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INTRODUCTION: Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are frequently overlapping conditions. Unlike in GCA, vascular inflammation is absent in PMR. Therefore, serum biomarkers reflecting vascular remodelling could be used to identify GCA in cases of apparently isolated PMR. MATERIALS AND METHODS: 45 patients with isolated PMR and 29 patients with PMR/GCA overlap were included. Blood samples were collected before starting glucocorticoids for all patients. Serum biomarkers reflecting systemic inflammation (interleukin-6 (IL-6), CXCL9), vascular remodelling (MMP-2, MMP-3, MMP-9) and endothelial function (sCD141, sCD146, ICAM-1, VCAM-1, vWFA2) were measured by Luminex assays. RESULTS: Patients with GCA had higher serum levels of sCD141 (p=0.002) and CXCL9 (p=0.002) than isolated PMR. By contrast, serum levels of MMP-3 (p=0.01) and IL-6 (p=0.004) were lower in GCA than isolated PMR. The area under the curve (AUC) was calculated for sCD141, CXCL9, IL-6 and MMP-3. Separately, none of them were >0.7, but combinations revealed higher diagnostic accuracy. The CXCL9/IL-6 ratio was significantly increased in patients with GCA (p=0.0001; cut-off >32.8, AUC 0.76), while the MMP-3/sCD141 ratio was significantly lower in patients with GCA (p<0.0001; cut-off <5.3, AUC 0.79). In patients with subclinical GCA, which is the most difficult to diagnose, sCD141 and MMP-3/sCD141 ratio demonstrated high diagnostic accuracy with AUC of 0.81 and 0.77, respectively. CONCLUSION: Combined serum biomarkers such as CXCL9/IL-6 and MMP-3/sCD141 could help identify GCA in patients with isolated PMR. It could allow to select patients with PMR in whom complementary examinations are needed.
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Biomarcadores , Arterite de Células Gigantes , Interleucina-6 , Polimialgia Reumática , Humanos , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/sangue , Polimialgia Reumática/sangue , Polimialgia Reumática/diagnóstico , Biomarcadores/sangue , Feminino , Masculino , Idoso , Interleucina-6/sangue , Quimiocina CXCL9/sangue , Pessoa de Meia-Idade , Idoso de 80 Anos ou mais , Curva ROC , Metaloproteinase 3 da Matriz/sangue , Proteínas de Transporte VesicularAssuntos
Arterite de Células Gigantes , Polimialgia Reumática , Humanos , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/genética , Arterite de Células Gigantes/complicações , Polimialgia Reumática/diagnóstico , Polimialgia Reumática/genética , Análise da Randomização Mendeliana , Diagnóstico DiferencialRESUMO
Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are common conditions in older adults. Their clinical connection has been recognized over time, with many patients experiencing both conditions separately, simultaneously or in temporal sequence to each other. Early GCA detection is essential to prevent vascular damage, but identifying subclinical GCA in PMR patients remains a challenge and routine screening is not standard practice. Subclinical GCA prevalence in newly diagnosed PMR patients ranges from 23 to 29%, depending on the screening method. Vessel wall imaging and temporal artery biopsy can detect subclinical GCA. Epidemiology and trigger factors show similarities between the two conditions, but PMR is more common than GCA. Genetic and pathogenesis studies reveal shared inflammatory mechanisms involving dendritic cells, pro-inflammatory macrophages, and an IL-6 signature. However, the inflammatory infiltrates differ, with extensive T cell infiltrates seen in GCA while PMR shows an incomplete profile of T cell and macrophage-derived cytokines. Glucocorticoid treatment is effective for both conditions, but the steroid requirements vary. PMR overall mortality might be similar to the general population, while GCA patients with aortic inflammatory aneurysms face increased mortality risk. The GCA-PMR association warrants further research. Considering their kinship, recently the term GCA-PMR Spectrum Disease (GPSD) has been proposed.
