Isolated Polymyalgia Rheumatica Research Articles

Isolated polymyalgia rheumatica: a cross-sectional, single-center, retrospective cohort study

Aim: The aim of the study was to review sociodemographic variables, clinical and laboratory findings, disease progression and treatment response of patients with isolated polymyalgia rheumatica and to investigate the effect of initial steroid dose and maintenance treatment on the progression of clinical and laboratory outcome. 
 Material and Method: The medical records of 39 polymyalgia rheumatic patients who followed up between 2001 and 2020 were screened. After excluding patients with overlapped giant cell arteritis and malignancies, 19 polymyalgia rheumatica patients (female:16 and male:3) were included in the study. Demographic data, anthropometric measurements, comorbidities, initial symptom, physical examination, clinical and laboratory (acute phase reactants) findings, medications, changes in treatment status, and cumulative steroid dose were recorded. The medical data of first (1st month), second (4th month), and third (7th month) visits were noted according to the structured form. 
 Results: The mean age was 68±8 years and the mean initial steroid dose was 21.5±8.9 mg. While the complaints regarding polymyalgia rheumatica decreased in 52.6% of patients (n=10) in the first visit, those complaints regressed in 47.4% (n=9) and 73.7% (n=14) of patients in the second and third visit, respectively. In the first visit (1st month), the mean erythrocyte sedimentation rate and C-reactive protein values were 21.7±12.3 mm/h and 5.8±5.1 mg/dl. In the second (4th month) and third visits (7th month), while the mean erythrocyte sedimentation rate was found as 36.5±27.3 mm/h and 27.3±18.6 mm/h, the mean C-reactive protein was 27.2±43.0 mg/dl and 17.6±30.2 mg/dl, respectively. Clinical remission was observed in 47.4% of patients (n=9) in the second visit and in 31.6% of patients (n=6) in the third visit. Median cumulative corticosteroid doses were 600 mg in first visit, 960 mg in second visit, and 1346 mg in the third visit. 
 Conclusion: The clinical characteristics and the initial steroid dose were compatible with the literature. Even though cumulative corticosteroid doses were quite high, lower remission rates were observed in these patients. The possible reason for this was considered to be the non-adherence of the patients to the treatment.

Fever Correlation with Erythrocyte Sedimentation Rate (ESR) and C-Reactive Protein (CRP) Concentrations in Patients with Isolated Polymyalgia Rheumatica (PMR): A Retrospective Comparison Study between Hospital and Out-of-Hospital Local Registries.

Background: Polymyalgia rheumatica (PMR) is the most common systemic inflammatory rheumatic disease affecting the elderly. Giant cell arteritis (GCA) is a granulomatous vasculitis affecting the aorta and its branches associated with PMR in up to 20% of cases. In recent studies based on university hospital registries, fever correlated with the erythrocyte sedimentation rate (ESR) but not with C-reactive protein (CRP) concentrations at the time of diagnosis in patients with isolated PMR. A long delay to a PMR diagnosis was suggested to explain this discrepancy, possibly caused by laboratory alterations (for instance, anemia of chronic disease type) that can influence only ESR. We performed a retrospective comparison study between the university hospital and two out-of-hospital public ambulatory databases, searching for any differences in fever/low-grade fever correlation with ESR and CRP. Methods: We identified all patients with newly diagnosed PMR between 2013 and 2020, only including patients who had a body temperature (BT) measurement at the time of diagnosis and a follow-up of at least two years. We considered BT as normal at <37.2 °C. Routine diagnostic tests for differential diagnostics were performed at the time of diagnosis and during follow-ups, indicating the need for more in-depth investigations if required. The GCA was excluded based on the presence of suggestive signs or symptoms and routine ultrasound examination of temporal, axillary, subclavian, and carotid arteries by experienced ultrasonographers. Patients with malignancies, chronic renal disease, bacterial infections, and body mass index (BMI) > 30 kg/m2 were excluded, as these conditions can increase CRP and/or ESR. Finally, we used the Cumulative Illness Rating Scale (CIRS) for quantifying the burden of comorbidities and excluded patients with a CIRS index > 4 as an additional interfering factor. Results: We evaluated data from 169 (73 from hospital and 96 from territorial registries) patients with newly diagnosed isolated PMR. Among these, 77.7% were female, and 61.5% of patients had normal BT at the time of diagnosis. We divided the 169 patients into two cohorts (hospital and territorial) according to the first diagnostic referral. Age at diagnosis, ESR, CRP, median hemoglobin (HB), and diagnostic delay (days from first manifestations to final diagnosis) were statistically significantly different between the two cohorts. However, when we assessed these data according to BT in the territorial cohort, we found a statistical difference only between ESR and BT (46.39 ± 19.31 vs. 57.50 ± 28.16; p = 0.026). Conclusions: ESR but not CRP correlates with fever/low-grade fever at the time of diagnosis in PMR patients with a short diagnosis delay regardless of HB levels. ESR was the only variable having a statistically significant correlation with BT in a multilevel regression analysis adjusted for cohorts (β = 0.312; p = 0.014).

