ARTHRITIS & RHEUMATISM
Vol. 39, No. 1, January 1996, pp 73-80
Q 1956, American College of Rheumatology
DISTAL EXTREMITY SWELLING WITH
PITTING EDEMA IN POLYMYALGIA RHEUMATICA
Report of Nineteen Cases
CARL0 SALVARANI, SHERINE GABRIEL, and GENE G. HUNDER
Objective. To determine the frequency and clini-
cal characteristics of ditruse distal extremity swelling
with pitting edema occurring in polymyalgia rheumatica
(PMR).
Methods. Clinical features and laboratory find-
ings were recorded for all 245 residents of Ohsted
County, Minnesota who developed PMR over a 22-year
period (1970-1991). Those who exhibited 2 1 episode of
diffuse distal extremity edema with pitting were selected
for this study, and were evaluated further.
Results. Thirteen women and 6 men in this
incidence cohort of PMR had 2 1 episode of distal
extremity swelling with pitting edema. Giant cell arteri-
tis was also identified in 5 patients. In 11 patients, the
swelling and edema developed concurrently with proxi-
mal PMR symptoms. In 2 patients, the distal swelling
was the initial manifestation, and in 6 patients, the distal
symptoms developed during relapses or recurrences of
PMR. Both upper and lower extremities were affected,
usually in a symmetric manner. Other peripheral man-
ifestations were also common. The distal swelling and
pitting edema responded promptly to corticosteroids,
and slowly or incompletely to nonsteroidal anti-
inflammatory drugs; a similar response was observed in
the proximal symptoms. The distal swelling appeared
to represent tenosynovitis and synovitis of regional
structures.
Conclusion. Distal extremity swelling with pitting
edema represents a manifestation of PMR that has not
been well described in previous studies. Awareness of
Carlo Salvarani, MD, Sherine Gabriel, MD, MPH, Gene G.
Hunder, MD: Mayo Clinic and Mayo Foundation, Rochester, Min-
nesota. Dr. Salvarani's current address is Arcispedale S. Maria
Nuova, Reggio Emilia, Italy.
Address reprint requests to Gene G. Hunder, MD, Mayo
Clinic, 200 First Street Southwest, Rochester, MN 55905.
Submitted for publication May 10, 1995;accepted in revised
form August I , 1995.
73
this finding will help facilitate the proper diagnosis and
institution of appropriate therapy for this disease.
Polymyalgia rheumatica (PMR) is a clinical
syndrome that occurs in persons 250 years old ( 1 4 ) .
It is characterized by aching and morning stiffness in
the proximal portions of the body, and by evidence of
a systemic reaction, usually indicated by an elevated
erythrocyte sedimentation rate (ESR). The cause of
the musculoskeletal pain is not completely under-
stood, but inflammation in proximal joints and pen-
articular structures is a likely basis for much of the
discomfort.
In addition to axial discomfort in PMR, muscu-
loskeletal symptoms can commonly occur distal to the
elbows and knees ( I ,4-7). Such distal extremity symp-
toms tend to be less severe and more variable than
proximal pains. These distal symptoms have been less
well characterized, but include aching and stiffness
similar to proximal pains, synovitis in 2 1 joint, teno-
synovitis, and carpal tunnel syndrome. We have oc-
casionally noted patients with PMR who have diffuse
swelling in the distal extremities with pitting edema. In
the present report, we focus on this latter finding
associated with PMR, and have determined the fre-
quency of distal extremity swelling with pitting edema
and related clinical features in a population-based
cohort of patients with PMR.
PATIENTS AND METHODS
We recently reported incidence rates of PMR in
Olmsted County, Minnesota, over a 22-year period (1970-
1991) (8). We identified 245 residents of Olmsted County (173
women and 72 men) who met diagnostic criteria for PMR.
