Back to resultsTreatment of Granulomatous and Lymphocytic Interstitial Lung Disease in Patients With Common Variable Immunodeficiency
Primary Purpose
Granulomatous and Lymphocytic Interstitial Lung Disease
Status
Withdrawn
Phase
Phase 2
Locations
Study Type
Interventional
Intervention
Rituximab (RTX) and Azathioprine (AZA)
Placebos
Sponsored by
About this trial
This is an interventional treatment trial for Granulomatous and Lymphocytic Interstitial Lung Disease focused on measuring Granulomatous and lymphocytic interstitial lung disease (GLIILD), common variable immunodeficiency, Rituximab, Azathioprine, primary immunodeficiency, GLIILD, CVID
Eligibility Criteria
INCLUSION CRITERIA:
Age:
- Patients must be 18 years of age or older.
Diagnosis:
- Diagnosis of CVID in accordance with international criteria which includes: A) Serum immunoglobin G (IgG) at least 2 standard deviations below the age adjusted norm; B) Decreased serum immunoglobin A (IgA) and/or immunoglobin M (IgM); C) Age > 4 years.; D) Abnormal specific antibody production in response to immunization; E) Exclusion of secondary causes of hypogammaglobulinemia.
- Diagnosis of GLILD based on histopathologic abnormalities of lung tissue obtained by open lung biopsy within 12 months of enrollment and confirmed by Pathology Core.
Performance Level:
- Karnofsky Performance Status (KPS) ≥ 50%
Prior Therapy:
- Patients must have fully recovered from the acute toxic effects of all prior therapy.
- Systemic steroids need to be completed at least 60 days from the time of enrollment.
Organ Function:
Adequate Lung Function defined as:
• FVC > 60 % predicted and
• DLco > 35 % predicted
Adequate Bone Marrow Function defined as:
• Peripheral absolute neutrophil count (ANC) ≥ 750/mm3 and
• Platelet count ≥ 50,000/mm3
Adequate Hepatic Function as evidenced by:
- Direct Bilirubin < 1.5 x upper limit of normal (ULN) for age
- Serum glutamic pyruvic transaminase (SGPT) (ALT) < 135 U/L. For the purpose of this study, the ULN for SGPT is 45 U/L.
- Adequate Renal Function as defined by a normal serum creatinine
Reproductive Function:
o Female patients of childbearing potential must have a negative urine or serum pregnancy test confirmed prior to enrollment.
- Female patients with infants must agree not to breastfeed their infants while on this study.
- Male and female patients of childbearing potential must agree to use an effective method of contraception approved by the investigator during the study.
- Sexually active females of childbearing potential must agree to use adequate contraception (diaphragm, birth control pills, injections, intrauterine device (UD), surgical sterilization, subcutaneous implants, or abstinence, etc.) for the duration of treatment and for 6 months after the last dose of study therapy. Sexually active men must agree to use barrier contraceptive for the duration of treatment and for 6 months after the last dose of study therapy.
Regulatory Requirements
- All patients must sign a written informed consent.
- All institutional, FDA, and NIH requirements for human studies must be met.
EXCLUSION CRITERIA:
Infection:
- Patients with uncontrolled infection are not eligible.
- Patients with documented serious infection within 3 months of screening or opportunistic infection within 6 months of screening are not eligible.
Cardiac Function:
o Patients cannot be diagnosed with New York Heart Association (NYHA) Class III or IV congestive heart failure, ventricular arrhythmias, or uncontrolled hypertension.
Allergies:
o Known hypersensitivity to any of the components of RTX or AZA.
Current Therapy:
- Systemic immunosuppressive medications including steroids.
- Steroids can be used to prevent or to treat infusion-related RTX symptoms, but this should be used only prior to or immediately after the RTX infusion, and should not be continued beyond 3 days. The use of systemic steroids should be recorded.
- Inhaled steroids are acceptable.
Previous Therapy:
o Previous treatment with RTX or AZA for GLILD.