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Arterite de Células Gigantes , Polimialgia Reumática , Humanos , Idoso , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/epidemiologia , Polimialgia Reumática/complicações , Polimialgia Reumática/diagnóstico , Polimialgia Reumática/tratamento farmacológico , Glucocorticoides/uso terapêuticoRESUMO
OBJECTIVES: To assess the accuracy of self-reported giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) diagnoses in a large French population-based prospective cohort, and to devise algorithms to improve their accuracy. METHODS: The E3N-EPIC cohort study (Etude Epidémiologique auprès des femmes de la Mutuelle Générale de l'Education Nationale) includes 98,995 French women born between 1925 and 1950, recruited in 1990 to study risk factors of cancer and chronic diseases. They completed biennially mailed questionnaires to update their health-related information and lifestyle characteristics. In three questionnaires, women could self-report a diagnosis of GCA/PMR. Those women were additionally sent a specific questionnaire, designed to ascertain self-reported diagnoses of GCA/PMR. Four algorithms were then devised to improve their identification. Accuracies of self-reported diagnoses and of each algorithm were calculated by comparing the diagnoses with a blinded medical chart review. RESULTS: Among 98,995 participants, 1,392 women self-reported GCA/PMR. 830 women sent back the specific questionnaire, and 202 women provided medical charts. After independent review of the 202 medical charts, 87.6 % of the self-reported diagnoses of GCA/PMR were accurate. Using additional data from a specific questionnaire (diagnosis confirmation by a physician, and self-report of >3-month of glucocorticoids), and from a reimbursement database (at least two deliveries of glucocorticoids in less than 3 consecutive months) improved their accuracy (91.8 % to 92.8 %). CONCLUSION: The accuracy of self-reported diagnosis of GCA/PMR was high in the E3N-cohort but using additional data as a specific GCA/PMR questionnaire and/or corticosteroid reimbursement database further improved this accuracy. With nearly 600 detected cases of GCA/PMR, we will be able to investigate risk factors for GCA/PMR in women.
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Arterite de Células Gigantes , Polimialgia Reumática , Humanos , Feminino , Polimialgia Reumática/diagnóstico , Polimialgia Reumática/epidemiologia , Polimialgia Reumática/complicações , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/epidemiologia , Arterite de Células Gigantes/etiologia , Autorrelato , Estudos de Coortes , Estudos ProspectivosRESUMO
OBJECTIVES: Diagnosing polymyalgia rheumatica (PMR) can be difficult as many conditions present with similar symptoms and findings. This study aimed to analyse how often the diagnosis of PMR changes during follow-up in a university hospital setting and to determine the most common clinical conditions initially misdiagnosed as PMR. METHOD: All patients with a new primary diagnosis of PMR on at least one visit during the years 2016-2019 were identified from the hospital discharge register of Turku University Hospital, Finland. A diagnosis of PMR was confirmed if the patient met at least one of the five classification criteria, complete clinical follow-up (median 34 months) was compatible with PMR, and no other diagnosis better explained their condition. RESULTS: Of the patients initially diagnosed with PMR, 65.5% were considered to have PMR after further evaluation and clinical follow-up. The most common conditions initially diagnosed as PMR were inflammatory arthritides (34.9%), degenerative or stress-related musculoskeletal disorders (13.2%), infection (9.3%), malignancy (9.3%), giant cell vasculitis (6.2%) and other vasculitis (6.2%), and a wide range of other less common diseases. The diagnosis of PMR remained in 81.3% of patients who fulfilled the 2012 American College of Rheumatology/European League Against Rheumatism PMR classification criteria and in 45.5% of patients who did not. CONCLUSIONS: Diagnosing PMR is challenging, even in a university hospital. One-third of the initial diagnoses of PMR changed during further evaluation and follow-up. There is a substantial risk of misdiagnosis, especially in patients with atypical presentation, and the differential diagnoses of PMR must be considered carefully.
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Arterite de Células Gigantes , Polimialgia Reumática , Humanos , Polimialgia Reumática/diagnóstico , Diagnóstico Diferencial , Finlândia/epidemiologia , Arterite de Células Gigantes/diagnóstico , HospitaisRESUMO
The combination therapy of atezolizumab, an anti-programmed cell death ligand-1 antibody, plus bevacizumab (Atz/Bev) is widely used to treat patients with advanced hepatocellular carcinoma (HCC). The development of polymyalgia rheumatica (PMR) during immune checkpoint inhibitor therapy for patients with HCC has not been reported to date. Two patients who developed PMR during Atz/Bev therapy for advanced HCC are reported. Both patients developed fever, bilateral symmetrical shoulder pain, morning stiffness, and an elevated C-reactive protein level. Their symptoms improved rapidly with prednisolone (PSL) 15-20 mg/d, and their C-reactive protein levels decreased. In PMR, long-term low-dose PSL should be administered. In the present patients who developed PMR as immune-related adverse events, starting with a small dose of PSL resulted in rapid improvement of symptoms.