These criteria were the following: 1) age 250 years; 2)
bilateral aching and morning stiffness (lasting %30minutes)
persisting for at least 1 month and involving 2 of the
74 SALVARANI ET AL

following areas: neck or torso, shoulders or proximal regions Table 1. Clinical features at diagnosis of polymyalgia rheumatica,
of the arms, and hips or proximal aspects of the thighs; 3) in 19 patients with distal extremity pitting edema, compared with
ESR (Westergren) elevated to 240 mdhour; and 4) the total cohort
absence of another disease that would explain the findings. Patients with Total
Information about the ascertainment of these cases of PMR edema population
in Olmsted County residents over the period of study was Feature (n = 19) (n = 245)
outlined in previous reports (1,8). We used a standardized
data collection form to record information on each patient’s Age, years, median (range) 75 (54-86) 74 (5 1-95)
age, sex, location of the aching and morning stiffness, joint Sex. F/M 13/6 173/72
abnormalities, the presence of systemic manifestations, on- Aching and stiffness, %
Shoulders/arms 95 94
set and duration of disease, the presence of giant cell arteritis Hips/thighs 74 78
(GCA), results of temporal artery biopsy if performed, type Nec Wtorso 42 62
of treatment used, along with dosage and duration, and the Giant cell arteritis, no. 5 39
development of relapses or recurrences. Laboratory and Hemoglobin, g d d l , median 12.3 (8.9-14.4) 12.4 (8.1-15.9)
radiologic data were recorded as available. Relapse was (range)
defined as an increase of articular symptoms or signs of PMR Erythrocyte sedimentation 73 (42-120) 69 (40-131)
that occurred < 1 month after reduction or discontinuation of rate, mm/hour, median
therapy, as well as regression of these symptoms or signs (range)
when the dosage of medication was increased or therapy was
resumed. Recurrence was defined as the return of musculo-
skeletal symptoms of PMR >1 month after the discontinua-
tion of therapy. The end of the disease was the date of symptoms of PMR gradually developed over the fol-
permanent discontinuation of therapy without a relapse or lowing months. In 11 patients, the swelling with pitting
recurrence. The end point of patient followup was the date of
edema and the symptoms of PMR developed concur-
the last clinic visit or the date of death.
Using this population incidence cohort, we collected rently. In 2 patients (patients 10 and 14), this manifes-
data for the present study on patients who had episodes of tation was noted during a relapse of PMR, and in 4
distal extremity swelling. We recorded information on the patients, the episode occurred at a time of a recurrence
presence of swelling of the joints and periarticular struc- of PMR after the initial course of therapy had been
tures, synovitis, tenosynovitis, and diffuse swelling with and stopped (Table 2).
without pitting edema. For patients with diffuse distal ex-
tremity swelling with pitting edema, we recorded its loca- In those patients with single episodes, 6 epi-
tion, duration, response to therapy, and any recurrences. sodes involved the upper extremities, 8 episodes the
lower extremities, and, in 4 episodes, both upper and
lower extremities were involved. In patient 18, who
RESULTS
had multiple episodes, both the upper and lower
Nineteen of the 245 patients in the population extremities were affected. In 5 patients, 1 extremity
cohort with PMR (8%) manifested 2 1 episode of was involved, and in 14 patients, multiple extremities
diffuse distal extremity swelling with pitting edema were involved. Fourteen episodes involved upper or
during the course of the illness. The clinical features of lower extremities in a symmetric manner.
