대한흉부영상의학회 Weekly Chest Cases

Case No : 1324 Date 2023-03-06

Courtesy of Jong Hyuk Lee, Woo Hyeon Lim, Hyungin Park, Eui Jin Hwang, Soon Ho Yoon / Seoul National University Hospital
Age/Sex 64 / F
Chief ComplaintDyspnea, underlying rheumatoid disease, incidentally detected lung nodules

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Diagnosis With Brief Discussion

Diagnosis: Lung parenchymal amyloidosis
Radiologic Findings: Fig 1. Chest AP shows multiple lung nodules disseminated in the bilateral lung parenchyma.
Fig 2-4. CT scans show multiple variable-shaped lung nodules in the bilateral lung parenchyma. The nodules occur most in the lower lobes and periphery. In the mediastinal window setting, several lung nodules have internal calcifications or cavitations.
Brief Review: Amyloidosis, a disorder of protein folding, is rare. It affects the respiratory tract in about 50% of cases. The most important proteins associated with respiratory tract disease are amyloid L and amyloid A.
There are three major forms of amyloidosis in the lower respiratory tract: tracheobronchial, nodular parenchymal, and diffuse parenchymal (alveolar septal, interstitial). The parenchymal nodules of localized pulmonary amyloid can be solitary or multiple and are usually fairly well-defined. Amyloid is often identifiable in the alveolar interstitium at the periphery of the nodule.
Pulmonary function tests may show evidence of restriction and impaired gas transfer in patients who have diffuse alveolar septal amyloidosis and air-trapping and fixed upper airway obstruction in those who have proximal tracheobronchial involvement.
In CT images, nodular primary parenchymal amyloidosis manifests as solitary or, less commonly, multiple nodules usually ranging from 0.5 to 5 cm in diameter. On occasion, nodular parenchymal amyloidosis may result in a large mass. Calcification is seldom evident on radiographs but is seen in 20% to 50% of nodules on CT. The nodules tend to be more common in the periphery of the lower lobes but may be seen anywhere in the parenchyma. Rarely, nodules cavitate. Cysts may occasionally be present adjacent to the nodules. Cysts and nodular amyloid deposits have been described most commonly in patients with Sjögren syndrome, with or without associated lymphoid interstitial pneumonia. The main differential diagnosis of the parenchymal nodular form of amyloidosis is granulomatous infection or primary or metastatic tumors.
The diagnosis of amyloidosis usually requires histologic confirmation. The diagnosis is based on demonstration of amyloid by Congo red staining, which produces characteristic apple-green birefringence under crossed polarized light. Nodular parenchymal amyloidosis is usually asymptomatic and remains stationary or progresses slowly. It usually has a good prognosis and seldom requires treatment.
References: 1. Christopher M. Walker, Jonathan H. Chung. Imaging of the Chest, 2nd Edition. Chapter 38. Metabolic and Storage Lung Diseases.
Keywords

No. of Applicants : 88

▶ Correct Answer : 30/88, 34.1%

- , Japan HIROAKI ARAKAWA
- Jichi Medical University Hospital , Japan JUN KANZAWA
- , Japan KYOKO NAGAI
- Shiga University of Medical Science , Japan AKITOSHI INOUE
- NIMS, HYDERABAD , India BHASKAR K
- Seoul National University Hospital , Korea (South) HYUNGIN PARK
- Vita Hospital , Brazil DIOGO LAGO PINHEIRO
- Other , Korea (South) SEONGSU KANG
- Scans world , India PHILSON JOSEPH MUKKADA
- Oita University, Faculty of Medicine , Japan FUMITO OKADA
- Samsung Medical Center , Korea (South) JUHYUN KIM
- The University of Tokyo Hospital , Japan WATARU GONOI
- Osaka University , Japan AKINORI HATA
- , Japan HIKARI FUKUI
- Shimada General Medical Center , Japan HAYATO NOZAWA
- Soonchunhyang University Hospital Bucheon , Korea (South) HYEJOO PARK
- The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
- Gifu University Hospital , Japan Yo Kaneko
- Hukuda memorial hospital , Japan SHINTARO KANO
- Kyoto University Hospital , Japan YASUHISA KURATA
- Ajou University Hospital , Korea (South) YOU NA KIM
- Osaka Metropolitan University Hospital , Japan SHU MATSUSHITA
- Chungbuk National University Hospital , Korea (South) MIHYEON PARK
- University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
- Dokkyo Medical University , Japan HIROAKI ARAKAWA
- Toranomon Hospital , Japan KAORU SUMIDA
- , Japan YUMI MAEHARA
- Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
- , Korea (South) HM PARK
- the first affiliatited hospital of nanjing medical univercity , China HAI XU

▶ Correct Answer as Differential Diagnosis : 23/88, 26.1%

- Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
- ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
- , Italy PAOLO BALDASSARI
- Tiger Gate Hospital , Japan SHIN-ICHI CHO
- , Japan CHIAKI SATO
- , United Arab Emirates GAYATHRI GANAPATHY
- Seoul Medical Center , Korea (South) HYUK GI HONG
- Ajou University Hospital , Korea (South) HAEIN LEE
- Ajou University Hospital , Korea (South) HYUNSEUNG LEE
- Yamanashi Prefectural Central Hospital , Japan HIROAKI WATANABE
- Narayana Multispeciality Hospital Jaipur Rajasthan , India JAINENDRA JAIN
- Ajou University Hospital , Korea (South) JAYOUNG MOON
- Other , Korea (South) CHAEHUN LIM
- Jichi Medical University, School of Medicine , Japan MITSURU MATSUKI
- Osaka metropolitan university Hospital , Japan TATSUSHI OURA
- Korea University Anam Hospital , Korea (South) KYU-CHONG LEE
- Kyoto University , Japan SHO KOYASU
- Hyogo Prefectural Kobe Children , Japan SHUHEI NORIMOTO
- Nerimahikarigaoka hospital , Japan TAKANA HAYASHI
- Matsunami General Hospital , Japan TARO TAKEDA
- , Japan YUKI HAYASHI
- Kyoto university , Japan AKIHIKO SAKATA
- Jiangsu province hospital , China WANGJIAN ZHA

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 1324 Date 2023-03-06

Discussion

No. of Applicants : 88