The Role of Tumor Necrosis Factor Alpha Antagonists (Anti TNF-α) in Personalized Treatment of Patients with Isolated Polymyalgia Rheumatica (PMR): Past and Possible Future Scenarios.

Background: Glucocorticoids (GCs) are the cornerstone of polymyalgia rheumatica (PMR) therapy, but their long-term use (as is usually necessary in PMR patients) can induce many adverse events. Alternatives have long been sought. The primary aim of our narrative review is to provide an overview about the use of anti-tumor necrosis factor alpha (TNF-α) drugs in patients with PMR, and discuss advantages and disadvantages. Materials and methods: we performed a non-systematic literature search (PRISMA protocol not followed) on PubMed and Medline (OVID interface). Results and Conclusions: only two anti TNF-α drugs have been prescribed to PMR patients: infliximab in 62 patients and etanercept in 28 patients. These drugs were normally used in addition to GCs when significant comorbidities and/or relapsing PMR were present; less commonly, they were used as first-line therapy. In general, they have been scarcely successful in patients with PMR. Indeed, randomized controlled trials did not confirm the positive results reported in case reports and/or case series. However, an administration schedule and study design different from those proposed in the past could favour new scenarios in the interest of PMR patients.

Temporal artery biopsy for suspected giant cell arteritis: a retrospective analysis.

Temporal artery biopsy (TAB) is one of the diagnostic criteria of giant cell arteritis (GCA) according to 1990 ACR criteria and remains a tool for diagnosis. Although clinicians perform TAB with an intent to confirm suspected GCA, some biopsies result in negative and some lead to non-GCA diagnoses. We aim to review the diagnoses after TAB biopsy performed for suspected GCA and also wanted to evaluate the diagnostic changes and concomitant diseases that develop over time. The patients who had undergone TAB for suspected GCA were identified using the record entry code for TAB. Patients meeting the classification criteria for GCA were designated as the GCA group and not meeting criteria were designated as a non-GCA group. Other classification criteria were implemented for the non-GCA group diseases. A total of 51 patients (Female: 62.7%, median age: 72.1 ± 7.4years) who had undergone TAB for suspected GCA were evaluated. TAB was positive in 23 (69.6%) of the 33 patients who met the GCA classification criteria. No significant difference was found between TAB-positive and TAB-negative GCA patients in terms of clinical and laboratory parameters. In the non-GCA group, 12 patients had isolated polymyalgia rheumatica (PMR), and the diagnoses of the remaining six patients were as follows: four large vessel vasculitis (LVV) not satisfying GCA diagnostic criteria, one chronic myelomonocytic leukemia (CMML), and one amyloidosis. TAB was negative in all patients with isolated PMR. TAB showed primary amyloidosis in one patient. Out of 33 GCA patients, 21 had "isolated" GCA, four had GCA + Rheumatoid arthritis (RA), seven had GCA + PMR, and one had GCA + polymyositis. RA was diagnosed antecedent to GCA in two patients, and after GCA in the other two patients. One of the patients had developed GCA 20years after polymyositis had been diagnosed. TAB was found to be positive in two-thirds of patients with suspected GCA. Late-onset RA and rarely other inflammatory rheumatic diseases may develop in the course of GCA.

Negative temporal artery biopsy: predictive factors for giant cell arteritis diagnosis and alternate diagnoses of patients without arteritis.