Pregnant Females:
o Pregnant females will not be allowed to participate in this study.
Hepatic Disease:
o Known cirrhosis and/or portal hypertension.
Hepatitis B or Hepatitis C Infection:
- All patients will be screened for Hepatitis B and C by polymerase chain reaction (PCR).
- Hepatitis C positive as determined by PCR.
Hepatitis B Reactivation: Hepatitis B Reactivation is defined as Hepatitis B carrier patients with one of the following:
- Positive hepatitis B e-antigen (HBe-Ag)
- Quantitative hepatitis B Viral (HBV) DNA Load > 10^5 genomes/ml
Human Immunodeficiency Virus (HIV) 1 Positive:
o HIV 1 infection will be determined by PCR.
Homozygous Mutations:
o Patients with homozygous mutations of thiopurine methyltransferase (TPMT) will be excluded from the study.
Sites / Locations
Arms of the Study
Arm 1
Arm 2
Arm Type
Active Comparator
Placebo Comparator
Arm Label
Rituximab (RTX) and Azathioprine (AZA)
Placebo
Arm Description
Rituximab 375 mg/m2/dose IV over 4 hours first dose, IV over 2-3 hours each subsequent dose weekly for 4 weeks at enrollment and again at months 6 and 12. Azathioprine: Starting dose of azathioprine will be 50 mg and increased in 25 mg increments to a maximum dose of 150 mg or 2 mg/k/day (whichever is lowest) as tolerated. Azathioprine will be administered by mouth daily for 18 months.
IV placebo will be administered on the same schedule as Rituximab. Oral placebo will be administered by mouth daily for 18 months.
Outcomes
Primary Outcome Measures
The effect of treatment with RTX/AZA in patients with GLILD compared to placebo, based on change in forced vital capacity (FVC) at 18 months compared to baseline.
Pulmonary Function Tests (PFTs) will be performed to measure lung volumes and airflow for evidence of restrictive and obstructive lung disease. PFTs are used to measure lung volumes and airflow for evidence of restrictive and obstructive lung disease. Measurements will be obtained by standard techniques following guidelines outlined by the American Thoracic Society. Spirometry, during screening, needs to be done pre- and post-bronchodilator. All subsequent spirometry is done post-bronchodilator. Diffusion capacity for carbon monoxide is always done post-bronchodilator. Spirometry will be performed to access the forced expiratory volume (FEV1) and forced vital capacity (FVC). Carbon monoxide diffusion capacity will be performed to assess gas exchange.
Secondary Outcome Measures
The effect of treatment with RTX/AZA relative to placebo on the changes over time in high-resolution CT scans of the chest.
Non-contrast, low dose HRCT scans of the chest will be performed at the intervals and analyzed. Studies will be performed on high-end scanners (64 or above detector rows) capable of producing thin section (1-1.25mm) lung algorithm scans.
Correlate changes in pulmonary function (FVC, FEV1, DLco) with extent of pulmonary fibrosis obtained on open lung biopsy.
Pulmonary Function Tests (PFTs) will measure forced vital capacity, forced expiratory volume in one second, diffusing capacity of the lungs for carbon monoxide (DLCO) (e.g., DLco will be measured by the single-breath technique using a 10-second breath hold). Histopathologic abnormalities of lung tissue will be determined by open lung biopsy.
Correlate changes in pulmonary function (FVC, FEV1, DLco) with high-resolution CT scan scores over time in the two randomized groups of patients.
Pulmonary Function Tests (PFTs) will measure forced vital capacity, forced expiratory volume in one second, diffusing capacity of the lungs for carbon monoxide (DLCO) (e.g., DLco will be measured by the single-breath technique using a 10-second breath hold). These will be compared with high-resolution CT scan scores.
