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Weekly Chest CasesArchive of Old Cases

Case No : 631 Date 2009-11-30

  • Courtesy of Mi Nyong Choi, Eil Seong Lee / Hallym University College of Medicine
  • Age/Sex 67 / F
  • Chief ComplaintMuscle weakness of both upper and lower extremity (3 month), Chronic cough (over 1 year)
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Diagnosis With Brief Discussion

Diagnosis
Interstitial lung disease associated with Polymyositis
Radiologic Findings
Fig 1. Chest PA shows subsegmental consolidation in right middle lung zone.
Fig 2-1 to 2-4. High-resolution CT images shows irregular linear increased attenuations, small consolidations, interlobular septal thickening and intralobular intertitial increased attenuations seen in subpleural regions of both basal lungs. These findings are suggesting NSIP or UIP. Subsegmental consolidation in right middle lobe is thought to be seuqle of previous aspiration or pneumonia.
Brief Review
Polymyositis is an autoimmune inflammatory myopathy characterized by symmetric weakness of the limb girdle and anterior neck muscles. Dermatomyositis is similar to polymyositis except for the presence of a characteristic skin rash. Polymyositis and dermatomyositis have an incidence of approximately 5-10 cases per million per year and occur twice as often in women as in men, with a bimodal peak age of occurrence during childhood and middle adulthood [1,2,3]. The thorax is commonly affected, generally in one or more of three forms: (a) hypoventilation and respiratory failure as a result of involvement of the respiratory muscles; (b) interstitial pneumonitis, usually with a histologic pattern of usual interstitial pneumonia or nonspecific interstitial pneumonia ; and (c) aspiration pneumonia secondary to pharyngeal muscle weakness (probably the most common pulmonary complication) [1, 4].
Interstitial lung disease associated with polymyositis or dermatomyositis has a wide spectrum of histopathologic features. Three major groups can be identified on the basis of histologic patterns: BOOP, usual or nonspecific interstitial pneumonia, and diffuse alveolar damage. Histologic appearance is useful for determining the prognosis.
Patients with diffuse alveolar damage r usual interstitial pneumonia have a poor prognosis, with only a 33% survival rate at 5 years; however, patients with nonspecific interstitial pneumonia have a good prognosis. Patients with BOOP have an excellent prognosis.
The frequency of radiographic parenchymal abnormalities is low (about 5%). The most common is a symmetric, predominantly basal reticular pattern that may become diffuse over time and progress to honeycombing [1,3]. Bilateral areas of consolidation develop in some patients over a 2- to 3-week period. This abnormality usually corresponds histologically to diffuse alveolar damage or BOOP. Initial high-resolution CT findings of pulmonary involvement in patients with polymyositis or dermatomyositis are prominent interlobular septa, ground-glass attenuation, patchy consolidation, parenchymal bands, irregular eribronchovascular thickening, and subpleural lines. Honeycombing may be seen in up to 16% of patients who have abnormal chest radiographic findings or pulmonary function.[1,5]
References
1. Eun A Kim, Kyung Soo Lee, Takeshi Johkoh, Tae Sung Kim, Gee Young Suh, O Jung Kwon, Jounho Han. Interstitial lung diseases associated with collagen vascular diseases: Radiologic and histoloic findings. Radiographics 2002; 22:151-165.
2. Jennifer P. Mayberry, Steven L. Primack, Nestor L.Muller. Radiographics 2000; 20:1623-1635.
3. Schwarz MI. The lung in polymyositis. Clin Chest Med 1998;19:701?02.
4. Tazelaar HD, Viggiano RW, Pickersgill J, et al. Interstitial lung disease in polymyositis and dermatomyositis: clinical features and prognosis as correlated with histologic findings. Am Rev Respir Dis 1990;141:727?33.
5. Ikezoe J, Johkoh T, Kohno N, Takeuchi N, Ichikado K, Nakamura H. High-resolution CT findings of lung disease in patients with polymyositis and dermatomyositis. J Thorac Imaging 1996; 11:250?59.
Keywords
Lung, Lung, Interstitial lung disease, Polymyositis, Connective tissue diseases, ILD,

