angioedema, idiopathic angioedema, idiopathic non-mast-cell angioedema, bradykinin-mediated angioedema recurrent swellings
Conditions
Interventions
Subjects with idiopathic non-mast cell-mediated angioedema will be evaluated for underlying OSA using validated questionnaires and polygraphy (WatchPAT One, Itamar Medical Ltd., CE certified). If OSA
in case of an attack, further specifications are asked via the digital application. 
Sponsors
Universitair Medisch Centrum Groningen
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: = 18 years old = 1 episode of angioedema in the last 3 months = 4 episodes of angioedema in the last year = 1 episode(s) of angioedema objectified by a physician - Angioedema attacks predominantly (>80%) during sleep or <2 hours after awakening - Insufficient response to treatment with high-dose antihistamines (4 tablets a day or the maximum tolerated dose in case of intolerance and/or renal insufficiency)) - Insufficient response to standard mast cell directed therapy**Consisting of treatment with high-dose H1-antihistamines (at least 4 tablets a day or the maximum tolerated dose in case of intolerance and/or renal insufficiency); at the discretion of the physician extended with other mast cell-directed therapies such rotation of antihistamines, short course(s) of steroids or anti-IgE therapy
Exclusion criteria
Exclusion criteria: = 1 episodes of spontaneous wheals (urticaria) in the last year - clear alternative explanation for all episodes of angioedema present (e.g. large local reaction due to insect bites) Current ACE-i usage or complete remission of angioedema episodes following cessation of ACE-I usage -confirmed (or suspected) genetic mutations associated with (hereditary) angioedema - patients already on continuous positive airway pressure (CPAP) or mandibular repositioning apparatus (MRA) treatment for underlying OSA - patients with severe chronic obstructive pulmonary disease (COPD GOLD IV) - absolute contra-indications for the use of CPAP/MRA
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Primary objective: To evaluate whether adequate OSA treatment can reduce angioedema attacks in a predefined subgroup of patients with idiopathic non-mast-cell angioedema and underlying OSA, in terms of: - Total number of attacks - Mean duration of attacks - Mean severity of attacks | — |
Secondary
| Measure | Time frame |
|---|---|
| 1. To improve the characterization and management of non- mast cell- angioedema (non-MC-AE) patients by collecting a rich multicenter dataset regarding clinical signs and symptoms of non-MC-AE as well as (routine/investigational) laboratory tests, genetic tests, (radiographic) images, treatment (effect and side-effects), disease activity, and quality of life 2. To assess readily available clinical characteristics to stratify non-MC-AE into clinically relevant phenotypes for future trials and patient-tailored treatment. 3. To gain further insight in the pathophysiology underlying the different phenotypes of idiopathic non-MC-AE patients. 4. To reduce the need for prophylactic and on demand treatment in nocturnal intermittent hypoxia induced angioedema5. To improve patient reported quality of life for all non-MC-AE patients. 6. To detected undiagnosed and thus untreated OSA and treat this group according to the general guidelines to lower the risk for OSA-related comorbidities. | — |
Countries
Netherlands
Contacts
Public ContactA.A.J.M. Ven
Universitair Medisch Centrum Groningen
Outcome results
None listed