Changes in FVC and HRCT of the chest (maintained for 6 months after completion of therapy in both randomized groups)
Pulmonary Function Tests (PFTs) will measure forced vital capacity, forced expiratory volume in one second, diffusing capacity of the lungs for carbon monoxide (DLCO) (e.g., DLco will be measured by the single-breath technique using a 10-second breath hold). High-resolution CT scans will be performed.
Incidence of lymphoma in patients treated with RTX/AZA or placebo over the time of enrollment in the study.
Patients will be monitored with physical examinations, laboratory tests (CBC, auto diff, ALT, bilirubin, serum creatinine, platelets, comprehensive lymphocyte phenotype and cell sort).
Changes in quality of life in the two randomized groups of patients as measured by SGRQ total score.
Quality of life will be measured by the St. George's Respiratory Questionnaire (SGRQ), a pulmonary disease specific questionnaire measuring self-reported dyspnea symptoms and their relationship to activities of daily living and psychological functioning.
Changes in quality of life in the two randomized groups of patients as measured by Karnofsky Performance Status Scale (KPS).
Karnofsky Performance Status Scale (KPS) will be performed at baseline, six months, 12 months, 18 months and 24 months.
Changes in quality of life in the two randomized groups of patients as measured by 6-minute Walking Test.
The 6-minute Walking Test will performed at baseline, six months, 12 months, 18 months and 24 months.
Dysregulated molecular pathway determined by performing whole transcriptome sequencing.
This will be done by performing whole transcriptome sequencing on GLILD, idiopathic pulmonary fibrosis (IPD), sarcoidosis and normal lung tissue.
Lung transcriptome predicts response to RTX/AZA therapy (performing whole transcriptome sequencing on GLILD, IPD, sarcoidosis and normal lung tissue) and confirm that lung transcriptome predicts response to RTX/AZA therapy.
This will be done by performing whole transcriptome sequencing on GLILD, IPD, sarcoidosis and normal lung tissue.
Peripheral blood biomarkers as indicators of GLILD disease activity.
The research team will examine blood specimens and evaluate Human Leukocyte Antigen - DR isotype (HLA-DR) negative and positive T cells.
Presence of bacterial (16S rRNA), fungal (Internal Transcribed Spacer region/ITS) and viral sequences (unbiased high-throughput sequencing) in the lungs of GLILD patients.
This will be done by screening lung biopsies from patients with GLILD, idiopathic pulmonary fibrosis, pulmonary sarcoidosis, or no known pulmonary disease.
Prevalence and abundance of bacterial, fungal and viral sequences.
This will be measured with quantitative PCR analysis of lung tissues obtained from patients with GLILD, idiopathic pulmonary fibrosis, pulmonary sarcoidosis or no known lung disease.
Full Information
NCT ID
NCT02789397
First Posted
May 16, 2016
Last Updated
March 6, 2020
Sponsor
Medical College of Wisconsin
1. Study Identification
Unique Protocol Identification Number
NCT02789397
Brief Title
Treatment of Granulomatous and Lymphocytic Interstitial Lung Disease in Patients With Common Variable Immunodeficiency
Official Title
Clinical Trial to Assess the Efficacy of Rituximab and Azathioprine in the Treatment of Granulomatous and Lymphocytic Interstitial Lung Disease (GLILD) in Adult Patients With Common Variable Immunodeficiency (CVID)
Study Type
Interventional
2. Study Status
Record Verification Date
March 2020
Overall Recruitment Status
Withdrawn
Why Stopped
No funding for study.
Study Start Date
May 2, 2016 (Actual)
Primary Completion Date
March 6, 2018 (Actual)
Study Completion Date
March 6, 2018 (Actual)
3. Sponsor/Collaborators
Responsible Party, by Official Title
Principal Investigator
Name of the Sponsor
Medical College of Wisconsin
4. Oversight
Studies a U.S. FDA-regulated Drug Product
Yes
Studies a U.S. FDA-regulated Device Product
No
Product Manufactured in and Exported from the U.S.
