[CIS PIDD] [cis-pidd] Auto-inflammatory Patient?

[CIS-PIDD]

I agree it smells infectious.  If all this started after this "dental appointment"  (was this a routine cleaning, or an extraction of a some lesion?), then chances are there is something which made its way out during the procedure, seeded some distal site/s, causing an inflammatory response that is blunted when he is on steroids, but re-manifests itself when the steroids are weaned down.

Or, alternatively, with the presentation of respiratory failure, was something introduced by inhalation?

The best sample (short of a biopsy) would probably be the BAL.  Was it tested for Pneumocystis, fungi, and AFB?  A galactomannan antigen level on the BAL and blood?  What was the cytology like?  (PCP and other invasive fungi may explain the lung --> liver hit, as well as the high LDH, and the "response" to steroids)

If all actually were negative, then I would just pull the trigger and send a lung + liver biopsy to U Washington for a pathogen PCR.  I have had some experience where culture negative specimens did get something identified.

Blood PCR's for EBV and CMV are not a bad idea.  If positive (and actually high ... >5000/ml), the workup suggested thus far (XIAP, etc.) should be useful.

Good luck.

  - K 

Karl O. A. Yu, M.D., Ph.D., F.A.A.P.
Instructor of Pediatrics (Pediatric Infectious Diseases)
University of Chicago - Comer Children's Hospital
5841 S Maryland Ave, MC 6054, Chicago IL 60637
Pager:  773-702-6800   x1744
Fax:  773-702-1196
Lab phone (Bubeck Wardenburg laboratory): 773-834-6976


________________________________________
From: CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
Sent: Monday, June 20, 2016 4:53 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] Auto-inflammatory Patient?

I still think its infection..lung, omentun/livre bipsy?!

Dr Leonardo Oliveira Mendonça

Médico do Serviço de Imunologia Clínica e Alergia Hospital Das Clinicas, Faculdade de Medicina da Universidade de São Paulo
Unidade Convênios e Particulares
Leonardo Oliveira Mendonça,MD
Departament of Clinical Immunology and Allergy  at Hospital das Clínicas, School of Medicine - University of São Paulo
Private Healthcare Unit
email: leonardo.mendonca at hc.fm.usp.br<mailto:leonardo.mendonca at hc.fm.usp.br>
telefones/phone number: +55-11-26619571<tel:%2B55-11-26619571>/ +55-11-26617825<tel:%2B55-11-26617825>/ FAX: 011-2661 8173


All,

We are currently caring for a 5 year old boy with fevers x 2 months and worsening respiratory status.

He was in good health until he had a dental appointment. The next day, he developed fevers to 103, a right ear rash, pain and swelling of his feet, diffuse muscle pain, decreased energy and PO intake. He was admitted to an OSH from 4/6/16 - 5/5/16. Had extensive workup during the hospitalization which was negative for infection and auto-immunity. The pt was empirically started on high dose steroids (on which he improved) and discharged to home with plan to taper steroids slowly over time and follow up with PCP and rheumatology.

Mom reports that as soon as she started to taper the steroids, he worsened again- with fevers and jaundice. He was then admitted to our hospital and found to have transaminitis, ascites, peritonitis, pancreatitis, persistent fevers, tachycardia, and tachypnea .

Yesterday, he was transferred to the PICU for continued fevers and respiratory failure requiring intubation. So far, no source has been found for fevers and respiratory distress.

Her work-up has included:

Ferritin- highest of 1133 on 4/20 now down to 333
CBC- just slight normocytic anemia
AST high of 1368 on 5/2 now down to 482
ALT high of 743 on 5/2 now down to 104
GGT high of 1431 on 5/20 now down to 394
LDH high of 707 on 5/2,
Triglycerides- high of 514 on 5/16, now down to 161
EBV ab to nuclear Ag IGG 4/7: >600 (high)
EBV ab to viral capsid ag IGG 4/7: 291 (high)
EBV ab to viral capsid ag IGM 4/7: 12.4
Viral respiratory panel- negative
ANA- negative
ESR- high of 78 on 4/28 now down to 68
C3 : 124
C4: 22.6
IgA: 257 (high)
IgG: 1160 (high)
IgM: 114 (nml)
Lymphocyte subsets: normal except low CD56 cells
Soluble IL-2R: 1462
Perforin/granzyme- normal
Glycogen storage disease work-up- normal
BAL- negative
Chest CT- Bilateral extensive groundglass/consolidation which is most characteristic for diffuse alveolar damage/acute lung injury

Abdomen CT- Enlarged, hypoattenuating liver. Interval decrease in now moderate volume ascites, partially surrounded by thick/enhancing walls compatible with organizing, loculated fluid. Superimposed infection is not excluded.

Edematous appearing pancreas with normal enhancement relative to the liver and spleen.


Blood/CSF/urine cxs- negative

He is currently on solumedrol 1mg/kg Q12 and his condition is continuing to deteriorate requiring more oxygen and ventilator support.

A cytokine panel has been sent out and is pending.

Any other ideas or considerations would be greatly appreciated.

Thanks.

-Evan


Evan Shereck, MD
Associate Professor of Pediatrics
Director, Pediatric Hematology/Oncology Fellowship
Block Co-Director, Blood and Host Defense
Oregon Health & Science University I  3181 SW Sam Jackson Park Rd, Mail Code: CDRCP  I  Portland, OR 97239
(Office): 503-494-0829<tel:503-494-0829>  I  (Fax): 503-494-0714<tel:503-494-0714>
 (E-mail): shereck at OHSU.edu

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