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Polyarthralgia disclosing hyperthyroidism: Report
of two cases
Molinier S.; Paris J.F.; Marlier S.; Galzin M.; Amah Y.;
Carli P.
S. Molinier, Serv. Med. Int. Malad. Infect./Trop., HIA
Laveran, F 13998 Marseille Armees France
Presse Medicale (France), 1998, 27/26
(1324-1326)
BACKGROUND: We report two cases of rheumatism associated
with hyperthyroidism. In both cases, arthralgia totally
regressed after thyroid treatment.
CASE REPORTS: Two 79-year-old and 59-year-old women
developed manifestations of polymyalgia rheumatica and
psoriasis arthritis respectively. Corticosteroid therapy was
ineffective and followed by manifestations of hyperthyroidism.
The first patient was treated with carbimazole and the second
with thyroidectomy. Once the hyperthyroidism was controlled,
both patients experienced a dramatically rapid cure of their
arthralgias.
DISCUSSION: Scalpulo-humeral periarthritis is the main
articular complication of hyperthyroidism. True manifestations
of <<thyrotoxicosis rheumatism>> are unusual and
may be linked with a direct toxicity of the thyroid hormones
on joint cartilage or with an autoimmune manifestation of
hyperthyroidism.
A 67-year-old woman with polymyalgia rheumatica and
left hemispatial neglect
Mahfood J.P.; Gold M.; Gonzalvo A.; Valeriano-Marcet J.
Dr. M. Gold, Univ. of South Florida Coll. of Med., Department
of Neurology, MDC, 12901 Bruce B. Downs Boulevard, Tampa, FL
33612 United States
Journal of Neuroimaging (United States), 1998, 8/4
(222-227)
A 67-year-old woman with a diagnosis of polymyalgia
rheumatica presented initially with periods of confusion and
incontinence. ACT scan of the brain was normal and she was
treated with tapering doses of corticosteroids and clinical
improvement. After a brief period off steroids, the patient
presented with a progressive dementia, left-sided clumsiness,
gait disturbances and left hemispatial neglect. An MRI at this
time demonstrated a large area of edema over the right
parietal lobe and intense cortical enhancement. A chest CT
demonstrated multiple nodules. Biopsies of the lung and brain
failed to identify any infectious organisms or malignant
tissue. The leptomeningeal biopsy revealed multiple
granulomatous areas with central necrosis and hystiocytic
cells consistent with idiopathic hypertrophic
pachymeningitis.
Effects of glucocorticoids on bone mass in patients
with polymyalgia rheumatica. A longitudinal study [4]
Mur E.; Watfah C.; Kinigadner U.; Fridrich L.; Riccabona G.;
Moncayo R.
Dr. E. Mur, Department of Internal Medicine, University of
Innsbruck, Anichstrasse 35, A-6020 Innsbruck Austria
Clinical and Experimental Rheumatology (Italy), 1998, 16/5
(623)
No abstract.
Polymyalgia rheumatica in biopsy proven giant cell
arteritis does not constitute a different subset but differs
from isolated polymyalgia rheumatica
Gonzalez-Gay M.A.; Garcia-Porrua C.; Vazquez-Caruncho
M.
Dr. M.A. Gonzalez-Gay, Division of Rheumatology, Hospital
Xeral-Calde, c/Dr. Ochoa s/n, 27004 Lugo Spain
Journal of Rheumatology (Canada), 1998, 25/9
(1750-1755)
Objective. To assess clinical and laboratory features that
may be useful in differentiating isolated polymyalgia
rheumatica (PMR) from PMR associated with biopsy proven giant
cell arteritis (GCA); and in differentiating biopsy proven GCA
associated with PMR from GCA without manifestations of
PMR.
Methods. Clinical records of patients with PMR and biopsy
proven GCA diagnosed at Hospital Xeral, Lugo, Spain from
January 1987 through May 1997 were reviewed. Patients with a
positive temporal artery biopsy were categorized into 2
different subgroups according to the presence or absence of
associated PMR. The patients with biopsy proven GCA associated
with PMR were compared with a group of patients with isolated
PMR (not associated with GCA).
Results. From a total of 108 biopsy proven patients with
GCA, 45 had associated PMR. Apart from a predominance of women
and a longer delay to diagnosis, patients with PMR associated
with GCA did not differ from the patients with GCA without PMR
manifestations. In comparing patients with isolated PMR (n =
117) with patients with PMR associated with GCA, we observed
that PMR associated with GCA was a more severe disease, with
significant abnormality in most laboratory variables,
including constitutional syndrome, higher elevation of
erythrocyte sedimentation rate and platelet counts, and lower
values of hemoglobin.
Conclusion. In both isolated PMR and PMR associated with
GCA we observed a predominance of women. While there are no
differences in the type of polymyalgia symptoms in patients
with isolated PMR versus PMR associated with GCA, severe
abnormalities associated with the inflammatory response in PMR
may have prognostic value for more severe disease, which may
be linked to the presence of GCA.
A case of anterior ischemic optic neuropathy
associated with polymyalgia rheumatica
Hayama F.; Hasegawa S.; Takagi M.; Suzuki K.; Takada R.; Abe
H.