No
Data Monitoring Committee
Yes
5. Study Description
Brief Summary
This phase II study will assess the effect of a treatment combination of Rituximab and azathioprine in patients with Granulomatous and Lymphocytic Interstitial Lung Disease (GLILD) compared to placebo, based on change in lung function at 18 months compared to baseline. The researchers will also assess if the drugs improved quality of life.
Detailed Description
BACKGROUND Common Variable Immunodeficiency (CVID) is one of the most clinically important primary immunodeficiencies due to its frequency, serious complications, and long-term costs of therapy. A form of lung disease known as granulomatous and lymphocytic interstitial lung disease (GLILD) occurs in 10-15% of patients with CVID. The causes of GLILD are unknown; no long-term study has defined the natural course of GLILD; and no clinical trials have been done to define the best possible treatment for this condition. As a result, currently there is no proven standard of care for the treatment of GLILD.
The best treatment for individuals with GLILD is not currently known. Some doctors believe that GLILD does not always continue to get worse and patients should only be treated unless this happens. Other doctors believe GLILD is always progressive and should be treated early to prevent more problems later.
There is compelling evidence to support that treatment using rituximab (RTX) in conjunction with azathioprine (AZA), may improve the lung function and abnormalities seen on high resolution CT (HRCT) scans of the chest.
STUDY GROUPS Patients in this study will either receive a placebo or a combination of Rituximab and azathioprine. These drugs are approved by the US Food and Drug Administration for other conditions, but not yet for this disease.
Because no one knows which of the treatments is best, patients will be "randomized" into one of the two study groups. Randomization means that you are put into a group by chance.
TREATMENT Eligible patients will be randomized to receive either 18 months of Rituximab and Azathioprine (20 patients) or placebo (20 patients). Rituximab will be administered intravenously (IV) weekly for four consecutive weeks at enrollment and months 6 and 12. IV placebo will be administered on the same schedule as Rituximab. Azathioprine or oral placebo will be administered by mouth daily for 18 months.
SUMMARY OF STUDY PROCEDURES
-Month 1, 6, 12
Patients will be required to travel to a study site weekly for four consecutive weeks at enrollment and at 6 and 12 months to receive study infusions. At each of these visits, patients will be given:
Your study infusions
Physical exams with vital signs
Blood tests to check your organ function
Every six months (Enrollment, 6, 12 & 18 months) while receiving study treatment, patients will be asked to complete the following study tests:
Lung Function testing
High resolution CT of the chest
Quality of Life Questionnaire, 6-min walk distance test and Karnofsky performance scale.
Blood for research - approximately 10 teaspoons of blood will be collected
Monthly Labs Following the first month of study treatment, patients will be required to visit their local clinic/hospital for a blood draw to monitor their lab values twice monthly for the second and third months of treatment, then monthly.
Final Study Visit
The final study visit will take place at Month 24 after start of study treatment. Patients will also have the following tests done:
Physical exams with vital signs
Lung Function testing
High resolution CT of the chest
Quality of Life Questionnaire, 6-min walk distance test and Karnofsky performance scale.
Blood for research - approximately 10 teaspoons of blood will be collected
6. Conditions and Keywords
Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study
Granulomatous and Lymphocytic Interstitial Lung Disease
Keywords
Granulomatous and lymphocytic interstitial lung disease (GLIILD), common variable immunodeficiency, Rituximab, Azathioprine, primary immunodeficiency, GLIILD, CVID
7. Study Design
Primary Purpose
Treatment
Study Phase
Phase 2
Interventional Study Model
Parallel Assignment
Masking
ParticipantCare ProviderInvestigator
Allocation
Randomized
Enrollment
0 (Actual)
8. Arms, Groups, and Interventions
Arm Title
Rituximab (RTX) and Azathioprine (AZA)
Arm Type
Active Comparator
Arm Description
Rituximab 375 mg/m2/dose IV over 4 hours first dose, IV over 2-3 hours each subsequent dose weekly for 4 weeks at enrollment and again at months 6 and 12. Azathioprine: Starting dose of azathioprine will be 50 mg and increased in 25 mg increments to a maximum dose of 150 mg or 2 mg/k/day (whichever is lowest) as tolerated. Azathioprine will be administered by mouth daily for 18 months.
