P086 Kawasaki syndrome and Epstein Barr virus: a case report (again!)

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
N Boutrid ◽  
H Rahmoune ◽  
B Bioud ◽  
M Amrane ◽  
A S Chehad

Abstract Background Kawasaki disease (kDa), also known as mucocutaneous lymph node syndrome, is always updating. We present the case of a toddler boy with concomitant positive EBV serology. Material The infant is referred to the pediatric ward for stomatitis with cheilitis, fever> 40 ° C, asthenia, irritability evolving for 4–5 days. In addition, clinical examination revealed multiple, small cervical lymph nodes, < 1 cm. There is no conjunctivitis, no skin rash, nor reactivation of the BCG scar. Biology reveals hyperleukocytosis with very high ESR and CRP. ECG and Echo-heart, requested for suspicion of Kawasaki syndrome, are normal. Results Empirical treatment with Aciclovir—Cefalexin with topical care was started. Viral serologies return HSV (-), CMV (-), but EBV (+). Clinical improvement was noted within 36 h. However, inflammatory markers improve within 2–3 days. At 15 days, the child presents with typical convalescent peeling of the fingers and toes; associated with marked thrombocytosis. The diagnosis of Kawasaki Syndrome is definitively confirmed. Discussion The exact aetiology of Kawasaki syndrome remains unclear. Among the factors frequently encountered, the Epstein Barr Virus is reported for almost 30 years. The syndrome frequency is highest before the age of 5 years; similar to Infectious Mono-Nucleosis (due to EBV…). Our case depicts a concomitant Kawaski syndrome and positive EBV serology; without any detectable coronary artery abnormality. Conclusion Kawasaki syndrome is a real “chameleon” condition with possible cardiovascular complications. The viral aetiology springs up through “an old friend”: the Epstein Barr Virus ...Even without any aneurysm (also associated with EBV), the American Heart Association recommends long-term monitoring for all Kawasaki syndromes.

2009 ◽  
Vol 30 (3) ◽  
pp. 274-281 ◽  
Author(s):  
Jun Muneuchi ◽  
Shouichi Ohga ◽  
Masataka Ishimura ◽  
Kazuyuki Ikeda ◽  
Kenichiro Yamaguchi ◽  
...  

2018 ◽  
Vol 185 (2) ◽  
pp. 377-380 ◽  
Author(s):  
Amie E. Hwang ◽  
Vickie Marshall ◽  
David V. Conti ◽  
Bharat N. Nathwani ◽  
Thomas M. Mack ◽  
...  

2019 ◽  
pp. practneurol-2019-002356 ◽  
Author(s):  
Andrew Lee ◽  
Leslie R Bridges ◽  
Mark Lloyd ◽  
Robert Barker ◽  
Damian R Wren ◽  
...  

The incidence of Epstein–Barr virus (EBV)associated lymphoproliferative disorders has increased with greater use of immunomodulatory therapies. We present a woman who developed subacute cognitive decline and unilateral weakness while taking long-term mycophenolate mofetil for granulomatosis with polyangiitis; her postmortem brain histopathology confirmed an EBV-driven lymphoproliferative disorder. Clinicians must have a high index of suspicion for EBV-driven lymphoma in people taking long-term immunosuppression who develop new neurological problems. We review the role of mycophenolate mofetil in EBV-driven lymphoproliferative disorders, and discuss checking EBV status in all patients starting immunosuppression and in older people already taking immunosuppression.


Blood ◽  
2001 ◽  
Vol 97 (4) ◽  
pp. 1131-1133 ◽  
Author(s):  
Maurizio Arico ◽  
Shinsaku Imashuku ◽  
Rita Clementi ◽  
Shigeyoshi Hibi ◽  
Tomoko Teramura ◽  
...  

Abstract The hemophagocytic lymphohistiocytoses (HLH) comprise a heterogeneous group of disorders characterized by dysregulated activation of T cells and macrophages. Although some patients with HLH harbor perforin gene mutations, the cause of the remaining cases is not known. The phenotype of HLH bears a strong resemblance to X-linked lymphoproliferative disease (XLP), an Epstein-Barr virus (EBV)-associated immunodeficiency resulting from defects in SH2D1A, a small SH2 domain-containing protein expressed in T lymphocytes and natural killer cells. Here it is shown that 4 of 25 male patients with HLH who were examined harbored germline SH2D1A mutations. Among these 4 patients, only 2 had family histories consistent with XLP. On the basis of these findings, it is suggested that all male patients with EBV-associated hemophagocytosis be screened for mutations in SH2D1A. Patients identified as having XLP should undergo genetic counseling, and be followed long-term for development of lymphoma and hypogammaglobulinemia.


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