Dr. F. Hayama, Dept of Ophthalmol, Niigata Univ School of
Med, 1-754 Asahimachi-dori, Niigata 951-8510 Japan
Folia Ophthalmologica Japonica (Japan), 1998, 49/8
(720-725)
We treated a case of anterior ischemic optic neuropathy
(AION) following polymyalgia rheumatica (PMR) that seemed to
be associated with temporal arteritis (TA). A 70-year-old
female developed AION in her right eye 9 years after the onset
of PMR and 1 month after the onset of TA. She had also
suffered for 4 years from maculopathy, of unknown etiology, in
her left eye. Magnetic resonance (MR) angiography revealed
stenosis of the right internal carotid artery at the point of
branching of the ophthalmic artery. Visual function in both of
this patient's eyes improved with topical steroid pulse
therapy, although her visual field worsened when she
interrupted topical steroid therapy for a course of systemic
steroid therapy. Little is known about AION associated with
PMR. However, this case suggests that cerebral angiography is
essential to locating the source of AION associated with
arteritis and that steroid therapy is strikingly effective and
should be maintained for the long term to preserve visual
function.
Polymyalgia rheumatica and giant cell
arteritis
Evans J.M.; Hunder G.G.
Dr. J.M. Evans, Mayo Clinic, 200 First Street SW, Rochester,
MN 55905 United States
Clinics in Geriatric Medicine (United States), 1998, 14/3
(455-473)
Polymyalgia rheumatica and giant cell arteritis are related
conditions that may represent a continuum of disease. These
conditions are relatively common and appear to be mediated by
an autoimmune cellular inflammatory response. A growing body
of evidence suggests an infectious cause or causes
precipitating this immune response in genetically susceptible
individuals. Although previously thought to affect primarily
branch vessels of the aortic arch, GCA is now thought of as a
disease in which proximal aortic involvement is common.
Despite the potential for serious, even fatal complications,
overall prognosis for patients with GCA or PMR is excellent.
Corticosteroids remain the standard treatment, although likely
not curative. While the ESR continues to be a useful indicator
of disease activity, other markers that may be more precise
are currently being investigated in order to improve disease
diagnosis and treatment.
Epidemiology and optimal management of polymyalgia
rheumatica
Labbe P.; Hardouin P.
Dr. P. Labbe, Department of Rheumatology, Centre Hospitalier
Specialise, Institut Calot, 62608 Berck sur Mer France
Drugs and Aging (New Zealand), 1998, 13/2
(109-118)
Polymyalgia rheumatica (PMR) is a disease of unknown
aetiology that occurs in elderly patients, predominantly
affecting the Caucasian population. The disease has a slightly
higher prevalence in women than in men. There is ongoing
discussion regarding the relationship between PMR and giant
cell arteritis; an increasing number of studies indicate that
they are closely related. PMR has also been linked with
rheumatoid arthritis, myopathy and malignant disease. Oral
corticosteroids remain the mainstay of drug therapy for PMR.
These drugs usually induce prompt relief of symptoms, and some
authors consider this dramatic response to be diagnostic for
PMR. However, the ideal initial dosage, the duration of
treatment and the optimal tapering schedule are much debated.
Other drugs, such as methotrexate and azathioprine, have been
suggested as corticosteroid sparing agents. Nonsteroidal
anti-inflammatory drugs are generally considered to be
unsuitable for the long term treatment of PMR.
Systemic corticosteroid therapy in rheumatology:
Benefit-risk ratio
Gerster J.C.; So A.K.L.; Burckhardt P.
Dr. J.C. Gerster, Service de Rhumatologie, CHUV, 1011
Lausanne Switzerland
Schweizerische Rundschau fur Medizin/Praxis (Switzerland),
1998, 87/33 (1024-1027)
Apart from the therapy of autoimmune diseases,
corticosteroids have an important position in the treatment of
rheumatoid arthritis. Corticosteroids are used after the
failure of non-steroidal antiinflammatory agents or of the
basis therapies to control the illness. When the rheumatoid
arthritis is accompanied by a systemic disease, they will be
used earlier and in higher dosages. For polymyalgia
rheumatica, independently of an association with temporal
arteritis, corticosteroids are the therapy of choice. Risks of
long- time corticosteroid therapy are a higher incidence of
infection and bone demineralisation, especially in
postmenopausal women. A careful prevention with Calcium and
Vitamin D must be carried out systematically. The
demineralisation can be limited by the use of Deflazacort, a
corticosteroid, which decreases the loss of calcium.
Ophthalmoplegia in treated polymyalgia rheumatica
and healed giant cell arteritis
Brilakis H.S.; Lee A.G.
Dr. A.G. Lee, Dept. Ophthalmol., Baylor Coll. Medicine, 6565
Fannin St, NC-205, Houston, TX 77030 United States
Strabismus (Netherlands), 1998, 6/2 (71-75)
Diplopia may be a sign of giant cell arteritis (GCA).
Polymyalgia rheumatica (PMR) is a systemic rheumatic
inflammatory disease characterized by shoulder and hip girdle
pain, and PMR can be associated in some patients with GCA. The
authors report diplopia in two patients with treated PMR and
biopsy-proven GCA, and review the literature on diplopia in
GCA.
Polymyalgia rheumatica in a patient with acute
tubulointerstitial nephritis due to Sjogren's syndrome
Hisahara S.; Ohkoshi N.; Shoji S.; Muro K.; Yamaguchi N.;
Kobayashi M.; Koyama A.
Dr. N. Ohkoshi, Department of Neurology, Institute of
Clinical Medicine, University of Tsukuba, 1-1-1 Tennoudai,
Tsukuba, Ibaraki 305 Japan
Journal of Internal Medicine (United Kingdom), 1998, 244/1
(83-86)
A 68-year-old Japanese woman with polymyalgia rheumatica
associated with acute tubulointerstitial nephritis and
subclinical Sjogren's syndrome is described. Gallium
scintigraphy showed marked accumulation in both kidneys and
the salivary glands. Renal biopsy revealed lymphocytic
infiltration in the tubulointerstitium. She was treated with
intravenous high-dose methylprednisolone followed by oral
prednisolone. Her clinical symptoms improved. This is the
first report of this particular association.