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Carcinoma Hepatocelular , Neoplasias Hepáticas , Polimialgia Reumática , Humanos , Polimialgia Reumática/tratamento farmacológico , Polimialgia Reumática/diagnóstico , Carcinoma Hepatocelular/tratamento farmacológico , Bevacizumab/efeitos adversos , Proteína C-Reativa/uso terapêutico , Neoplasias Hepáticas/tratamento farmacológico , Prednisolona/uso terapêuticoRESUMO
Polymyalgia rheumatica (PMR) is a syndrome characterized by pain and morning stiffness in the neck and shoulder and pelvic girdles, as well as raised acute-phase reactants, with or without systemic symptoms, such as fever. Giant cell arteritis (GCA) is a systemic vasculitis of unclear etiology that involves systemic arteries, principally affecting medium- and large-sized arteries with skipped, segmental alterations and granulomatous vasculitis seen on histopathology. In China, epidemiological data describing GCA are still limited; thus, the prevalence might be underestimated. The involvement of vessels in GCA can cause irreversible visual impairment or loss and stroke, which are serious complications. PMR is three times more prevalent than GCA, and other specific diseases should be excluded before the diagnosis is established. PMR symptoms can be present in 40%-60% of patients with GCA. Conversely, GCA can develop in 15% of patients with PMR. Chinese Rheumatology Association, based on the clinical diagnosis and treatment guidelines in 2005, utilizing the experience and guidelines of diagnosis and treatment at home and abroad, formulated this specification to standardize the diagnosis and treatment of GCA and PMR and improve the patient's prognosis.
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Arterite de Células Gigantes , Polimialgia Reumática , Humanos , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/tratamento farmacológico , Polimialgia Reumática/complicações , Polimialgia Reumática/diagnóstico , Polimialgia Reumática/tratamento farmacológico , Mialgia , Prognóstico , Artérias/patologiaRESUMO
A 77-year-old man arrived at our hospital with bilateral shoulder pain persisting for several months and headache for 1 month. Giant cell arteritis with polymyalgia rheumatica was suspected. However, considering his medical history of testing positive for syphilis, we submitted a sample for a syphilis serology test, which yielded positive results. The Treponema pallidum hemagglutination assay of cerebrospinal fluid was positive, and a temporal artery biopsy revealed vasculitis, confirming the diagnosis of tertiary syphilis. He was successfully treated for two weeks with penicillin G infusions. Symptoms reminiscent of giant cell arteritis and polymyalgia rheumatica may reveal syphilis, which is called the "great imitator."
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Arterite de Células Gigantes , Polimialgia Reumática , Sífilis , Masculino , Humanos , Idoso , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/tratamento farmacológico , Polimialgia Reumática/diagnóstico , Sífilis/complicações , Sífilis/diagnóstico , Sífilis/tratamento farmacológico , Treponema pallidum , Artérias Temporais/patologiaRESUMO
Giant cell arteritis (GCA) is closely associated with polymyalgia rheumatica (PMR). We herein report an 82-year-old woman who developed GCA during PMR treatment. She initially presented with shoulder pain and was diagnosed with PMR based on elevated serum C-reactive protein (CRP) levels and bursitis detected in both shoulders on ultrasonography (US). Treatment was initiated with a daily dose of 15 mg prednisolone (PSL), which led to rapid symptom alleviation, and the dosage was tapered to 1 mg/day. One month later, she developed myalgia extending from the lumbar region to the thigh and tenderness in the left temporal region. However, no abnormalities in the temporal artery were observed on US. Although the PSL dose was increased to 2 mg for relapse of PMR, the symptoms did not improve. One week later, she developed occipital pain with an increased CRP level of 9 mg/dL. She was diagnosed with GCA based on the 1990 ACR Classification Criteria. Fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT) detected anomalous accumulations in the bilateral superficial temporal and vertebral arteries, but not in the larger vessels. We therefore diagnosed her with cranial-type GCA. At this time point, we repeated US and found a halo sign in the temporal artery. Although epithelioid and giant cells were not observed in the temporal artery biopsy, vascular inflammatory findings such as disruption of the internal elastic lamina and chronic inflammatory cell infiltration were noted. Symptoms improved immediately and CRP levels decreased after the PSL dose was increased to 30 mg daily. To mitigate the risk of steroid-induced diabetes, tocilizumab was introduced, and gradual tapering of PSL was implemented. In conclusion, we encountered a case of GCA that developed after PSL reduction during the course of PMR. PET/CT confirmed intracranial artery inflammation and facilitated a definitive diagnosis. Although PET/CT cannot be routinely performed for diagnose in Japan, we consider it useful as an adjunctive diagnostic tool.
