Polymyalgia rheumatica

FRI0200 TIME TO FLARE IN GIANT CELL ARTERITIS AND POLYMYALGIA RHEUMATICA AND REVIEW OF THE LITERATURE

To elucidate epidemiologic and clinical manifestations of Japanese patients with giant cell arteritis (GCA), the first nationwide survey for GCA was conducted in 1998 in Japan. The first questionnaire on GCA for patients treated in 1997 was sent to 10,717 medical departments in Japan. A total of 177 patients were reported. Among the 177 patients, 66 GCA patients with detailed clinical and epidemiologic features on second survey were analyzed. Prevalence in patients 50 years of age and older in 1997 was 1.47 per 100,000 population in Japan. The average age at onset was 71.5 years old. The male:female ratio was 1:1.7. The association with permanent and complete visual loss (6.5%), jaw claudication (15.2%), and polymyalgia rheumatica (PMR) (30.3%) were low in frequency compared with those reported from other countries. All patients were treated with corticosteroids. Only 3 (4.5%) patients were reported as deceased due to other causes. The prevalence of GCA in Japan was revealed to be extremely low compared with other countries. Clinical findings of permanent and complete visual loss, jaw claudication, and PMR were infrequent among Japanese patients with GCA.

Giant cell arteritis (GCA) is a form a large- to medium-vessel vasculitis that involves the aorta and its branches. Polymyalgia rheumatica (PMR), a syndrome of muscle pain and stiffness in the neck, shoulders, and hips, often occurs with GCA but can occur independently. GCA predominantly affects the second- to fifth-order aortic branches, often in the extracranial arteries of the head. The mean age at diagnosis of GCA is approximately 72. The diagnosis of GCA is made usually by biopsy of the temporal artery. GCA associated with granulomatous inflammation within the blood vessel wall, but granulomatous changes are not always evident early in the disease. Giant cells occur in a slight majority of cases of positive temporal artery biopsies. Estimates of the frequency of giant cells in temporal artery biopsy tissues are on the order of 50–80%. In GCA, clinical symptoms of local vascular inflam-mation and vascular insufficiency are usually accompanied or preceded by a systemic inflammatory process. Visual loss is the most feared complication of GCA. Visual loss may occur through the syndrome of anterior ischemic optic neuropathy, caused by narrowing of the posterior ciliary artery, as well as through other arterial insufficiency syndromes. Glucocorticoids are the cornerstone of treatment for GCA and PMR. PMR occurring in the absence of GCA requires a lower dose of prednisone for disease control.

AB0382 DOES THE ULTRASOUND IMAGE OF THE LARGE VESSEL WALLS DIFFER IN THE SUBTYPES OF GCA AND PMR OR THERE IS A SYSTEMIC SUBCLINICAL COMMON INFLAMMATION?

AB0439 Contribution of anti-ferritin antibodies to the diagnosis of giant cell arteritis

On the 125th anniversary of the first recognised publication on polymyalgia rheumatica, a review of the literature was undertaken to assess what progress has been made from the point of view of the clinical care of affected patients. The authors searched Medline and PubMed using the search terms 'polymyalgia rheumatica', 'giant cell arteritis' and 'temporal arteritis'. As much as possible, efforts were made to focus on studies where polymyalgia rheumatica and giant cell arteritis were treated as separate entities. The selection of articles was influenced by the authors' bias that polymyalgia rheumatica is a separate clinical condition from giant cell arteritis and that, as yet, the diagnosis is a clinical one. Apart from the elevation of circulating acute phase proteins, which has been recognised as a feature of polymyalgia rheumatica for over 60 years, the diagnosis receives no significant help from the laboratory or from diagnostic imaging. This review has shown that, following the recognition of polymyalgia as a distinct clinical problem of the elderly, the results of a considerable amount of research efforts including those using the advances in clinical imaging technology over the past 60 years, have done little to change the ability of clinicians to define the disease more accurately. Since the introduction of corticosteroids in the 1950s, there has been also very little change in the clinical management of the condition. Polymyalgia rheumatica remains a clinical enigma, and its relationship to giant cell arteritis is no clearer now than it has been for the past 125 years. Diagnosing this disease is still almost exclusively dependent on the clinical acumen of a patient's medical attendant. Until an objective method of identifying it clearly in the clinical setting is available, uncovering the aetiology is still unlikely, and until then, preventing the pain and stiffness of the disease while avoiding the problems of prolonged exoposure to corticosteroids is likely to remain elusive or serendipitous.

