Journal of Clinical Immunology

, Volume 33, Issue 1, pp 30–39 | Cite as

Use of Combination Chemotherapy for Treatment of Granulomatous and Lymphocytic Interstitial Lung Disease (GLILD) in Patients with Common Variable Immunodeficiency (CVID)

  • Nicole M. Chase
  • James W. Verbsky
  • Mary K. Hintermeyer
  • Jill K. Waukau
  • Aoy Tomita-Mitchell
  • James T. Casper
  • Sumit Singh
  • Kaushik S. Shahir
  • William B. Tisol
  • Melodee L. Nugent
  • R. Nagarjun Rao
  • A. Craig Mackinnon
  • Lawrence R. Goodman
  • Pippa M. Simpson
  • John M. Routes
Original Research

Abstract

Purpose

A subset of patients with common variable immunodeficiency (CVID) develops granulomatous and lymphocytic interstitial lung disease (GLILD), a restrictive lung disease associated with early mortality. The optimal therapy for GLILD is unknown. This study was undertaken to see if rituximab and azathioprine (combination chemotherapy) would improve pulmonary function and/or radiographic abnormalities in patients with CVID and GLILD.

Methods

A retrospective chart review of patients with CVID and GLILD who were treated with combination chemotherapy was performed. Complete pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) scans of the chest were done prior to therapy and >6 months later. HRCT scans of the chest were blinded, randomized, and scored independently (in pairs) by two radiologists. The differences between pre- and post-treatment HRCT scores and PFT parameters were analyzed.

Results

Seven patients with CVID and GLILD met inclusion criteria. Post-treatment increases were noted in both FEV1 (p = 0.034) and FVC (p = 0.043). HRCT scans of the chest demonstrated improvement in total score (p = 0.018), pulmonary consolidations (p = 0.041), ground-glass opacities (p = 0.020) nodular opacities (p = 0.024), and both the presence and extent of bronchial wall thickening (p = 0.014, 0.026 respectively). No significant chemotherapy-related complications occurred.

Conclusions

Combination chemotherapy improved pulmonary function and decreased radiographic abnormalities in patients with CVID and GLILD.

Keywords

Common variable immunodeficiency (CVID) primary immunodeficiency lung disease granulomatous and lymphocytic interstitial lung disease (GLILD) rituximab azathioprine 

Notes

Acknowledgements

We would like to acknowledge and thank Dr. Mitchell Grayson for his thoughtful review of the manuscript.

This research was supported by National Institutes of Health grant R01CA122539 (www.nih.gov), and the Children’s Research Institute of the Children’s Hospital of Wisconsin (www.chw.org/display/PPF/DocID/30477/router.asp).

Conflict of interest

The authors of this manuscript declare that they have no financial conflicts of interest.

Supplementary material

10875_2012_9755_MOESM1_ESM.docx (17 kb)
ESM 1 (DOCX 17.3  kb)
10875_2012_9755_MOESM2_ESM.doc (21 kb)
ESM 2 (DOC 21 kb)

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Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Nicole M. Chase
    • 1
    • 2
  • James W. Verbsky
    • 1
    • 3
  • Mary K. Hintermeyer
    • 1
    • 2
  • Jill K. Waukau
    • 1
    • 2
  • Aoy Tomita-Mitchell
    • 4
    • 5
  • James T. Casper
    • 1
    • 6
  • Sumit Singh
    • 7
    • 8
  • Kaushik S. Shahir
    • 7
    • 8
  • William B. Tisol
    • 4
    • 5
  • Melodee L. Nugent
    • 10
  • R. Nagarjun Rao
    • 7
    • 9
  • A. Craig Mackinnon
    • 7
    • 9
  • Lawrence R. Goodman
    • 7
    • 8
  • Pippa M. Simpson
    • 10
  • John M. Routes
    • 1
    • 2
    • 11
  1. 1.Department of PediatricsMedical College of WisconsinMilwaukeeUSA
  2. 2.Division of Allergy/Clinical ImmunologyMedical College of WisconsinMilwaukeeUSA
  3. 3.Division of RheumatologyMedical College of WisconsinMilwaukeeUSA
  4. 4.Department of SurgeryMedical College of WisconsinMilwaukeeUSA
  5. 5.Division of Cardiothoracic SurgeryMedical College of WisconsinMilwaukeeUSA
  6. 6.Division of Hematology/Oncology/BMTMedical College of WisconsinMilwaukeeUSA
  7. 7.Department of MedicineMedical College of WisconsinMilwaukeeUSA
  8. 8.Division of Diagnostic RadiologyMedical College of WisconsinMilwaukeeUSA
  9. 9.Division of PathologyMedical College of WisconsinMilwaukeeUSA
  10. 10.Department of Quantitative Health SciencesMedical College of WisconsinMilwaukeeUSA
  11. 11.MilwaukeeUSA

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