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Weekly Chest CasesArchive of Old Cases

Case No : 1112 Date 2019-02-18

  • Courtesy of Min Jae Cha, MD / Chung-Ang University Hospital
  • Age/Sex 62 / F
  • Chief ComplaintCouph, dyspnea, systemic lupus erythematosus
  • Figure 1
  • Figure 2
  • Figure 3

Diagnosis With Brief Discussion

Diagnosis
Diffuse alveolar hemorrhage in patient with SLE
Radiologic Findings
Figs 1. Chest PA shows diffuse bilateral airspace opacification, especially Left lung.
Fig 2-3. Lung window setting of the high resolution chest CT scan shows diffuse ground-glass opacity and consolidation with mild interlobular septal thickening in both lungs, especially left lung.

Clinical Presentation
The patient visited our emergency department because of cough, dyspnea and hemoptysis, which have been aggravated for last 5 days. She was diagnosed with systemic lupus erythematosus (SLE) and lupus nephritis 5 years ago, but she stopped taking medication 2 years ago. Initial laboratory tests revealed severe anemia (hemoglobin: 5.7 g/dL), thrombocytopenia (79  109/L), and elevation of serum CRP (135.8 mg/L) and creatinine (4.8 mg/dL) with proteinuria (280 mg/dL) and hematuria (3+).



Brief Review
Diffuse alveolar hemorrhage (DAH) is a potentially catastrophic pulmonary complication of systemic lupus erythematosus (SLE) associated with a high mortality rate. DAH has been reported to complicate 2–5% of all cases of SLE. DAH is typically defined by the presence of three major components: (1) signs (bronchoscopy with bloody return) or symptoms (dyspnea, cough, hemoptysis) of pulmonary hemorrhage, (2) drop of hemoglobin (typically 1.5–2 g/dL) and (3) new onset of diffuse infiltrates on chest imaging. Lupus nephritis has frequently been linked to DAH, with evidence of active kidney disease in patients with lupus DAH.
The pathophysiology of DAH is not well understood, suggested theories postulate immune-mediated alveolar capillary endothelial damage and inflammation that would result in local or diffuse bleeding from those capillaries. Imaging studies are commonly consistent with unexplained new consolidation and ground-glass opacity, usually diffuse but can be seen in the perihilar regions, while apices are spared.
As with most organ-threatening manifestations of SLE, cyclophosphamide has been the mainstay of therapy for DAH, albeit in association with poor outcomes, which probably reflect its preferential use in the most critically ill patients. Other reported therapies include plasmapheresis, extracorporeal membrane oxygenation (ECMO), rituximab, mycophenolate mofetil, recombinant factor VII, and stem cell transplantation. However, the evidence to support any particular therapy is not strong.
References
1. Kazzaz NM, Coit P, Lewis EE, McCune WJ, Sawalha AH, Knight JS. Systemic lupus erythematosus complicated by diffuse alveolar haemorrhage: risk factors, therapy and survival. Lupus science & medicine. 2015;2(1):e000117.
2. Abdalla AO, Al-Khafaji J, Akella PM, Taha M. A fatal case of diffuse alveolar hemorrhage as the initial presentation of systemic lupus erythematosus: A case report and literature review. Respiratory Medicine Case Reports. 2018;24:55-7.
Keywords
Lung, Connective tissue diseases,

No. of Applicants : 79

▶ Correct Answer : 48/79,  60.8%
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - ZIGONG TCM HOSPITAL OF CHINA , China Cao Cunyou
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - Niigata University , Japan ATSUSHI UEHARA
  • - The University of Tokyo Hospital , Japan MASAFUMI KAIUME
  • - Higashi-Ohmi General Medical Center , Japan AKITOSHI INOUE
  • - King Abdulaziz University Hospital , Saudi Arabia Amr M. Ajlan
  • - MD , India DEEPANDER SINGH RATHORE
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Other , Korea (South) HEE SEOK CHOI
  • - DELTA CARE HOSPITAL, THANJAVUR, INDIA , India SIVARAJA SUBRAMANIAM
  • - Seoul National University Hospital , Korea (South) Ju Gang Nam
  • - Asan Medical Center , Korea (South) EUN JIN CHAE
  • - 罹…stanbul , Turkey AYHAN YILMAZ
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - The Jikei university , Japan TAKU GOMI
  • - university of montreal , Canada Andrei Bogdan Gorgos I
  • - Onomichi municipal hospital , Japan Hirofumi Mifune
  • - , Japan HISATAKA ITO
  • - Fujieda Municipal General Hospital , Japan HAYATO NOZAWA
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Narayana Multispeciality Hospital Jaipur Rajasthan , India JAINENDRA JAIN
  • - Yeungnam University Medical Center , Korea (South) JONGSOO PARK
  • - Asan Medical Center , Korea (South) JOOAE CHOE
  • - Gifu University Hospital , Japan Yo Kaneko
  • - Samsung Medical Center , Korea (South) MIN YEONG KIM
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - National Taiwan University Hospital Hsin-Chu Branch , Taiwan LI-TA KENG
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - , Japan NAOMI YUASA
  • - Ajou University Hospital , Korea (South) HYERIN KIM
  • - Seoul National University Hospital , Korea (South) SE WOO KIM
  • - Azienda Ospedaliera di Cremona , Italy Pietro Sergio
  • - Asan Medical Center , Korea (South) JIHOON KIM
  • - Columbia asia refferal hospital,Bengaluru , India PRAVIN KUMAR M
  • - Holy Family Hopsital , India RAJESH GOTHI
  • - Chonnam National University Hospital , Korea (South) SEUNGYOON CHAE
  • - TB centre kasaragod. , India rikhy krishnan
  • - Fortis hospital , Mohali , India SHALEEN RANA
  • - , China SHENG HUA JIANG
  • - Kizawa Memorial Hospital , Japan Shoji Okuda
  • - Kyoto Prefectural University of Medicine , Japan TADASHI TANAKA
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - Private sector , Greece VASILIOS TZILAS
  • - , Japan YUMI MAEHARA
  • - Hamamatsu University Hospital , Japan YUKI HAYASHI
▶ Correct Answer as Differential Diagnosis : 18/79,  22.8%
  • - The University of Kyoto Hospital , Japan TOMOAKI OTANI
  • - Ajou University School of Medicine , Korea (South) JUNG YONG JUN
  • - McGill University Health Center , Canada Alexandre Semionov
  • - NIMS, HYDERABAD , India BHASKAR K
  • - , Korea (South) CHOHEE KIM
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
  • - Gyeongsang National University Hospital , Korea (South) KWON HWANWOONG
  • - Chonbuk National University Hospital , Korea (South) KUM JU CHAE
  • - , Korea (South) JANG SEONG WON
  • - Ajou University Hospital , Korea (South) SUBIN HEO
  • - Teikyo University Hospital , Japan KAORU SUMIDA
  • - National Center for Global Health and Medicine , Japan HIROSHI TAKUMIDA
  • - Pavilho Pereira Filho , Brazil THIAGO KRIEGER BENTO DA SILVA
  • - Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
  • - Japanese Red Cross Wakayama Medical Center , Japan AKIHIKO SAKATA
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - Ajou University Hospital , Korea (South) YONG HO JANG
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Semi-Correct Answer : 1/79,  1.3%
  • - Lille , France BENOIST CAPON
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