대한흉부영상의학회 Weekly Chest Cases

Case No : 1163 Date 2020-02-17

Courtesy of Hwayoung Song, Junghoon Kim, Jihang Kim, Kyung Hee Lee, Kyung Won Lee / Seoul National University Bundang Hospital
Age/Sex 39 / M
Chief ComplaintFever, dyspnea / AML, s/p allogeneic stem cell transplantation (16 months ago)

Figure 1
Figure 2
Figure 3

Diagnosis With Brief Discussion

Diagnosis: Bronchiolitis obliterans syndrome due to chronic graft versus host disease
Radiologic Findings: Fig 1-2. CT scan reveals multiple peribronchial tiny nodules, bronchial wall thickening and minimal bronchiectasis in both lungs. Fig 3. worsening of bronchial dilatation and bronchial wall thickening after 3 months.
Brief Review: Bronchiolitis obliterans, or obliterative bronchiolitis, is the most common and most relevant late noninfectious pulmonary complication after HSCT. Its reported incidence varies from 2% to 48%, likely because of a lack of uniform classification. Most cases manifest within 6–12 months after transplant, although presentation as early as 3 months and as late as 10 years after transplant has been reported. Bronchiolitis obliterans is considered a manifestation of chronic GVHD in the lungs and therefore is a complication of allogeneic HSCT. Although bronchiolitis obliterans is not expected to occur after autologous HSCT, two fatal cases have been reported in the literature.
Histologically, bronchiolitis obliterans is characterized by constrictive bronchiolitis with submucosal bronchiolar fibrosis and luminal narrowing affecting the small airways. The pathogenesis, in most cases, may be related to immunomediated mechanisms secondary to GVHD.
Bronchiolitis obliterans is defined clinically by airflow obstruction. The term bronchiolitis obliterans syndrome describes a clinical syndrome characterized by an irreversible decline in forced expiratory volume in 1 second (FEV1) of at least 20% from the baseline. Patients with biopsy-proved bronchiolitis obliterans may not fulfill the clinical criteria for bronchiolitis obliterans syndrome on the basis of pulmonary function test results, whereas patients with a clinical diagnosis of bronchiolitis obliterans syndrome may not demonstrate the classic pathologic features. Nevertheless, these two terms are often used interchangeably in clinical practice.
Patients frequently present with an insidious onset of dry cough and progressive dyspnea. Fever is rare unless there is a superimposed infection due to underlying bronchiectasis. Early in the disease course, HRCT may show normal findings or may demonstrate hyperinflation with subtle areas of decreased attenuation. Bronchiectasis is seen later in the course of the disease. The most characteristic HRCT manifestation of bronchiolitis obliterans is the mosaic perfusion pattern, a finding of areas of decreased attenuation and vascularity interspaced with areas of normal or increased attenuation. The mosaic perfusion pattern is highly suggestive of bronchiolitis obliterans in this population, with 74%–91% sensitivity and 67%–94% specificity. The areas of decreased attenuation are accentuated on expiratory-phase images because of airtrapping. In a recent study that assessed HRCT findings of bronchiolitis obliterans in patients after HSCT, the mosaic perfusion pattern was seen in all patients and showed a predominant peripheral distribution. Airtrapping was seen in 56% of cases. Therefore, it is strongly rec-ommended that HRCT of HSCT recipients should include both inspiratory- and expiratory-phase image acquisition.
Please refer to
References: 1. Pena E, Souza CA, Escuissato DL, et al. Noninfectious pulmonary complications after hematopoietic stem cell transplantation: practical approach to imaging diagnosis. Radiographics 2014; 34: 663–683
Keywords: lung, airway, graft versus host disease, bronchiolitis obliterans,

No. of Applicants : 74

▶ Correct Answer : 41/74, 55.4%

- Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
- Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
- Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
- Niigata University , Japan ATSUSHI UEHARA
- Mayo Clinic , United States AKITOSHI INOUE
- , Korea (South) HYEYOUNG CHOI
- McGill University Health Center , Canada Alexandre Semionov
- IRSA LA ROCHELLE , France JEAN LUC BIGOT
- , Egypt KHALED ABDULGAWAD BORAEE
- , Korea (South) CHOHEE KIM
- Vita Hospital , Brazil DIOGO LAGO PINHEIRO
- Inje University Pusan Paik Hospital , Korea (South) DA SOM KIM
- Oita University, Faculty of Medicine , Japan Fumito Okada
- University of Michigan , United States GABIN YUN
- Other , Korea (South) HONG SANG HYUP
- National Center for Global Health and Medicine , Japan MASATOSHI HOTTA
- Gifu University Hospital , Japan Yo Kaneko
- Seoul National University Hospital , Korea (South) JI HEE KANG
- Soonchunhyang University Hospital Bucheon , Korea (South) SEONGHWAN BYUN
- Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
- Hallym University Dongtan Medical Center , Korea (South) MINSU KIM
- , India SUSHANT JOSHI
- University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
- , Japan NAOMI YUASA
- Ajou University Hospital , Korea (South) HYERIN KIM
- Seoul National University Hospital , Korea (South) SE WOO KIM
- Asan Medical Center , Korea (South) JIHOON KIM
- Kitano hospital , Japan SATOSHI IKEDA
- , India SAURABH MAHESHWARI
- University of Tsukuba Hospital , Japan SODAI HOSHIAI
- , India SHYJUMON GEORGE
- Toranomon Hospital , Japan KAORU SUMIDA
- Ichinomiya Nishi Hospital , Japan Takao Kiguchi
- , Japan KAZUMA TERAUCHI
- Private sector , Greece VASILIOS TZILAS
- Hamamatsu University Hospital , Japan YUKI HAYASHI
- Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
- Chonnam National University Hwasun Hospital , Korea (South) WONGI JEONG
- the first affiliatited hospital of nanjing medical univercity , China HAI XU
- Ajou University Hospital , Korea (South) YONG HO JANG
- Jiangsu province hospital , China WANGJIAN ZHA

▶ Correct Answer as Differential Diagnosis : 8/74, 10.8%

- , Saudi Arabia ALAAELDIN MOHAMED
- CH de la Cote Basque , France PAUL ARDILOUZE
- NIMS, HYDERABAD , India BHASKAR K
- Hamamatsu University Hospital , Japan HAYATO NOZAWA
- Medicheck health care , Korea (South) Chae hun Lim
- TB centre kasaragod. , India rikhy krishnan
- Nishida Hospital , Japan SHOJI OKUDA
- National Center for Global Health and Medicine , Japan HIROSHI TAKUMIDA

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 1163 Date 2020-02-17

Discussion

No. of Applicants : 74