To investigate whether among patients with a negative temporal artery biopsy (TAB) there are clinical features that may differentiate between patients with an eventual diagnosis of giant cell arteritis (GCA) and those without arteritis, and to assess the eventual diagnoses of patients without arteritis. Retrospective analysis of patients with a negative TAB performed between 1/1/2000 and 31/12/2015. Information collected included baseline clinical and laboratory data. Patients' final diagnoses were obtained from medical records. Patients eventually diagnosed with GCA were compared with those without arteritis, and predictive features for GCA diagnosis were assessed. A total of 154 patients with a negative TAB were included in the study. Among them, 31 (20%) were eventually diagnosed with GCA. The leading alternative diagnoses of patients without arteritis were self-limited disease (23%), isolated polymyalgia rheumatica (PMR) (18%), and neurological conditions (17%). In the multivariate analysis, predictors for diagnosis of GCA among patients with a negative TAB included PMR (OR = 2.86, 95% CI 1.06-7.69), platelet count (OR = 1.28, 95% CI 1.07-1.53), and ACR score > 2 (OR = 13.4, 95% CI 4.27-42.03). Among patients with a negative TAB, the best predictors for diagnosis of GCA are fulfillment of the ACR criteria, a clinical diagnosis of PMR, and high platelet levels. These features may aid in the diagnostic work-up of patients with a negative TAB.

Development of Giant Cell Arteritis after Treating Polymyalgia or Peripheral Arthritis: A Retrospective Case-control Study.

We investigated the development of giant cell arteritis (GCA) in patients with prior diagnoses of isolated polymyalgia rheumatica and/or peripheral arthritis (PMR/PA), and the potentially relevant characteristics of both illnesses in such patients. We retrospectively compared the features of 67 patients at the onset of PMR/PA, and their outcomes, to those of a random group of 65 patients with PMR/PA who did not develop late GCA. We also compared the features and outcomes of patients with late GCA to those of a random sample of patients with more usual GCA (65 with concurrent PMR/PA and 65 without). Patients with late GCA represented 7.4% of all patients with GCA included in a large hospital-based inception cohort. PMR/PA preceded overt GCA by 27 months on average. Permanent visual loss developed in 10 patients, including 8 of 48 (17%) patients featuring cranial arteritis. A questionable female predominance was the only distinguishing feature of PMR/PA evolving into GCA; late GCA more often featured subclinical aortitis (OR 6.42, 95% CI 2.39-17.23; p < 0.001), headache (OR 0.44, 95% CI 0.19-1.03; p = 0.06), and fever (OR 0.29, 95% CI 0.13-0.64; p = 0.002) less often compared to the more usual form of GCA. Patients with either form of GCA experienced similar outcomes. A cranial arteritis pattern of late GCA is associated with a significant risk for ischemic blindness. However, compared to the usual form of GCA, late GCA is often less typical, with a higher frequency of silent aortitis. Patients with relapsing/refractory PMR may not be at increased risk for late GCA.

MMP-3 can distinguish isolated PMR from PMR with GCA: A retrospective study regarding PMR and GCA in Japan

Objective. We sought to identify clinical features at diagnosis that can distinguish isolated polymyalgia rheumatica (PMR) without giant cell arteritis (GCA) from PMR with GCA, and clinical features at diagnosis of isolated PMR that can predict subsequent relapse and corticosteroid discontinuation.Methods. A retrospective study of 115 patients with isolated PMR and 29 patients with GCA was performed. A comparison between isolated PMR patients, GCA patients (with or without PMR), and PMR with GCA patients was performed. Predictors of relapse and corticosteroid discontinuation were identified using a logistic regression in the patients with isolated PMR.Results. Matrix metalloproteinase-3 (MMP-3) level was significantly different among the patient groups. MMP-3: 230.5 ± 201.5 ng/mL in isolated PMR, 80.5 ± 47.5 ng/mL in GCA (p < 0.01), and 96.8 ± 54.8 ng/mL in PMR with GCA (p = 0.03). In the patients with isolated PMR, female gender (odds ratio [OR], 2.73; 95% confidence interval [CI], 1.16–6.41; p < 0.05) and creatinine (Cr) < 50 μmol/L (OR, 2.48; 95% CI, 1.02–5.99; p < 0.05) were significant prognostic factors that predicted relapse.Conclusion. A low level of MMP-3 is an excellent positive predictor for PMR with GCA. Among patients with isolated PMR, female gender and Cr < 50 μmol/L were significant prognostic factors that predicted relapse.

