1. to describe NVC findings in patients with pediatric (systemic) auto-immune/-inflammatory diseases:- Juvenile systemic/localized sclerosis (jSSc/lSc)- Childhood-onset systemic lupus erythematosus (cSLE)- Juvenile dermatomyositis (JDM)- Juvenile…
ID
Source
Brief title
Condition
- Autoimmune disorders
- Joint disorders
- Vascular disorders NEC
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
NVC measurements consist of describing the following variables per 1 mm image
(4 images per finger, digits 2-5 on both sides):
- Density: number of capillaries per mm (mean 9, range 6-12)
- Abnormal shapes described as not-hairpin/tortuous/crossing (1)
- Capillary apex maximum diameter (20-50 mcm described as enlarged, >50mcm
described as giant capillary)
- Large micro-hemorrhages (pathological bleedings with apical cap-like
appearance)
- Small multiple point-shaped hemorrhages surrounding the capillary loop
(extravasations)
Specific endpoint for study 3: diagnosis of a (systemic) autoimmune disease.
Secondary outcome
not applicable
Background summary
Nail fold video-capillaroscopy (NVC) is a non-invasive, easy-to-perform
magnification method to visualize the capillary bed of the fingertips. It is
used in adults with Raynaud*s phenomenon as a diagnostic tool for the presence
of vasculopathy which is correlated with the risk of development of systemic
sclerosis (SSc). Classification of NVC-findings in adults with SSc also seem to
correlate with severity of organ involvement. However, in children data on
capillaroscopy are scarce.
Study objective
1. to describe NVC findings in patients with pediatric (systemic)
auto-immune/-inflammatory diseases:
- Juvenile systemic/localized sclerosis (jSSc/lSc)
- Childhood-onset systemic lupus erythematosus (cSLE)
- Juvenile dermatomyositis (JDM)
- Juvenile idiopathic arthritis (JIA)
- Kawasaki disease (KD)
- Raynaud*s phenomenon (RP)
and compare them with NVC findings in healthy children (participants in the
sub-study *Nailfold capillaroscopy in pediatric rheumatic diseases and healthy
children*)
2. to establish classification criteria for abnormal NVC findings in patients
with cSLE
3. to assess if this classification of NVC findings in cSLE:
- is associated with disease activity
- is associated with the type of organ involvement and with future disease
activity/-organ involvement
- can be changed due to medication effects
4. to assess the risk for developing a systemic auto-immune disease in patients
with abnormal NVC findings and Raynaud*s phenomenon with a pediatric onset
Study design
Part 1. Observational cross-sectional international study in healthy children
and pediatric patients with (systemic) autoimmune/-inflammatory diseases: SSc,
lSc, SLE, JDM, RP, JIA, KD and RP
This sub-study is a part of the international multicenter cross-sectional study
*Nail fold capillaroscopy in pediatric rheumatic diseases and healthy
children*, Belgian registration number B670201627545)
Capillaroscopy: once
Part 2. Prospective international multicenter cohort study in children and
young adults with cSLE
Capillaroscopy: t=0, t=6 months and then yearly for 5 years (+optional 5 years)
Part 3. Prospective international multicenter cohort study in children and
young adults with RP
Capillaroscopy: t=0 and then yearly for 5 years (+optional 5 years)
Study burden and risks
NVC is a non-invasive, non-painful method to visualize the smallest blood
vessels in the nail fold area of the fingertips. It is already part of standard
clinical care in systemic autoimmune diseases for diagnostic purpose. The
patient is in a sitting position at a table and does not need to remove any
clothing. There is no risk and negligible burden for the participant.
A possible burden for some, namely cSLE- and RP-patients, consists of extra
blood sampling of 10-15 ml blood, which will only be taken if a venipuncture is
part of standard clinical care. If there is a request for blood sampling as
part of standard care, the 10-15 ml will be additional. This means no
additional venipuncture will be necessary and therefore minimally invasive.
Meibergdreef 9
Amsterdam 1105 AZ
NL
Meibergdreef 9
Amsterdam 1105 AZ
NL
Listed location countries
Age
Inclusion criteria
1. Healthy children, 6-18 years old2. Pediatric patients < 18 years old, with
one of the following diagnoses:
- Systemic lupus erythematosus (SLE), according to Systemic Lupus International
Collaborating Clinics (SLICC) criteria
- Systemic sclerosis (SSc), according to EULAR criteria
- lokalized scleroderma (lSc), differentiated from SSc by the absence of
sclerodactyly, RP, nailfold capillary changes, and organ involvement
- Juvenile dermatomyositis (JDM), according to Bohan and Peter criteria
- Juvenile idiopathic arthritis (JIA), according to the International League of
Associations for Rheumatology (ILAR) criteria
- Kawasaki disease (KD), according to the 5th revision of diagnostic criteria
- Raynaud's phenomenon (RP), diagnostic questions as described by Wigley3.
Adult patients >=18 years old, with a diagnosis childhood-onset SLE / RP,
diagnosed before the age of 18#*¶*l/+
Exclusion criteria
Lack of written informed consent
Design
Recruitment
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 | NL60885.018.17 |