these 19 patients compared with the overall group of The episodes of soft tissue swelling with pitting
245 are shown in Table 1. Although some differences edema developed either subacutely or gradually over
between the groups were present, none were statisti- several days or longer. The area involved was reported
cally significant. Five of the patients (patients 1, 2, 13, to be painful, and the pain was made worse by moving
16, and 19) also had biopsy-proven GCA. the joints in the affected area. The tissues affected
Characteristics of the swelling with pitting were usually tender to palpation and sometimes warm,
edema. Table 2 lists the 19 patients and describes the but erythema was not prominent. The swelling and
site of the swelling and pitting edema, the relationship pitting edema usually occurred at the time PMR de-
to the stage of the disease, and the influence of therapy veloped, relapsed, or recurred, indicating a connection
on the swelling and edema. In 18 patients, 1 episode of with disease activity. In some cases, the areas of
swelling with edema of the distal extremity occurred. swelling and edema conformed to underlying joints or
In the other patient (patient 18), 3 episodes occurred. tendons, such as the posterior tibial tendon in patient
In 2 patients (patients 4 and 18), the distal swelling 5 and Achilles tendons in patient 14. However, in most
with pitting edema was the initial manifestation. These instances, the swelling was more diffuse and articular
2 patients were treated with nonsteroidal antiinflam- in location, but it extended beyond specific joint
matory drugs (NSAIDs) and the swelling persisted as margins or other structures. The swelling and pitting
DISTAL EXTREMITY SWELLING WITH PITTING EDEMA IN PMR 75

Table 2. Characteristics of 19 patients with polymyalgia rheumatica and distal extremity pitting edema*
Response to therapy
Sexlage
Patient (years) Site of swelling with edema Stage of disease NSAID Corticosteroids
1 FI84 Hands and wrists Onset 0 +
2 Fl76 Hands and wrists Recurrence 7 years after onset 0 +
3 Fl69 Hands, wrists, ankles, feet Onset 0 +
4 Fl82 Left ankle and foot, medial calf 3 months prior to onset 0 +
5 Fl54 Left ankle, especially along Recurrence 3% years after onset 0 +
course of posterior tibia1
tendon
6 Fl70 Feet and pretibial areas Onset + -
7 Fl75 Right foot Recurrence 1 l/2 years after onset + -
8 FI80 Left hand and wrist Onset - +
9 MI73 Left hand and wrist Onset 0 t
10 M/86 Hands and wrists Relapse 9 months after onset - +
11 MI82 Hands and wrists Onset 0 +
12 FI84 Ankles and dorsum of feet Onset - +
13 MI72 Ankles, feet, pretibial region Onset + +
14 Fl57 Achilles tendons and distal calves Relapse 8 months after onset - +
I5 MI57 Hands, wrists, ankles, feet Recurrence 13 years after onset + -
16 Fl79 Ankles, feet, left hand Onset 0 +
17 ~n5 Hands, wrists, ankles, feet Onset + -
18 MI74 Left ankle 5 months prior to onset - -
Right hand, both ankles Onset + -
Right hand, ankles, feet Relapse 14 months after onset 0 +-
19 Fno Ankles and feet Onset +
* 0 indicates lack of response; + indicates response to therapy. NSAlD = nonsteroidal antiinflammatory drug.

edema were most visible and prominent over the
dorsum of the hands and wrists, the ankles, and the
tops of the feet.
The swelling and edema usually responded
promptly to corticosteroids, and more slowly or in-
completely to NSAIDS. In some cases, NSAID ther-
apy resulted in no improvement and, when cortico-
steroids were substituted, the symptoms promptly
improved (Table 2). In patients 3 and 9, carpal tunnel
syndrome was also present at the time the dorsa of the
hands and wrists were swollen and edematous (Tables
2 and 3). In both patients, carpal tunnel release surgery
was performed and histologic examination of the teno-
synovial membranes in the carpal tunnel showed evi-
dence of lymphocytic synovitis.