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Arterite de Células Gigantes , Polimialgia Reumática , Humanos , Feminino , Idoso de 80 Anos ou mais , Arterite de Células Gigantes/tratamento farmacológico , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/diagnóstico , Polimialgia Reumática/tratamento farmacológico , Polimialgia Reumática/complicações , Polimialgia Reumática/diagnóstico , Prednisolona/efeitos adversos , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Redução da Medicação , InflamaçãoRESUMO
BACKGROUND: Polymyalgia rheumatica and giant cell arteritis are systemic inflammatory conditions of the elderly. Polymyalgia rheumatica classically presents as a bilateral proximal muscle pain and stiffness syndrome. Biceps tenosynovitis is the commonest pathology in polymyalgia rheumatica. However according to literature, erosive sternoclavicular arthritis is a rare association of polymyalgia rheumatica. Giant cell arteritis is an inflammatory granulomatous arteritis predominantly involving large cerebral arteries. Thus, its classic clinical presentation includes severe headache with scalp tenderness, jaw claudication, and sudden painless loss of vision. Urological manifestations (prostatic vasculitis and epididymo-orchitis) were seldom reported in giant cell arteritis. CASE PRESENTATION: A 53-year-old Sinhalese man presented with progressive right-sided shoulder joint pain and neck pain associated with constitutional symptoms and episodic generalized headache. Examination revealed restricted movements of the right shoulder joint with nontender pulsatile bilateral temporal arteries. Blood testing showed elevated erythrocyte sedimentation rate and C-reactive protein. Color Doppler ultrasound of the superficial temporal artery revealed "halo sign." The temporal artery showed infiltration of mononuclear cells in the arterial media and adventitia. Computed tomography revealed right sternoclavicular arthritis with incidental finding of ureteric stricture. The patient was treated with high-dose oral prednisolone, and good clinical and biochemical response was observed during follow-up. CONCLUSION: Polymyalgia rheumatica-giant cell arteritis may rarely present as erosive sternoclavicular arthritis as the initial manifestation, mimicking many rheumatological conditions. Urological involvement such as ureteric strictures may be rare associations of primary systemic vasculitis. A high degree of suspicion combined with targeted investigations would allow early identification the polymyalgia rheumatica-giant cell arteritis syndrome in the presence of atypical manifestations, leading to improved patient outcomes.
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Artrite , Arterite de Células Gigantes , Polimialgia Reumática , Masculino , Humanos , Idoso , Pessoa de Meia-Idade , Polimialgia Reumática/complicações , Polimialgia Reumática/diagnóstico , Polimialgia Reumática/tratamento farmacológico , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/tratamento farmacológico , Artérias Temporais , Artrite/complicações , CefaleiaRESUMO
BACKGROUND: Giant cell arteritis (GCA) is a large vessel vasculitis that may cause significant morbidity in the elderly population. We aimed to evaluate presentation characteristics, treatment, and outcome in a cohort of patients with GCA diagnosed and followed in a single center. METHODS: A retrospective chart review revealed 84 (41 M/43 F) registered patients diagnosed with GCA between 1990 and 2020. Clinical features at presentation and follow-up, radiographical imaging, temporal artery biopsy (TAB), and laboratory findings were retrieved from digital medical records or hard-copy patient files. Of these, 33 patients' follow-up period was less than 12 months; hence, relapses and treatment outcomes were examined in the remaining 51 (60.5%) patients. RESULTS: A total of 84 patients were included in the cohort. The mean age at diagnosis was 68.4 ± 7.9 years (range: 49-85). At presentation, 60 (71.4%) patients had headache, 22 (26.2%) had symptoms compatible with polymyalgia rheumatica (PMR), and 23 (27.4%) had visual loss. Three (3.6%) patients had solid organ malignancies while two had hematologic malignancies (2.4%) before GCA diagnosis. TAB was obtained in 63 (75%) patients, in 47 of whom (74.6%) the pathological findings were consistent with GCA. A PET/ CT scan has been performed before glucocorticoids (GCs) initiation in 43 (51.2%) patients and of these, 37 (86.0%) revealed uptake consistent with large vessel involvement. The median follow-up time of the 51 patients was 3.7 (IQR: 1.8-6.8) years. GCs were started promptly after the diagnosis. During the follow-up period, 28 (54.9%) patients experienced a relapse. Thirty-nine (78%) patients were under GC treatment, with a mean dosage of 4.8 ± 2.8 g/day at the final visit. At the final visit, 20.3% (17:84) had died whereas 9.8% (5:51) had permanent vision loss. DISCUSSION: Treatment of GCA is challenging. GCA causes serious morbidities and increased mortality. PET/CT is highly effective in detecting large vessel vasculitis in GCA and could perhaps replace TAB in the future.