SAT0171 FDG PET-CT in Polymyalgia Reumatica and Giant Cell Arteritis

Risk factors for visual loss in giant cell (temporal) arteritis: a prospective study of 174 patients

POS0808 CHARACTERISTICS OF GIANT CELL ARTERITIS FLARES AFTER SUCCESSFUL TREATMENT WITH TOCILIZUMAB: RESULTS FROM THE LONG-TERM EXTENSION OF A RANDOMIZED CONTROLLED PHASE 3 TRIAL

One of the unresolved challenges posed in giant cell (temporal) arteritis (GCA) is the detection and monitoring of large-artery complications, particularly aortitis. Recent investigations support vascular magnetic resonance imaging (MRI) studies in this issue. We report our preliminary experience with this imaging technique in the study of the aorta and its proximal branches in patients with GCA and/or polymyalgia rheumatica (PMR). Between 2000 and 2003, six patients with GCA and/or PMR seen in our department were diagnosed with aortitis using vascular MRI studies. In all cases, the study was performed according to a specifically designed protocol that included MRI and MR angiography (MRA). MRI was a hepful non-invasive method for diagnosis of aortitis in all cases, providing accurate information about its extent. In particular, MRI had a higher ability to detect earlier stages of vasculitis disclosing subclinical aortitis in five of the six patients. The main signs of early vascular inflammation observed were vessel wall thickness and oedema (six cases) and increased mural enhancement on postcontrast T1-weighted images (four cases). MRA disclosed lumen changes (stenosis) in two patients. On follow-up studies, whereas vascular stenosis and vessel wall thickness remained invariable, vascular wall oedema and contrast enhancement improved significantly when disease activity decreased. MRI may be a useful technique for diagnosing patients with occult major artery involvement in GCA, whether presenting with classic symptoms of temporal arteritis or PMR. Its utility for monitoring the course of the disease and response to treatment requires further confirmation.

Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) as 2 types of autoimmune diseases are frequently concomitant, and Mendelian randomization (MR) was applied in this study to assess the causal relationship between them. In this study, single-nucleotide polymorphism (SNP) was used as the instrumental variable for Mendelian analysis, and the SNP data of GCA and PMR were obtained from the FinnGen Biobank databases. SNPs are significantly correlated with GCA and PMR and were screened based on preset thresholds. Inverse variance weighted analysis was used as the main analysis, supplemented with MR-Egger and weighted median. The evidence of the impact of GCA on PMR risk was found in inverse variance weighted results (odds ratio, 1.22 [95% confidence interval, 1.11-1.34]; P < .01), and the evidence of the impact of PMR on GCA risk has also been found (odds ratio, 1.58 [95% confidence interval, 1.28-1.96]; P < .01). Finally, the stability and reliability of the results were tested using the retention method, heterogeneity test, and horizontal gene pleiotropy test. MR analysis indicates that GCA increases the risk of PMR and PMR is an important risk factor for GCA, with a causal relationship. The potential value of reasonable management of PMR in patients with GCA has received high attention. In addition, novel GCA therapeutics may be indicated for PMR, and it is a potential for further investigation.

Objectives: Giant cell arteritis (GCA) is a systemic disease with extensive vascular involvement. The aim of this study was to investigate the cumulative incidence of large vessel involvement (LVI) in GCA, the distribution of vessels involved, and predictors for LVI.Method: Patients with biopsy-proven GCA in a defined area in southern Sweden, diagnosed between 1997 and 2010, were identified through the register of the regional Department of Clinical Pathology. A structured review of all medical records and imaging and histopathology reports was performed. Imaging studies for an age- and sex-matched reference cohort were also reviewed.Results: A total of 164 patients with GCA were investigated, of whom 24 (15%) had LVI. LVI manifestations were detected a median of 3.7 [interquartile range (IQR) 0.7–7.5] years after GCA diagnosis. Aortic involvement was found in 16 patients (10%), mainly aneurysms/ectasias of the thoracic aorta. Two patients had aortic dissections. Fourteen patients had tributary involvement. In the reference population, the cumulative incidence of LVI overall was 10.8% and aortic involvement was found in 5.4%. The presence of giant cells in the biopsy was significantly less frequent among GCA patients with LVI (23% vs. 52%; p = 0.01), and a presentation with polymyalgia rheumatica (PMR) was more frequent (44% vs. 20%, p = 0.01).Conclusions: The estimated incidence of LVI, detected by imaging in a clinical setting, was higher among patients with GCA than the reference population. The aorta was the most commonly affected vascular territory. The negative association with giant cells may suggest particular mechanisms in this subset of GCA.

