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

[Mahboobeh Mahdavinia]

 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 CXRdespite 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.
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