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Weekly Chest CasesArchive of Old Cases

Case No : 1481 Date 2026-03-09

  • Courtesy of Jae Wook Lee / Soonchunhyang University Bucheon Hospital
  • Age/Sex 74 / F
  • Chief ComplaintDyspnea, Rashes on both hands
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6
  • Figure 7

Diagnosis With Brief Discussion

Diagnosis
MDA-5 associated ILD
Radiologic Findings
Figs. 1–3. Initial chest radiographs demonstrated subpleural reticulation and patchy consolidations with lower lobe predominance, and chest CT revealed peribronchial and subpleural consolidations.
Figs. 4–5. Follow-up chest CT obtained 2 weeks later showed increased extent of consolidations and ground-glass opacities.
Figs. 6–7. Follow-up chest CT at 4 weeks demonstrated further progression of consolidations and ground-glass opacities, with newly developed pneumomediastinum.
Brief Review
The patient presented with dyspnea and had no significant past medical history. Chest CT demonstrated an organizing pneumonia pattern, for which steroid therapy was initiated; however, subsequent imaging showed progression of pulmonary abnormalities in conjunction with clinical deterioration. Antinuclear antibody testing revealed a titer of 1:160, and a palmar rash was noted. Additional serologic evaluation demonstrated positivity for anti–MDA-5 antibody.
Anti–melanoma differentiation–associated gene 5 (MDA-5) antibody–positive disease represents a distinct subset of dermatomyositis, most commonly associated with clinically amyopathic dermatomyositis (CADM) and a high risk of rapidly progressive interstitial lung disease (RP-ILD). Patients typically exhibit minimal or absent muscle weakness with normal or mildly elevated creatine kinase levels, while characteristic cutaneous manifestations—such as painful digital ulcerations, palmar papules, and Gottron’s papules—are prominent. This phenotype is reported more frequently in East Asian populations and is associated with a particularly aggressive pulmonary course.
Pulmonary involvement is the major determinant of prognosis in anti–MDA-5–positive patients. High-resolution computed tomography commonly demonstrates bilateral ground-glass opacities and consolidations with peribronchovascular or subpleural distribution, often showing a mixed pattern of nonspecific interstitial pneumonia and organizing pneumonia. Disease progression may occur over weeks to months, frequently leading to respiratory failure despite intensive immunosuppressive therapy. Elevated serum ferritin levels have been consistently associated with disease activity and poor outcomes, underscoring the importance of early recognition and prompt initiation of combination immunosuppressive treatment.
References
1. Sato S, Hirakata M, Kuwana M, et al. Autoantibodies to a 140-kDa polypeptide, CADM-140, in patients with clinically amyopathic dermatomyositis. Arthritis Rheum. 2005;52(5):1571–1576.
2. Koga T, Fujikawa K, Horai Y, et al. The diagnostic utility of anti–MDA-5 antibody testing for predicting rapidly progressive interstitial lung disease in dermatomyositis. Rheumatology (Oxford). 2012;51(7):1278–1285.
3. Tsuji H, Nakashima R, Hosono Y, et al. Multidisciplinary treatment for anti–MDA-5 antibody–positive dermatomyositis with rapidly progressive interstitial lung disease. Arthritis Rheumatol. 2020;72(3):488–498.
Keywords