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Arterite de Células Gigantes , Polimialgia Reumática , Humanos , Idoso , Pessoa de Meia-Idade , Idoso de 80 Anos ou mais , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/epidemiologia , Arterite de Células Gigantes/tratamento farmacológico , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Estudos Retrospectivos , Recidiva Local de Neoplasia , Polimialgia Reumática/diagnóstico , Polimialgia Reumática/tratamento farmacológico , Polimialgia Reumática/epidemiologia , Glucocorticoides/uso terapêuticoRESUMO
Polymyalgia rheumatica and giant cell arteritis are inflammatory conditions that occur predominantly in people 50 years and older, with peak incidence at 70 to 75 years of age. Polymyalgia rheumatica is more common and typically presents with constitutional symptoms, proximal muscle pain, and elevated inflammatory markers. Diagnosis of polymyalgia rheumatica is clinical, consisting of at least two weeks of proximal muscle pain, constitutional symptoms, and elevated erythrocyte sedimentation rate or C-reactive protein. Treatment of polymyalgia rheumatica includes moderate-dose glucocorticoids with a prolonged taper. Giant cell arteritis, also known as temporal arteritis, usually presents with new-onset headache, visual disturbances or changes, constitutional symptoms, scalp tenderness, and temporal artery symptoms. Inflammatory markers are markedly elevated. Temporal arterial biopsy should be used for diagnosis. However, color duplex ultrasonography, magnetic resonance imaging, and fluorodeoxyglucose positron emission tomography may be helpful when biopsy is negative or unavailable. All patients with suspected giant cell arteritis should receive empiric high-dose glucocorticoids because the condition may lead to blindness if untreated. Tocilizumab is approved by the U.S. Food and Drug Administration for giant cell arteritis and should be considered in addition to glucocorticoids for initial therapy. Polymyalgia rheumatica and giant cell arteritis respond quickly to appropriate dosing of glucocorticoids but typically require prolonged treatment and have high rates of relapse; therefore, monitoring for glucocorticoid-related adverse effects and symptoms of relapse is necessary. Methotrexate may be considered as an adjunct to glucocorticoids in patients with polymyalgia rheumatica or giant cell arteritis who are at high risk of relapse.
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Arterite de Células Gigantes , Polimialgia Reumática , Humanos , Polimialgia Reumática/diagnóstico , Polimialgia Reumática/tratamento farmacológico , Polimialgia Reumática/epidemiologia , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/tratamento farmacológico , Glucocorticoides/uso terapêutico , Metotrexato , Proteína C-Reativa , Mialgia , RecidivaRESUMO
Importance: Few treatments are available for patients with glucocorticoid-dependent polymyalgia rheumatica. IL-6 antagonists may reduce disease activity in patients with active glucocorticoid-dependent polymyalgia rheumatica. Objective: To compare the efficacy of tocilizumab vs placebo in patients with glucocorticoid-dependent polymyalgia rheumatica. Design, Setting, and Participants: This double-blind, parallel-group, placebo-controlled randomized clinical trial enrolled 101 patients with polymyalgia rheumatica at 17 hospitals in France from February 2017 to October 2019. Final follow-up occurred in November 2020. Inclusion criteria were persistent disease activity (polymyalgia rheumatica activity score computed using the C-reactive protein level [CRP PMR-AS] >10) and prednisone dose greater than or equal to 10 mg per day. Interventions: Patients were randomly assigned to receive intravenous tocilizumab (8 mg/kg; n = 51) or placebo (n = 50) every 4 weeks for 24 weeks, combined with predefined standardized tapering of oral prednisone. Main Outcomes and Measures: The primary efficacy end point was CRP PMR-AS less than 10 (range, 0-100; higher values indicate greater activity; no minimal clinically important difference defined) combined with either prednisone dose less than or equal to 5 mg per day or a decrease in prednisone dose greater than or equal to 10 mg from baseline at week 24. There were 11 secondary outcomes assessed at week 24 included in this report, including disease activity (measured by CRP PMR-AS) and the proportion of patients no longer taking prednisone. Results: Of the 101 randomized patients (mean age, 67.2 years; 68 [67.3%] women), 100 (99%) received at least 1 infusion and 100 completed the trial. The primary end point was achieved in 67.3% of patients in the tocilizumab group and 31.4% of patients in the placebo group (adjusted difference, 36.0% [95% CI, 19.4%-52.6%]; adjusted relative risk, 2.3 [95% CI, 1.5-3.6]; P < .001). Of 11 reported secondary end points at 24 weeks, 7 showed significant differences favoring tocilizumab, including mean CRP PMR-AS score (7.5 [95% CI, 5.4-9.6] vs 14.9 [95% CI, 11.4-18.4]; adjusted difference, -7.5 [95% CI, -11.2 to -3.8]; P < .001) and the percentage of patients no longer receiving prednisone (49.0% vs 19.6%; adjusted difference, 29.3% [95% CI, 18.9%-39.7%]; adjusted relative risk, 2.5 [95% CI, 1.8-3.5]; P < .001). The most frequent adverse events were infections, experienced by 23 patients (46.9%) in the tocilizumab group and 20 (39.2%) in the placebo group. Conclusions and Relevance: Among patients with active polymyalgia rheumatica despite prednisone therapy, tocilizumab, compared with placebo, resulted in a significantly greater percentage of patients with a CRP PMR-AS less than 10 with reduced prednisone requirements at week 24. Further research is needed to confirm efficacy and to determine the balance of potential benefits and harms. Trial Registration: ClinicalTrials.gov Identifier: NCT02908217.
