[CIS PIDD] [cis-pidd] VS: 10yo with Campylobacter pericarditis and chronic oligosymptomatic interstitial lung disease

[CIS-PIDD]

Hi Markus, 

...exciting case! The closest known PIDDs I can think of would be IRAK4/MyD88, at least experimentally... Given the largely normal thus far tested labs, would further want me to exclude these? I do not know if you have a lab in Austria to test these, but in Brexit-Europe Cambridge / Rainer Doffinger and of course Inserm do.

See on the theoretical scope of infections in these Eur. J. Immunol. 2012. 42: 3126-3135 by von Bernuth, Picard and Casanova, an excellent review. 

Hope this helps, his story smells like PIDD to me anyway.... for what it is worth... Maybe even a novel in the same TLR signaling pathway, if not either???

ATB,

Mikko

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Lähettäjä: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org] 
Lähetetty: 17. elokuuta 2016 17:07
Vastaanottaja: CIS-PIDD <cis-pidd at lyris.dundee.net>
Aihe: [cis-pidd] 10yo with Campylobacter pericarditis and chronic oligosymptomatic interstitial lung disease

Dear colleagues,
this 10yo boy suffered from shingles at 3 years of age after a normal course of chickenpox, had a mycoplasma pneumonia at 8yrs of age that produced a similar Xray like now with structural thickening (interstitial picture) that resolved clinically, but was never confirmed to be normal in the meantime. He is a competition soccer player, and did not have any breathing problems, but returned now with campylobacter-positive pericardial effusion and bacteriemia and fever. Responded well to antibiotics and steroids but relapsed one week after end of treatment. IgE moderatly increased.

Does this sound typical of any PID? Your help is highly appreciated. 

Lab: IgG incl subclasses and specific Ab's normal, IgA normal, IgM mildly increased, IgE 400, Eos mildly increased (500-1000/ul), B cells incl subsets normal, T cells including naive, gdT, DNT and proliferation tests were normal, granulocyte function tests normal. 
Parasitology pending; NK function (CD107a degranulation) pending. No exposition to tropical areas/infections known. Other bacteriological and virological tests negative. Autoantibodies incl ANCA subsets, basal membrane Ab's and ENA negative. 

Thanks for your helpful ideas or advice, Best Markus Seidel




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