Inclusion Criteria:
1. Participant and/or legally authorized representative must be able to understand and
provide informed consent prior to study procedures being performed.
2. Willing and able to comply with study visits and activities. 3. Age ≥ 18 to ≤ 70 years at study enrollment. 4. Histologically active eosinophilic esophagitis (EoE) at time of screening or within 12
weeks prior to enrollment, with a peak count of ≥ 15 eosinophils (eos)/high powered
field (hpf) in any region of the esophagus, with no other known cause for esophageal
eosinophilia; involvement of eosinophilic inflammation in other gastrointestinal
segments will be allowed but not required or sufficient.
5. History of moderate to severe symptoms of abdominal/chest pain or dysphagia an average
of ≥ 2 episodes per week during the 2 weeks prior to screening.
6. History of approximately 8 week standard of care (SOC) treatment (e.g., proton pump
inhibitors (PPI's), topical corticosteroids) that did not adequately control or treat
the EoE or documentation that such treatment was not tolerated. Participant may
re-screen if this is not met.
7. Stable medical management of EoE (and other eosinophilic disorders, if applicable)
including stable dosage of medications in the 8 weeks prior to study enrollment, if
applicable. Participants may be on baseline anti-EoE therapy (such as elimination
diet, elemental diet, proton pump inhibitors (PPI), topical or systemic
glucocorticoids (≤10 milligrams (mg) daily), immunosuppressive agents, cromolyn, and
H1 and H2 anti-histamines) as long as there is agreement not to change their dosage.
8. Willing to maintain current dietary regimen throughout the course of the study. Diet
must have been stable for 8 weeks prior to baseline endoscopy.
Exclusion Criteria:
1. Inability or unwillingness of a participant to give written informed consent or comply
with study protocol.
2. Current active H. pylori infection. A history of H. pylori infection needs to have
medical documentation of one of the three acceptable eradication tests: antigen,
breath or histology. Participants with current active H. pylori infections can be
re-screened for study participation in the future if they are treated and have medical
documentation of one of the three acceptable eradication tests: antigen, breath or
histology.
3. Another disorder that causes esophageal eosinophilia (e.g., hypereosinophilic
syndrome*, Churg Strauss vasculitis, eosinophilic granuloma or a parasitic infection).
*Hypereosinophilic syndrome defined by multiple organ involvement (with the exception
of atopic disease or eosinophilic gastrointestinal disorder (EGID)) and persistent
blood absolute eosinophil count ≥1500/microliter.
4. Systemic gastrointestinal disorders such as Crohn's disease, inflammatory bowel
disease, or Celiac disease not including chronic gastritis, chronic duodenitis,
mucosal eosinophilia or other EGID's.
5. Diagnosed with Chronic Obstructive Pulmonary Disease (COPD).
6. Known immunoglobulin A (IgA) deficiency (i.e., IgA level < 8 mg/dL at screening).
7. Current coronavirus disease of 2019 (COVID-19) infection (i.e., detection of the
presence of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) before
the screening endoscopy using testing done at the clinical site).
8. Hematological disorders that prevent the blood from clotting (e.g., hemophilia, Von
Willebrand disease, clotting factor deficiencies).
9. Currently on anti-coagulation medications (except aspirin/Non-steroidal
anti-inflammatory drugs).
10. Known history of hypersensitivity following infusions of human blood or blood
components (e.g., human immunoglobulins or human albumin).
11. Known history of hypersensitivity or anaphylaxis to Zemaira or other A1AT products.
12. Uncontrolled, or poorly controlled, comorbid conditions including, but not limited to,
cardiovascular diseases, hypertension and diabetes as defined by the following
criteria:
- - A myocardial infarction within the last 6 months.
- - Blood pressure > 179/99 mmHg.
- - Diabetics with a hemoglobin A1C (HbA1C) > 7% and that are deemed to have
uncontrolled diabetes as defined by the physician.
13. History of cancer: Individuals who have had basal cell carcinoma, localized squamous
cell carcinoma of the skin, or in situ carcinoma of the cervix are eligible provided
that the participant is in remission and curative therapy was completed at least 12
months prior to the date informed consent was obtained. Individuals who have had other
malignancies are eligible provided that the individual is in remission and curative
therapy was completed at least 5 years prior to the date informed consent was
obtained.
