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Weekly Chest CasesArchive of Old Cases

Case No : 599 Date 2009-04-20

  • Courtesy of Joo Yeon Cheung, Sung Shine Shim, Kim Yookyung / School of Medicine, Ewha Womans University, Seoul, Korea
  • Age/Sex 35 / F
  • Chief ComplaintBoth hand, knee and elbow joint pain (duration :2-3months)
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4

Diagnosis With Brief Discussion

Diagnosis
Systemic lupus erythematous with NSIP and pulmonary arterial hypertension
Radiologic Findings
Chest radiographs shows minimal reticular opacities in both lower lung zones with focal ground-glass opacity in left lower lung zone. Contrast CT scans show dilatation of pulmonary trunk with larger diameter than ascending aorta measuring 3.3 cm and enlargement of right cardiac chamber that are suggestive of pulmonary arterial hypertension.
Brief Review
Systemic lupus erythematosus (SLE) is an autoimmune disorder characterized by inflammation, immune complex deposition, vasculitis, and vasculopathy. Involvement of the respiratory system in SLE is relatively common. Over one-half of patients with SLE develop pulmonary disease, with pneumonia, pulmonary hemorrhage, and lupus pneumonitis being the most common manifestations. The most common patterns of parenchymal involvement are cellular, fibrotic, or mixed nonspecific interstitial pneumonia (NSIP). The most common HRCT pattern of interstitial lung disease is a combination of ground-glass opacity and reticular opacities. Other common abnormal features include parenchymal bands, centrilobular nodularity, and focal consolidation. These changes predominate in the mid- and lower-lung zones. One fifth of patients have mediastinal lymphadenopathy accompanying parenchymal opacities.
Pulmonary arterial hypertension is an increasingly recognized complication of SLE. Less than one-half of patients have pathologic abnormalities of pulmonary vessels. Vascular abnormalities typically afflict small vessels only and most commonly and most severely in the muscular arteries. Varying degrees of smooth muscle hypertrophy and hyperplasia, intimal fibrosis, inflammatory cellular infiltration of pulmonary arterioles (vasculitis), destruction of the elastic laminae, and in situ thrombosis may be observed. In the early phase of the disease, chest radiographic findings may be normal. Advanced radiographic findings include enlargement of the right ventricle and central pulmonary arteries with distal attenuation (pruning) of the arteries, reflecting obliteration of the more distal vessels.
References
1. Kim, JS, Lee KS, Koh E, Kim SY, Chung MP, Han J. Thoracic Involvement of Systemic Lupus Erythematosus: Clinical, Pathologic, and Radiologic Findings. J Comput Assist Tomogr.2000 Jan-Feb;24(1):9-18
2. Jeffrey J. Swigris, DO, MS; Aryeh Fischer, MD et al. Pulmonary and Thrombotic Manifestations of Systemic Lupus Erythematosus. Chest 2008;133;271-280
3. Bull TM, Fagan KA, Badesch DB. Pulmonary vascular manifestations of mixed connective tissue disease. Rheum Dis Clin North Am. 2005;31(3):451-464
Please refer to
Case 165 Case 225 Case 306 Case 500
Keywords
Lung, Vascular, Connective tissue diseases, SLE,