Prevalence of ischemic complications in patients with giant cell arteritis presenting with apparently isolated polymyalgia rheumatica

ObjectiveTo investigate the frequency and type of giant cell arteritis (GCA)-related ischemic complications in a series of patients with GCA who, for a substantial period of time (i.e., at least 3mo), lacked vascular symptoms and presented with apparently isolated polymyalgia rheumatica (PMR). MethodsRetrospective follow-up study of an unselected population of 167 patients with GCA diagnosed from 1985 to 2014. ResultsIn all, 18 patients (11%) developed GCA on a background of a prior history of PMR. They were diagnosed as having isolated PMR because they did not have clinical evidence of GCA at diagnosis and exhibited a prompt and complete response to low-dose steroid therapy. However, during the course of treatment, 17 patients later experienced an arteritic relapse with the development of typical craniofacial symptoms, and one patient developed signs of upper extremity vascular insufficiency, resulting in the diagnosis of large-vessel GCA. The median time to GCA diagnosis from the initiation of low-dose steroid therapy was 9 ± 14.4mo (range: 3−39).At the time of GCA diagnosis, severe ischemic complications were observed in 50% (9/18) of the patients. Of these patients 22% (4/18) were considered to have “true” occlusive disease (i.e., permanent visual loss, stroke, and/or limb claudication).Late inflammation of the aorta and its branches occurred in 4 (22%) of the patients during long-term follow-up. ConclusionPatients with GCA presenting with apparently isolated PMR have a significant risk of developing transient or permanent disease-related ischemic complications; these complications occurred in 50% of the cases.

AB0589 Non-Infectious Aortitis: Still an Underdiagnosed Entity. Report of 32 Cases from A Single Center

BackgroundNon infectious aortitis may present as an idiopathic isolated condition or associated with a wide spectrum of diseases. Aortitis often presents with nonspecific symptoms leading in many cases to an...

AB0597 FDG PET-CT versus Temporal Artery Biopsy in Patients Presenting with Gca/Pmr: A Retrospective Cross-Sectional Study

BackgroundGiant Cell Arteritis (GCA) is a large vessel vasculitis, which can occur concomitant with Polymyalgia Rheumatica (PMR). Both diseases may also be seen as isolated disease entities. The two conditions...

SAT0174 Clinical and Histological Features in Patients with Permanent Visual Los Due to Biopsy-Proven Giant Cell Arteritis

BackgroundAmaurosis is the most frequent ischemic complication and the main cause of permanent disability among patients with giant cell arteritis (GCA). Some premonitory symptoms of visual loss such as amaurosis...

Imaging for large-vessel vasculitis

Ultrasonography, MRI, and PET are increasingly studied in large-vessel vasculitis. They have broadened our knowledge on these disorders and have a place in the diagnostic approach of these patients. Temporal artery ultrasonography can be used to guide the surgeon to that artery segment with the clearest 'halo' sign to perform a biopsy, or in experienced hands can even replace biopsy. The distal subclavian, axillary, and brachial arteries can also be examined. High-resolution MRI depicts superficial cranial and extracranial involvement patterns in giant cell arteritis (GCA). Contrast enhancement is prominent in active inflammation and decreases under successful steroid therapy. Presence of aortic complications such as aneurysm or dissection can be ruled out within the same investigation. Large thoracic vessel FDG-uptake is seen in the majority of patients with GCA, especially at the subclavian arteries and the aorta. FDG-PET cannot predict which patients are bound to relapse, and once steroids are started, interpretation is hazardous, which makes its role in follow-up uncertain. Increased thoracic aortic FDG-uptake at diagnosis of GCA may be a bad prognostic factor for later aortic dilatation. In patients with isolated polymyalgia rheumatica - who have less intense vascular FDG uptake - symptoms are caused by inflammation around the shoulders, hips, and spine. Ultrasonography, MRI, and PET remain promising techniques in the scientific and clinical approach of large-vessel vasculitis.

