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

[Seppänen Mikko]

Dear Laia,

if this is enteroviral perimyocarditis, it would be prudent to decrease the dose of prednisone, and when pericardial fluid reappears (while controlling symptoms with NSAIDs), do entero-PCR from pericardial fluid sample. If this would be positive, I suggest You contact European Center for Disease Prevention and Control ECDC, and ask about possible available antienteroviral agents for compassionate use, since I seem to recall that there is a slight chance of acquiring pleconaril or newer still investigational agents through them? I do not think we still know if Enteroviridae are causative or innocent bystanders (much debated in ID field), but if this child's perimyocarditis would be entero-PCR +, and could be controlled with antienteroviral agents, it would of course clarify the issue somewhat?

On the other hand, this could be concurrent idiopathic recurrent pericarditis (IRP), where the use of prednisone before/instead of colchicine as the initial agent after NSAIDs actually may even be one of the reasons why it has become so steroid-dependent. The etiology of IRP is obscure for most patients. However, appr. 6 % of patients in one recent Italian study were carriers of a seemingly hypomorphic TNFRSF1A allele, so some of them might actually be TRAPS patients (which of course, if found mutated in this patient, would help to choose a treatment).

There are uncontrolled studies on IRP and IVIg, with favorable results, but there is a very real chance of positive publication bias. I have personally treated 2 hopeless cases with IVIg, both responded initially but eventually ended up having pericardiectomy... I personally would not try to increase the dose above those suggested by Mary Ellen, and would initiate colchicine ASAP instead of Prednisone, like Safa below suggests. And in between would try to check for entero-PCR from pericardial and endocardial biopsies.

hope this helps

mikko

__________________________________________________
Mikko Seppänen, MD, PhD, Docent (Associate professor/Senior Lecturer)
Specialist in Internal Medicine and Infectious Diseases
Senior Consultant, Physician in charge (PIDD)
EM(E)A Expert, PIDDs and Intravenous Immunoglobulin Therapy

Immunodeficiency Unit
Division of Infectious Diseases
Department of Medicine
Helsinki University Central Hospital
Hospital District of Helsinki and Uusimaa
Aurora Hospital, Ward 4-2 and Outpatient Clinic
P.O.Box 348
FI-00029 HUS, Helsinki
FINLAND
phone +358 9 47175923, fax +358 9 47175945
_________________________________________



Lähettäjä: Safa baris [mailto:safabaris at hotmail.com]
Lähetetty: 6. maaliskuuta 2013 19:31
Vastaanottaja: CIS-PIDD
Aihe: 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
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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