Distal musculoskeletal manifestations in
polymyalgia rheumatica: A prospective followup study
Salvarani C.; Cantini F.; Macchioni P.; Olivieri I.; Niccoli
L.; Padula A.; Boiardi L.
Dr. C. Salvarani, Servizio di Reumatologia, Arcispedale Santa
Maria Nuova, V.le Umberto 1 N50, 42100 Reggio Emilia
Italy
Arthritis and Rheumatism (United States), 1998, 41/7
(1221-1226)
Objective. To determine the frequency and the
characteristics of distal musculoskeletal manifestations in
polymyalgia rheumatica (PMR).
Methods. Prospective followup study of 177 consecutive
patients meeting clinical criteria for PMR, diagnosed over a
5-year period in 2 rheumatology secondary referral centers in
Italy.
Results. Seventy-nine of the 177 patients (45%) had distal
musculoskeletal manifestations. Peripheral arthritis occurred
in 45 patients (25%), carpal tunnel syndrome in 24 (14%),
distal extremity swelling with pitting edema in 21 (12%), and
distal tenosynovitis in 5 (3%). These manifestations were
usually associated with PMR proximal symptoms (69%); however,
31% of the episodes represented isolated relapse/recurrence at
distal sites. Distal symptoms responded promptly to
corticosteroids. No evidence of joint deformities, erosions,
or development of rheumatoid arthritis was observed during the
followup. The group of patients with peripheral arthritis
included a higher proportion of females, had a longer duration
of therapy, and had more relapses/recurrences. Patients who
had distal extremity swelling with pitting edema had a higher
age at disease onset, a shorter duration of therapy, and lower
initial and cumulative prednisone doses.
Conclusion. Inflammatory involvement of distal articular
and/or tenosynovial structures occurs in approximately half of
the cases of PMR. Peripheral arthritis is associated with more
severe disease, while distal extremity swelling with pitting
edema appears to identify a more benign disease subset.
Anticardiolipin antibodies in giant cell arteritis
and polymyalgia rheumatica: A study of 40 cases
Manna R.; Latteri M.; Cristiano G.; Todaro L.; Scuderi F.;
Gasbarrini G.
R. Manna, Istituto Medicina Interna Geriatria, Policlinico A.
Gemelli, L.go F. Vito 8, 00168 Roma Italy
British Journal of Rheumatology (United Kingdom), 1998, 37/2
(208-210)
The occurrence and clinical value of anticardiolipin
antibodies (aCL) were studied in 33 patients with giant cell
arteritis (GCA) and in seven patients with polymyalgia
rheumatica (PMR), at onset and during follow-up. aCL were
present in 19/40 (47.5%) GCA/PMR cases, most of them of the
IgG isotype, whereas all controls (21 subjects) were aCL
negative. The presence of aCL was not associated with
inflammatory parameters or clinical signs of arteritis;
however, they disappeared in a significant percentage (56%) of
patients during steroid therapy. No correlation was found
between ischaemic events and aCL, suggesting that they are not
important for the development of vascular complications in
GCA/PMR patients. Moreover, a retrospective evaluation of our
data showed a correlation between aCL positivity and anaemia,
whose significance remains to be elucidated.
An initially double-blind controlled 96 week trial
of depot methylprednisolone against oral prednisolone in the
treatment of polymyalgia rheumatica
Dasgupta B.; Dolan A.L.; Panayi G.S.; Fernandes L.
B. Dasgupta, Department of Rheumatology, Southend Health Care
Trust, Prittlewell Chase, Westcliff-on-Sea, Essex SS0 0RY
United Kingdom
British Journal of Rheumatology (United Kingdom), 1998, 37/2
(189-195)
The objective was to compare the effiacy and safety of
intramuscular methylprednisolone acetate (i.m. MP) with oral
prednisolone (OP) in the treatment of polymyalgia rheumatica
(PMR), a common steroid-treated illness where prolonged
therapy can lead to steroid side-effects. The cumulative dose
with i.m. MP injections given every 3-4 weeks is considerably
smaller than that with conventional OP, and may therefore be
associated with fewer long-term side-effects. A hybrid design
was used with an initial 12 week double-blind
placebo-controlled phase followed by an open phase on active
treatment up to 96 weeks. The study was multicentre hospital
out-patient based and included 60 patients with untreated PMR.
In the doubleblind phase, either 120 mg 3-weekly i.m. MP or
gradually tapering daily OP (initial dose 15 mg) were
administered. In the open phase, subjects continued their
active treatment with gradual tapering of the steroid dosage.
The remission rate at 12, 48 and 96 weeks, and other measures
of disease activity, i.e. sedimentation rate, pain and morning
stiffness, and percentage of adverse reactions and serious
complications such as fractures, were the main outcome
measures. Sixty patients entered (30 OP:30 i.m. MP) and 49 (25
OP:24 i.m. MP) completed the study. There were similar
remission rates after the double-blind phase (60.6% OP and
66.6%, i.m. MP, respectively) and similar disease control in
the succeeding open phase. With steroid tapering, the mean
erythrocyte sedimentation rate for the entire cohort
registered a significant increase in the absence of an
increase in symptoms. At 96 weeks, the cumulative mean steroid
dose in subjects treated with i.m. MP was equivalent to 56%
that of subjects treated with OP. There were eight fractures
with OP compared to one on i.m. MP. Mean weight gain was
significantly greater with OP than i.m. MP (3.42 vs 0.82 kg, P
< 0.005). Minor adverse reactions were similar in both
groups apart from slightly increased bruising with i.m. MP.