Development of Ischemic Complications in Patients With Giant Cell Arteritis Presenting With Apparently Isolated Polymyalgia Rheumatica

Several studies suggest that patients with giant cell arteritis (GCA) presenting with isolated polymyalgia rheumatica (PMR) with no cranial symptoms are at low risk of suffering GCA-related ischemic events. However, the issue remains controversial. In the current study we assessed the development of ischemic events in a large series of GCA patients who suffered from apparently isolated PMR during the main course of their disease. One hundred GCA patients presenting with PMR only for at least 2 months were selected from among 347 individuals with biopsy-proven GCA. Clinical manifestations and their chronologic appearance before diagnosis were recorded. Seventy-three patients presented with isolated PMR for a median of 8 months (range, 2 mo-5 yr) and later developed cranial symptoms for a median of 3 weeks (range, 0 wk-1 yr), which eventually led to GCA diagnosis (Group 1). The remaining 27 patients, after presenting a self-limiting course of dismissed mild cranial symptoms lasting for a median of 2 weeks (range, 1 wk-4 mo), developed PMR, which was their chief complaint for a median of 3 months (range, 2 mo-1.5 yr) and the reason for medical evaluation (Group 2). Twenty (27.4%) patients in Group 1 suffered disease-related ischemic complications at the time of diagnosis. No patient in Group 2 developed ischemic events. Patients with GCA presenting with apparently isolated PMR are not a benign subset and have a significant risk of developing ischemic complications. Among them, the only patients who appear to be at low risk of developing ischemic events are those in whom a self-limiting episode of cranial symptoms can be recorded.

Repetitive 18-fluorodeoxyglucose positron emission tomography in isolated polymyalgia rheumatica: a prospective study in 35 patients

To study fluorodeoxyglucose (FDG) deposition in different vascular beds and in the large joints of patients with isolated polymyalgia rheumatica (PMR), and to investigate whether there is a relation between FDG-positron emission tomography (PET) results and risk of relapse. All consecutive patients with isolated PMR underwent a FDG-PET scan before treatment with steroids was started and--if logistics allowed--at 3 and 6 months. PET scans were scored at seven different vascular areas and a total vascular score (TVS) was calculated, ranging from 0 to 21. FDG uptake in the shoulders, the hips and the processi spinosi of the vertebrae was scored as 0 (no uptake), 1 (moderate uptake) or 2 (intense uptake). Thirty-five patients entered the study. At diagnosis, vascular FDG uptake was noted in 11 patients (31%), predominantly at the subclavian arteries. Mean TVS was low. FDG uptake in the shoulders was noted in 94% of patients, in the hips in 89% and in the processi spinosi of the vertebrae in 51%. The intensity of FDG uptake in the large vessels or in the shoulders, hips or processi spinosi did not correlate with the risk of relapse. Only one in three patients has an (moderately) increased vascular FDG uptake, especially in the subclavian arteries. The vast majority has inflammation of shoulders and hips, and half of them have increased FDG-uptake at the processi spinosi. Results of FDG-PET scans in patients with PMR did not correlate with their risk of relapse.

PP1. REPETITIVE 18-FLUORODEOXYGLUCOSE POSITRON EMISSION TOMOGRAPHY IN ISOLATED POLYMYALGIA RHEUMATICA: A PROSPECTIVE STUDY IN 35 PATIENTS

Objective: To study fluorodeoxyglucose (FDG)-deposition in different vascular beds and in the large joints of patients with isolated polymyalgia rheumatica (PMR) and to investigate whether there is a relation between FDG-positron emission tomography (PET) results and risk of relapse. Methods: All consecutive patients with isolated PMR underwent a FDG- PET scan before treatment with steroids was started and – if logistics allowed – at 3 and 6 months. PET-scans were scored at 7 different vascular areas and a total vascular score (TVS) was calculated, ranging from 0 to 21. FDG uptake in the shoulders, the hips and the processi spinosi of the vertebrae was scored as 0 (no uptake), 1 (moderate uptake) or 2 (intense uptake). Results: Thirty-five patients entered the study. At diagnosis, vascular FDG-uptake was noted in 11 patients (31%), predominantly at the subclavian arteries. Mean TVS was low. FDG-uptake in the shoulders was noted in 94% of patients, in the hips in 89% and in the processi spinosi of the vertebrae in 51%. The intensity of FDG-uptake in the large vessels or in the shoulders, hips or processi spinosi did not correlate with the risk of relapse. Conclusions: Only 1 in 3 patients has a (moderately) increased vascular FDG-uptake, especially in the subclavian arteries. The vast majority has inflammation of shoulders and hips and half of them have increased FDG-uptake at the processi spinosi. Results of FDG-PET scans in patients with PMR do not correlate with their risk of relapse.

