[CIS PIDD] [cis-pidd] XIAP
CIS-PIDD
cis-pidd at lists.clinimmsoc.org
Mon Aug 21 16:56:24 EDT 2017
He is not sexually active.
I will follow up on unusual exposures.
Thank you for the responses!
Jason W Caldwell DO FAAAAI
Associate Professor of Internal Medicine and Pediatrics
Section of Pulmonary, Critical Care, Allergic and Immunological Diseases
Program Director of Allergy/Immunology Fellowship
Wake Forest University School of Medicine
Office: 336-716-5166
Administrative: 336-716-4843
Pager: 336-806-8330
jcaldwel at wakehealth.edu<mailto:jcaldwel at wfubmc.edu>
[Wake Forest School of Medicine]
From: cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] On Behalf Of CIS-PIDD
Sent: Monday, August 21, 2017 4:50 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Subject: RE:[cis-pidd] XIAP
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Dr. Caldwell:
I agree with Dr. Atkinson. I've had the U Washington lab be helpful once in a while. Not often, but enough to want to use them if I'm suspicious for a zebra.
Pus, fever, and elevated and rising inflammatory markers strongly suggest an infection. Linezolid x 3 weeks would have done something if it were either staph or strep (or most other Gram positives). But, the differential diagnosis - even for immunocompetent patients - does not end there.
Kingella would be the classic culture negative arthritis in a kid (this is because most orthopedic surgeons do not know to send an anaerobic culture of the fluid).
If he's an outdoorsy-type adolescent, the line of investigation should include the tick-borne diseases (it is summer, after all ...). Going by your history, this is a subacute and unilateral process that seems to have started from the L medial ankle and is making its way up (3 weeks does not count as "chronic" in my book). 'Smells suspicious enough. (Was there any history of rash or trauma??)
If outsdoorsy with river / pond water exposure ... this'll be painful. The differential will stretch to include several Gram negatives that are not easy to treat).
If sexually active, then the usual suspects should be added for testing (early syphilis, Chlamydia and gonococcus could all look like this). Of course, because of the PID, I would lean towards non-serologic diagnostics - unless you have sufficient evidence that he's seroconverted to routine vaccines.
With XIAP - viral arthritis should go up the differential ... in theory. I do not have enough experience with this immunodeficiency to say if this happens in practice. If the joint fluid cytology was classic neutrophil-predominant, I probably wouldn't go down this path. Do you have results on this?
Good luck.
- K
Karl O. A. Yu, M.D., Ph.D., F.A.A.P.
Scientist II and Assistant Director, Center for Infectious Diseases and Immunology
RGH Research Institute | Rochester General Hospital | Rochester Regional Health
1425 Portland Ave., Room R-403, Rochester, NY 14621
Tel 585-922-3709 | Fax 585-922-2415
From: cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] On Behalf Of CIS-PIDD
Sent: Monday, August 21, 2017 4:20 PM
To: CIS-PIDD
Subject: RE:[cis-pidd] XIAP
Hi Jason - I suggest you order the 16S/NGS testing for bacterial infection available through the University of Washington on a sample of the fluid. This low-grade infection could also be some type of mycoplasma infection - we have recently seen a case of multifocal septic arthritis in an untreated CVID patient due to M. salivarium. It evolved over a period of several months.
Prescott
T. Prescott Atkinson, MD PhD, Professor and Director
Division of Pediatric Allergy, Asthma & Immunology
University of Alabama at Birmingham
Tel: 205-996-9582
Fax: 205-975-7080
________________________________
From: cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net> [cis-pidd at lyris.dundee.net] on behalf of CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
Sent: Monday, August 21, 2017 3:00 PM
To: CIS-PIDD
Subject: [cis-pidd] XIAP
Good day,
I have a 12 year old patient with confirmed XIAP. He presented as an infant with fulminant HLH secondary to EBV. He has done well since with no other complications until recently.
About 3 weeks ago his PCP called secondary to swelling of the left foot and ankle. No pain, normal range of motion, but obviously swollen.
MRI:
1. No evidence of osteomyelitis of bone erosion identified. No subperiosteal abscess.
2. Ankle joint effusion and synovial enhancement noted, concerning for joint inflammation.
3. There is fluid surrounding the flexor digitorum longus tendon, with postcontrast enhancement of the synovium, findings concerning for tenosynovitis, unclear whether this is reactive to soft tissue inflammation and joint effusion/inflammation versus infected tenosynovitis.
4. Extensive subcutaneous and deep soft tissue edema of the lower leg, ankle, foot identified, medial side worse than lateral side concerning for soft tissue inflammatory changes/cellulitis. No focal fluid collection identified.
Then 1 weeks ago went to PCP for acute knee swelling and pain. He came into our facility with difficulty putting weight on left leg at the knee, swelling, pain, and fever of 101.
MRI:
1. There are no findings to indicate osteomyelitis of left knee. Small joint effusion and synovial enhancement noted suggesting inflammatory change/septic arthritis. No articular erosions noted.
2. There is reactive edema surrounding the distal femoral anterior compartment muscles and in the proximal lead predominantly involving the posterior compartment, likely reactive changes. No focal fluid collection
Ortho did an I & D of his knee. (did not want to look at the ankle since it was "chronic")
The I & D was remarkable for pus which was drained and closed. He was started on antibiotics and sent home on 3 weeks of Linezolid.
He returned today with improvement in knee swelling, normal temp, but zero improvement in the ankle foot.
Labs:
Hgb platelets Sed rate Ferritin crp
Admission labs: : 9.9, 693000, 64, 1100
Discharge 9.8 653000 64 295 55.5
Today 77 414 72.2
Triglcerdies and LDH are normal.
All cultures are no growth to date. (acid fast and fungal are no growth for 7 days.
There is mention of arthritis in these patient in the literature, but I did not see any details.
Has anyone had experience with this manifestation in XIAP?
Thoughts on treatment if cultures remain negative?
Any other thoughts??
Thank you in advance.
Jason
Jason W Caldwell DO FAAAAI
Associate Professor of Internal Medicine and Pediatrics
Section of Pulmonary, Critical Care, Allergic and Immunological Diseases
Program Director of Allergy/Immunology Fellowship
Wake Forest University School of Medicine
Office: 336-716-5166
Administrative: 336-716-4843
Pager: 336-806-8330
jcaldwel at wakehealth.edu<mailto:jcaldwel at wfubmc.edu>
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