Only patients on OP reported moon face, hypertension,
cataracts, back pain and depression, but the numbers were
small. It is possible to achieve equivalent long-term disease
control in PMR with i.m. MP compared to OP. I.m. MP was
associated with far fewer fractures and lesser weight gain,
presumably related to lower cumulative dose. These findings
may have implications in the steroid treatment of PMR, and
other rheumatic and nonrheumatic diseases.
The deleterious effects of low-dose corticosteroids
on bone density in patients with polymyalgia rheumatica
Pearce G.; Ryan P.F.J.; Delmas P.D.; Tabensky D.A.; Seeman
E.
E. Seeman, Department of Endocrinology, Austin Repatriation
Medical Centre, Heidelberg 3084 Australia
British Journal of Rheumatology (United Kingdom), 1998, 37/3
(292-299)
The beneficial effects of corticosteroid therapy in the
treatment of rheumatic diseases may be offset by the
occurrence of corticosteroid-related osteoporosis. This
problem may be overcome by using low-dose corticosteroids;
however, the dose of corticosteroids that is both efficacious
and skeletal sparing is uncertain. Therefore, the aim of this
study was to determine whether low-dose prednisolone treatment
results in bone loss and modifies bone turnover. Nineteen
patients (12 female, seven male) suffering from polymyalgia
rheumatica received 10 mg or less daily, given in reducing
dosage, with a range of 2.5-10 mg and an average of 6.0 plus
or minus 0.2 mg daily (plus or minus S.E.M.). Prior to the
commencement of therapy and at regular intervals during
treatment, bone mineral density (BMD) using dual X-ray
absorptiometry and circulating biochemical and hormonal
determinants of bone turnover were measured. The patients were
followed for 14.4 plus or minus 1.6 months (range 6-27). They
were compared to 19 age-matched controls. Despite a mean
exposure dose of 6 mg/day and disease remission, BMD decreased
in the patients at the lumbar spine (2.6 plus or minus 0.8%, P
< 0.01), femoral neck (2.9 plus or minus 1.5%, P = 0.06),
Ward's triangle (5.5 plus or minus 2.9%, P = 0.06) and the
trochanter (4.3 plus or minus 1.9%, P < 0.05). Total body
bone mass decreased by 50 plus or minus 19 g in the first 6
months (P < 0.02), and by 39 plus or minus 30 g in the
remaining 8 months of follow-up [not significant (NS)]. In the
first 6 months, BMD decreased at the lumbar spine (1.7 plus or
minus 0.9%, P = 0.06). From 6 months to the end of follow-up,
BMD decreased by 8.5 plus or minus 3.5% at Ward's triangle (P
< 0.05) and by 4.8 plus or minus 2.5% at the femoral neck
(P = 0.08). The fall in BMD correlated with the cumulative
prednisolone dose at trabecular-rich regions (trunk r = -0.72,
P < 0.001; ribs r = -0.53, P < 0.05). Bone resorption,
assessed by urinary cross-laps, was 54.7% higher than controls
before treatment was started (P < 0.05) and decreased by
23.5 plus or minus 7.1% in the first month of treatment when
the mean prednisolone dose was 9.1 mg/day, range 5-10 (P <
0.0001). Serum osteocalcin was not suppressed by disease
before treatment, decreased by 27.4 plus or minus 5.1% during
the first month of treatment (P < 0.001), remained
suppressed while the daily dose of prednisolone was > 5
mg/day, but returned to baseline below this dose. Serum
parathyroid hormone was 19.3% lower in the patients than
controls at baseline (NS), and increased by 46.1% (P <
0.05) but was no higher than controls at any time. Muscle
strength increased by 20-60% (P < 0.05 to < 0.01).
Prophylaxis should be considered in patients receiving less
than or equal to 5 mg/day prednisolone daily as bone loss is
2- to 3-fold expected rates. Earlier trabecular bone loss may
predispose to spine and rib fracture; later cortical bone loss
may predispose to hip fractures. Doses of prednisolone of <
5 mg daily may be skeletal sparing, but may not be
effcacious.
Cytokines and adhesion molecules in patients with
polymyalgia rheumatica
Uddhammar A.; Sundqvist K.G.; Ellis B.; Rantapaa-Dahlqvist
S.
A. Uddhammar, Department of Rheumatology, University Hospital
Northern Sweden, S-901 85 Umea Sweden
British Journal of Rheumatology (United Kingdom), 1998, 37/7
(766-769)
Serum levels of interleukin-1beta (IL-1beta), IL-1 receptor
antagonist (IL-1ra), tumour necrosis factor alpha (TNF-alpha),
IL-6, soluble IL-6 receptor (sIL-6R), soluble intercellular
adhesion molecule-1 (sICAM-1) and soluble E-selectin were
measured in 15 patients with newly diagnosed polymyalgia
rheumatica (PMR) before and after 3 months of corticosteroid
therapy. Both IL-6 and IL-1ra were significantly increased in
untreated PMR and remained elevated compared with controls
during therapy, although significantly only for sIL-1ra.
sICAM-1 was raised in 12/15 (87%) patients at diagnosis and
remained high in 10/14 (71%) patients; soluble E-selectin
levels were initially raised in 6/15 (40%) patients and
decreased with therapy in those with the highest levels. IL-6,
IL-1ra and sICAM-1 are sensitive indicators of continuing
immunological activation in PMR; the advantages of these
markers in assessing the response to therapy should be
investigated in a longitudinal study.
