Connective Tissue Disease, Interstitial Lung Disease
Conditions
Keywords
Interstitial lung disease, Connective tissue disease, Scleroderma, Rheumatoid arthritis, Sjogren's syndrome, Mixed connective tissue disease, Dermatomyositis, Polymyositis, Systemic lupus erythematosis
Brief summary
The first objective of this protocol is to assess the tolerability and safety of N-acetylcysteine (NAC) in patients with connective tissue disease related interstitial lung disease (CTD-ILD).
Detailed description
The investigators will assess tolerability and safety at scheduled intervals via standard of care physical exam findings, monitoring complete blood count, serum chemistry, and pulmonary function tests, along with patient reports of medication intolerance and change in symptoms of dyspnea.
Interventions
DRUGN-Acetylcysteine
600mg by mouth, three times daily for 12 months
Sponsors
University of Michigan
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
Inclusion: 1. Subject gives voluntary written informed consent to participate in the study. 2. Subject has been diagnosed with a connective tissue disease and associated interstitial lung disease confirmed by computed tomography of the lungs along with symptoms of cough and/or shortness of breath. 3. Males and females age greater than 18 years at time of screening. 4. Females of childbearing potential must be willing to use a reliable form of medically acceptable contraception and have a negative pregnancy test confirmed at baseline before starting NAC as per standard of care. Women who are surgically sterile or have been post-menopausal for at least two years are not considered to be of child-bearing potential. Exclusion: 1. History of severe chronic kidney disease defined as a glomerular filtration rate of less than 30. 2. Hemoglobin concentration less than 70% of the lower limit of the normal range at time of screening. 3. Moderate to severe hepatic impairment, i.e., Child-Pugh Class B or C.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Pulmonary Function Tests | Every 3 months | Not recorded. Study terminated due to departure of PI. |
Countries
United States
Participant flow
Recruitment details
5 subjects consented but did not participate in study due to departure of PI from institution.
Pre-assignment details
5 subjects consented to participate due to the PI departed the institution. Numbers in patient flow were updated to reflect this.
Participants by arm
| Arm | Count |
|---|---|
| N-Acetylcysteine This is an open label trial, all patient will be entered into one treatment arm.
N-Acetylcysteine: 600mg by mouth, three times daily for 12 months | 5 |
| Total | 5 |
Baseline characteristics
| Characteristic | N-Acetylcysteine |
|---|---|
| Age, Categorical <=18 years | 0 Participants |
| Age, Categorical >=65 years | 0 Participants |
| Age, Categorical Between 18 and 65 years | 5 Participants |
| Age, Continuous | 50 years STANDARD_DEVIATION 5 |
| Region of Enrollment United States | 5 participants |
| Sex: Female, Male Female | 4 Participants |
| Sex: Female, Male Male | 1 Participants |
Adverse events
| Event type | EG000 affected / at risk |
|---|---|
| deaths Total, all-cause mortality | — / — |
| other Total, other adverse events | 0 / 5 |
| serious Total, serious adverse events | 3 / 5 |
Outcome results
Primary
Pulmonary Function Tests
Not recorded. Study terminated due to departure of PI.
Time frame: Every 3 months
| Arm | Measure | Value |
|---|---|---|
| N-Acetylcysteine | Pulmonary Function Tests | 0 |