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Cyclosporine Vs Steroids in DRESS

Early Phase 1
Conditions
Drug-Induced Hypersensitivity Syndrome
DRESS Syndrome
Interventions
Registration Number
NCT04988256
Lead Sponsor
University of Southern California
Brief Summary

Current treatments for patients with drug reaction with eosinophilia and systemic symptoms (DRESS) include supportive care, steroids and cyclosporine. No randomized controlled trial (RCT) exists in comparing these treatments and all available literature comes in the form of case reports and case series. These two treatments are considered standard of care and this trial seeks only to compare outcomes of DRESS between these two therapies. No additional labs, therapies or procedures will be used apart from those that are routinely done for patients with this diagnosis.

This will be a pilot study to determine efficacy of the two therapies with particular endpoints in mind so that the investigators can study the safety of these two therapies in patients with DRESS.

Data suggests a potential benefit for adults with DRESS using either steroids or cyclosporine but the investigators are seeking a comparison of efficacy of these two therapies. The study population will include adults with a Registry of Severe Cutaneous Adverse Reaction (RegiSCAR) score of greater than 4 (i.e. a likely diagnosis of DRESS). The investigators will exclude patients with sepsis, active Hepatitis B or C, active tuberculosis, a documented allergy to steroids or cyclosporine, and patients with an estimated glomerular filtration rate (eGFR) \< 30 (unless on dialysis in which case the participants will be included).

Detailed Description

Current treatments for patients with drug reaction with eosinophilia and systemic symptoms (DRESS) include supportive care, steroids, cyclosporine and to a lesser extent, intravenous immunoglobulin (IVIG). Regarding IVIG, a recent case series suggests no improved benefit in adults. No randomized controlled trial (RCT) exists in comparing these treatments and all available literature comes in the form of case reports and case series. These two treatments are considered standard of care and this trial seeks only to compare outcomes of DRESS between these two therapies. No additional labs, therapies or procedures will be used apart from those that are routinely done for patients with this diagnosis.

This will be a pilot study to determine efficacy of the two therapies with particular endpoints in mind so that the investigators can study the safety of these two therapies in patients with DRESS. Hopefully, this study will allow the investigators to better power a full prospective trial in the future.

This is a potentially life-threatening severe cutaneous adverse reaction (SCAR) with significant potential morbidity. Data suggests a potential benefit for adults with DRESS using either steroids or cyclosporine but the investigators are seeking a comparison of efficacy of these two therapies. The study population will include adults with a Registry of Severe Cutaneous Adverse Reaction (RegiSCAR) score of greater than 4 (i.e. a likely diagnosis of DRESS). The investigators will exclude patients with sepsis, active Hepatitis B or C, active tuberculosis, a documented allergy to steroids or cyclosporine, and patients with an estimated glomerular filtration rate (eGFR) \< 30 (unless on dialysis in which case the participants will be included).

Participants will be randomized using a randomization protocol at all sites. Primary endpoints will include percentage of participants with complete or near complete resolution of organ involvement as well as erythema resolution at day 7 and day 30.

Secondary endpoints will be:

1. fever presence, resolution of facial edema, resolution of pruritus, lymphadenopathy, eosinophil count at days 7 and 30

2. days of hospitalization

3. mortality at days 7, 30 and 90

4. viral reactivation at days 30, 60 and 90

5. those with autoimmune development by day 30 and day 90

6. 30 day re-admission rate.

Recruitment & Eligibility

Status
ENROLLING_BY_INVITATION
Sex
All
Target Recruitment
50
Inclusion Criteria
  • Adults with a RegiSCAR score of greater than 4 (i.e a likely diagnosis of DRESS)
Exclusion Criteria
  • Active sepsis
  • Active hepatitis B or C
  • Active tuberculosis
  • Documented allergy to steroids or cyclosporine
  • Estimated glomerular filtration rate (eGFR) < 30 (unless on dialysis, in which case they will be included)

