40). The lymphoid infiltrate composed of small lymphocytes surrounding germinal centers percolates through the alveolar interstitium (H&E, x100). A mixture of small and medium-sized lymphocytes and plasma cells is noted within the widened alveolar interstitium (H&E, x200). Submandible, right, excisional biopsy shows loss of acinus, ductal proliferation, lymphplasma cell infiltration with germinal centers, stromal fibrosis, consistent with SJOGREN'S DISEASE.
Brief Review
LIP is a benign lymphoproliferative disorder characterized by a diffuse and exquisitely interstitial proliferation of small lymphoctytes and plasma cells. LIP occurs most commonly in patients who have Sjogren syndrome, autoimmune thyroid disease, acquired immunodeficiency syndrome (AIDS), or Castleman disease.
HRCT findings in 22 patients who had biopsy-proved LIP is characterized by the presence of ground-glass attenuation, poorly defined centrilobular nodules, and thickening of the interstitium along the lymphatic vessels (1).
The natural history of LIP is highly variable. Some patients recover or have slowly improving or stable respiratory disease following treatment with corticosteroids. Over one-third of reported patients have progressive disease. Lymphoma has supervened in some patients, although is likely that this group of patients had malignant lymphoma from the outset.
References
1. Johkoh T, Muller N, Pickford H, et al. Lymphocytic interstitial pnumonia: thin section CT findings in 22 patients. Radiology 1999;212:567-572.
2. Deheinzelin D, Capelozzi VI, Kairalla RA, et al. Intersititial lung disease in primary Sjoren sundrome: clinical-pathological evaluation and response to treatment. Am. J Respir Crit Care Med 1996;154:794-799.
3. Koss MN, Hochholzer L, Langloss JM, Wehunt WD, Lazrus AA. Lymphoid interstitial pneumonia: clinicopathological and immunopathological finding in 18 cases. Pathology 1987;19:178-185.
Keywords
Lung, Interstitial lung disease, LIP, ILD,

No. of Applicants : 29

▶ Correct Answer : 16/29,  55.2%
  • - Annecy Hospital, France Gilles Genin
  • - Asan Medical Center, Korea Eun Jin Chae
  • - Pusan National University Hospital, Pusan, Korea Soo Jin Kim
  • - CHU Nancy-Brabois, France Denis Regent
  • - Chungju Hospital, Konkuk University, Korea Chang Hee Lee
  • - Chungju Saeroun hospital, Korea Cheolkyu Jung
  • - Ewha Women's University Hospital, Korea Sung Shine Shim
  • - Geneva Hospital, Switzerland Dumont Philippe
  • - Gwangmyoung Sung-Ae Hospital, Korea Jiyong Rhee
  • - Hangang Sacred Heart Hospital, Korea Eil Seong Lee
  • - Mallinckrodt Institute of Radiology, USA Jin Mo Goo
  • - Ohio State University Med Cntr, Ohio, USA Sumit Seth
  • - Ondokuz Mayis University, Samsun, Turkey Cetin Celenk
  • - Shin Kong Wu Ho-Su Memorial Hsopital, Taipei, Taiwan Chen-Chun Lin
  • - Tokyo Kouseinenkin Hospital , Japan Noriatsu Ichiba
  • - Upasana Hospital, Kollam, India Joy A Thomas
▶ Semi-Correct Answer : 12/29,  41.4%
  • - Annecy Hospital, France Arnaud Gregoire
  • - Busan Armed Force Hospital Young-Min Cho
  • - Centre d'imagerie Jacques Callot, Nancy, France Lionel Cannard
  • - Cheongju St. Mary Hospital, Korea Jeong-Geun Yi
  • - Chung Li Ten-Chen Hospital,Taiwan Kui-Lin Zheng
  • - CIM Saint Dizier, France JC Leclerc
  • - EPM-UNIFESP and Fleury Med. Ctr, Sao Paulo, Brazil Gustavo Meirelles
  • - Geoje Baek Hospital , Gyungnam, Korea James Lim
  • - Homs National Hospital, Homs, Syria Rami Abou Zalaf
  • - Incheon Sarang Hospital, Korea Jung Hee Kim
  • - Kyunghee University Hospital, Korea Kyung Ran Ko
  • - Social Security Hospital, Ankara, Turkey Meric Tuzun
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