Other peripheral joint manifestations. In addi-
tion to the episodes of swelling and pitting edema, 17
of the 19 patients also had other peripheral findings
distal to the arms and thighs during the course of the
illness (Table 3). These included an additional episode
of diffuse swelling of the hands, wrists, or feet in 6
patients, with pitting edema not recorded as being
present (Table 3). Other musculoskeletal findings were
generally diagnosable as synovitis of joints or teno-
synovitis. Thirteen patients had further single epi-
sodes, 2 patients had 2 episodes (patients 2 and lo),
and 2 others had 3 episodes (patients 18 and 19). None
of the patients developed these other manifestations
prior to the onset of PMR. However, 9 patients
developed the symptoms concurrently with disease
onset, and 6 developed them later during a relapse or
recurrence of PMR (Table 3). Patients 18 and 19
developed these manifestations at onset and also later
in the course of the disease. Patients 2, 5 , and 18
developed transient peripheral symptoms in the ab-
sence of proximal aching and stiffness. Overall, these
other musculoskeletal manifestations included synovi-
tis of the knees in 11 patients, of the wrists in 1 patient,
and of the rnetacarpophalangeal (MCP) joints or prox-
imal interphalangeal (PIP) joints in 3 patients. Teno-
synovitis developed in 6 patients (Table 3). As noted
above, carpal tunnel syndrome occurred in 2 patients.
These manifestations resolved completely after cortico-
steroid therapy was started.
In 2 instances, synovial fluid was aspirated from
a swollen joint when the pitting edema was also
present, although the fluid was aspirated from a differ-
ent extremity in each instance. In patient 16, the left
knee yielded fluid with a leukocyte count of 10.0 x
109/liter, with 83% neutrophils. Right knee fluid from
patient 7 yielded a leukocyte count of 1.2 X lO’Aiter,
with 17% neutrophils. In an additional patient (patient
76 SALVARANI ET AL

Table 3. Peripheral musculoskeletal findings other than swelling with pitting edema in 19 patients with polymyalgia rheumatica (PMR)*
Patient Finding Stage of disease PMR present
1 Synovitis of wrists, MCPs, PIPs Relapse 4 months after onset Yes
2 Synovitis, left knee Recurrence 7 years after onset Yes
Synovitis of MCPs, PIPs; extensor carpi ulnaris 9 years after onset No
tendinitis
3 Bilateral CTS Onset Yes
4 Synovitis, left knee; left thumb extensor tendinitis Onset Yes
5 Tenosynovitis, right posterior tibial tendon Relapse 3 years after onset No
6 None - -
7 Swelling, right wrist and hand; no pitting noted Onset Yes
8 Synovitis, left knee Onset Yes
9 Bilateral CTS Onset Yes
10 Synovitis, right knee; tenosynovitis, right third Relapse 9 months after onset Yes
palmar flexor tendon
Swelling, both hands; pitting not noted 2 years after onset Yes
11 Synovitis of both knees Onset Yes
12 None - -
13 Swelling, right hand; pitting not noted; synovitis of Onset Yes
both knees
14 Swelling, dorsum left hand, especially over middle Relapse 16 months after onset Yes
finger extensor tendon
15 Synovitis of both knees Recurrence 13 years after Yes
onset
16 Synovitis of both knees Onset Yes
17 Synovitis of both knees Onset Yes
18 Swelling, left hand; pitting not noted; synovitis, Onset Yes
right knee
Swelling, dorsum left wrist; synovitis of both knees Relapse 5 months after onset No
Synovitis of both knees, wrists, MCPs, PIPs Relapse 1% years after onset No
19 Synovitis, right knee; left carpi ulnaris tendinitis Onset Yes
with swelling
Swelling, dorsum right foot Relapse 11 months after onset Yes
Synovitis of both knees Relapse 13 months after onset Yes
* MCPs = metacarpophalangeal joints; PIPs = proximal interphalangeal joints; CTS = carpal tunnel syndrome.

9), synovial fluid leukocytes were aspirated from the
right knee at a time when PMR was present, but
without swelling with pitting edema. The leukocyte
count was 1.4 x lO’iliter, with 11% neutrophils. Find-
ings of rheumatoid factor tests were negative in all 17
patients tested. Fluorescent antinuclear antibody tests
gave negative results in 10 patients, had a positive
result with a serum titer of 1:20 in 1 patient, and had 1
negative and 1 positive result with a titer of 1:40 in 2
testings in another patient. Joint radiography was
performed in all patients at some time point during the
course of the illness. The radiographs showed either
normal joints or evidence of osteoarthritis in all pa-
tients; none showed erosions or changes associated
with rheumatoid arthritis (RA).