AB0465 ANALYSIS OF VASCULITIS PATTERNS IN PATIENTS WITH GIANT CELL ARTERITIS COMPARED TO PATIENTS WITH GIANT CELL ARTERITIS AND POLYMYALGIA RHEUMATICA

Giant cell arteritis (GCA) is a severe form of vasculitis in the elderly. The recent discovery of varicella zoster virus (VZV) in the temporal arteries and adjacent skeletal muscle of patients with GCA, and the rationale and strategy for antiviral and corticosteroid treatment for GCA are reviewed. The clinical features of GCA include excruciating headache/head pain, often with scalp tenderness, a nodular temporal arteries and decreased temporal artery pulsations. Jaw claudication, night sweats, fever, malaise, and a history of polymyalgia rheumatica (aching and stiffness of large muscles primarily in the shoulder girdle, upper back, and pelvis without objective signs of weakness) are common. ESR and CRP are usually elevated. Diagnosis is confirmed by temporal artery biopsy which reveals vessel wall damage and inflammation, with multinucleated giant cells and/or epithelioid macrophages. Skip lesions are common. Importantly, temporal artery biopsies are pathologically negative in many clinically suspect cases. This review highlights recent virological findings in temporal arteries from patients with pathologically verified GCA and in temporal arteries from patients who manifest clinical and laboratory features of GCA, but whose temporal artery biopsies (Bx) are pathologically negative for GCA (Bx-negative GCA). Virological analysis revealed that VZV is present in most GCA-positive and GCA-negative temporal artery biopsies, mostly in skip areas that correlate with adjacent GCA pathology. The presence of VZV in Bx-positive and Bx-negative GCA temporal arteries indicates that VZV triggers the immunopathology of GCA. However, the presence of VZV in about 20% of temporal artery biopsies from non-GCA postmortem controls also suggests that VZV alone is not sufficient to produce disease. Treatment trials should be performed to determine if antiviral agents confer additional benefits to corticosteroids in both Bx-positive and Bx-negative GCA patients. These studies should also examine whether oral antiviral agents and corticosteroids are as effective as intravenous acyclovir and corticosteroids. Appropriate dosage and duration of treatment also remain to be determined.

Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are inflammatory disorders predominantly affecting older adults. PMR is primarily characterized by proximal muscle pain and systemic inflammation, while GCA involves large-vessel inflammation, posing risks of vision loss and stroke. Due to overlapping clinical features and age-related prevalence, diagnosing these conditions remains challenging. While PMR is largely diagnosed based on clinical symptoms and laboratory markers, GCA often requires histopathological confirmation via temporal artery biopsy (TAB). This study aims to assess the clinical presentation, diagnostic approaches, and treatment outcomes of PMR and GCA in geriatric patients, emphasizing the role of clinical versus laboratory and histopathological diagnosis. A cross-sectional study was conducted at Khyber Teaching Hospital, Peshawar, Pakistan, from September 2023 to September 2024, involving 118 patients aged 50 years or older diagnosed with PMR and/or GCA. Data were collected through patient interviews, medical records, and diagnostic reports. Statistical analyses were performed to evaluate clinical characteristics, laboratory markers, and treatment responses. The mean age of participants was 68.5 ± 7.4 years, with 65% female. PMR diagnosis was based on clinical symptoms, supported by laboratory markers such as elevated CRP (85%) and ESR (90%). GCA was clinically suspected in 38 patients, with 76% confirmed through TAB. Additionally, 67% of GCA patients exhibited the "halo sign" on vascular ultrasound, while 12% required magnetic resonance angiography for further evaluation. Glucocorticoid therapy was initiated in all cases, leading to clinical remission in 84% of patients. However, 28% developed hyperglycemia, and 14% experienced osteoporosis as treatment-related complications. Delayed glucocorticoid initiation caused a significantly higher relapse rate (p = 0.04). Glucocorticoid therapy remains effective in most cases, though adverse effects necessitate careful monitoring. Tocilizumab was required in 18% of patients due to glucocorticoid resistance or intolerance, but it causedhigher relapse rates and metabolic complications. The findings emphasize the need for steroid-sparing therapies and personalized treatment approaches to improve long-term disease control. Future research should explore newer biologic treatments, optimize imaging strategies, and address resource limitations to enhance early detection and management outcomes in elderly patients.