A 24-year-old man with symptoms and signs of
polymyalgia rheumatica
Whittaker P.E.; Fitzsimons M.G.
Dr. P.E. Whittaker, MAMC (ATTN: MCHJ-FP), Tacoma, WA 98431
United States
Journal of Family Practice (United States), 1998, 47/1
(68-71)
Most physicians regard polymyalgia rheumatica (PMR) as a
disease that affects only the elderly. This case report of a
24-year-old man with proximal limb girdle muscle pain,
stiffness, tenderness, weakness, and an elevated erythrocyte
sedimentation rate, who had a dramatic response to steroids,
supports the premise that PMR may affect a wider range of our
population. Increased physician awareness that this illness
does occur in patients younger than 50 may prevent delays in
diagnosis, and decrease the needless suffering and
incapacitation of younger patients with this disorder.
Deltoid muscle in patients with polymyalgia
rheumatica
Uddhammar A.; Dahlqvist S.R.; Hedberg B.; Thornell
L.-E.
Dr. A. Uddhammar, Department of Rheumatology, Univ. Hospital
of Northern Sweden, S-901 85 Umea Sweden
Journal of Rheumatology (Canada), 1998, 25/7
(1344-1351)
Objective. To investigate muscle tissue in patients with
polymyalgia rheumatica (PMR). Methods. Deltoid muscle biopsies
obtained from 10 female patients with PMR before
corticosteroid therapy and from 5 healthy female controls of
similar age were studied using light microscopy. Serial
cryosections were stained for the activity of myofibrillar
ATPases at various pH and for oxidative and glycolytic
enzymes. Sections were also incubated with antibodies against
different myosin heavy chain (MyHC) isoforms, and the laminin
alpha1 chain, identifying capillaries and cell borders, and
against ligands for selected adhesion molecules. Results. The
muscle fibers in patients and controls were mainly of type I
containing slow MyHC, or type II AB containing both fast A and
fast B MyHC isoforms. The oxidative capacity of the muscle
fibers was in general low. In the patients with PMR the
numbers of capillaries per unit area of type I and type II
fibers were higher than in controls (p = 0.05 and p < 0.5,
respectively). No accumulation of inflammatory cells or
increased expression of adhesion molecules in the muscle
sections from patients with PMR was found. Conclusion. There
is increased microvascularization of the deltoid muscle fibers
in patients with PMR. Whether this is directly associated with
the systemic inflammation and the musculoskeletal symptoms or
is mainly due to muscle fiber atrophy could not be
determined.
Polyarthritis associated with myelodysplastic
syndromes: 5 cases
Bonnotte B.; Chauffert B.; Roubert X.; Milas L.; Caillot D.;
Solary E.; Francois M.; Bernard L.
Prof. B. Lorcerie, Service de Medecine Interne, Hopital du
Bocage, 2 Bd Marechal de Lattre de Tassigny, F-21034 Dijon
Cedex France
European Journal of Internal Medicine (Italy), 1998, 9/1
(57-60)
We analyzed the clinical and biological characteristics of
5 patients who demonstrated polyarthritis associated with a
myelodysplastic syndrome. Polyarthritis either preceded (3
cases) or appeared during the course (2 cases) of the
hematological malignancy. Two patterns of rheumatic
manifestations were observed: rheumatoid type arthritis and
manifestations of polymyalgia rheumatica. X-ray demonstrated
non erosive lesions. Biological evaluations showed a non
specific inflammatory syndrome and rheumatoid factor was
negative. In 3 patients prednisolone improved the
symptomatology while chemotherapy cured the polyarthritis in
the two remaining cases. These observations indicate that
hematological diseases can be revealed by rheumatic
manifestations and that both diseases have a parallel
evolution.
Clinical outcome of 149 patients with polymyalgia
rheumatica and giant cell arteritis
Bahlas S.; Ramos-Remus C.; Davis P.
Dr. P. Davis, 562 Heritage Medical Research Centre,
University of Alberta, Edmonton, Alta. T6G 2S2 Canada
Journal of Rheumatology (Canada), 1998, 25/1
(99-104)
Objective. To assess the clinical outcome of patients with
polymyalgia rheumatica (PMR) and giant cell arteritis
(GCA).
Methods. All charts of consecutive patients with a
diagnosis of PMR and/or GCA attending a tertiary referral
center from June 1989 to February 1996 were reviewed following
a predetermined protocol. Subsequently, the majority of
patients (90%) were assessed clinically or by telephone
interview. Registered variables included demographic data,
disease characteristics, prednisone dosage and duration,
comorbidities, and clinical outcomes.
Results. There were 149 patients (133 with PMR alone, 7
with GCA alone, 9 with both); 94 (63%) were females; the mean
age was 68 plus or minus 9 years, and the mean disease
duration from the first symptom to the rheumatology
consultation was 13 plus or minus 12 weeks (1-99). Typical
clinical features of PMR were present in patients with PMR.
Synovitis was observed in 26 patients. The presenting symptoms
for GCA were typical features in 13 patients and blindness in
3 (2%) patients. Mean follow-up was 3.7 plus or minus 2 years.
