Incidence and Prevalence of Polymyalgia Rheumatica and Giant Cell Arteritis in a Healthcare Management Organization in Buenos Aires, Argentina.

OBJECTIVE: To estimate incidence and prevalence of polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) in a university hospital-based health management organization (Hospital Italiano Medical Care Program) in Argentina. METHODS: Overall and sex-specific incidence rates (IRs) and prevalence were calculated (age ≥ 50 yrs). Incidence study followed members with continuous affiliation ≥ 1 year from January 2000 to December 2015. Diagnosis as per the 2012 European Alliance of Associations for Rheumatology/American College of Rheumatology (ACR) criteria for PMR or the ACR 1990 criteria for GCA. Prevalence was calculated on January 1, 2015. RESULTS: There were 176,558 persons who contributed a total of 1,046,620 person-years (PY). Of these, 825 developed PMR, with an IR (per 100,000 PY) of 78.8 (95% CI 73.4-84.2) overall, 90.1 (95% CI 82.9-97.2) for women, and 58.9 (95% CI 51.1-66.6) for men. Ninety persons developed GCA; the IR was 8.6 (95% CI 6.8-10.4) overall, 11.1 (95% CI 8.5-10.6) for women, and 4.2 (2.2-6.3) for men. There were 205 prevalent PMR cases and 23 prevalent GCA cases identified from a population of 80,335. Prevalence of PMR was 255 per 100,000 (95% CI 220-290) overall, 280 (95% CI 234-325) for women, and 209 (95% CI 150-262) for men; and the prevalence of GCA was 28.6 per 100,000 (95% CI 16.9-40.3) overall, 36.4 (95% CI 20.1-52.8) for women, and 14.2 (95% CI 0.3-28.1) for men. CONCLUSION: This is the first study of incidence and prevalence of PMR and GCA in Argentina. There were similarities and differences with cohorts from other parts of the world, but population-based epidemiologic studies in Latin America are needed.

Usefulness of Methotrexate in the Reduction of Relapses and Recurrences in Polymyalgia Rheumatica: An Observational Study.

OBJECTIVES: Methotrexate (MTX) has been studied with conflicting results in patients with polymyalgia rheumatica (PMR). Our objective was to evaluate the effectiveness of MTX to reduce relapses and recurrences in patients with PMR. METHODS: This observational longitudinal cohort study included 94 consecutive patients with PMR. Patients were assigned to 3 groups according to the prescribed treatment: group 1, treated with glucocorticoids (GCs) alone; group 2, treated with GCs initially plus MTX after a relapse or recurrence; and group 3, treated with GCs plus MTX since diagnosis.Factors associated with a first relapse were studied in the population. To evaluate MTX effect, patients from group 2 were evaluated comparing results from the first treatment period (GCs alone) to the second treatment period with GCs plus MTX. RESULTS: Ninety-four patients were included. The median follow-up time was 21.3 months (interquartile range [IQR], 11.7-56.2). Fifty-three patients (56.4%) were in group 1, 33 (35.1%) in group 2, and 8 (8.5%) in group 3. We found that female sex had a tendency to be associated with a first relapse (p = 0.07).In group 2, 35 relapses were identified during the first treatment period and only 8 relapses during the combined treatment period (p < 0.001). In this group, after the addition of MTX, the GCs dose at relapse was lower (5.1 vs 3 mg/d, p = 0.02) and the time to accomplish remission was shorter (22.9 vs 8.7 months, p = 0.01). There were no differences in the number of recurrences. CONCLUSIONS: The use of MTX in PMR patients who already had a relapse reduced the number of future relapses and decreased the time to achieve remission. Adding MTX allowed a reduction of GCs dose at relapse.

Prevalence of polymyalgia rheumatica in Colombia: data from the national health registry 2012-2016.

Polymyalgia rheumatica (PMR) affects elderly patients and is characterized by pain and stiffness of the shoulder girdle, pelvic girdle and cervical region, which can be associated with the presence of giant cell arteritis. Data on the epidemiology of this disease in Latin America are scarce. In Colombia, the Ministry of Health introduced SISPRO, a tool to collect nation-wide information from the health system. The information collected from SISPRO is available for scientific analysis. Using SISPRO data for the years 2012-2016, an analysis was made on the prevalence and characteristics of patients diagnosed with PMR. This is a descriptive epidemiological study using the International Statistical Classification of Diseases and Related Health Problems as search terms related to PMR, based on SISPRO data. Criteria for diagnosis are not explicitly addressed in each individual case. National records report 19,901 individuals diagnosed with PMR and estimated prevalence of 2 cases per 1000 inhabitants over 50 years old (based on a total population of 47,663,162), being more frequent in women (86% of cases), with a female/male ratio of 6.2:1. This is the first study that describes the demographic characteristics of PMR in Colombia. Our results are consistent with the age-related increase in prevalence and gender ratio. Likewise, there are differences between regions, which may be related to ancestry and environmental factors, which require further studies.