Audiovestibular manifestations in giant cell arteritis: a prospective study.

Giant cell arteritis (GCA) is a multisystemic vasculitis of elderly people that involves large and medium-sized blood vessels with predisposition to the cranial arteries. Some cranial ischemic manifestations, in particular permanent visual loss, have been widely described. Audiovestibular manifestations have been less commonly reported. In the present study we assessed the frequency and outcome of audiovestibular manifestations in a series of GCA and isolated polymyalgia rheumatica (PMR) patients examined prospectively between June 1999 and May 2001 at the single hospital for a defined population. Patients were included in the study if a temporal artery biopsy had been performed and they were examined within a week after beginning corticosteroid treatment. Patients with abnormal otoscopy or tympanogram, history of cerebrovascular complications, syphilis, Ménière and other vestibular syndromes, infections involving the inner ear, barotrauma, or being treated with ototoxic drugs were excluded. During the study period 44 patients with GCA and 10 patients with biopsy-negative isolated PMR were examined. Patients with isolated PMR were younger. Audiovestibular dysfunction was significantly more frequent in GCA patients than in those with isolated PMR and matched controls. Almost 90% of the GCA patients had vestibular dysfunction, which was generally reversible after several days of steroid treatment; after 3 months of treatment, vestibular dysfunction was observed in only 13 (29.6%) of the 44 GCA patients. These patients with persistent vestibular dysfunction were more likely to have persistent head-shaking nystagmus. Twelve (27.3%) of the 44 GCA patients had hearing improvement after 3 months of therapy. After 6 months of therapy, only 1 of the 44 GCA patients had abnormal vestibular tests. However, no additional improvement in hearing function was observed. The present study confirms a high frequency of audiovestibular manifestations in GCA. It also suggests that audiovestibular damage may be reversible in some patients with GCA.

Antiphospholipid antibodies and thrombophilic factors in giant cell arteritis

Objectives: To evaluate the prevalence of thrombophilic risk factors known to induce intravascular clotting and to assess their relationship with ischemic manifestations in giant cell arteritis (GCA). Methods: Eighty consecutive patients with established GCA were included: 36 with isolated temporal arteritis (TA), 14 with isolated polymyalgia rheumatica (PMR), and 30 with TA and PMR. Forty-four patients (67%) had ischemic phenomena due to GCA. Twelve patients (15%) had thrombotic events unrelated to GCA (6 strokes, 5 deep venous thrombosis, and 1 myocardial infarction). A control group of 100 age- and sex-matched individuals without autoimmune disease, bleeding disorders, thrombosis, or clinical picture of TA or PMR also was analyzed. All participants were tested for the antiphospholipid antibody (aPL) profile, protein C, protein S, antithrombin activity, factor V Leiden mutation, and prothrombin gene G20210A mutation. We also studied fibrinolysis parameters: plasminogen, tissue-type plasminogen activator (t-PA) antigen, t-PA activity, type-1 plasminogen activator inhibitor (PAI-1) antigen, PAI-1 activity, and the 4G/5G polymorphism of the promoter region of the PAI-1 gene. Results: Eleven patients (18%) tested positive for lupus anticoagulant, 24 (30%) for anticardiolipin antibodies, 9 (11%) for anti–β2-glycoprotein I antibodies, and 29 (36%) for antiprothrombin antibodies. No relationship was found between these autoantibodies and ischemic manifestations. None of the patients had decreased protein C, protein S or antithrombin activity. Two patients and 2 controls were heterozygous for factor V Leiden, and only 1 patient and 2 controls were heterozygous for the prothrombin gene G20210A mutation. No statistically significant correlation was found between any thrombophilic factor and GCA-related or GCA-unrelated ischemic events. Conclusion: GCA patients have a high prevalence of aPL that is not related to ischemic manifestations. Moreover, GCA-related or GCA-unrelated ischemic manifestations do not appear to be due to congenital thrombophilic risk factors. Semin Arthritis Rheum 31:12-20. Copyright © 2001 by W.B. Saunders Company