Comorbid conditions were present in 71 patients: 12 patients
had hypertension, 13 had fractures, 8 diabetes, 29 cataract, 8
major infection, and 37 had other complications. Cancer was
diagnosed in 4 patients and 6 patients had died. Prednisone
was prescribed in 148 patients (mean dose 23 plus or minus 14
mg) for a mean time of 28 plus or minus 29 mo. Nonsteroidal
antiinflammatory drugs were prescribed in 51 (34%) patients
and methotrexate in 2. Disease remission was achieved in 81
(54%) patients (72 remissions, 9 presumed remissions) in whom
steroid therapy had been stopped. Another 54 (36%) patients
were still taking prednisone at the time of the interview, all
were in clinical remission. Seventeen patients developed
rheumatoid arthritis subsequent to the diagnosis of PMR.
Conclusion. PMR and GCA should not necessarily be
considered diseases with favorable outcome. In many of our
patients, steroids were required for a prolonged period. Some
patients developed significant complications attributable to
steroid therapy. A significant number of patients progressed
to rheumatoid arthritis.
Large vessel vasculitides
Cid M.C.; Font C.; Coll-Vinent B.; Grau J.M.
Dr. M.C. Cid, Department of Internal Medicine, Hospital
Clinic, University of Barcelona, Barcelona Spain
Current Opinion in Rheumatology (United States), 1998, 10/1
(18-28)
During the past few years remarkable progress has been
achieved in the understanding of the pathogenic mechanisms
leading to vascular inflammation and injury in giant cell
(temporal) arteritis. T lymphocytes are activated by specific
recognition of a putative antigen residing in the arterial
wall and, subsequently, activate macrophages that undergo a
functional differentiation and contribute to vessel damage
through various pathways. Vascular response to inflammation
amplifies the inflammatory response through neovascularization
and adhesion molecule expression. We are beginning to
appreciate that products released by infiltrating inflammatory
cells may play an important role in vessel occlusion and
resulting ischemic complications. Concomitantly, synovitis
underlying polymyalgia rheumatica musculoskeletal symptoms has
been immunopathologically characterized and the nature of its
relationship to giant cell arteritis is discussed. Although
some components of the disease are highly corticosteroid
responsive, other underlying pathogenic mechanisms may remain
active. Long-term outcome is heterogeneous in patients with
giant cell arteritis. Much less is known about the
pathogenesis of Takayasu's arteritis. Recent work supports its
association with HLA class I antigens, which may differ in
different geographic areas or ethnic groups. Because
Takayasu's disease expression may vary in different ethnic
settings, this possibility has led to the proposal of new
diagnostic criteria. Finally, the role of new imaging
techniques in diagnosis and assessment of disease activity is
discussed.
Diagnosis and management of polymyalgia
rheumatica/giant cell arteritis
Salvarani C.; Macchioni L.; Olivieri I.; Cantini F.; Boiardi
L.
Dr. C. Salvarani, Unita Reumatologica, Arcispedale S. Maria
Nuova, Viale Umberto 1 50, 42100 Reggio Emilia Italy
BioDrugs (New Zealand), 1998, 9/1 (25-32)
There are no standardised diagnostic criteria for
polymyalgia rheumatica. The combination of persistent pain (at
least 1 month) with marked morning stiffness in at least 2 of
the neck, shoulder or pelvic girdle is characteristic of
polymyalgia rheumatica. The other criteria are age >50
years, erythrocyte sedimentation rate (ESR) >40 mm/hour,
rapid response to corticosteroids and an absence of other
diseases capable of causing the musculoskeletal symptoms. A
normal ESR does not exclude a diagnosis of polymyalgia
rheumatica. Diagnostic temporal artery biopsy is recommended
in all patients suspected of having giant cell arteritis. The
segment of temporal artery with abnormality on physical
examination should be biopsied. The drugs of choice in the
treatment of polymyalgia rheumatica/giant cell arteritis are
corticosteroids. An initial prednisone dosage of 40 to 60
mg/day is adequate in almost all cases of giant cell
arteritis. Higher dosages and/or intravenous pulse
methylprednisolone can be tried on patients with partial
response or with recent visual loss. Polymyalgia rheumatica in
the absence of giant cell arteritis requires an initial dose
of prednisone 10 to 20 mg/day. In some cases of mild
polymyalgia rheumatica, a short course of nonsteroidal
anti-inflammatory drugs may be tried. Long term corticosteroid
therapy in polymyalgia rheumatica and giant cell arteritis is
complicated by serious adverse effects in between 48 and 65%
of patients. Vertebral fractures and infections are among the
most dangerous and frequent complications. Although there are
limited data on the use of cytotoxic or immunosuppressive
drugs, such as methotrexate, azathioprine and cyclosporin, in
these indications, they might be effective either in sparing
corticosteroids or in treating patients who do not respond to
treatment with corticosteroids.
Adverse outcomes of antiinflammatory therapy among
patients with polymyalgia rheumatica
Gabriel S.E.; Sunku J.; Salvarani C.; O'Fallon W.M.; Hunder
G.G.
Dr. S.E. Gabriel, Dept. of Health Sciences Research, Mayo
Clinic Rochester, 200 First Street, SW, Rochester, MN 55905
USA
Arthritis and Rheumatism (USA), 1997, 40/10
(1873-1878)
Objective. To evaluate the incidence and risks of adverse
events associated with therapy (both corticosteroids (CS) and
nonsteroidal antiinflammatory drugs (NSAIDs)) among a
previously identified, population- based cohort of patients
first diagnosed with polymyalgia rheumatica (PMR) between 1970
and 1991 who were followed up over the long term.