Arm Title
Placebo
Arm Type
Placebo Comparator
Arm Description
IV placebo will be administered on the same schedule as Rituximab. Oral placebo will be administered by mouth daily for 18 months.
Intervention Type
Drug
Intervention Name(s)
Rituximab (RTX) and Azathioprine (AZA)
Other Intervention Name(s)
Truxima
Intervention Description
Rituximab 375 mg/m2/dose IV over 4 hours first dose, IV over 2-3 hours each subsequent dose weekly for 4 weeks at enrollment and again at months 6 and 12 for the active comparator arm Rituximab (RTX) and Azathioprine (AZA).
Intervention Type
Drug
Intervention Name(s)
Placebos
Other Intervention Name(s)
Saline
Intervention Description
IV placebo will be administered on the same schedule as Rituximab and oral placebo will be administered by mouth daily for 18 months.
Primary Outcome Measure Information:
Title
The effect of treatment with RTX/AZA in patients with GLILD compared to placebo, based on change in forced vital capacity (FVC) at 18 months compared to baseline.
Description
Pulmonary Function Tests (PFTs) will be performed to measure lung volumes and airflow for evidence of restrictive and obstructive lung disease. PFTs are used to measure lung volumes and airflow for evidence of restrictive and obstructive lung disease. Measurements will be obtained by standard techniques following guidelines outlined by the American Thoracic Society. Spirometry, during screening, needs to be done pre- and post-bronchodilator. All subsequent spirometry is done post-bronchodilator. Diffusion capacity for carbon monoxide is always done post-bronchodilator. Spirometry will be performed to access the forced expiratory volume (FEV1) and forced vital capacity (FVC). Carbon monoxide diffusion capacity will be performed to assess gas exchange.
Time Frame
Baseline and 18 months
Secondary Outcome Measure Information:
Title
The effect of treatment with RTX/AZA relative to placebo on the changes over time in high-resolution CT scans of the chest.
Description
Non-contrast, low dose HRCT scans of the chest will be performed at the intervals and analyzed. Studies will be performed on high-end scanners (64 or above detector rows) capable of producing thin section (1-1.25mm) lung algorithm scans.
Time Frame
Baseline, six months, 12 months, 18 months, 24 months
Title
Correlate changes in pulmonary function (FVC, FEV1, DLco) with extent of pulmonary fibrosis obtained on open lung biopsy.
Description
Pulmonary Function Tests (PFTs) will measure forced vital capacity, forced expiratory volume in one second, diffusing capacity of the lungs for carbon monoxide (DLCO) (e.g., DLco will be measured by the single-breath technique using a 10-second breath hold). Histopathologic abnormalities of lung tissue will be determined by open lung biopsy.
Time Frame
24 months
Title
Correlate changes in pulmonary function (FVC, FEV1, DLco) with high-resolution CT scan scores over time in the two randomized groups of patients.
Description
Pulmonary Function Tests (PFTs) will measure forced vital capacity, forced expiratory volume in one second, diffusing capacity of the lungs for carbon monoxide (DLCO) (e.g., DLco will be measured by the single-breath technique using a 10-second breath hold). These will be compared with high-resolution CT scan scores.
Time Frame
24 months
Title
Changes in FVC and HRCT of the chest (maintained for 6 months after completion of therapy in both randomized groups)
Description
Pulmonary Function Tests (PFTs) will measure forced vital capacity, forced expiratory volume in one second, diffusing capacity of the lungs for carbon monoxide (DLCO) (e.g., DLco will be measured by the single-breath technique using a 10-second breath hold). High-resolution CT scans will be performed.
Time Frame
Baseline, six months, 12 months, 18 months and 24 months
Title
Incidence of lymphoma in patients treated with RTX/AZA or placebo over the time of enrollment in the study.
