lymphangioleiomyomatosis
Conditions
Interventions
Intervention type:DRUG
Name of intervention:Srolimus
Dose form / Japanese Medical Device Nomenclature:TABLET
Route of administration / Site of application:ORAL
Dose per administration:2
2mg
mg
Dosing
Sponsors
Koh Nakata, MD, PhD
Yoshikazu Inoue
Kuniaki Seyama
Ryushi Tazawa
Toshinori Takada
Eligibility
Sex/Gender
Female
Inclusion criteria
Inclusion criteria: a. written informed consent b. diagnosis of LAM by chest HRCT etc.
Exclusion criteria
Exclusion criteria: a. history of myocardial infarction, angina or stroke b. pregnant, breastfeeding or plans to become pregnant c. unable or unwilling to use adequate contraception d. significant hematologic or hepatic abnormality e. intercurrent infection or HBV career f. any surgery within 8 weeks of initiation of study drug g. uncontrolled hyperlipidemia h. previous lung transplant or currently on lung transplant list etc.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| All adverse events | — |
Secondary
| Measure | Time frame |
|---|---|
| 1) QOL tests 2) change of pulmonary function test (FEV1, FVC) 3) serum VEGF-D concentration 4) pharmacokinetics of sirolimus in 10 patients and sequential trough concentration of sirolimus in all patients 5) change of bone mineral dencity 6) change of plasma estrogen, progesterone and testosterone 7) comparison of trough concentration between 1mg and 2mg administration in same patients | — |
Countries
Japan
Contacts
Public ContactShigeru Imai
Sogo Rinsho Medefi Co. Ltd.
Outcome results
None listed