No. of Applicants : 95

▶ Correct Answer : 13/95,  13.7%
  • - SMC , Korea (South) Jihoon Cha
  • - NTUH , Taiwan Kuei-pin Chung
  • - hospital Sao Paulo , Brazil israel missrie
  • - Bundang CHA hospital , Korea (South) Jung Jin Young
  • - kyungpook national university hospital , Korea (South) jiwon park
  • - Kyungpook National Univ. Hospital , Korea (South) Sang-yub Lee
  • - Seoul National University Hospital , Korea (South) Kwang Nam Jin
  • - Gimpo Woori Hospital , Korea (South) Son Youl Lee
  • - Apollo Gleneagles hosp kolkata , India salim parvez
  • - All India Institute of medical sciences , India Justin Moses
  • - Hospital Sotiria, Athens, Greece , Greece Vasilios Tzilas
  • - Hanmaeum Hospital , Korea (South) Kwon Hyoung Kim
  • - Armed Forces Chun-Cheon Hospital , Korea (South) ChaeHun Lim
▶ Correct Answer as Differential Diagnosis : 6/95,  6.3%
  • - Kasturba Medical College Manipal , India Paresh Desai
  • - IRSA , France jean BIGOT
  • - Saga University , Japan Ryoko Egashira
  • - IRSA La Rochelle France , France Denis Chabassiere
  • - DCA, , India Rajesh Gothi
  • - Mallinckrodt Institute of Radiology , United States Naganathan Mani
▶ Semi-Correct Answer : 51/95,  53.7%
  • - Father Muller Medical College , Mangalore , India nabil mahmood
  • - PingTung Christian Hospital ,China Medical University ,Taiwan,R.O.C. , Taiwan Jun Jun Yeh
  • - Institute of Chest Diseases,Calicut , India sijith raghavan
  • - Gauhati medical College Hospital , India Hari Krishnan
  • - Chungnam national university hospital , Korea (South) Jeoung Eun Lee
  • - Shiga University of Medical Science , Japan Norihisa Nitta
  • - john h stroger hsp , United States dheeraj reddy gopireddy
  • - Mackay Memorial Hospital , Taiwan Sheng-Yeh Shen
  • - POPOVO HOSPITAL , Bulgaria VLADISLAV RUSINOV
  • - Soonchunhyang university hospital BUCHEON , Korea (South) Yang ji yeon
  • - HOSPITAL SOTIRIA , Greece AIKATERINI DELAVERI
  • - Chonnam national university hospital , Korea (South) Jang-hyeon Song
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - Samsung Medical Center , Korea (South) Hye Sun Hwang
  • - Vital Imaging Centre, Mumbai,India , India Ganesh Agrawal
  • - McGill University Health Center , Canada Faiza Al Kindi
  • - Trakya University School of Medicine , Turkey Umut Ugur
  • - Seoul Nationial University Bundang Hospital, Korea (South) , Korea (South) Kyoung Jin Oh
  • - WARABI CITY HOSPITAL , Japan YASUO OKUBO
  • - won ju christian hospital , Korea (South) youngwook jeon
  • - Univ. of Miami/Jackson Memorial , United States mantosh rattan
  • - Kashan University of Medical Sciences, kashan , Iran Ebrahim Razi
  • - kims,narkatpally , India k bhaskar
  • - Hangang Sacred Heart Hospital , Korea (South) Eil Seong Lee
  • - CHUV , Switzerland Andrea Ojanguren
  • - National Taiwan University Hospital , Taiwan Chin-Chung Shu
  • - Assam medical college , India Karunakaran M
  • - McGill University Health Center , Canada Alexandre Semionov
  • - The Armed Forces Daegu Hospital , Korea (South) Hyungwoo Oh
  • - Medical IT Consulting , Japan Kazumasa Nishimura
  • - Armed Force Capital Hospital , Korea (South) Dae Kun Oh
  • - All India Institute of Medical Sciences , India Ashish Gupta
  • - Soonchunhyang University Cheonan Hospital , Korea (South) Park Sang Hyun
  • - wonju christial hospital , Korea (South) Daekeon Lim
  • - Changhua Christian Hospital , Taiwan Chia-Fu Tsai
  • - CHU caen , France nicolas gautier
  • - chest hospital , Egypt assaad assaad
  • - Toyama University Hospital, Laboratory of Pathology , Japan TANAKA TOMONORI
  • - Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
  • - Homs National Hospital , Syria Rami Abou Zalaf
  • - Deptt of Radiodiagnosis & Imaging, PGIMER chandigarh , India Ram Galwa
  • - clinique de SAVOIE , France, Metropolitan gay-depassier philippe
  • - jaslok hospital & research centre mumbai , India JAINENDRA JAIN
  • - Shinsegye Rad Clinic, Daegu , Korea (South) Gi Beom Kim
  • - The Armed Forces HamPyeong Hospital , Korea (South) Bae Geun Oh
  • - Beaulieu clinic Geneva , Switzerland gilles GENIN
  • - CHRU Lille , France Benjamin Damarey
  • - UZ GASTHUISBERG , Belgium Johan COOLEN
  • - Soonchunhyang university hospital, Bucheon , Korea (South) Hyelin Kim
  • - Seoul Nationial University Bundang Hospital , Korea (South) Hee Seok Choi
  • - Kangnam Sacred Heart Hospital , Korea (South) Jaewon Kim
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