No. of Applicants : 97

▶ Correct Answer : 48/97,  49.5%
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - , Japan Z Y
  • - The University of Tokyo Hospital , Japan ISSEI FUKUDA
  • - Affilitated Hospital of Jining Medical college , China JIANG SHENG HUA
  • - Oita university , Japan AYUMI KAMEI
  • - Kyoto University Hospital , Japan YUSUKE UTSUNOMIYA
  • - Chonnam National University Hospital , Korea (South) JUNYEOL YANG
  • - , Korea (South) DONG HEE LEE
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Kyeongpook National University Hospital , Korea (South) JUNGMIN LEE
  • - Other , Korea (South) SEONGSU KANG
  • - Kyung Hee University Medical Hospital , Korea (South) INKEON YEO
  • - Secomedic Hospital , Japan FUMINORI MIYOSHI
  • - Oita University, Faculty of Medicine , Japan FUMITO OKADA
  • - University of Yamanashi , Japan HIROYUKI MORISAKA
  • - Chiba University Hospital , Japan HIROKI MIYAZAKI
  • - The Catholic University of Korea Seoul St. Mary , Korea (South) JIHO PARK
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Nara Medical University , Japan AYA YAMADA
  • - , Japan YOSHIKI ISHII
  • - University of Yamanashi , Japan HIROAKI WATANABE
  • - Japanese Red Cross Otsu Hospital , Japan JUN YOSHIDA
  • - The Catholic University of Korea Yoeuido St. Mary , Korea (South) OHMIN KWON
  • - Chonbuk National University Hospital , Korea (South) SEUNG HO LEE
  • - Osaka Metropolitan University Hospital , Japan SHU MATSUSHITA
  • - Etlik City Hospital, Ankara , Turkey MERIC TUZUN
  • - Kyoto City Hospital , Japan AKIHIKO MINAMI
  • - Jichi Medical University, School of Medicine , Japan MITSURU MATSUKI
  • - , Japan MASAMICHI IWAI
  • - , Korea (South) MYUNG-JEAN LEE
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - Osaka metropolitan university Hospital , Japan TATSUSHI OURA
  • - Kyung Hee University Medical Hospital , Korea (South) TAE JUN PARK
  • - Kyoto University , Japan SHO KOYASU
  • - Yonsei University,Severance Hospital , Korea (South) SEO BUM CHO
  • - Fukuoka university , Japan KEISUKE SATO
  • - Kantou Rousai Hospital , Japan KAORU SUMIDA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - Matsunami General Hospital , Japan TARO TAKEDA
  • - Ehime University , Japan TOMOHISA OKADA
  • - Ehime University , Japan KOTARO MATSUMOTO
  • - , Japan YUMI MAEHARA
  • - Chonbuk National University Hospital , Korea (South) HYEONG RYUN CHO
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - University of Yamanashi , Japan KOJIRO ONOHARA
  • - Chonnam National University Hospital , Korea (South) JAEBONG YANG
  • - , Japan YUKIHISA TAKAYAMA
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 2/97,  2.1%
  • - Kyoto University , Japan AKIHIKO SAKATA
  • - Osaka University , Japan AKINORI HATA
▶ Semi-Correct Answer : 29/97,  29.9%
  • - Chonnam National University Hwasun Hospital , Korea (South) KWANGKUN CHO
  • - Kyeongpook National University Hospital , Korea (South) JOHN BAEK
  • - medical scanning , Japan HIROAKI ARAKAWA
  • - , Japan HIROKI SAKURADA
  • - Shiga University of Medical Science , Japan AKITOSHI INOUE
  • - Central Hospital Atasehir Istanbul , Turkey ARMAGAN SARAC
  • - , Korea (South) KYUNG HYUN WOO
  • - Chonnam National University Hospital , Korea (South) HYUNJIN KIM
  • - , Italy PAOLO BALDASSARI
  • - , China FANG YING GEN
  • - Seoul Medical Center , Korea (South) HATAEK JEONG
  • - Inje University Pusan Paik Hospital , Korea (South) JIYEON HAN
  • - , Switzerland JULIA LANGHART
  • - Kyung Hee University Medical Hospital , Korea (South) DONGIL KIM
  • - Other , Korea (South) CHAEHUN LIM
  • - Other , Korea (South) MARI A
  • - , Japan SHUNJIRO NOGUCHI
  • - , Korea (South) HEONSEOK LEE
  • - Shiga General Hospital , Japan YUSAKU MORIBATA
  • - GHICL , France MANUEL TOLEDANO
  • - Dokkyo Medical University , Japan HIROAKI ARAKAWA
  • - Seoul Medical Center , Korea (South) HANSEUL CHO
  • - , Japan TOMOHIRO KAWAJI
  • - Kyung-hee University Medical Center , Korea (South) HYESUN RYU
  • - , Japan MATSUNO MAI
  • - Paras hospital, Panchkula , India SHALEEN RANA
  • - Kyung Hee University Medical Hospital , Korea (South) JEONG TAEK YOON
  • - The Catholic University of Korea Yoeuido St. Mary , Korea (South) CHAWOONG JEON
  • - , Japan YUJI OHIZUMI
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