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doi:10.1093/rheumatology/kem353
Concise Report
KEY
WORDS:
was 46 yrs and the range was 1871 yrs. The median duration of
symptoms was 47 months, range 2540 months. Only patients
with definite RP (defined by at least two colour changes, one of
them being white in response to cold or emotion), 18 yrs or older
and able to give consent were recruited.
Fourteen patients (2 males, 12 females) fulfilled the LeRoy and
Medsger [6] criteria for PRP. Thirty patients (4 males, 26 females)
were classified as SRP [6, 7]. Twelve patients had SSc, 10 patients
had the limited cutaneous and two had the diffuse cutaneous
subtype [8]. Thirteen patients had undifferentiated CTD (UCTD)
(2 males, 11 females). These patients had features that precluded
the diagnosis of PRP but did not have a specific CTD. All patients
with UCTD were antinuclear antibody (ANA)-positive (positivity
was defined by an IgG titre of >1/100). Nailfold capillaroscopy
was done in 9 of these 13 patients and three had abnormal
findings. One patient had mixed CTD (MCTD) and was positive
for UI-RNP antibody, three patients (all female) had primary
Sjogrens syndrome (one patient was positive for anti-Ro/La
antibodies) and one patient had cold agglutinin disease.
Twelve patients (27%) were on vasoactive drugs such as
calcium channel blockers and angiotensin-converting enzyme
inhibitors. Of the 12 patients who were on vasoactive drugs, 10
patients (83%) fulfilled the criteria for SRP, and two patients
(17%) the criteria for PRP. Twenty-four patients (55%) had a
history of smoking and 13 patients (30%) were current smokers.
Introduction
In RP, classically the digits turn white (vasospasm), then blue
(deoxygenation of static venous blood), then red (reperfusion) in
response to precipitating factors such as cold exposure or
emotional stress [1, 2]. RP can be divided into primary and
secondary depending on absence or presence of an underlying
disease/disorder, respectively. For example, connective tissue
diseases (CTDs) such as SSc [3].
There are many methods that have been developed over the
years to quantify digital vascular involvement in RP. These
include nailfold capillaroscopy, laser Doppler flowmetry, thermography and finger systolic blood pressure [4, 5]. It has been
observed using these techniques that digital involvement is not
uniform and anecdotally, many patients when specifically asked
report that the thumb is spared. Yet, to our knowledge there are
no previous studies that have looked into thumb involvement. If
the thumb is not involved, this raises the question as to why this is
the case. The other question is whether thumb involvement is of
prognostic value, i.e. do those patients who have RP affecting
thumbs have more severe disease than those without? The
difference in the degree of thumb involvement between primary
Raynauds phenomenon (PRP) and secondary Raynauds phenomenon (SRP) has not been previously investigated. This could
be clinically important and might assist in clinical classification. If
there is a difference in thumb involvement between PRP and SRP
this could imply different pathophysiologies.
Methods
Patients
Forty-four patients (6 males, 38 females) were recruited at Hope
Hospital between November 2006 and April 2007. The median age
1
The Royal Liverpool University Hospital, Liverpool, 2Rheumatic Diseases
Centre and 3Biostatistics Group, University of Manchester, Hope Hospital,
Salford, UK.
219
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Objectives. To conduct a prospective study to determine which digits are affected (and whether the thumb is spared or not) in a cohort of
patients with RP as assessed by symptoms and thermography and to determine whether the degree of thumb involvement differs between
primary (PRP) and secondary Raynauds phenomenon (SRP).
Methods. This was a cross-sectional study of 44 patients with RP. The following characteristics were recorded to allow comparisons between
digits: symptoms of RP in each digit (graded on a scale of never, sometimes and always affected during an attack of RP) and
thermography at 238C. A distaldorsal difference (DDD) in temperature at 238C of 18C or less was considered to be clinically relevant.