Polymyalgia rheumatica: 125 years of epidemiological progress?

OBJECTIVES: 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 epidemiology of this disease and whether such studies have advanced our knowledge of its aetiopathogenesis and management. METHODS: 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 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. RESULTS: 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 investigating the populations susceptible, the geographic distribution of these affected populations and the associated sociological and genetic elements that might contribute to its occurrence, polymyalgia rheumatica remains a difficult problem for the public health services of the developed world. CONCLUSIONS: 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. Until then, clear guidelines on the future incidence and prevalence of polymyalgia rheumatica and the public health problems of the disease and its management, especially in relation to the use of long term corticosteroids, will be difficult to provide.

Polymyalgia rheumatica: 125 years of progress?

OBJECTIVES: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Giant cell arteritis in Mexican patients.

BACKGROUND: Giant cell arteritis (GCA) is the most common primary systemic vasculitis worldwide, although it seems to be very rare in some areas, such as Latin America. OBJECTIVES: The objective of the study was to describe the clinical, laboratory, and treatment features in a Mexican Mestizo population with GCA. METHODS: Retrospective data chart review (1989-2010). RESULTS: Twenty-two patients with GCA were identified, 18 women and 4 men. Mean age was 73 (SD, 7.9) years. Diagnosis was made at a mean of 67 (SD, 83.6) days from symptom onset. Most frequent presenting symptoms included headache (90%), constitutional symptoms (86%), and polymyalgia rheumatica (59%). Severe cranial ischemic complications were present in 32%. Amaurosis fugax and blindness were present in 36% and 27%, respectively. High erythrocyte sedimentation rate was present in 89% of patients. Rapid response to prednisone treatment was seen, but in 10 patients, relapse occurred, possibly related to fast tapering. Additional treatment was methotrexate (n = 8), azathioprine (n = 5), and cyclophosphamide (n = 3). Median follow-up was 242 (SD, 214) weeks. CONCLUSIONS: Giant cell arteritis is rarely recognized in Latin America. We report on characteristics of GCA in a population of Mexican Mestizos, as ours is the largest series to be reported from Latin America so far. When compared with other series, age at onset is similar, females are more affected, and although a good response to corticosteroid treatment was seen, a higher frequency of amaurosis fugax and blindness was observed, accounting for an unfavorable functional outcome in 6 (27%) of 22 patients.

Magnetic resonance angiography in extracranial giant cell arteritis.

BACKGROUND: Noninvasive diagnosis of giant cell arteritis (GCA) remains challenging, particularly with regard to evaluation of extracranial arterial disease. OBJECTIVES: The objective of the study was to retrospectively review extracranial involvement in patients with GCA and/or polymyalgia rheumatica (PMR), evaluated with magnetic resonance imaging (MRI), especially 3-dimensional contrast-enhanced magnetic resonance angiography images of the aortic arch and its branches. METHODS: Clinical information, biopsy status, and MRI examinations of 28 patients with GCA/PMR were reviewed. Patient images were mixed randomly with 20 normal control images and were independently reviewed by 2 radiologists. Interobserver agreement for detection of arterial stenosis was determined by the k coefficient. RESULTS: Both readers described vascular alterations in keeping with extracranial GCA in 19 of 28 patients (67%) with good interobserver agreement (k = 0.73) and with even higher agreement on diagnosing nonocclusive versus occlusive disease (k = 1.00). The most common lesions were bilateral axillary stenosis or obstructions, observed by both readers in 8 patients (28%). Among the 19 patients with magnetic resonance angiography lesions in the subclavian/axillary arteries, 12 (75%) had biopsy-proven GCA, but only 5 (41%) of these patients had clinical features of large artery disease. CONCLUSIONS: In our series review, MRI could provide accurate information on involvement of the aortic arch and its branches in extracranial GCA, depicting different degrees of stenosis. Our analysis also illustrates that occult large artery vasculitis should be considered in patients without biopsy-proven GCA, patients with classic GCA but without clinical signs of large artery disease, and in patients initially diagnosed as having PMR.

[Conditions mimicking polymyalgia rheumatica]. / Enfermedades que simulan polimialgia reumática.