CASE REPORTS
Swelling with pitting edema near the onset of
PMR. Patient 12, an 84-year-old woman, developed
gradual onset of aching and morning stiffness in the
shoulder and hip girdles. She felt tired, became ano-
rectic, and had a weight loss of 3 kg. The symptoms
became worse, and she was unable to function inde-
pendently. Two months after onset, she developed
pain, swelling, and pitting edema in the ankles and
mid-dorsal areas of both feet. The ESR was 47 mm/
hour. A diagnosis of PMR was made. Prednisone, 15
mg/day , was started, with rapid resolution of joint pain
within 48 hours after treatment. When she was seen
for followup 18 days later, the aching and pain were
absent and the foot swelling was nearly gone. The ESR
was 12 m d h o u r . The prednisone dosage was gradu-
ally decreased, and was discontinued at the end of 1
year. The patient died of a myocardial infarction 7
years later, without a recurrence of PMR or foot
swelling.
Swelling with pitting edema at onset of PMR.
Patient 1, an 84-year-old woman, developed dif€use
swelling in the dorsum of the right hand and wrist over
a 1-2-week period. Shortly thereafter, she noted sim-
DISTAL EXTREMITY SWELLING WITH PITTING EDEMA IN PMR
ilar swelling in the left hand and wrist, as well as
marked aching and morning stiffness of the neck,
shoulders, and proximal regions of the arms. She
developed marked fatigue, anorexia, and had a weight
loss of 7 kg. Physical examination revealed tenderness
and pitting edema in the dorsa of both hands. The
patient’s fingers were not affected, but she could not
make complete fists. The ESR was 54 m d h o u r .
Indomethacin, 25 mg 3 timedday, was started. Two
weeks later, her condition had not improved, and the
ESR was 82 mm/hour. Prednisone, 40 mg/day, was
given. When she was seen 10 days later, the patient’s
symptoms had resolved.
Four months later, the prednisone dosage had
been reduced to 5 mg/day, and the proximal symptoms
and diffuse swelling of the hands had returned. Pitting
edema was not identified at this time. On this exami-
nation, however, it was believed that synovitis was
present in the wrists, MCP joints, and PIP joints. The
prednisone dosage was increased to 10 mglday, and
joint symptoms and swelling resolved.
Three months later, the patient stopped the
prednisone treatment on her own. The proximal ach-
ing returned without hand o r wrist swelling. She
developed headache and jaw claudication. A temporal
biopsy showed changes associated with GCA.
Swelling with pitting edema on recurrence of
PMR. Patient 2, a 76-year-old woman, developed
aching and morning stiffness in the neck, torso, and
shoulders. The ESR was 62 mmhour. Prednisone, 45
mg/day, was begun, and her symptoms resolved within
72 hours. Five months later, the prednisone was
stopped after the dosage was gradually reduced. After
an additional 3 months, her proximal aching and
stiffness returned, and the ESR was 47 mm/hour.
Prednisone was reinstituted, and her condition im-
proved again. Six months later, the prednisone was
discontinued without recurrence of symptoms.
Seven years later, at age 83, the patient expe-
rienced the same aching and stiffness in the neck,
shoulders, and hips. At the same time, she also devel-
oped painful swelling and pitting edema in both hands
and a left knee effusion. The ESR was 86 mm/hour.
She was started on a regimen of ibuprofen, but the
symptoms did not improve. She was then given pred-
nisone, 20 mg/day, and the proximal aching, as well as
the hand and knee swelling, resolved. The ESR re-
mained at 60 m d h o u r . When the prednisone dosage
was lowered to 15 mg/day, she noted headache and
jaw claudication. A temporal artery biopsy showed
changes associated with partially treated GCA.