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Anti-Inflamatórios , Anticorpos Monoclonais Humanizados , Glucocorticoides , Polimialgia Reumática , Prednisona , Administração Intravenosa , Administração Oral , Idoso , Anti-Inflamatórios/administração & dosagem , Anti-Inflamatórios/efeitos adversos , Anti-Inflamatórios/uso terapêutico , Anticorpos Monoclonais Humanizados/administração & dosagem , Anticorpos Monoclonais Humanizados/uso terapêutico , Proteína C-Reativa/análise , Método Duplo-Cego , Redução da Medicação , Feminino , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/tratamento farmacológico , Glucocorticoides/administração & dosagem , Glucocorticoides/efeitos adversos , Glucocorticoides/uso terapêutico , Humanos , Interleucina-6/antagonistas & inibidores , Masculino , Polimialgia Reumática/diagnóstico , Polimialgia Reumática/tratamento farmacológico , Prednisona/administração & dosagem , Prednisona/efeitos adversos , Prednisona/uso terapêuticoRESUMO
Polymyalgia rheumatica (PMR) is the most common inflammatory rheumatic disease affecting people older than 50 years and is 2-3 times more common in women. The most common symptoms are pain and morning stiffness in the shoulder and pelvic girdle and the onset may be acute or develop over a few days to weeks. General symptoms such as fatigue, fever and weight loss may occur, likely driven by systemic IL-6 signalling. The pathology includes synovial and periarticular inflammation and muscular vasculopathy. A new observation is that PMR may appear as a side effect of cancer treatment with checkpoint inhibitors. The diagnosis of PMR relies mainly on symptoms and signs combined with laboratory markers of inflammation. Imaging modalities including ultrasound, magnetic resonance imaging and positron emission tomography with computed tomography are promising new tools in the investigation of suspected PMR. However, they are still limited by availability, high cost and unclear performance in the diagnostic workup. Glucocorticoid (GC) therapy is effective in PMR, with most patients responding promptly to 15-25 mg prednisolone per day. There are challenges in the management of patients with PMR as relapses do occur and patients with PMR may need to stay on GC for extended periods. This is associated with high rates of GC-related comorbidities, such as diabetes and osteoporosis, and there are limited data on the use of disease-modifying antirheumatic drugs and biologics as GC sparing agents. Finally, PMR is associated with giant cell arteritis that may complicate the disease course and require more intense and prolonged treatment.
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Antirreumáticos , Produtos Biológicos , Arterite de Células Gigantes , Polimialgia Reumática , Antirreumáticos/uso terapêutico , Produtos Biológicos/uso terapêutico , Biomarcadores , Feminino , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/tratamento farmacológico , Glucocorticoides/uso terapêutico , Humanos , Inflamação/complicações , Interleucina-6 , Polimialgia Reumática/complicações , Polimialgia Reumática/diagnóstico , Polimialgia Reumática/tratamento farmacológico , Prednisolona/uso terapêuticoRESUMO
OBJECTIVE: Giant cell arteritis (GCA) is the most frequent vasculitis affecting adults aged > 50 years. Cardiac involvement in GCA is considered rare, and only a few cases of pericarditis have been reported. The aim of this study was to determine the characteristics and prognosis of GCA patients suffering from pericardial involvement at diagnosis. METHODS: We conducted a single-centre, retrospective chart review of patients with GCA in internal medicine departments (from 2000 to 2020). Patients were identified through a centralized hospital database. We retrospectively collected demographic, clinicobiological, histological, imaging, treatment and outcome data. Patients with pericardial effusion, defined as an effusion visible on the CT-scan performed at GCA diagnosis were compared to those without pericardial involvement. RESULTS: Among the 250 patients with GCA, 23 patients (9.2%) had pericardial effusion on CT-scan. The comparison between the groups revealed similar distribution of age, gender, cranial symptoms and ocular ischaemic complications. Patients with pericardial effusion had a higher frequency of weight loss. They also had lower haemoglobin levels and higher platelet levels (p = 0.006 and p = 0.002, respectively), and they more frequently had positive temporal artery biopsy. There were no differences concerning the treatment, relapses, follow-up duration or deaths. CONCLUSIONS: This case series sheds light on GCA as a cause of unexplained pericardial effusion or symptomatic pericarditis among adults aged > 50 years and elevated inflammatory biological markers. Fortunately, pericardial involvement is a benign GCA manifestation. In that context, the search for constitutional symptoms, cranial symptoms and associated signs of polymyalgia rheumatica is crucial for rapidly guiding GCA diagnosis.
