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Title : 28 year-old female w/ persistent fever
Date : October, 5, 2011
Contributed by

Contributed by  Eun-Jeong Joo, M.D, Samsung Medical Center, Korea

Modified by  Young Eun Ha, M.D., Samsung Medical Center, Korea

Patient History
Age/Sex 28-year-old female
Chief complaint Fever for 3 weeks
Present illness

A 28-year-old female was admitted with a persistent fever for 3 weeks and flu-like symptoms. On admission, her temperature was 38.6°C, blood pressure was 130/70 mmHg, and respiration rate was 30/min. Her inguinal lymph nodes were palpable, about 0.5 cm in diameter, tender, and fixed. She had decreased breath sounds in both lower lung fields and hepatosplenomegaly.

Initial laboratory findings

WBC : 2,770 /uL

Hb : 13.2g/dL

Platelet : 56,000/uL

Bilirubin : 1.3mg/dL,  AST / ALT  : 610/ 697 U/L

Serum protein : 6.3 g/dL,   albumin : 3.5 g/dL,  LDH : 2,327 ng/mL

Pleural fluid exam : transudate

Blood, urine and pleural fluid culture : no bacteria or fungi were isolated.

Serology : EBV viral capsid antigen (VCA)-IgG (+), EBV VCA-IgM (–), EBV- early antigen (EA) (+), EBNA (+), anti-HBs Ab (+), and anti-HCV Ab (–).

EBV-DNA by RT PCR : 946.1 copies/5 μL.

Radiologic findings

The chest radiograph showed diffuse ground glass opacities in both lower lung fields with bilateral pleural effusions . Computed tomography (CT) of the abdomen and chest revealed hepatosplenomegaly and diffuse ground glass opacities and interlobular septal thickening of the lungs .

Hospital course

Flexible bronchoscopy showed normal bronchial anatomy without inflammation of the airway mucosa. Biopsy of inguinal lymph node showed reactive hyperplasia without evidence of EBV DNA . Six days after admission, the patient was transferred to the intensive care unit because of impending respiratory failure. At this point, a lung biopsy was performed with video-assisted thoracoscopic surgery. Twenty-six days after admission, her symptoms and signs had resolved spontaneously and she was discharged from the hospital.





 
Question - ID Case of the Week ( October, 5, 2011 )
What is your presumptive diagnosis for this patient?
 
Correct Answer

Chronic active EBV infection with interstitial pneumonitis

 
Review

 

The diagnostic criteria of chronic active EBV infection syndrome (CAEBV) includes :

The major clinical features of CAEBV are fever, hepatosplenomegaly, liver dysfunction, pancytopenia, lymphadenopathy, hypersensitivity to mosquito bites, skin rash, and uveitis. In addition, CAEBV often results in life-threatening complications, such as hemophagocytic syndrome, disseminated intravascular coagulopathy, hepatic failure, coronary artery aneurysm, central nervous system involvement, myocarditis, and interstitial pneumonitis. 1) persistent or recurrent infectious mononucleosis-like symptoms; 2) an unusual pattern of EBV antibodies with elevated anti-VCA and anti-EA, or detection of the EBV genome in affected tissues including the peripheral blood; and 3) chronic illness that cannot be explained by any other known disease processes at the time of diagnosis

The literature describes three pulmonary manifestations associated with EBV infection: hilar/mediastinal lymphadenopathy, pleural effusion, and interstitial pneumonitis. Few reports describe pulmonary parenchymal involvement as a complication of acute or chronic active EBV infection in immunocompetent patients . A report on two children with CAEBV stated that the histopathology of their lung tissues showed interstitial infiltration of mature lymphocytes, which spread into the interalveolar septa, and similar to our case, EBV-positive T lymphocytes were detected throughout the alveolar septae and vascular lumens .

 

References :

 

Okano M, Kawa K, Kimura H, et al. Proposed guidelines for diagnosing chronic active Epstein-Barr virus infection. Am J Hematol 2005;80:64-69.

Ankermann T, Claviez A, Wagner HJ, Krams M, Riedel F. Chronic interstitial lung disease with lung fibrosis in a girl: uncommon sequelae of Epstein-Barr virus infection. Pediatr Pulmonol 2003;35:234-238.

Schooley RT, Carey RW, Miller G, et al. Chronic Epstein-Barr virus infection associated with fever and interstitial pneumonitis. Clinical and serologic features and response to antiviral chemotherapy. Ann Intern Med 1986;104:636-643.

 


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