Absence of mitochondrial dysfunction in polymyalgia rheumatica: Evidence based on a simultaneous molecular and biochemical approach

Objective: To investigate the molecular and biochemical profile of skeletal muscle mitochondria of patients with isolated polymyalgia rheumatica (PMR). Patients and Methods: We included patients with a recent diagnosis of PMR and as control healthy individuals submitted to orthopedic surgery. Skeletal muscle was obtained from quadriceps, thus was mitochondria immediately isolated. Long polymerase chain reaction and Southern blot transference were performed to detect deleted mtDNA molecules. Mitochondrial oxidative activity using different substrates and individual enzyme activity of respiratory chain complexes were assessed to search for any biochemical dysfunction. Results: Fifty-one individuals (PMR=25, controls=26) were included. Mean age was 72 (11) years; 45% were females. We found no significant increase of deleted mtDNA molecules in PMR patients compared to controls. Both groups differed neither on oxygen consumption (p=NS for all substrates) nor enzymatic activity (p=NS for all complexes). Conclusions: Skeletal muscle mitochondria are molecularly and biochemically unaffected in PMR.

Clinical features in patients with permanent visual loss due to biopsy-proven giant cell arteritis.

The objective was to determine associated clinical findings in patients with visual loss due to giant cell arteritis (GCA) by means of a record review of 146 patients with biopsy-proven GCA. Twenty-three (15.75%) patients had lost vision. All of these patients complained of classical GCA cranial symptoms for an average of 1.3 months, 34.8% had an apparent isolated polymyalgia rheumatica for an average of 10.8 months and 65.2% had premonitory visual symptoms before visual loss for an average of 8.5 days. A clear delay in diagnosis and treatment was present in 15 patients (65.2%) who complained of at least two classical cranial symptoms for longer than 3 weeks and/or who had presented premonitory visual symptoms for longer than 72 h before blindness. Two additional patients lost vision while receiving standard steroid therapy. In conclusion, a high proportion of patients with permanent visual loss have a delayed diagnosis and treatment. A wider recognition of the disease would potentially reduce the prevalence of irreversible visual loss among GCA patients.

Von Willebrand factor in the outcome of temporal arteritis.

To determine fluctuation in circulating von Willebrand factor (vWF) in the outcome of patients with temporal arteritis. Plasma vWF antigen concentrations were measured in 65 patients with biopsy proven temporal arteritis at different disease activity stages, in 12 with isolated polymyalgia rheumatica, and in 16 controls. Fourteen temporal arteritis patients underwent serial determinations during the course of their disease. vWF concentrations were significantly raised in temporal arteritis (mean 220 [arbitrary units], range 96 to 720) and in polymyalgia rheumatica (mean 196, range 103 to 266) compared with healthy controls (mean 98, range 75 to 137) (P < 0.05). Although vWF values tended to be higher in temporal arteritis, no significant differences were found between temporal arteritis and polymyalgia rheumatica patients nor between temporal arteritis patients with ischaemic complications (mean 269, range 130 to 720) and those who presented with polymyalgia rheumatica or constitutional symptoms only (mean 179, range 140 to 220). The highest levels were obtained in patients with associated, mainly infectious, diseases (mean 631, range 240 to 1680). Raised vWF values found in active temporal arteritis patients (mean 220, range 96 to 720) persisted within the first two years after the beginning of treatment (mean 244, range 102 to 510) but tended to normalise in patients in long term remission (mean 143, range 50 to 260). Persistent elevation of vWF during early remission of temporal arteritis might represent an endothelial activation status induced by a remaining inflammatory microenvironment rather than a marker of endothelial cell injury. In long term remission, decreasing vWF concentrations might reflect progression of inflammatory lesions to a healing stage.