Description
Patients will be monitored with physical examinations, laboratory tests (CBC, auto diff, ALT, bilirubin, serum creatinine, platelets, comprehensive lymphocyte phenotype and cell sort).
Time Frame
24 months
Title
Changes in quality of life in the two randomized groups of patients as measured by SGRQ total score.
Description
Quality of life will be measured by the St. George's Respiratory Questionnaire (SGRQ), a pulmonary disease specific questionnaire measuring self-reported dyspnea symptoms and their relationship to activities of daily living and psychological functioning.
Time Frame
Baseline, six months, 12 months, 18 months and 24 months
Title
Changes in quality of life in the two randomized groups of patients as measured by Karnofsky Performance Status Scale (KPS).
Description
Karnofsky Performance Status Scale (KPS) will be performed at baseline, six months, 12 months, 18 months and 24 months.
Time Frame
Baseline, six months, 12 months, 18 months and 24 months
Title
Changes in quality of life in the two randomized groups of patients as measured by 6-minute Walking Test.
Description
The 6-minute Walking Test will performed at baseline, six months, 12 months, 18 months and 24 months.
Time Frame
Baseline, six months, 12 months, 18 months and 24 months
Title
Dysregulated molecular pathway determined by performing whole transcriptome sequencing.
Description
This will be done by performing whole transcriptome sequencing on GLILD, idiopathic pulmonary fibrosis (IPD), sarcoidosis and normal lung tissue.
Time Frame
24 Months
Title
Lung transcriptome predicts response to RTX/AZA therapy (performing whole transcriptome sequencing on GLILD, IPD, sarcoidosis and normal lung tissue) and confirm that lung transcriptome predicts response to RTX/AZA therapy.
Description
This will be done by performing whole transcriptome sequencing on GLILD, IPD, sarcoidosis and normal lung tissue.
Time Frame
24 Months
Title
Peripheral blood biomarkers as indicators of GLILD disease activity.
Description
The research team will examine blood specimens and evaluate Human Leukocyte Antigen - DR isotype (HLA-DR) negative and positive T cells.
Time Frame
24 Months
Title
Presence of bacterial (16S rRNA), fungal (Internal Transcribed Spacer region/ITS) and viral sequences (unbiased high-throughput sequencing) in the lungs of GLILD patients.
Description
This will be done by screening lung biopsies from patients with GLILD, idiopathic pulmonary fibrosis, pulmonary sarcoidosis, or no known pulmonary disease.
Time Frame
24 Months
Title
Prevalence and abundance of bacterial, fungal and viral sequences.
Description
This will be measured with quantitative PCR analysis of lung tissues obtained from patients with GLILD, idiopathic pulmonary fibrosis, pulmonary sarcoidosis or no known lung disease.
Time Frame
24 Months
10. Eligibility
Sex
All
Minimum Age & Unit of Time
18 Years
Accepts Healthy Volunteers
No
Eligibility Criteria
INCLUSION CRITERIA:
Age:
Patients must be 18 years of age or older.
Diagnosis:
Diagnosis of CVID in accordance with international criteria which includes: A) Serum immunoglobin G (IgG) at least 2 standard deviations below the age adjusted norm; B) Decreased serum immunoglobin A (IgA) and/or immunoglobin M (IgM); C) Age > 4 years.; D) Abnormal specific antibody production in response to immunization; E) Exclusion of secondary causes of hypogammaglobulinemia.
Diagnosis of GLILD based on histopathologic abnormalities of lung tissue obtained by open lung biopsy within 12 months of enrollment and confirmed by Pathology Core.
Performance Level:
Karnofsky Performance Status (KPS) ≥ 50%
Prior Therapy:
Patients must have fully recovered from the acute toxic effects of all prior therapy.
Systemic steroids need to be completed at least 60 days from the time of enrollment.