No. of Applicants : 86

▶ Correct Answer : 58/86,  67.4%
  • - University of British Columbia , Canada Amr Ajlan
  • - Goa Medical College , India Paresh Desai
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - Shiga University of Medical Science , Japan Norihisa Nitta
  • - Assam Medical College , India Karunakaran M
  • - NTUH , Taiwan Kuei-pin Chung
  • - Institute of Chest Diseases Calicut , India Divya Nair
  • - EWHA WOMANS MOKDONG UNIVERSITY HOSPITAL , Korea (South) YOOKYUNG KIM
  • - Shinchon Severance Hospital , Korea (South) Ho-Joon Lee
  • - hospital Sao Paulo , Brazil israel missrie
  • - CHUV , Switzerland ANNE-SOPHIE KNOEPFLI
  • - partner, aditya imaging centre , India vivek patel
  • - Father Muller Medical College , Mangalore , India nabil mahmood
  • - KMC,Manipal , India satish maddukuri
  • - KING KHALID UNIVERSITY HOSPITAL,RIYADH , Saudi Arabia NABIL GHALEB
  • - CHRU lille , France manuel toledano
  • - POPOVO HOSPITAL , Bulgaria VLADISLAV RUSINOV
  • - Institute of Chest Diseases,Calicut , India sijith raghavan
  • - Uzunkopru Devlet Hastanesi , Turkey Armagan Sarac
  • - Univ. of Miami/Jackson Memorial , United States mantosh rattan
  • - Assam Medical college , India samudra borah
  • - SAISEIKAI KURIHASHI HOSPITAL , Japan YASUO OKUBO
  • - Samsung Medical Center , Korea (South) Eun Kim
  • - CHU NANCY , France, Metropolitan GREGORY LESANNE
  • - CSSS LAVAL , Canada olivier krief
  • - Apollo Gleneagles hosp kolkata , India SALIM PARVEZ
  • - Saga University , Japan Ryoko Egashira
  • - Osaka University , Japan Osamu Honda
  • - CHRU Lille , France Benjamin Damarey
  • - Trakya University School of Medicine , Turkey Umut Ugur
  • - Hospital M de Deus , Brazil Thiago Bento da Silva
  • - McGill University Health Center , Canada Alexandre Semionov
  • - Asan Medical Center , Korea (South) Hye Jeon Hwang
  • - All India Institute of medical sciences , India Justin Moses
  • - Seoul National University Hospital , Korea (South) Sang Min Lee
  • - Asan Medical Center , Korea (South) Seong Yun Kim
  • - seoul national university hospital , Korea (South) Ijin Joo
  • - CHU Nancy , France kollen marie
  • - HOSPITAL SOTIRIA , Greece AIKATERINI DELAVERI
  • - Kang buk samsung hospital, department of radiology , Korea (South) Hyojin Lim
  • - Shinsegye Rad Clinic, Daegu , Korea (South) Gi Beom Kim
  • - IRSA La Rochelle France , France Denis Chabassiere
  • - SMG-SNUH boramae medical center , Korea (South) Kwang Nam Jin
  • - HIA Legouest , France Alban Gervaise
  • - Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
  • - CHRU Lille , France guillaume lefebvre
  • - SacrCoeur , Canada Andrea Ojanguren
  • - All India Institute of Medical Sciences , India Jitesh Ahuja
  • - IRSA , France jean-luc BIGOT
  • - Hospital Sotiria, Athens, Greece , Greece Vasilios Tzilas
  • - clinique de SAVOIE , France, Metropolitan gay-depassier philippe
  • - Louisiana State University - Shreveport , United States Carlos Previgliano
  • - Hel-Dieu Montreal , Canada nicolas gautier
  • - CHU Grenoble , France Fabrice Bing
  • - CHU BRABOIS , France Sab Ariane
  • - Seoul Nationial University Bundang Hospital , Korea (South) Kyoung Jin Oh
  • - Mallinckrodt Institute of Radiology , United States Naganathan Mani
  • - Hospital du Sacre Coeur , Canada Abraham Bohadana
▶ Correct Answer as Differential Diagnosis : 14/86,  16.3%
  • - EKH-Berlin , Germany Michael Weber
  • - PingTung Christian Hospital ,China Medical University ,Taiwan,R.O.C. , Taiwan Jun JunYeh
  • - jaslok hospital & research centre mumbai , India JAINENDRA JAIN
  • - Toyama University Hospital, Laboratory of Pathology , Japan TOMONORI TANAKA
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - Kashan University of Medical Sciences, kashan , Iran Ebrahim Razi
  • - NASA SCANS , India RAKESH BHATIA
  • - Homs National Hospital , Syria Rami Abou Zalaf
  • - Vital Imaging Centre, Mumbai,India , India Ganesh Agrawal
  • - Beaulieu clinic Geneva , Switzerland gilles GENIN
  • - Deptt of Radiodiagnosis & Imaging, PGIMER chandigarh , India Ram Galwa
  • - ROK air force aerospacemedical center , Korea (South) Daekeon Lim
  • - clinique de rochefort , France viviane pages
  • - Trakya University School of Medicine, Edirne , Turkey Ahmet POLAT
▶ Semi-Correct Answer : 1/86,  1.2%
  • - IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
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