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

Sat Apr 23 17:14:18 EDT 2016

Lynn
In order to give a better differential, I think the following  information is essential. Was the lymphocyte subset analysis done before or after steroids were started? What is the distribution of the lung parenchymal abnormalities (diffuse, upper lung zones, lower lung zones?). The result of the BAL (cell differential and presence of lipid laden macrophages, cultures or PCRs performed for pathogens, evidence of pulmonary alveolar proteinosis). What is the IgA and IgM? I would not rely on titers to exclude infection, HIV-1 should be excluded by PCR and would suggest PCR for other viral RT pathogens (RV, RSV, influenza, PIV etc…) as well Herpes viruses (CMV, EBV).
The differential diagnosis of diffuse ILD in infants and children is long and includes infection, lung intrinsic causes, systemic diseases with lung involvement (PIDs, lymphoproliferative disorders etc..), drugs etc. Frequently a transbronchial biopsy does not get sufficient tissue for definitive diagnosis and open lung biopsy may be necessary. The specific diagnosis is essential for proper treatment. If you are uncomfortable with the work up, I  would suggest that you might consider referral to a center with experience in the evaluation of such children. Let me know if I can be of help
best
Jack

John M. Routes, MD

Chief, Section of Allergy and Clinical Immunology
Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics
Department of Pediatrics
Children's Hospital of Wisconsin
Medical College of Wisconsin
9000 W. Wisconsin Ave.
Milwaukee, WI  53226-4874

Phone: 414-456-4802; 456-4803
Fax: 414-456-6487 (Clinical)
Fax: 414-456-6323 (Laboratory)
Email: jroutes at mcw.edu<mailto:jroutes at mcw.edu>

From: CLINICAL IMMUNOLOGY LISTSERV <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Reply-To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Date: Friday, April 22, 2016 at 12:19 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Subject: [cis-pidd] Case of ILD

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
[cid:7281F95A64691A4494D3A332B7513600 at saintfrancis.com]

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