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| Name | Class |
|---|---|
| Joseph Merola | UNKNOWN |
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This is an open-label, pilot study evaluating the efficacy of tildrakizumab on the treatment of bullous pemphigoid (BP) in eligible patients (see detailed study protocol). Three total doses of tildrakizumab 100mg will be administered at Weeks 0, 4, 16 a total of 16 weeks of treatment by the study staff to patients with bullous pemphigoid. The patients will be followed for a total of 24 weeks.
The investigators will recruit 16 patients with bullous pemphigoid 18 years of age and older with confirmed diagnosis of bullous pemphigoid on biopsy (both H&E and Direct Immunofluorescence (DIF) +/- IIF and ELISA) At the initial screening visit, demographic information will be obtained, inclusion and exclusion criteria, and informed consent obtained for those deemed eligible for enrollment. Three total doses of Tildrakizumab 100mg will be administered at Weeks 0, 4, 16 a total of 16 weeks of treatment by the study staff. Subjects will be evaluated for improvement in primary and secondary endpoints using clinical examination and questionnaires during initial and follow-up visits at screening, Weeks 0, 4, 16, and 24, a total of 24 week followup period.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Intervention | Experimental | Participants will receive Tildrakizumab 100mg at Weeks 0, 4, 16; three doses; a 16-week treatment course and 24-week followup |
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Tildrakizumab Prefilled Syringe | Drug | Patients will receive Tildrakizumab 100 mg subcutaneously at Weeks 0, 4, and 16. The study staff will administer each dose. |
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| Measure | Description | Time Frame |
|---|---|---|
| Change in Disease Severity | Disease severity category (mild: 1-9 lesion(s), moderate: 10-30 lesions, and severe: > 30 | Weeks 0, 4, 16, and 24 |
| Measure | Description | Time Frame |
|---|---|---|
| Change in Bullous Pemphigoid Disease Activity Index (BPDAI) | The BPDAI tool (9) computes 2 scores: total BPDAI activity and total BPDAI damage. The total BPDAI activity score is the arithmetic sum of the 3 subcomponents - cutaneous blisters/ erosions, cutaneous urticaria/erythema, and mucosal blisters/ erosions. The total BPDAI damage score is the arithmetic sum of the items rated regionally for damage caused by more permanent features such as post-inflammatory hyperpigmentation, scarring and other. BPDAI quantifies lesion number and size thresholds. Lesions are rated based on the regions affected. BPDAI gives additional weighting to areas of the skin primarily affected in BP, such as the limbs, and less emphasis to scalp and face, to better differentiate clinical response in BP. Scores can range from 0 to 360 for BPDAI total activity (maximum 240 for total skin activity and 120 for mucosal activity), and 0 to 12 for BPDAI damage, with higher scores indicating greater disease activity or damage. |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Liset Chacin | Contact | 617-264-5926 | lchacin@bwh.harvard.edu | |
| Alex Gionfriddo | Contact | agiofriddo@bwh.harvard.edu |
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| ID | Term |
|---|---|
| D010391 | Pemphigoid, Bullous |
| ID | Term |
|---|---|
| D012872 | Skin Diseases, Vesiculobullous |
| D012871 | Skin Diseases |
| D017437 | Skin and Connective Tissue Diseases |
| D001327 | Autoimmune Diseases |
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| Weeks 0, 4, 16, and 24 |
| Change in BPDAI-Pruritus | No evidence of itch (no excoriations) 0 Mild itch (isolated excoriation up to two body sites) 10 Moderate itch (excoriation on ≥ 3 body sites, impairment of daily activity 20 Severe itch (Generalized excoriation, sleep impairment) 30 | Weeks 0, 4, 16, and 24 |
| Change in Dermatology Life Quality Index (DLQI) | Each question is scored on a four-point Likert scale: Very much = 3 A lot = 2 A little = 1 Not at all = 0 Not relevant = 0 Question unanswered = 0 The DLQI is calculated by adding the score of each question, resulting in a maximum of 30 and a minimum of 0. The higher the score, the more quality of life is impaired. A score higher than 10 indicates that the patient's life is being severely affected by their skin disease. Meaning of scores 0-1 = no effect at all on patient's life 2-5 = small effect on patient's life 6-10 = moderate effect on patient's life 11-20 = very large effect on patient's life 21-30 = extremely large effect on patient's life | Weeks 0, 4, 16, and 24 |
| anti-BP180, BP230 IgG | Weeks 0, 4, 16, and 24 |
| Total IgE | Weeks 0, 4, 16, and 24 |
| Cytokine level | Weeks 0, 4, 16, and 24 |
| D007154 | Immune System Diseases |