Organ Function:
Adequate Lung Function defined as:
• FVC > 60 % predicted and
• DLco > 35 % predicted
Adequate Bone Marrow Function defined as:
• Peripheral absolute neutrophil count (ANC) ≥ 750/mm3 and
• Platelet count ≥ 50,000/mm3
Adequate Hepatic Function as evidenced by:
Direct Bilirubin < 1.5 x upper limit of normal (ULN) for age
Serum glutamic pyruvic transaminase (SGPT) (ALT) < 135 U/L. For the purpose of this study, the ULN for SGPT is 45 U/L.
Adequate Renal Function as defined by a normal serum creatinine
Reproductive Function:
o Female patients of childbearing potential must have a negative urine or serum pregnancy test confirmed prior to enrollment.
Female patients with infants must agree not to breastfeed their infants while on this study.
Male and female patients of childbearing potential must agree to use an effective method of contraception approved by the investigator during the study.
Sexually active females of childbearing potential must agree to use adequate contraception (diaphragm, birth control pills, injections, intrauterine device (UD), surgical sterilization, subcutaneous implants, or abstinence, etc.) for the duration of treatment and for 6 months after the last dose of study therapy. Sexually active men must agree to use barrier contraceptive for the duration of treatment and for 6 months after the last dose of study therapy.
Regulatory Requirements
All patients must sign a written informed consent.
All institutional, FDA, and NIH requirements for human studies must be met.
EXCLUSION CRITERIA:
Infection:
Patients with uncontrolled infection are not eligible.
Patients with documented serious infection within 3 months of screening or opportunistic infection within 6 months of screening are not eligible.
Cardiac Function:
o Patients cannot be diagnosed with New York Heart Association (NYHA) Class III or IV congestive heart failure, ventricular arrhythmias, or uncontrolled hypertension.
Allergies:
o Known hypersensitivity to any of the components of RTX or AZA.
Current Therapy:
Systemic immunosuppressive medications including steroids.
Steroids can be used to prevent or to treat infusion-related RTX symptoms, but this should be used only prior to or immediately after the RTX infusion, and should not be continued beyond 3 days. The use of systemic steroids should be recorded.
Inhaled steroids are acceptable.
Previous Therapy:
o Previous treatment with RTX or AZA for GLILD.
Pregnant Females:
o Pregnant females will not be allowed to participate in this study.
Hepatic Disease:
o Known cirrhosis and/or portal hypertension.
Hepatitis B or Hepatitis C Infection:
All patients will be screened for Hepatitis B and C by polymerase chain reaction (PCR).
Hepatitis C positive as determined by PCR.
Hepatitis B Reactivation: Hepatitis B Reactivation is defined as Hepatitis B carrier patients with one of the following:
Positive hepatitis B e-antigen (HBe-Ag)
Quantitative hepatitis B Viral (HBV) DNA Load > 10^5 genomes/ml
Human Immunodeficiency Virus (HIV) 1 Positive:
o HIV 1 infection will be determined by PCR.
Homozygous Mutations:
o Patients with homozygous mutations of thiopurine methyltransferase (TPMT) will be excluded from the study.
Overall Study Officials:
First Name & Middle Initial & Last Name & Degree
John M Routes, MD
Organizational Affiliation
Medical College of Wisconsin
Official's Role
Principal Investigator
12. IPD Sharing Statement
Plan to Share IPD
No
Citations:
PubMed Identifier
30713498
Citation
Zdziarski P, Gamian A. Lymphoid Interstitial Pneumonia in Common Variable Immune Deficiency - Case Report With Disease Monitoring in Various Therapeutic Options: Pleiotropic Effects of Rituximab Regimens. Front Pharmacol. 2019 Jan 18;9:1559. doi: 10.3389/fphar.2018.01559. eCollection 2018.
Results Reference
derived
Learn more about this trial
Treatment of Granulomatous and Lymphocytic Interstitial Lung Disease in Patients With Common Variable Immunodeficiency
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