[CIS PIDD] [cis-pidd] XIAP

Mon Aug 21 17:00:45 EDT 2017

He has not had hypogamm since I have been following over the last 8 years.

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 4:45 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Subject: RE:[cis-pidd] XIAP

This email contains a suspicious URL
Jason

Is the patient hypogam?

James

James W. Verbsky M.D./Ph.D.
Associate Professor of Pediatrics and Microbiology
Medical Director, Clinical Immunology Research Laboratory
Medical Director, Clinical and Translational Research
Medical College of Wisconsin
Milwaukee, WI

From: cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net> [mailto:cis-pidd at lyris.dundee.net] On Behalf Of CIS-PIDD
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>
[Wake Forest School of Medicine]

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