[CIS PIDD] [cis-pidd] question about dysuria in a CVID patient

[Harville, Terry O]

In peripubertal patients, it is common to see the beginnings of spondyloarthropathy features.  It appears that she has urethritis.  In the past, one may consider this as part of Reiter's syndrome.  I have seen many, many peripubertal females with non-specific urethritis, typically being attributed to some non-culturable or non-identifiable organism, where the patients would have been seen by their primary care or in ER settings and begun on antibiotics, ultimately to be stopped due to no organism cultured or no benefit to the patient.

Some 10% of the general population inherit HLA-B27 or related component which places them at risk for spondyloarthropathies.

Your description of your patient fits many of these that I have seen.  Albeit, the question of CVID versus other CID is not part of the spondyloarthropathy issue.

Terry Harville MD PhD
-Medical Director, Special Immunology Laboratory
-Medical Director, Histocompatibility Laboratory
-Medical Director, Immunogenetics and Transplantation Laboratory
-Specialist in Pediatric Immunology and Rheumatology
Departments of Pathology and Laboratory Services and Pediatrics
University of Arkansas for Medical Sciences
4301 West Markham
Mail Slot #502
Little Rock, AR  72205-7199

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From: YaeJean Kim [mailto:yaejeankim at skku.edu]
Sent: Monday, June 10, 2013 9:52 AM
To: CIS-PIDD
Subject: [cis-pidd] question about dysuria in a CVID patient

Dear All,

I have a 14 yo girl with CVID on IVIG replacement therapy every 3 weeks.
She has multiple conditions as below.
- s/p CMV infection diagnosed at outside hospital.
- A huge splenomegaly for which she received splenectomy -> amoxacillin prophylaxis
- Chronic kidney disease stage 4
- Hypertension
- Short stature, delayed puberty
- interstitial pneumonitis responded to steroid and now off

The reason that I am writing is about significant dysuria and urinary frequency. Multiple urine cultures did not grow anything.
But she complains of severe waxing and waning pain.

This time, she was rolling in the bed because of pain in the suprapubic area/perineum and fever. At ER, UA showed LE+++, numerous WBC/ HPF but gram stain and routine urine culture grew nothing. Cefotaxime was started empirically and the fever is gone, the pain is reduced (I am not sure whether it was truly due to cefotaxime effect or not)...Anyway, she got somewhat better but still complains of sudden pain that requires pain meds on and off.
Image studies showed no definite stones..

Urology and nephrology have seen her but no clear idea. To her, this is a significant symptom that bothers her daily life.
At this point, I would appreciate any suggestion or advice.

Sincerely,

YaeJean

-------------------------------------------------
Yae-Jean Kim, MD, PhD
Associate Professor
Division of Infectious Diseases
Department of Pediatrics
Sungkyunkwan University School of Medicine
Samsung Medical Center
50 Irwon-dong Gangnam-gu
Seoul, Korea
Tel)  +82-2-3410-3539, 0987  Fax) +82-2-3410-0043
yaejeankim at skku.edu<mailto:yaejeankim at skku.edu>

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