대한흉부영상의학회 Korean Society of Thoracic Radiology

대한흉부영상의학회 Weekly Case 메뉴

Close

대한흉부영상의학회 Weekly Case 검색
대한흉부영상의학회 Weekly Case 검색
Advanced Search..

Close

Weekly Chest CasesArchive of Old Cases

Case No : 545 Date 2008-04-07

  • Courtesy of Choong-Ki Park, MD, Jeong Nam Heo, MD. / Hanyang University Guri Hospital, Korea.
  • Age/Sex 58 / F
  • Chief ComplaintFever, sputum, joint pain. / Lymph node biopsy; histiocytic necrotizing lymphadenitis.
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6

Diagnosis With Brief Discussion

Diagnosis
Adult Onset Still’s Disease
Radiologic Findings
Figs 1. Chest PA shows prominent hila and widening of right paratracheal stripe.
Fig 2-3. Axial and coronal chest CT scans show multiple enhancing and ehlarged lymph nodes in the neck, mediastinum and both hila.
Figs 4. Follow-up chest CT scans show reducing the size of lymphadenopathy.
Fig 5. Follow-up chest PA shows enlarged cardiac silhouette.
Fig. 6. Hand AP shows multiple joint swelling of the hand and wrist.
Brief Review
Adult-onset Still's disease (AOSD) is a rare, systemic inflammatory disease of unknown aetiology, characterized by daily high spiking fevers, evanescent rash and arthritis. There is no single diagnostic test for AOSD, and diagnosis is based on clinical criteria and usually necessitates the exclusion of infectious, neoplastic and autoimmune diseases.

Adult Still’s disease is defined as the clinical and biological signs:
- fever > higher than 39°C;
- joint pain and swelling;
- skin rash on the trunk and limbs during the fever spikes;
- muscle pain;
- sore throat, corresponding pharyngitis;
- palpable lymph nodes;
- leukocytosis, especially neutrophils;
- biological hepatitis, elevated AST/SGOT;
- elevated ferritin, useful finding for orienting the diagnosis;
- no sign of autoimmunity in the blood;
- no microbial agent in throat swabs.

In our case, clinical findings include fever, sputum, joint pain and swelling, skin rash (6 month ago), palpable lymph nodes on the neck, leukocytosis (18400) and elevated neutrophils (95%), elevated AST/ALT(247/83), elevated ferritin (>2000), LDH (2720), elevated ESR, AIT (-), DNAT (-), ANCAT (-), C3/C4 80/25, normocytic normochromic anemia

The major radiographic abnormalities of AOSD are multiple cervical, mediastinal and hilar lymphadenopathy, joint swelling, pericardial effusion. Differential diagnosis of multiple lymphadenopathy include lymphoma, tuberculosis, infectious disease, neoplastic disease, Kikuchi disease, etc.

The diagnosis of AOSD could be applied most confidently to an illness of adults in which fever, skin rash, remission of multiple lymphadenopathy, joint swelling, pericardial effusion and/or pleural effusion as well as specific laboratory findings of leukocytosis, elevated ferritin, elevated LDH, and elevated AST/ALT.

Treatment includes the use of corticosteroids, often in combination with immunosuppressants (e.g. methotrexate, gold, azathioprine, leflunomide, tacrolimus, ciclosporin and cyclophosphamide) and intravenous immunoglobulin. Biological agents (e.g. anti-TNFα, anti-IL-1 and anti-IL-6) have been successfully used in refractory cases.

Approximately 1/3 of patients have chronic persistent disease with progressive joint damage. Half of all AOSD patients require some medication 10 years after their illness.
Still we have interest why the biopsy-obtained lymph node demonstrated histiocytic necrotizing lymphadenitis as same as Kikuchi disease.
References
1. Fautrel B. Adult onset Still’s disease. Orphanet encyclopedia, Janvier 2004. http://www.orpha.net/data/photo/GB/uk-still.pdf
2. Shin JW, Park NJ, Lee JW, Kwon OH, Lee SK. Increased serum ferritin concentration in adult-onset Still’s disease: a case report. Korean J Clin Pathol 16(4) 1996
3. Kontzias, Apostolos, Efthimiou, Petros. Adult onset Still's disease: pathogenesis, clinical manifestations and therapeutic advances. Drugs 2008; 68(3); 319-337
Keywords
Mediastinum, Multiple organ, Non-infectious inflammation,

