대한흉부영상의학회 Weekly Chest Cases

Case No : 1357 Date 2023-10-24

Courtesy of Suji Lee, Na Young Kim, Hye-Jeong Lee / Severance Hospital
Age/Sex 54 / F
Chief ComplaintDyspnea R.O.S/P.E: xerostomia, skin rash Serology test: ANA(-), Anti CCP Ab(-), P-ANCA(-), C-ANCA(-), MPO(-), Anti-Jo1(-), Anti-SS-A/Ro(-), Anti-SS-A/La (-) Past history: None

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2021.10.20

Diagnosis With Brief Discussion

Diagnosis: MDA-5 associated ILD
Radiologic Findings: Fig 1. Serial chest radiographs show progressive subpleural reticulation and ground glass opacities in both lungs.
Fig 2. Initial chest CT scan shows mild subpleural GGO and consolidation in both lungs with mild basal lung predominancy.
Fig 3. Follow-up chest CT scan after 2 months shows rapidly progressed subpleural GGO and consolidations in both lungs.
Brief Review: The patient had no past history and visited the emergency room with acute dyspnea. Her ROS and physical exam showed xerostomia and skin rash for 2 months. However, her serum antibody tests showed negative for common autoimmune antibodies. Her initial chest radiograph and chest CT scan showed subpleural GGO and consolidation with mild basal lung predominance, and these findings are suspicious for interstitial lung disease such as organizing pneumonia or nonspecific interstitial pneumonia (NSIP). After 2months later, her follow-up chest CT scan revealed rapidly progressed interstitial lung disease with organizing pneumonia pattern. With the clinician��s suspicion, a myositis panel study was done, and the MDA-5 antibody (Ab) was positive.
The anti-MDA5 Ab was initially called the anti-CADM-140 Ab since it was first found in patients with clinically asymptomatic dermatomyositis (CADM). It was renamed after MDA5 was found to be the corresponding antigen. Patients who are positive for Anti-MDA5 Ab usually present with a rapidly progressive acute to subacute course of the disease, and often present to the emergency department with severe dyspnea. The prognosis of Anti-MDA5 Ab-positive patients is determined by the ability to save the patient in the early stages of the disease. When the patient presents with rapidly progressive ILD, a diagnosis of dermatomyositis may not be possible to make clinically. Notably, the indication of Anti-MDA5 Ab positivity based on imaging findings may be lifesaving.
The image findings of Anti-MDA5 Ab ILD are 1) Acute or subacute onset, often rapidly progressive; 2) Severe organizing pneumonia pattern leading to DAD, but without chronic lesions; 3) Diffuse, or lower lobe predominant consolidation/GGO, or both; 4) Ground glass attenuation was more frequent and craniocaudal distribution was more extensive in Anti-MDA5 Ab positive group. More aggressive treatment and careful follow-up are desirable when CT shows extensive lesions, multiple GGOs with random distribution, and a typical diffuse alveolar damage (DAD) pattern.
References: 1. Mariampillai K, Granger B, Amelin D, Guiguet M, Hachulla E, Maurier F, et al. Development of a new classification system for idiopathic inflammatory myopathies based on clinical manifestations and myositis-specific autoantibodies. JAMA neurology. 2018;75(12):1528-37.
2. Egashira R. High-Resolution CT Findings of Myositis-Related Interstitial Lung Disease. Medicina. 2021;57(7):692.
3. Laporte A, Mariampillai K, Allenbach Y, Pasi N, Donciu V, Toledano D, et al. Idiopathic inflammatory myopathies: CT characteristics of interstitial lung disease and their association (s) with myositis-specific autoantibodies. European Radiology. 2022;32(5):3480-9.
Keywords

No. of Applicants : 72

▶ Correct Answer : 16/72, 22.2%

- Yonsei University,Severance Hospital , Korea (South) SEO BUM CHO
- Jiangsu province hospital , China WANGJIAN ZHA
- Oita University, Faculty of Medicine , Japan FUMITO OKADA
- Osaka Metropolitan University Hospital , Japan SHU MATSUSHITA
- Vita Hospital , Brazil DIOGO LAGO PINHEIRO
- Healthy Longevity Medical Center , Japan SHIN-ICHI CHO
- Seoul Medical Center , Korea (South) HYUK GI HONG
- , Japan KENTARO KOTANI
- Osaka University , Japan AKINORI HATA
- , Japan HIKARI FUKUI
- OITA UNIVERSITY , Japan FUMIKA ISHITOBI
- Toyota Kosei Hospital , Japan YUKI HAYASHI
- Gifu University Hospital , Japan Yo Kaneko
- Secomedic Hospital , Japan FUMINORI MIYOSHI
- Matsunami General Hospital , Japan TARO TAKEDA
- Hyogo Prefectural Kobe Children , Japan SHUHEI NORIMOTO

▶ Correct Answer as Differential Diagnosis : 6/72, 8.3%

- Kyoto university , Japan AKIHIKO SAKATA
- Ichinomiya Nishi Hospital , Japan Takao Kiguchi
- The University of Tokyo Hospital , Japan WATARU GONOI
- , Japan SUZUNE TSUKAMOTO
- Sotiria Hospital, Athens , Greece VASILIOS TZILAS
- Kyoto University Hospital , Japan YASUHISA KURATA

▶ Semi-Correct Answer : 17/72, 23.6%

- , Japan HIROAKI ARAKAWA
- Kyoto City Hospital , Japan YUSUKE UTSUNOMIYA
- The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
- , Japan SHUNJIRO NOGUCHI
- Soonchunhyang University Hospital Bucheon , Korea (South) HYEJOO PARK
- Affilitated Hospital of Jining Medical college , China JIANG SHENG HUA
- , Japan YUMI MAEHARA
- The Catholic University of Korea Yoeuido St. Mary , Korea (South) CHAWOONG JEON
- The University of Tokyo Hospital , Japan HIROSHI TAKUMIDA
- Dokkyo Medical University , Japan HIROAKI ARAKAWA
- , Korea (South) KANGHWI LEE
- Chonnam National University Hospital , Korea (South) KIM SANG GYUN
- Others , India PURUSHOTHAMA RAO TUMMALA
- , Taiwan SHIHHAN PAN
- the first affiliatited hospital of nanjing medical univercity , China HAI XU
- Mie university , Japan SHIKO OKABE
- Jichi Medical University, School of Medicine , Japan MITSURU MATSUKI

대한흉부영상의학회 Korean Society of Thoracic Radiology

Cases by Disease Category

Case No : 1357 Date 2023-10-24

Discussion

No. of Applicants : 72