Methods. Information on demographics, PMR diagnosis,
disease course, and drug therapy, in addition to data on
adverse events commonly associated with CS and NSAID
treatment, was obtained from the Rochester Epidemiology
Project database. Cox proportional hazards and regression
analysis models were used to evaluate the relationship between
the occurrence of these events and therapy.
Results. Of the 232 patients (69 male, 163 female) included
in the study, the mean age at PMR diagnosis was 72.9 years,
the average followup was 8.0 years, and 30 patients were also
diagnosed with giant cell (temporal) arteritis. Among the 175
patients (49 male, 126 female) treated with CS, the mean
duration of CS therapy was 2.4 years, the average daily dose
was 9.6 mg, and the mean cumulative dose was 8.4 gm. In total,
65% of the 124 patients treated with CS alone experienced at
least 1 adverse event, compared with 67% of the 57 patients
treated with NSAIDs alone and 80% of the 51 patients treated
with CS and NSAIDs. The average time from initiation of
therapy to the first adverse event was 1.6 years (n = 160).
Proportional hazards modeling identified 3 variables that
independently increased the risk of adverse events: age at PMR
diagnosis, a cumulative dose of prednisone less than or equal
to1,800 mg, and female sex. Person-year analysis revealed that
the risks of diabetes mellitus, vertebral fractures, femoral
neck fractures, and hip fractures were 2-5 times greater among
PMR patients compared with age- and sex-matched individuals
from the same population. Medical care or consultation by a
rheumatologist was a highly significant predictor of a lower
initial CS dose.
Conclusion. The use of CS and NSAIDs in the treatment of
PMR is associated with important long- term morbidity.
The sequential analysis of T lymphocyte subsets and
interleukin-6 in polymyalgia rheumatica patients as predictors
of disease remission and steroid withdrawal
Corrigall V.M.; Dolan A.L.; Dasgupta B.; Panayi G.S.
V.M. Corrigall, Rheumatology Unit, Thomas Guy House, Guy's
Hospital, London SE1 9RT United Kingdom
British Journal of Rheumatology (United Kingdom), 1997, 36/9
(976-980)
CD4 and CD8 T lymphocyte subsets, the late T cell
activation marker, HLA-DR, and serum interleukin-6 (IL-6)
levels of 57 polymyalgia rheumatica (PMR) patients were
followed over 2 yr to investigate whether they could be used
to predict the safe withdrawal of steroid therapy. Cell
phenotypes were studied by flow cytometry and IL-6 levels by
ELISA. %CD8 cells were reduced below the normal range in PMR
patients prior to steroid therapy. In 56% of patients, the
%CD8 T lymphocytes failed to return to normal levels when
quiescent disease allowed cessation of steroid therapy.
Activated CD8 T cells, as detected by HLA-DR positivity, were
above the normal range at the initiation of therapy and showed
a negative correlation with %CD8 T cells. The serum
concentration of IL-6 fluctuated over 24 months, and the
correlation between IL-6 and erythrocyte sedimentation rate
(ESR) seen prior to treatment was not seen at later intervals.
The %CD8 T cell and serum IL-6 levels are not a good indicator
of disease activity in PMR and are, therefore, unable to
predict the safe withdrawal of steroids.
Polymyalgia rheumatica - Therapy, course of
disease, complications
Ehlert A.; Stoyanova-Scholz M.
Dr. A. Ehlert, Rheumatologische Klinik, Stadt. Kliniken, Zu
den Rehwiesen 9, D-47055 Duisburg Germany
Geriatrie Forschung (Germany), 1997, 7/1 (13-18)
Polymyalgia rheumatica is a typical disease of the elderly.
If vascular complications of giant cell arteritis have not
developed before treatment starts, the prognosis is generally
favourable. However, the course of the disease is often
characterised by relapses requiring an increase in the
corticosteroid dosage. This is associated with a higher rate
of adverse reactions that modifies the otherwise favourable
prognosis. We evaluated the course of the disease in 78
polymyalgia rheumatica patients who were observed for a mean
of 28 plus or minus 20 months. Temporal arteritis was
histologically confirmed in 20 out of 71 patients (28%). Of
the 70 patients who were observed for more than six months, 18
(26%) suffered a relapse of corticosteroid dosages of 6.25
plus or minus 3.1 (0-12.5) mg. After 24 months 36% had been in
remission without treatment for a period of 9.3 plus or minus
6.1 (3-18) months. Therapy-associated complications arose in
21 (34%) of 64 patients who were observed for more than nine
months. The most common were steroid-induced diabetes mellitus
or aggravation of a known diabetic metabolic condition (33%).
The most severe adverse reaction osteoporotic vertebral
fracture - was reported in 3 patients. Further complictions of
therapy were various frequencies of arterial hypertension,
cataract, glaucoma, subjectively disturbing weight gain and
hypokalaemia. Overall, our data confirmed that the usually
favourable course of polymyalgia rheumatica is modified by
relapses and complications of therapy. Hence, we would tend to
reduce steroids or use immunosuppressants at an early stage,
especially in high risk cases such as patients with
inadequately controllable diabetes mellitus or manifest
osteoporosis.
Antibodies against Chlamydia pneumoniae,
cytomegalovirus, enteroviruses and respiratory syncytial virus
in patients with polymyalgia rheumatica
Uddhammar A.; Boman J.; Juto P.; Dahlqvist S.R.