No. of Applicants : 75

▶ Correct Answer : 4/75,  5.3%
  • - China Medical University,Taiwan,R.O.C. , Taiwan jun jun yeh
  • - Gimpo Woori Hospital , Korea (South) Son Youl Lee
  • - Armed Forces Chun-Cheon Hospital , Korea (South) ChaeHun Lim
  • - OSWAL CANCER HOSPITAL , India LOVKESH MITTAL
▶ Correct Answer as Differential Diagnosis : 7/75,  9.3%
  • - Kasturba Medical College Manipal , India Paresh Desai
  • - Pe d , France Fabrice Bing
  • - Shiga University of Medical Science , Japan Norihisa Nitta
  • - Seoul National University Hospital , Korea (South) Sang Min Lee
  • - National Taiwan University Hospital , Taiwan Nin Chieh Hsu
  • - IRSA La Rochelle , France Denis Chabassie
  • - Hospital Sotiria, Athens , Greece Vasilios Tzilas
▶ Semi-Correct Answer : 51/75,  68.0%
  • - Shinchon Severance Hospital , Korea (South) Ho-Joon Lee
  • - Kashan University of Medical Sciences, kashan , Iran Ebrahim Razi
  • - Trakya Univercity School of Medicine , Turkey Armagan Sarac
  • - Osaka University , Japan Osamu Honda
  • - Yonsei university Shinchon Severance hospital , Korea (South) Na-young Shin
  • - MD PG Resident, PIMS,LONI, MAHARASHTRA, INDIA , India Sushant Bhadane
  • - Apollo Hospital, Ahmedabad. , India Sandip Shah.
  • - Lilavati Hospital & Research Centre , India Mahavir Swami
  • - hospital Sao Paulo , Brazil israel missrie
  • - Konkuk university hospital , Korea (South) Jeong Geun Yi
  • - Chungnam National University hospital , Korea (South) Kyoung Jin Oh
  • - Shinsegye Rad Clinic, Daegu, Korea , Korea (South) Gi Beom Kim
  • - Almana.G.Hospital , Saudi Arabia Jamal Abazid
  • - Hopital universitaire Lausanne , Switzerland abed kourhani
  • - National Taiwan University Hospital , Taiwan Chin-Chung Shu
  • - Mackay Memorial Hospital , Taiwan Shen ShengYeh
  • - Yonsei University college of medicine Severance hospital , Korea (South) Hua Sun Kim
  • - Regional Imaging Ltd. - Riverina , Australia Rashid Hashmi
  • - Ansan Hando hospital , Korea (South) Hyun Seok Lee
  • - Samsung Medical Center , Korea (South) Dae Kun Oh
  • - Samsung medical center , Korea (South) Hee Jung Kim
  • - seoul national university hospital , Korea (South) Ijin Joo
  • - EKH-Berlin , Germany Michael Weber
  • - Marien Hospital, Hamm , Germany Davis Chiramel
  • - Yongsan Hospital, College of Medicine, Chung-Ang University , Korea (South) Jae Seung Seo
  • - Deptt of Radiodiagnosis & Imaging, PGIMER chandigarh , India Ram Galwa
  • - EWHA WOMANS MOKDONG UNIVERSITY HOSPITAL , Korea (South) YOOKYUNG KIM
  • - Chung-Ang univ. , Korea (South) JIN KYOUNG KIM
  • - lilavati hospital and research center , India dr vivek gupta
  • - Command Hospital (Eastern Command) , India Vivek Sharma
  • - CH de la Ce Basque , France Paul ARDILOUZE
  • - Trakya University School of Medicine , Turkey Derya Demir
  • - Daegu Fatima Hospital , Korea (South) Tae-Hun Kim
  • - SNUH , Korea (South) Mi-Jin Kang
  • - Private Clinic , Bahamas Trupti Dabholkar
  • - Homs National Hospital , Syria Rami Abou zalaf
  • - Saga University, , Japan Ryoko Egashira
  • - IRSA LA ROCHELLE , France jean BIGOT
  • - Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
  • - christie hopsital , United Kingdom rohit kochhar
  • - All India Institute of Medical Sciences , India Ashish Gupta
  • - Vitalife Clinics , Pune , India rahul deshmukh
  • - Mackay Memorial Hospital, Taipei , Taiwan Chia-Fu Tsai
  • - All India Institute of medical sciences , India Justin Moses
  • - E-DA Hospital / I-Shou University , Taiwan Yu-Feng Wei
  • - Jackson Memorial Hospital , United States Naganathan Mani
  • - Polyclinique de SAVOIE , France, Metropolitan GAY-DEPASSIER Philippe
  • - EKH-Berlin , Germany Holger Hartmann
  • - MAX HEALTH CARE , India vickrant malhotra
  • - PITTWATER RADIOLOGY , Australia Julie Arora
  • - PITTWATER RADIOLOGY , Australia Saurabh Khandelwal
  • Top
  • Back

Each Case of This Site Supplied by the Members of KSTR.
Copyright of the Images is in the KSTR and Original Supplier.
Current Editor : Sang Young Oh, M.D., Ph.D Email : sangyoung.oh@gmail.com

This website is optimized for IE 10 and above.