[CIS PIDD] [cis-pidd] Case of ILD

[CIS-PIDD]

I have a 2 y.o. white male with biopsy proven ILD (see biopsy results below).  He also has 0 antibody production titers to s pneumonia despite both Pneumovax and PCV-13 and I've subsequently started him on IVIG (one dose received).  Genetic testing (screen) for immunodeficiency is pending for 35 deletions (ARUP). He continues to struggle with high dose prednisone, frequent exacerbations, and very labile asthma.

Questions:
1.       In addition to prednisone, does anyone have any experience with using other immunomodulators such as cyclophosphamide?
2.      I've read an article from Pediatric Pulmonology regarding genetic testing for surfactant protein C mutations and use of hydroxychloroquine for treatment in these patients-any thoughts?
3.      If a lung transplantation is a possibility, what centers are close to Oklahoma where this patient is from?

I have included his labs and biopsy information at the bottom of the page.  I appreciate any feedback! Dr. Lynn Wiens Warren Clinic Tulsa, OK

4/19/2016  3:02 PM - Background User Lab


Component Results

        Component       Value   Ref Range & Units       Status
        IGG     732     345-1213 mg/dL  Final
4/4/2016  5:36 PM - Background User Lab


Component Results

        Component       Value   Ref Range & Units       Status
        HIV-1/2 Ag/Ab   Non-reactive    Non-reactive    Final
        HIV Ag/Ab s/co ratio    0.06    <1.00   Final
T-cell fixation test: poor response to antigens & mitogens.
DHR which screens for phagocytic function is normal.
Lymphocyte function, detected by T-cell fixation test, shows a subpar response to viral peptides.
Patient does not perform adequate antibodies to streptococcus pneumonia
3/28/2016 12:25 PM - Janna L. Dierker


Component Results

        Component       Value   Ref Range & Units       Status
        CD3 T Lymphs (%)        58.0    55-82 % Final
        CD3 T Lymphs (Absolute) 1856 (L)        2300-3300 cumm  Final
        CD4+/CD3+ (%)   27 (L)  38-46 % Final
        CD4+/CD3+ (Absolute)    838 (L) 1500-2200 cumm  Final
        CD8+/CD3+ (%)   32 (H)  8-31 %  Final
        CD8+/CD3+ (Absolute)    988     350-2500 cumm   Final
        CD4/CD8 Ratio   0.85 (L)        1.17-6.22       Final
        CD19 B Lymphs (%)       36      11-45 % Final
        CD19 B Lymphs (Absolute)        1178    430-3300 cumm   Final
        NK Cells (%)    4 (L)   5-27 %  Final
        NK Cells (Absolute)     133     90-590 cumm     Final

No organisms cultured on BAL
This lung biopsy shows a primarily airway-centered, follicular bronchiolitis pattern of lymphocytic inflammation, mixed with scattered areas of interstitial involvement by a lymphocytic interstitial pneumonia-like pattern. Both are interspersed around areas of spared, relatively uninvolved parenchyma. There is evidence of small airway injury and areas of mucostasis and foamy macrophages with cholesterol aggregates are indicative of more proximal airway obstruction. No features are seen to suggest other entities in the clinical differential including primary surfactant deficiency, pulmonary interstitial glycogenosis, or organizing pneumonia/infection. The possibility of chronic aspiration injury was considered due to the prominence of airway involvement however due to the overall histologic pattern and absence of aspirated foreign material this would be considered less likely. Otherwise the primary consideration for this combination or follicular bronchiolitis and LIP pattern in a child would be underlying immunodeficiency syndrome, including inherited immunodeficiency syndromes but also acquired causes (infectious HIV, EBV, etc.). It is understood that clinical workup for such is ongoing at the moment.



Lynn A. Wiens, MD
Office: 918-495-2636
Cell: 918-550-1200
http://tulsaallergynews.com




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