Assuntos
Arterite de Células Gigantes , Derrame Pericárdico , Pericardite , Polimialgia Reumática , Biomarcadores , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/diagnóstico por imagem , Hemoglobinas , Humanos , Derrame Pericárdico/diagnóstico por imagem , Derrame Pericárdico/etiologia , Pericardite/complicações , Polimialgia Reumática/diagnóstico , Estudos RetrospectivosRESUMO
BACKGROUND: It has been over a year since the first documented case of the COVID-19 virus was recorded. Since then, our understanding of this virus has continually evolved, however, its wide-ranging effects are still unfolding. Similar to previously studied viral infections, severe acute respiratory syndrome-associated coronavirus-2 (SARS-CoV-2) has been shown to lead to a degree of autoimmunity in patients who are recovering from its effects. Due to its effects on the innate immune system, such as the toll-like receptors and complement system, a varying degree of proinflammatory markers can become widespread in those who continue to recover from the virus. This case series offers a unique perspective on how COVID-19 has had dramatic effects on those already suffering from inflammatory rheumatic conditions, such as rheumatoid arthritis, systemic lupus erythematosus, or fibromyalgia. As the ever-lasting effects of COVID-19 are still unfolding, this case series is one of few to discuss the development and changes of patients with rheumatic conditions. This study hopes to encourage larger studies to be conducted on the effects of COVID- 19 on autoimmune conditions. CASE PRESENTATION: Seven patients were identified with new manifestations of rheumatic conditions, which included 3 cases of rheumatoid arthritis, 2 cases of polymyalgia rheumatica, 1 case of reactive arthritis, and 1 case of cutaneous lupus. Post-COVID syndrome was also diagnosed in 7 other patients. Patients with rheumatoid arthritis presented with symptoms 4-5 weeks after being diagnosed with COVID-19. Symptoms of polyarticular joint pain, swelling, and morning stiffness were reported in this group. These patients were treated with disease-modifying anti-rheumatic drugs and experienced an improvement in symptoms on follow-up. Two cases of polymyalgia rheumatica were identified in patients that were previously diagnosed with COVID-19 six weeks prior. One patient had no significant past medical history and the other patient had a history of rheumatoid arthritis, which was well controlled. These patients experienced weakness and tenderness in the proximal joints with elevated levels of ESR and CRP. They were treated with prednisone and showed improvement. Reactive arthritis was diagnosed in 1 patient who presented with swelling in both hands and wrists 2 days after being diagnosed with COVID-19. This patient began to experience symptoms of reactive arthritis 2 days after resolution of initial COVID-19 symptoms and this persisted for 3 months. The patient was managed with methylprednisolone injections and NSAIDs, which improved her symptoms. Post-COVID syndrome was identified in 7 patients. All patients were female and had a history of well-controlled fibromyalgia. Patients generally experienced fatigue, headaches, and memory fog, which had variable onset from a few days and up to 4 weeks after being diagnosed with COVID-19. One patient had a complete recovery of her symptoms at follow-up 3 months after the initial presentation. The other 6 patients continued to report symptoms of post-COVID syndrome at follow-up. Patients were managed with lifestyle modifications and their previous fibromyalgia treatment. CONCLUSION: While cases of COVID-19 continue to rise, complications of this disease are still being discovered. Those who initially recover from COVID-19 may experience new-onset rheumatic conditions, worsening of previously diagnosed rheumatic conditions, or post-COVID syndrome. As we continue to learn more about the effects of COVID-19, the awareness of these manifestations will play a key role in the appropriate management of these patients.
