- To study the clinical characteristics and disease course of patients with LEMS with or without SCLC- To identify the characteristics of the humoral or cellular immune response that are associated with the prolonged survival of patients with SCLC…
ID
Source
Brief title
Condition
- Autoimmune disorders
- Neuromuscular disorders
- Respiratory tract neoplasms
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
Description of epidemiology, clinical characteristics and disease course in
LEMS patients.
Comparison of the humoral and cellular immune response in LEMS patients and
SCLC patients with and without LEMS.
Secondary outcome
- Tumour size on lung imaging studies and growth rate upon follow-up.
- DELTA-P score tumor prediction score in LEMS patients, diagnosis of SCLC upon
follow-up
- Presence of other auto-antibodies possibly related to LEMS
- Tumour infiltration of SCLC by lymphocytes and macrophages (+- LEMS)
- Anti-tumour effect of serum VGCC antibodies and related macrophage and
adaptive response in vitro
- Presence of specific T cells against tumour-related antigens
- Patients' experience with symptomatic medication for LEMS
Background summary
A detailed description of clinical characteristics and disease course of
patients with Lambert-Eaton myasthenic syndrome (LEMS) will enable earlier
diagnosis and treatment of this rare, disabling, but well-treatable,
neuromuscular disorder and the associated tumour This study will provide
detailed knowledge about the immune response against voltage-gated calcium
channels and the small cell lung cancer (SCLC) in patients with LEMS. This is
essential for the development of future therapeutical interventions or
immune-mediated therapies.
Study objective
- To study the clinical characteristics and disease course of patients with
LEMS with or without SCLC
- To identify the characteristics of the humoral or cellular immune response
that are associated with the prolonged survival of patients with SCLC and LEMS.
Study design
Single center prospective study
Study burden and risks
Patient burden and risk in this study will be minimal and mostly almost
completely limited to the period shortly after inclusion. If a patient
participates in all elements of the study this will include:
- detailed history and physical examination
- electromyogram to determine the severity of the LEMS
- blood donation. We will draw 90 mL at inclusion and up to 50 mL at follow-up
for selected patients with SCLC.
Personal benefit would be the contact with the clinical researchers experienced
in the treatment of this rare disorder, with the opportunity to be completely
informed on all aspects of the disease and get detailed therapeutical advice.
At a group level the results will contribute to improved understanding of the
pathogenesis, and possibly improved therapies.
Albinusdreef 2
Leiden 2333 ZA
NL
Albinusdreef 2
Leiden 2333 ZA
NL
Listed location countries
Age
Inclusion criteria
Diagnosis of LEMS
Criterion 1 and 2 or 1 and 3 have to be fulfilled
1. Clinical features consistent with LEMS (proximal muscle weakness, reduced tendon reflexes, autonomic symptoms)
2. Abnormalities upon repetitive nerve stimulation (decrement of CMAP amplitude and/or increment after voluntary contraction or high frequency stimulation)
3. Presence of VGCC antibodies;Diagnosis of SCLC
Definitive diagnosis of SCLC based on pathology results (either cytology or histology).
Exclusion criteria
* Age < 18 years
* Unable to give informed consent
Design
Recruitment
metc-ldd@lumc.nl
metc-ldd@lumc.nl
metc-ldd@lumc.nl
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL44216.058.13 |