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

[Conley, Mary Ellen]

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
mconley at uthsc.edu<mailto:mconley at uthsc.edu<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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