Assuntos
Antirreumáticos , Artrite Reativa , Artrite Reumatoide , COVID-19 , Fibromialgia , Polimialgia Reumática , Doenças Reumáticas , Humanos , Feminino , Masculino , COVID-19/complicações , Polimialgia Reumática/complicações , Polimialgia Reumática/diagnóstico , Polimialgia Reumática/tratamento farmacológico , SARS-CoV-2 , Fibromialgia/complicações , Artrite Reativa/tratamento farmacológico , Prednisona/uso terapêutico , Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Doenças Reumáticas/tratamento farmacológico , Metilprednisolona/uso terapêutico , Anti-Inflamatórios não Esteroides/uso terapêuticoRESUMO
Inflammatory pseudotumors of the liver are rare, non-neoplastic liver tumors. Due to the nonspecific clinical presentation, imaging features, and histopathological findings, they can mimic malignant tumors requiring invasive diagnostics. We present a case of a 61-year-old female patient with a history of type 2 diabetes mellitus, hypothyroidism, hyperlipidemia, and polymyalgia rheumatica who had initially presented with abdominal pain for 3 weeks. Further workup showed normal liver chemistries and tumor markers: AFP and CA 19-9. Magnetic resonance imaging (MRI) of the abdomen showed a segment 6 lesion measuring 4.1 × 4.0 × 3.7 cm. A liver biopsy then confirmed the diagnosis of an inflammatory pseudotumor of the liver with negative IgG4. On follow-up imaging, a rapid growth of this liver lesion was noted. Laparoscopy was done but did not show any distinct liver lesion. Follow-up imaging confirmed a decrease in the size of the mass. Interestingly, the patient had been on a higher dose of steroids for her polymyalgia rheumatic leading up to the follow-up imaging. This is the first case of an inflammatory pseudotumor of the liver in a patient with polymyalgia rheumatica. With this case, we would like to increase the awareness for inflammatory pseudotumors of the liver as a differential diagnosis of liver lesions in patients with underlying autoimmune disorders.
Assuntos
Diabetes Mellitus Tipo 2 , Arterite de Células Gigantes , Granuloma de Células Plasmáticas , Polimialgia Reumática , Diabetes Mellitus Tipo 2/complicações , Feminino , Granuloma de Células Plasmáticas/complicações , Granuloma de Células Plasmáticas/diagnóstico , Humanos , Fígado , Pessoa de Meia-Idade , Polimialgia Reumática/complicações , Polimialgia Reumática/diagnósticoRESUMO
Elderly-onset rheumatoid arthritis (EORA) is characterized by acute onset and clinical features of high disease activity. Anti-cyclic citrullinated peptide antibody (ACPA) positivity or the presence of bone erosions predicts a radiological joint destruction of EORA, but ACPA-negative EORA with a polymyalgia rheumatica (PMR) phenotype may also present. Biological disease-modifying antirheumatic drugs (bDMARDs) and Janus kinase inhibitors were beneficial both in older and in younger patients in terms of risk-benefit balance. Implementation of a treat-to-target strategy could improve EORA outcomes, but older patients have more age-related comorbidities and interstitial lung disease than younger patients. Baseline comorbidities, more frequent methotrexate dose-dependent adverse events, serious infections, cardiovascular disease events, and malignancy all influence the choice of treatment and the treatment goals for older patients. Based on articles reviewed here, it is suggested that current treatment strategies for younger patients are also useful for ACPA-positive EORA and for ACPA-negative EORA with bone erosion. Differential diagnosis of ACPA-negative EORA without erosive arthritis and PMR with peripheral manifestations is challenging, and the treatment strategy of patients presenting with this overlap phenotype remained unclear. An appropriate treatment strategy for all patients with EORA still needs to be developed.
Assuntos
Antirreumáticos , Artrite Reumatoide , Polimialgia Reumática , Idade de Início , Idoso , Antirreumáticos/uso terapêutico , Artrite Reumatoide/diagnóstico , Artrite Reumatoide/tratamento farmacológico , Autoanticorpos , Humanos , Polimialgia Reumática/diagnósticoRESUMO
Giant cell arteritis is a devastating condition that can lead to permanent vision loss. Early diagnosis is vital to initiate corticosteroid treatment before irreversible visual symptoms develop. This case study examines an 81-year-old woman with a history of polymyalgia rheumatica who initially presented with sore throat and headache. She was ultimately diagnosed with giant cell arteritis. However, her diagnosis was delayed by multiple emergency and outpatient providers who failed to evaluate her for giant cell arteritis. Her story reflects the importance of assessing polymyalgia rheumatica patients for giant cell arteritis even when presenting with more unusual symptoms, such as sore throat.