Results. Symptom scores in the thumb were significantly better, i.e. less severe than in each finger (P < 0.001). As only three participants
had any finger better than the thumb, there was no power to compare whether the thumb was spared more in PRP compared with SRP.
Mean DDD was significantly higher (i.e. better) in the thumb compared with each finger (P < 0.001). Although DDD scores were higher in PRP
compared with SRP (P 0.01), there was no evidence that the relative effect of the thumb differed between the two groups (P 0.26).
Conclusions. Our findings confirm that the thumbs are spared in RP, both primary and secondary, as demonstrated by both symptoms and
thermography. The reasons for sparing of the thumb were not addressed in this study but raised questions regarding pathophysiolgy.
B. Chikura et al.
220
right thumb was never, sometimes involved was considered for analysis.
The study was approved by the Warrington, Wigan and Leigh
Research Ethics Committee and all subjects gave informed
consent.
Never
Always
Total, n (%)
0
7
15
22 (50)
0
0
4
4 (9)
1 (2)
13 (30)
30 (68)
44
<0.001
0
9
13
22 (50)
0
0
4
4 (9)
0 (0)
13 (30)
31 (70)
44
<0.001
0
11
11
22 (50)
0
2
2
4 (9)
1 (2)
19 (43)
24 (55)
44
<0.001
1
13
8
22 (50)
0
2
2
4 (9)
3 (7)
23 (52)
18 (41)
44
<0.001
This table shows comparison of reporting of symptoms of always, sometimes and never
between the thumbs and other digits. The worst score from each pair of digits was considered for
analysis. The percentages of the totals for symptom frequencies are shown to allow comparison
between digits. The P-values obtained using the McNemars tests for each comparison are
shown.
TABLE 2. DDD in the different digits showing (i) numbers of digits with a clinically
relevant temperature gradient, i.e. DDD of 18C or less and (ii) the mean DDD for
each digit for the whole patient cohort and for the subgroups with PRP and SRP.
Thermography
Proportions of different digits with a DDD of 18C or
less. The proportions of thumbs with clinically relevant DDD,
i.e. a DDD of 18C or less, showed that the thumb was the least
affected digit; 18% of thumbs compared with 64% in the index
finger had clinically relevant DDD (Table 2).
McNemars
P-value
Sometimes
DDD of 18C
or less at 238C
(All patients), n (%)
Thumb
Index
Middle
Ring
Little
8
28
24
25
23
(18)
(64)
(55)
(57)
(52)
PRP
(N 14)
SRP
(N 30)
0.09
0.82
1.02
0.98
1.19
0.63
0.52
0.11
0.03
0.49
0.43
1.44
1.54
1.42
1.50
ANOVA
P-value
(vs thumb)
<0.001
<0.001
<0.001
<0.001
DDD, distal dorsal difference. Values are the worse score from each pair of digits.
Discussion
This is the first study to look specifically at thumb sparing in
RP. Our findings show that the thumb is spared as reported
by patients. Symptoms were comparable in all digits with
the exception of the thumb, which was significantly less frequently
involved. This was confirmed by thermography. The mean
DDD of the thumbs was higher (i.e. their tips were warmer)
compared with other digits, and the proportion of thumbs with
a DDD of 18C or less at 238C was lower than that for the
other digits.
The degree of thumb sparing was more pronounced in the SRP
group compared with PRP group (Table 2). Although DDD
scores were higher (better) in PRP compared with SRP, there was
no evidence that the relative effect of the thumb differed between
the PRP and SRP. If the thumb is more involved in SRP as
compared with PRP, this observation raises the question as to
whether thumb involvement is of prognostic value, i.e. do those
patients who have RP affecting the thumb have more severe
disease than those without? The question as to whether thumb
involvement differs between PRP and SRP is clinically relevant to
a rheumatologist in that this may help distinguish the two
conditions and imply different pathophysiologies. Vascular
problems in PRP are mainly functional; in contrast, structural
vascular abnormalities can be seen in patients with SRP,
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