Dr. A. Uddhammar, Department of Rheumatology, University
Hospital, S-901 85 Umea Sweden
Clinical and Experimental Rheumatology (Italy), 1997, 15/3
(299-302)
Objectives: To investigate the association between the
onset of polymyalgia rheumatica (PMR) and prior or persistent
infection with Chlamydia pneumoniae or cytomegalovirus (CMV)
(both known to infect the vessel wall) enteroviruses (EV) or
respiratory syncytial virus (RSV).
Methods: Serum samples were collected from 48 patients with
newly-diagnosed PMR and from 22 controls of the same age. The
presence of IgG, IgA and IgM antibodies to C. pneumoniae, IgG
and IgM antibodies to CMV and EV, and complement fixing
antibodies to RSV were analysed.
Results: Clinical symptoms of infection preceding PMR
symptoms were associated with the presence of synovitis at the
first visit. There were no significant differences in the
seroprevalence rates of antibodies to C. pneumoniae, CMV, EV
or RSV between PMR patients and controls. IgM antibodies to EV
were found in two patients and IgM antibodies to CMV in
another two patients.
Conclusion: Serological evidence of an association between
newly-diagnosed PMR and prior or chronic infection with C.
pneumoniae was not found. IgM antibodies to EV in two
patients, consistent with ongoing or recent infection, suggest
that EV could represent one of perhaps several microbes which
are able to trigger PMR.
False diagnosis: A common occurrence in autoimmune
diseases
Seitz M.
Switzerland
Schweizerische Medizinische Wochenschrift (Switzerland),
1997, 127/9 (349-354)
False diagnosis of autoimmune diseases may have many
different reasons. It may be caused by the relative rareness
of these rather complicated diseases, by sometimes mono- or
oligosymptomatic courses, and by the highly variable clinical
presentations. In addition, many physicians lack experience in
the treatment of these rheumatologic-immunological diseases. A
first shot diagnosis, inadequate technique in obtaining a
correct history or in performing a physical examination, as
well as an incomplete evaluation of differential diagnosis,
are possible causes of wrong diagnosis in the context of
autoimmune diseases and may delay effective therapy. To
illustrate these problems, three examples of patients with
autoimmune diseases are discussed, namely a patient with
polyarthritis and fever, a patient with polymyalgia
rheumatica, and a patient with an ulcerative skin lesion.
These examples serve to discuss the difficulty of correct
diagnosis in complicated courses of autoimmune diseases, which
are also relatively common in general practice.
Risk factors and predictive models of giant cell
arteritis in polymyalgia rheumatica
Rodriguez-Valverde V.; Sarabia J.M.; Gonzalez-Gay M.A.;
Figueroa M.; Armona J.; Blanco R.; Fernandez-Sueiro J.L.;
Martinez-Taboada V.M.
Dr. V. Rodriguez-Valverde, Rheumatology Division, Hosp.
Universitario 'M. Valdecilla', Av Valdecilla s/n, 39008
Santander Spain
American Journal of Medicine (USA), 1997, 102/4
(331-336)
OBJECTIVE: To identify in polymyalgia rheumatica the best
set of predictors for a positive temporal artery biopsy and to
define predictive models with either a high or low probability
of giant cell arteritis (GCA).
PATIENTS AND METHODS: Retrospective study of 227 patients,
137 with polymyalgia rheumatica unassociated with arteritis
(group A) and 90 with polymyalgia associated with
biopsy-proven giant cell arteritis (group B or training set).
Data on demographic features, clinical and laboratory
abnormalities were collected. Risk factors for arteritis were
estimated by nonlinear logistic regressions. Simple predictive
models were constructed with those predictors more related to
arteritis by multivariable analysis. These models were then
tested in group B and in 89 cases of arteritis without
polymyalgia rheumatica (group C or test set).
RESULTS: The best predictors of arteritis were a new
headache odds ratio (OR) 13.6 (95% confidence interval (CI)
4.7 to 39.3); age at onset < 70 years OR 0.11 (CI 0.04 to
0.35); abnormal temporal arteries OR 4.2 (CI 1.3 to 13.7);
raised liver enzymes OR 2.9 (CI 1.1 to 7.8), and jaw
claudication OR 4.8 (CI 1.0 to 22.7). Amaurosis was only
observed in patients with arteritis. Three subsets had a very
high risk of arteritis: (1) Patients with recent headache,
abnormal arteries, and less than or equal to70 years at
disease onset: sensitivity 44%, positive predictive value
(PPV) 93%, likelihood ratio (LR) 20.3; (2) patients with a new
headache, jaw claudication, and abnormal arteries: sensitivity
34.4%, PPV 96.9%, LR 47.2; and (3) those, that in addition to
the last 3 features, were less than or equal to70 years of age
at disease onset: sensitivity 26.7%, PPV 100%. We could also
identify a subset with a very low risk of arteritis
constituted by patients < 70 years, without headache, and
with clinically normal temporal arteries: sensitivity 1.1%,
PPV 1.7%, LR 0.03. In group C or the test set, these four
predictive models correctly identified 57.3%, 29.2%, 23.6, and
3.4% of patients, respectively.
CONCLUSIONS: In polymyalgia rheumatica it is feasible to
identify subsets with a very high likelihood of GCA. Although
in some of these subsets the diagnosis of arteritis is almost
certain, we suggest that even then it should be confirmed by
temporal artery biopsy. By contrast, in those patients with
polymyalgia < 70 years and without cranial features of
giant cell arteritis, the risk of vasculitis is so low that
the biopsy could be initially avoided and the patient treated
with low-dose corticosteroids.
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