Weekly Chest CasesArchive of Old Cases

Case No : 523 Date 2007-11-05

  • Courtesy of Dae Yoon Kim, MD, Eun Jin Chae, MD, Kyung-Hyun Do, MD. / Department of radiology, University of Ulsan, College of Medicine, Asan medical center, Korea.
  • Age/Sex 36 / F
  • Chief ComplaintCough, sputum for 2 yrs
  • Figure 1
  • Figure 2
  • Figure 3

Diagnosis With Brief Discussion

Diagnosis
Churg-Strauss syndrome
Radiologic Findings
Additional past history: Asthma, peripheral eosinophilia (31%), h/o sinusitis, allerginc rhinitis.
This patient had migrating infiltrations in the lung. The initial HRCT showed non-segmental and bilateral, lobular GGO in both cental lungs. Three months later, this patient had new, multiple, mass-like consolidations without cavitation and GGO in the right lung. At admission to our hospital after 20 months from the initial presentation, HRCT showed new, multiple, centrilobular nodules with non-segmental distribution in both lungs and mild bronchial wall thickening. Biopsy revealed eosinophilic infiltration in the interstitium and blood vessels. On PNS CT, the patient also had bilateral, maxillary sinusitis.
Brief Review
Churg-Strauss syndrome (allergic angiitis and granulomatosis) is a clinicopathologic entity of necrotizing vasculitis of small-sized vessel and extravascular granulomas by infiltration of eosinophil, mainly involving the lung, skin, kidney, GI tract, nervous system, paranasal sinus. Asthma and peripheral eosinophilia are observed in this syndrome. In Churg-Strauss syndrome, transient migrating pulmonary opacity is observed and bilateral, non-segmental and geographic lobular consolidation or ground glass opacity is common finding on CT. Multifocal centrilobular nodules with mild bronchial wall thickening is also observed. Differential dianosis includes chronic eosinophilic pneumonia and Wegener’s granulomatosis. Clinically, there are three distinct phases: (1) a prodromal phase that may persist for many years, consisting of asthma, often preceded by allergic rhinitis; (2) a second phase of marked peripheral blood eosinophilia and eosinophilic tissue infiltrates, which may recur over a period of years; and (3) a third, life-threatening vasculitic phase. 5-year survival rate is 60%.
American College of Rheumatology (ACR) 1990 criteria for the classification of Churg-Strauss syndrome (CSS). Classified as CSS if at least four of six criteria are present.
1. Asthma: History of wheezing or diffuse high-pitched expiratory rhonchi.
2. Eosinophilia: Eosinophilia >10% on differential white blood cell count.
3. Mono- or polyneuropathy: Development of mononeuropathy, multiple mononeuropathies, or polyneuropathy (glove/ stocking distribution) attributable to systemic vasculitis.
4. Pulmonary infiltrates, non-fixed: Migratory or transitory pulmonary infiltrates (not including fixed infiltrates). attributable to vasculitis.
5. Paranasal sinus abnormality: History of acute or chronic paranasal sinus pain or tenderness or radiographic opacification of the paranasal sinuses.
6. Extravascular eosinophils: Biopsy including artery, arteriole or venule showing accumulations of eosinophils in extravascular areas.
Please refer to
Case 262,
References
1. Choi YH, Im JG, Han BK, Kim JH, Lee KY, Myoung NH. Thoracic manifestation of Churg-Strauss syndrome: radiologic and clinical findings. Chest. 2000;117:117-124.
2. Seo JB, Im JG, Chung JW, Song JW, Goo JM, Park JH, Yeon KM. Pulmonary vasculitis: the spectrum of radiological findings. Br J Radiol 2000;73:1224-1231.
3. Lanham, JG, Elkon, KB, Pusey, CD, et al. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine 1984;63:65-81.
Keywords
Lung, Eosinophilic lung disease, Churg Strauss syndrome, Vasculitis,

No. of Applicants : 50

▶ Correct Answer as Differential Diagnosis : 6/50,  12.0%
  • - Dept. of radiology, Assam medicalcollege, Assam, India Karunakaran M
  • - IRSA, La Rochelle, France Denis Chabassiere
  • - Korea Han Na Kim
  • - Gimpo Woori Hospital, Korea Son Youl Lee
  • - Yongsan Hospital, College of Medicine, Chung-Ang University, Korea Jae Seung Seo
  • - China Medical University Hospital, Taiwan Jun-Jun Yeh
▶ Semi-Correct Answer : 40/50,  80.0%
  • - Registrar in Radiology, UHL NHS Trust, UK A. Donuru
  • - Shiga University of Medical, Japan Norihisa Nitta
  • - Dept. of radiology, Inje University Ilsan Paik Hospital, Korea Bae Geun Oh
  • - Dept. of Radiodiagnosis and Imaging, PGIMER, Chandigarh, India Ram Prakash Galwa
  • - UCLA Medical center, Los Angeles, USA Fereidoun Abtin
  • - Shinsegye Rad Clinic, Daegu, Korea Gi Beom Kim
  • - Poitiers, France Jerome Hannequin
  • - Vitalife Clinic, Pune, India Rahul Deshmukh
  • - Osaka University, Japan Osamu Honda
  • - CHRU Lille hopital calmette, France Toledano Manuel
  • - KAUMS, Kashan, Iran Ebrahim Razi
  • - Kangbuk Samsung hospital, Korea Ji Na Kim
  • - EKH-Berlin, Germany Michael Weber
  • - Mackay Memorial Hospital, Taipei, Taiwan Chia-Fu Tsai
  • - St. Mary's Hospital, The Catholic University of Korea, Korea Sun Jin Lee
  • - Annecy hospital, France Gilles Genin
  • - Hospital Sotiria, Athens, Greece Vasilios Tzilas
  • - Armed Forces ChunCheon Hospital (AFCC), Korea Chae Hun Lim
  • - Homs National Hospital, Homs - Syria Rami Abou Zalaf
  • - Annemasse, Polyclinique De Savoie, France Gay-Depassier Philippe
  • - St. Mary's Hospital, The Catholic University of Korea, Korea Jeong Hyun Wi
  • - Bollineni superspeciality hospital nellore, India jignesh dubal
  • - Annecy Hospital, France Olivier Segall
  • - Vital imaging centre, Mumbai, India Ganesh Agrawal
  • - Institute Of chest diseases, Calicut, India Arun P
  • - Government Medical College, Kozhikkode, India Jineesh
  • - University of Virginia, USA Rachita Khot
  • - IRSA La Rochelle, France Jean-Luc BIGOT
  • - E-Da hospital, Taiwan Yu-Feng Wei
  • - Ultracare, Coimbatore, India Debabrata Das
  • - Jackson Memorial Hospital, Florida, USA N.B.S.Mani
  • - Soonchunhyang University Cheonan Hospital, Korea Sang Hyun Park
  • - Nassau, Bahamas Trupti Dabholkar
  • - Armidale Radiology, Australia Saurabh Khandelwal
  • - Pittwater Radiology, Australia Julie Arora
  • - Aims, New Delhi, India Ashish Gupta
  • - Max Hospital, New Delhi, India Vickrant Malhotra
  • - Erciyes University Thoracic Surgery Department , Kayseri, Turkiye Omer Onal
  • - CHU Grenoble, France Bing Fabrice
  • - Hangang Sacred Heart Hospital, Korea Eil Seong Lee
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