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
CorticosteroidsMethylprednisolone and PrednisoneAll Patients start with 500 mg IV Methylprednisolone for 3 days 1. If \>25% improvement (must be \>25% in all involved organs), start the taper regimen 2. If 0-25% improvement (in ≥1 involved internal organ), give 500 mg IV Methylprednisolone for 4 days 1. If no improvement, switch to cyclosporine arm of treatment 2. If 0-25% improvement, give 500 mg IV Methylprednisolone for 3 days i. If labs are down-trending, start the taper regimen ii. If labs are not down-trending, switch to cyclosporine arm of the study c. If \>25% improvement, start the taper regimen Taper Regimen set as: 1. 125 mg IV Methylprednisolone x3 days 2. 1.2 mg/kg PO prednisone x1 week 3. 1 mg/kg PO prednisone x1 week 4. 0.8 mg/kg PO prednisone x1 week 5. 0.6 mg/kg PO prednisone x1 week 6. 0.4 mg/kg PO prednisone x1 week 7. 0.2 mg/kg PO prednisone x1 week 8. 0.1 mg/kg PO prednisone x1 week 9. 0.05 mg/kg PO prednisone x1 week
CyclosporineCyclosporineAll Patients start with 5 mg/kg/day (3 mg/kg/day if renal impairment) PO divided bid for 7 days (or IV if patient is NPO) 1. If complete resolution, stop cyclosporine and monitor closely for relapse a. If patient relapses, give 5 (3 if renal impairment) mg/kg/day PO divided bid PO for 7 days i. If down-trending, start oral taper regimen ii. If not down-trending, switch to steroid arm 2. If \>25% improvement and labs are down-trending, start the oral taper regimen. 3. If 0-25% improvement, give 5 (3 if renal impairment) mg/kg/day PO divided bid for 3 days 1. If down-trending, start oral taper regimen 2. If not down-trending, switch to steroid arm 4. If no improvement or up-trending labs at 7 days, switch to steroid arm Oral Taper Regimen set as 3 mg/kg PO divided bid for 14 days, then 2 mg/kg PO divided bid for 20 days. If renal impairment, oral taper regimen set as 2 mg/kg PO divided bid for 14 days, then 1 mg/kg PO divided bid for 20 days
Primary Outcome Measures
NameTimeMethod
Percentage of patients with complete or near complete resolution of organ involvement at day 30, on steroid therapy and cyclosporine therapyDay 30

Measured by the following quantitative metrics:

* Creatinine within 1.25x of baseline or upper limit of normal, whichever is higher

* Aspartate Aminotransferase (AST) within 1.5x of baseline or upper limit of normal, whichever is higher

* Troponin-T, Creatine Kinase Myocardial Band (CK-MB), Pro B-type Natriuretic Peptide (Pro-BNP), and lipase within 1.25x of baseline or upper limit of normal, whichever is higher

* Resolution of interstitial pneumonitis on chest x-ray

Percentage of patients with complete or near complete resolution of erythema at day 7, on steroid therapy and cyclosporine therapyDay 7

Clinical measurement of erythema

Percentage of patients with complete or near complete resolution of erythema at day 30, on steroid therapy and cyclosporine therapyDay 30

Clinical measurement of erythema

Secondary Outcome Measures
NameTimeMethod
30-day readmission rateDay 30

Proportion of patients re-admitted to the hospital within 30 days of discharge

Percentage of patients with resolution of facial edemaDay 30

Clinical resolution of edema for at least 24 hours

Percentage of patients with resolution of lymphadenopathyDay 30

Clinical resolution of lymphadenopathy for at least 24 hours

Patients with autoimmune disease developmentDay 90

Measured by titers of autoimmune markers (Antinuclear Antibody (ANA), Thyroid Stimulating Hormone (TSH)) compared to baseline

Total days of hospitalization after initial dermatology consult0-120 days

Number of days

Percentage of patients with resolution of feverDay 30

Less than 38 degrees Celsius for at least 24 hours

Percentage of patients with resolution of pruritusDay 30

Resolution for at least 24 hours

Absolute eosinophil proportion compared to peak valueDay 30

Proportion of absolute eosinophils

MortalityDay 90

Proportion of patient mortality

Viral reactivationDay 90

Measured by titers of Human Herpesvirus 6 (HHV6), Cytomegalovirus (CMV), and Epstein-Barr Virus (EBV)

Trial Locations

Locations (1)

USC

🇺🇸

Los Angeles, California, United States

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