77
Additional similar cases. In the cohort of 245 pa-
tients with PMR (8), there were 5 other patients who
had diffuse swelling of 2 1 distal extremity, similar to
the cases outlined above, except that pitting edema
was not described in these 5 patients. This group
included 3 men and 2 women, ages 67-80, with a
median pretreatment ESR of 74 mmhour (range 42-
106). In 3 patients, dif€use swelling in both hands and
wrists was present. In the fourth patient, the dorsum
of the right hand and wrist was swollen. In the fifth
patient, the dorsum of the left hand and wrist was
affected. Each of these 5 patients had 1 episode of
distal extremity swelling.
In 4 patients, the swelling of the hands and
wrists began early in the course of the disease, in
conjunction with proximal symptoms of PMR, and
resolved along with the other symptoms when treat-
ment with corticosteroids was started. In the fifth
patient, diffuse swelling of both hands and wrists
developed during a recurrence of PMR 1 year after the
original onset of the disease. This last patient had a
pretreatment ESR of 106 mmhour, and the symptoms
showed a typical rapid response to treatment with
prednisone, 20 mg/day. The prednisone was reduced
and discontinued completely after 1 year. About 5
weeks later, the proximal aching and stiffness, and
also diffuse swelling of the dorsal aspects of the hands,
recurred. It was also noted that the digits and PIP
joints in both hands appeared swollen. All symptoms
resolved with administration of 10 mg of prednisone
per day. The ESR, which had risen again to 82
mm/hour, dropped to 27 mm/hour after reinstitution of
the prednisone. The prednisone was later reduced and
eventually stopped without recurrence of PMR or
hand swelling.
One additional resident of Olmsted County,
seen during the same period of time, developed typical
manifestations of PMR, and also pitting edema of the
hands and wrists, but was not included in the study
cohort because the ESR did not reach the criterion of
40 mmhour (8). The patient was a 59-year-old man
who developed aching in the shoulders, proximal
aspects of the arms, hips, and proximal regions of the
thighs and neck, with 2-3 hours of morning stiffness.
The ESR rose from 9 mmhour before disease onset, to
27 mm/hour at the time of the diagnosis. The patient
began treatment with 40 mg of prednisone/day and all
the symptoms resolved rapidly, including the swelling
of the hands and wrists. However, when the pred-
nisone was reduced to 15 mg/day, the proximal aching
and stiffness and bilateral hand pitting edema returned.
78
Sulindac was then added to the treatment regimen
(prednisone maintained at 15 mglday). The symptoms
gradually improved and eventually resolved, and did
not recur 6 months later when the prednisone was
discontinued completely.
DISCUSSION
This study was performed to determine the
clinical characteristics of distal extremity swelling
with pitting edema that may be seen in PMR. In an
effort to provide an accurate clinical picture of the
frequency and clinical spectrum of this manifestation,
and to reduce selection and referral bias, we evaluated
our community cohort of 245 patients with PMR.
Among this patient cohort, 19 were observed to have
had an episode of distal swelling with pitting edema.
Five of the 19 patients also had GCA. Although there
are no pathognomonic findings in PMR, the patients in
the cohort fulfilled the preset diagnostic criteria, and
none have developed another disease since onset that
would explain the findings. None of the patients de-
veloped persistent synovitis, as seen in RA, that may
occasionally occur in patients who initially develop
PMR or GCA (9).
All patients in this study cohort had 1 episode of
swelling with pitting edema, except patient 18, who
had 3 episodes. The episodes were observed during all
phases of the disease, but occurred most often (in 11
patients) at the onset of PMR. In 2 other patients, the
swelling with edema was the first manifestation; in 2
patients, it developed initially during a relapse of
PMR; and in 4 patients, it occurred during a recur-
rence. Patient 18 had an episode at onset and during 2
relapses. In different members of the group, the upper
and lower extremities were aEected singly or together.
There was a tendency toward symmetric involvement.
