[CIS PIDD] [cis-pidd] patient with Bruton and refractory pericarditis

[beatriz]

Dear Laia

We are also following an XLA patient who presented pericarditis a year ago when he was 16 y old. He was treated with antibiotics and steroids and improved but unfortunately no microorganism was isolated (virus, bacteria or mycobacteria). He had lost considerable weight before pericarditis but during this time we couldn´t diagnose any specific infection or neoplasia. Before pericarditis and after discharge, his IVIG dosage was approx 700mg/kg/month but his trough IgG levels were 300-400mg/dL that increased to 600- 700mg/dL after metronidazole (again no microorganism was isolated in faces (cryptosporidium, giardia, isospora ). He has been released by cardiologists and is doing well.


Beatriz Tavares Costa Carvalho
Profa Adjunto da Disciplina de Alergia, 
Imunologia Clínica e Reumatologia
Depto Pediatria - UNIFESP-EPM
Fone: 50840285
Fax: 55791590
e-mail: beacarvalho at terra.com.br ou 
beatrizt.dped at epm.br
www.imunopediatria.org.br



From: Safa baris 
Sent: Wednesday, March 06, 2013 2:30 PM
To: CIS-PIDD 
Subject: RE: [cis-pidd] patient with Bruton and refractory pericarditis

I suggest to start colchicum therapy for your patient. if you cant detect any infection causes, colchicum can aid to resolve pericarditis caused by increases of inflamation (like FMF). 

best regards.

safa baris,MD.
pediatric immunology, 


--------------------------------------------------------------------------------
From: mconley at uthsc.edu
To: cis-pidd at lists.clinimmsoc.org
Subject: RE:[cis-pidd] patient with Bruton and refractory pericarditis
Date: Wed, 6 Mar 2013 16:57:13 +0000


I have not seen pericarditis in XLA but I wonder if an enterovirus might be causing the problem.  It may be that Coxsackie caused the knee arthritis at presentation and it is now causing the pericarditis.  I have been impressed that the enteroviruses can be asymptomatic for many years and then cause encephalitis. When the patients are on good doses of gammaglobulin, it is very, very hard to document an enteroviral infection, even by PCR.  



There is a paper in the February 2013 issue of Journal of Cardiology that suggest that steroids help Coxsackie induced pericarditis in a mouse model but only early after infection.  If your trough levels of IgG are greater than 1000 mg/dl, I doubt that more will help.

Mary Ellen



Mary Ellen Conley, MD

West Research Tower

LeBonheur Children's Hospital

50 N. Dunlap St.

Memphis TN  38103-2800

Tel     901-287-4657

FAX  901-287-4551

mailto:mconley at uthsc.edu%3cmailto:mconley at uthsc.edu>



From: Laia Alsina Manrique de Lara [mailto:lalsina at hsjdbcn.org] 
Sent: Wednesday, March 06, 2013 5:59 AM
To: CIS-PIDD
Subject: [cis-pidd] patient with Bruton and refractory pericarditis



Dear all, 



I contact you regarding an 8-yo boy diagnosed with Bruton disease at 20 months of age in the context of a reactive arthitis of the knee. Since then, on IG substitution (though levels >10 gr) with no main infections nor other complications. He is well grown.



He was diagnosed 1.5 months ago with a pericarditis of undefined origin after extensive studies (see biochemical and microbiological studies below) that was finally treated with steroids.  



The diagnosis of pericarditis was performed based on clinical symptoms (central chest pain) along with EKG and ecography with 20mm of pericardial liquid. He had significant inflammatory signs (fever 38,5ºC, leucocitosis and elevated CRP and ESR). He was empirically treated with antibiotics (cefotaxime + vancomycin after pericardiocentesis) 



After reducing the dose of steroids due to initial good response to treatment, the pericarditis has rapidly reproduced.



Has anybody experience with this  complication ocurring in Bruton disease? 

--is the pericarditis viral and I am not controlling a virus I haven't found? Should I give more IGIV for this purpose?

--can Bruton patients have reactive/inflammatory pericarditis?




 
 
Pericardial liquid (22/01/2013): serohematic. Proteines 75 g/L, Glucose 52 mg/dl (blood 92 mg/dl). Leucocites 3.150/mm3 (Mononuclear 50%, PMN 50%). ADA 101.9 UI/L. 
PCR enterovirus, rinovirus, coronavirus, influenza, parainfluenza, adenovirus, bocavirus: negative.
Ziehl Neelsen in pericardial liquid: negative. ELISPOT BK: negative. PPD + chest X-ray patient and parents is negative.
Culture: negative. 







Thank you in advance, 



Laia







Dra. Laia Alsina
Sección de Alergia e Inmunología Clínica
Hospital Sant Joan de Déu
Passeig Sant Joan de Déu nº2
08950 Esplugues de Llobregat, Barcelona
+34932804000 ext 3330




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