[CIS PIDD] a case of recurrent pneumonias and culture negtive endocarditis

[Richard Wasserman]

You didn't provide the actual Ig values nor the pneumococcal antibody
values; they would be interesting. The complement abnormalities are a
reflection of active infection and consumption. This lady sounds quite ill.
I don't think 500mg/kg/mo is adequate in this setting. I would have given
her 500mg/kg/dose on two consecutive days, repeat an IgG at 48-72 hours and
follow her levels and clinical response. Good luck.
Richard Wasserman
Dallas

On Wed, Jun 20, 2012 at 4:46 PM, Mahboobeh Mahdavinia <
mahboobeh-mahdavinia at fsm.northwestern.edu> wrote:


> Dear Colleagues,

> We appreciate your input with suggestions for possible diagnosis, work up

> and treatment for the following case.

>

> Regards,

>

> Maha Mahdavinia,MD

> Allergy/Imuunolog fellow

> Northwestern University

>

> Pt is a 37 y/o Female with Hx of multiple pneumonias and otitis since age

> 2 months old. By age 18 years she had documented bronchiectasis and

> continues to have multiple hard to treat otitis, frequent pneumonias

> documented by

> CXR despite prophylactic clarithromycin.

>

>

>

> Her w/u so far showed normal immunoglobulin levels, normal lymphocyte

> numbers and function, negative workup for cystic fibrosis (both sweat test

> and mutation analysis), negative ANCA, ANA of 1:80  (speckled) and

> negative nRNP, SS-A, SS-B, Sm and Scl-70 Autoantibodies.

>

>

>

> She has been in hospitals for the past 4 months with recurrent and

> refractory culture negative endocarditis .She has no history of valve

> disease in past. During this period, she has already undergone 3 cardiac

> surgeries for MVR twice, AVR, and repairs of aortic-atrial fistula. She

> is currently intubated due to respiratory failure, is in renal failure and

> has had prolonged thrombocytopenia (improved after IVIG in last 3 days).

>

>  All cultures during her hospitalization and throughout her life,

> including bronchoalveolar  lavages , blood cultures, and biopsy cultures

> have been negative (fungal, TB, special cultures and viral PCRs).

>

>

>

> During this endocarditis admission, her C3 was 38 (NL:90-180), C4 was 17

> (NL:10-40), and CH50 was 54 and 48  (NL:63-145).

>

> Pneumococcus Ab titers showed minimal levels to all 14 serotypes despite

> Pneumovax  in 2008. We started IVIG 500mg/kg IV last Friday 6/15.

>

> * *

>

> Pending labs include: AH50 level ,Dihydrorhodamine (DHR) , NK function,

> C1 and C2.

>

>

>

>

>




-- 
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211
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