We observed no instances, in this group, in which the
distal swelling and edema episodes occurred distinctly
separate from the proximal symptoms of PMR. Six of
the 19 patients had an additional episode of diffuse
distal extremity swelling in which pitting edema was
not described. It is possible that pitting may have been
present in some of these instances, but not recorded
by the examiner. Alternatively, the absence of pitting
edema in these episodes, and in the 5 other patients,
may simply indicate that the manifestation of diffuse
swelling occurs in varying degrees.
The nature of the episodes of swelling with
pitting edema was not completely defined. However,
they appeared to be part of the inflammatory processes
SALVARANI ET AL
of PMR, based on the location over or nearjoints, the
association with the development of other articular
symptoms of this syndrome, and the rapid response to
corticosteroid therapy. The areas affected conformed
best to the distribution of tenosynovial membranes.
The swelling was also most prominent in areas where
subcutaneous tissues are most distensible, such as the
dorsum of the hand, foot, and ankle. The palmar and
plantar surfaces, and the fingers and toes were also
noted to be swollen in some instances, but without
pitting. The swollen tissues were painful and tender
and often warm, but erythema was seldom described.
Motion of the joints in the region of the swollen tissues
was painful and limited. For example, patients with
swelling of the dorsum of the hand and wrist were
unable to move the wrist through a full range of motion
or make a complete fist. N o biopsies of the obviously
edematous tissues were obtained. But, in patients 3
and 9, flexor tenosynovitis of the wrist was observed
in specimens that were obtained during surgery for
carpal tunnel syndrome at the same time the dorsum of
the hands were swollen. In patients 5 and 14, swelling
and edema clearly conformed to the courses of ten-
dons. There was no evidence of venous obstruction to
account for the swelling. Lymphedema was not ex-
cluded. However, the distribution of soft tissue swell-
ing appeared less extensive in an extremity, and more
reversible in response to treatment, than would seem
likely if the findings were secondary to lymphedema.
Seventeen of the 19 patients also had other
evidence of peripheral joint inflammation (Table 3). In
some instances, these findings were more clearly rec-
ognizable as synovitis because soft tissue swelling
occurred either in the anatomic location of the syno-
vium or in the tenosynovial membranes, or joint
effusions were present. The diffuse swelling without
definite pitting edema may represent a spectrum of
manifestations of a single pathologic process. Synovial
fluid was aspirated from 3 joints, and the leukocyte
count was moderately elevated in 1, and minimally
elevated in 2 other patients.
We conclude that the episodes of swelling and
pitting edema were peripheral manifestations of the
inflammatory processes associated with PMR and,
most likely, secondary to a vigorous tenosynovitis.
Synovitis of underlying joints also may be a compo-
nent in some instances. The tenosynovitis with pitting
edema may be one end of a spectrum of musculoskel-
etal manifestations of PMR. The 5 additional patients
with PMR and diffuse swelling who were not described
to have pitting edema may have had less marked
DISTAL EXTREMITY SWELLING WITH PITTING EDEMA IN PMR
tenosynovitis. The findings in these 5 patients, and the
1 other with typical PMR symptoms plus peripheral
swelling and pitting edema but with an ESR <40
mmhour, further indicate that the manifestation of
diffuse distal swelling in PMR is not rare.
We are uncertain if these patients represent a
specific subset of PMR. The relatively high frequency
of other peripheral joint findings may suggest this, but
further study is needed. Although peripheral joint
involvement in PMR has been previously described
(1,4-6), swelling with pitting edema in this condition
has been poorly recognized. The most characteristic
symptoms in PMR are proximal, and distal symptoms,
when they occur, are usually mild. Thus, prominent
distal swelling may focus attention away from the
proximal symptoms. However, at least a few cases of
PMR with pitting edema have been described previ-
ously (10-13). In some instances, the authors were
uncertain about the link between the 2 conditions.