14. Current or expected treatment with sublingual immunotherapy (SLIT) or oral
immunotherapy (OIT).
15. Current or recent use of any investigational drug (within 3 months or 5 half-lives,
whichever is longer, prior to screening).
16. Currently participating or planning to participate in another clinical study involving
an investigational drug during the course of this study.
17. Presence of steroid-responsive diseases (e.g., asthma) with a recent (within the last
6 months) history of disease exacerbations requiring steroid treatment.
18. Use of systemic corticosteroids within 3 months prior to screening, with the exception
of the use of systemic steroids < 10 mg or a steroid burst ≤3 days (see Exclusion
Criterion #12).
19. Current use of systemic steroids with daily dose > 10 mg for any reason or steroid
burst for > 3 days within 1 month of screening.
20. Current pregnancy or breastfeeding.
21. Any esophageal stricture unable to be passed with a standard, diagnostic upper
endoscope.
22. Esophageal varices or interventional treatment for esophageal varices that, in the
opinion of the investigator, would put the participant at undue risk for significant
complications from an endoscopy procedure.
23. History of alcohol or drug abuse within 6 months prior to screening.
24. Participant or his/her immediate family is a member of the investigational team.
25. Presence of clinically significant laboratory abnormalities at the screening that
meets one or more of the following criteria:
1. Serum alanine aminotransferase (ALT) ≥ 3 times upper limit of normal (ULN)
2. Serum total bilirubin ≥2 times ULN. 3. Absolute neutrophil count (ANC) ≤ 1000 cells/mm3. 4. Hemoglobin (Hgb) ≤ 10.0 g/dL. 5. Platelet count ≤ 100,000/mm3. 6. Glomerular Filtration Rate (GFR) ≤ 44 mL/min/1.73 m2. 26. Planned or anticipated major surgical procedure during the study.
27. Known or suspected immunodeficiency disorder, including human immunodeficiency
disorder (HIV) or a history of invasive opportunistic infections (e.g., tuberculosis,
non-tuberculous mycobacterial infections, histoplasmosis, listeriosis,
coccidioidomycosis, pneumocystosis, aspergillosis, or an infectious etiology of
esophagitis (e.g. cytomegalovirus or Candida) despite infection resolution or
otherwise recurrent infections of abnormal frequency or prolonged infections
suggesting an immune-compromised status as judged by the investigator.
28. Planned or anticipated use of any prohibited medications and procedures during the
study.
29. Currently on Zemaira for other indications.
30. Initiation, discontinuation or change in the dosage regimen of the following
medications within 8 weeks prior to the baseline endoscopy:
Proton pump inhibitors Leukotriene inhibitors Nasal and/or inhaled corticosteroids
Participants on a stable dose of these medications for at least 8 weeks prior to the
baseline endoscopy may be included in the study. PPI and leukotriene inhibitor dosing
must not change during the study, but nasal and/or inhaled corticosteroids can be
increased if there is a deterioration in the condition for which the medications are
prescribed.
31. Presence of the following esophageal disorders: achalasia cardia, Barrett's esophagus
or precancerous lesions noted on the endoscopy.
32. Women of childbearing potential, or male participants with female partners of
childbearing potential, who are unwilling to practice highly effective contraception
prior to the initial dose/start of the first treatment, during the study, and for at
least 12 weeks after the last dose. Highly effective contraceptive measures include:
1. Stable use of combined (estrogen and progestogen containing) hormonal
contraception (oral, intravaginal, transdermal) or progestogen-only hormonal
contraception (oral, injectable, implantable) for one month prior to screening. 2. Intrauterine device; intrauterine hormone-releasing system. 3. Bilateral tubal ligation. 4. Vasectomized partner. 5. And/or sexual abstinence. 33. Past or current medical problems or findings from physical examination or laboratory
testing that are not listed above, which, in the opinion of the investigator, may pose
additional risks from participation in the study, may interfere with the participant's
ability to comply with study requirements or that may impact the quality or
interpretation of the data obtained from the study.
