Weekly Chest CasesArchive of Old 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%
  • - Healthy Longevity Medical Center , Japan SHIN-ICHI CHO
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Secomedic Hospital , Japan FUMINORI MIYOSHI
  • - Oita University, Faculty of Medicine , Japan FUMITO OKADA
  • - Seoul Medical Center , Korea (South) HYUK GI HONG
  • - Osaka University , Japan AKINORI HATA
  • - , Japan HIKARI FUKUI
  • - OITA UNIVERSITY , Japan FUMIKA ISHITOBI
  • - Gifu University Hospital , Japan Yo Kaneko
  • - , Japan KENTARO KOTANI
  • - Osaka Metropolitan University Hospital , Japan SHU MATSUSHITA
  • - Hyogo Prefectural Kobe Children , Japan SHUHEI NORIMOTO
  • - Yonsei University,Severance Hospital , Korea (South) SEO BUM CHO
  • - Matsunami General Hospital , Japan TARO TAKEDA
  • - Toyota Kosei Hospital , Japan YUKI HAYASHI
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 6/72,  8.3%
  • - , Japan SUZUNE TSUKAMOTO
  • - The University of Tokyo Hospital , Japan WATARU GONOI
  • - Kyoto University Hospital , Japan YASUHISA KURATA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - Sotiria Hospital, Athens , Greece VASILIOS TZILAS
  • - Kyoto university , Japan AKIHIKO SAKATA
▶ Semi-Correct Answer : 17/72,  23.6%
  • - , Japan HIROAKI ARAKAWA
  • - Affilitated Hospital of Jining Medical college , China JIANG SHENG HUA
  • - Kyoto City Hospital , Japan YUSUKE UTSUNOMIYA
  • - , Korea (South) KANGHWI LEE
  • - Soonchunhyang University Hospital Bucheon , Korea (South) HYEJOO PARK
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - , Japan SHUNJIRO NOGUCHI
  • - Jichi Medical University, School of Medicine , Japan MITSURU MATSUKI
  • - Mie university , Japan SHIKO OKABE
  • - Dokkyo Medical University , Japan HIROAKI ARAKAWA
  • - Others , India PURUSHOTHAMA RAO TUMMALA
  • - Chonnam National University Hospital , Korea (South) KIM SANG GYUN
  • - , Taiwan SHIHHAN PAN
  • - The University of Tokyo Hospital , Japan HIROSHI TAKUMIDA
  • - , Japan YUMI MAEHARA
  • - The Catholic University of Korea Yoeuido St. Mary , Korea (South) CHAWOONG JEON
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
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