Distal extremity swelling with pitting edema has
been reported occasionally in various other rheumatic
diseases. Conditions that most closely resemble our
cases of PMR include late-onset seronegative RA,
remitting seronegative symmetric synovitis with pit-
ting edema, and late-onset peripheral seronegative
spond ylarthropathy .
Seronegative RA is considered as a diagnosis
most clearly when distal polyarthritis persists chroni-
cally over years, with eventual destructive changes in
joints. When limb edema occurs in RA, it is usually
associated with destructive joint changes at the site of
edema, and a rapid and complete or near-complete
response to corticosteroid therapy is unusual (10,14-
15). The swelling in all our patients resolved com-
pletely after corticosteroids were started, and eventu-
ally remained absent when treatment was reduced or
discontinued. None of our patients experienced a
course of prolonged and persistent synovitis.
In remitting, seronegative, symmetric synovitis
with pitting edema, described by McCarty et a1
(16,17), older patients had an acute onset of symmetric
synovitis predominantly affecting the flexor digitorum
tendon sheaths in the extremities, with pitting edema
of the hands and feet. A high ESR was present, and
symptoms responded promptly to small doses of pred-
nisone. A remission was achieved without relapse
within 3-36 months. However, many of the patients
also were reported to have pain and stiffness and
restricted motion in the shoulders, suggesting some
similarity to PMR. Yet, some differences were also
present. The patients in the McCarty et a1 study
79
(16,17), as well as those in other studies, have been
predominantly male, not all have had proximal limb-
girdle symptoms, and residual flexor contraction of the
fingers and wrists may have developed, which was not
seen in our patients. HLA-B7 was positive in 15 of 23
patients tested by McCarty et al, whereas levels of
HLA-DR4 are increased in patients with PMR (18,19).
In addition, patients in our study also had unilateral or
single lower extremity involvement, and the distal
swelling and edema occurred during relapses or recur-
rences in some instances. However, some cases of the
RS3PE syndrome have recently been reported to have
unilateral involvement (13,20,21).
Until more is understood about the etiology and
pathogenetic factors in PMR, late-onset seronegative
RA, and the syndrome described by McCarty and
colleagues, the exact nature of the interrelationships
among them will remain uncertain. In diseases of
unknown etiology, without pathognomonic findings,
categorization of all patients is unlikely to be achieved.
Healey (11) noted 2 patients who had 3 steroid-
responsive episodes, which, at different times, were
typical of PMR, remitting, seronegative, symmetric
synovitis with pitting edema, and seronegative RA. H e
has speculated that these 3 syndromes, at least in some
cases, may be variants of the same process.
Dubost and Sauvezie reported 10 patients with
late-onset peripheral spondylarthropathy (22). All
were men over the age of 50 with mild involvement of
the axial skeleton, but who had lower limb oligoarthri-
tis with swelling and pitting edema. They had promi-
nent systemic symptoms and a high ESR. The condi-
tions did not respond well to corticosteroids. All were
HLA-B27 positive. Five patients were found later to
have sacroiliitis. Olivieri et a1 (23) have described 2
similar cases. Compared with our cohort, these pa-
tients appeared different in several ways, including the
response rate to corticosteroids, which we observed to
be rapid in our patients. All 19 patients with PMR and
pitting edema in our study, and the 5 additional similar
cases, fulfilled the criteria for PMR, as defined by Bird
and coworkers (24).
In summary, we have described 13 women and
6 men with PMR from a population cohort of 245
patients who had 2 1 episode of distal extremity swell-
ing with pitting edema, in conjunction with the char-
acteristic proximal symptoms seen in this condition.
The distal swelling with pitting edema responded fa-
vorably to therapy, as did the proximal symptoms. We
conclude that the distal swelling with pitting edema in
these patients, and the diffuse swelling without pitting

edema in others, are likely due to tenosynovitis, and
represent a manifestation of this syndrome that has
been poorly recognized previously. Awareness that
distal swelling may occur in PMR will help reduce
diagnostic confusion and facilitate institution of appro-
priate therapy.
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