OBJECTIVE: To examine the main clinical and laboratory data of patients initially diagnosed with polymyalgia rheumatica (PMR), which then developed another conditions. MATERIAL AND METHODS: We reviewed the clinical records of patients diagnosed with PMR in three hospitals in Argentina. Patients had a diagnosis of PMR if they met the following criteria: age ≥ 50 years, erythrocyte sedimentation rate (ESR) at the time of diagnosis > 40 mm, persistent pain and stiffness of at least one month of evolution in two of the following areas: neck, shoulders or proximal arms, hips or proximal lower limbs. Special attention was paid to symptoms or signs of "alarm" (beginning or during disease progression) for suspecting the presence of other non PMR disease within a period of ≤ 12 months. RESULTS: Sixteen of the 200 patients (8%) had other diseases during follow up. Malignancies (n=4) and rheumatic diseases (n=4) were the most common entities, in addition to infective endocarditis (n=1), narrow cervical canal (n=1), Parkinson's disease (n=1), statin-related myalgia (n=1), hypothyroidism (n=1), vitamin D deficiency (n=1) and Calcium Pyrophosphate Deposition Disease (CPDD) (n=2). The average length change of diagnosis was 4.5±3 months. Ten patients had no response to steroids and two had persistently elevated ESR. CONCLUSION: In this study we highlight the importance of recognizing signs and symptoms along with laboratory data and lack of response to treatment as suspects for the diagnosis of other disease manifestations in patients with PMR symptoms.

Polimialgia reumática: experiencia basada en 22 casos / Polymyalgia rheumatica: experience based on 22 cases

Introducción. La polimialgia reumática (PMR) se caracteriza por dolor y rigidez de las cinturas cervicoescapular y pelviana; preséntase en mayores de 50 años, asociada frecuentemente a arteritis de células gigantes (ACG), y tiene excelente respuesta a dosis bajas de corticosteroides. Objetivo. Evaluar sus características clínicas, de laboratorio, terapéuticas y evolutivas en nuestro medio. Pacientes y método: 22 pacientes diagnosticados según criterios establecidos y controlados entre 1998 y 2008. Según protocolo se analizan sexo, edad, diagnósticos previos, clínica, laboratorio, tratamiento y evolución. Resultados. Edad 52 a 81 años (mediana 69,3); predominio femenino (17/5), y 9,2 meses promedio de evolución prediagnóstico. Todos presentaron dolor y rigidez cervicoescapular y pelviana, 2 pacientes (9 por ciento) tuvieron artritis y 2 (9 por ciento), características clínicas de arteritis de células gigantes (ACG). El laboratorio fue inespecífico, excepto velocidad de sedimentación elevada entre 38 y 131 mm/hora (promedio 80,7). Todos tuvieron factor reumatoídeo (-) y anticuerpos antinucleares (-). Todos, excepto los portadores de ACG, respondieron a dosis bajas de corticosteroides (20 mg/día de prednisona o menos), con dosis de mantención entre 5 y 10 mg/día. Excelente evolución en todos, excepto uno, con desaparición de síntomas, normalización de VHS y reintegración a actividades previas. Conclusiones. La PMR constituye un diagnóstico diferencial en personas mayores de 50 años con dolor de cintura escapular y pelviana, que suele asociarse con ACG. El diagnóstico se basa en criterios bien establecidos, destacando la VHS persistentemente alta y la respuesta a dosis bajas de corticosteroides, que es además la terapéutica de elección.

Polymyalgia rheumatica (PMR) is characterized by cervicoscapular and pelvic pain and rigidity, it manifests in people older than 50 years and is frequently associated with giant cell arteritis (GCA). It responds very well to low doses of corticosteroids. Objective: Evaluate clinical, laboratory, therapeutic and evolutionary characteristics in our environment. Patients and method: 22 patients diagnosed according to set criteria and controlled between 1998 and 2008. Sex, age, previous clinical and laboratory diagnosis, treatment and evolution were analyzed. Results: Age 52 to 81 (average 69.3); 17 females,5 males; and 9.2 month average prediagnostic evolution. All presented cervicoscapular and pelvic pain and rigidity, 2 patients (9 percent) had arthritis, and 2 (9 percent) presentedclinical characteristics of GCA. Laboratory tests were nonspecific, except for high sedimentation speed between 38 and 131 mm/hour (average 80.7). All patients presented rheumatoid factor (-) and antinuclear antibodies (-). All, except GCA carriers, responded to low doses of corticosteroids (20 mg/day or less of Prednisona), followed by a maintenance dose of 5 - 10 mg/day. Evolution was excellent in all patients except one, symptoms disappeared, ESR normalized and patients were reintegrated to their previous activities. Conclusions: PMR constitutes a differential diagnosis in people over 50 years of age, with cervicoscapular and pelvic pain, and is commonly associated with GCA. Diagnosis is based on well established criteria, with persistently high ESR and response to low doses of corticosteroids - the treatment therapy of choice - among the most significant.

Fiebre de origen desconocido en colangenopatías y vasculitis / Fever of unknown origen in collagen diseases and vasculitis

La fiebre de origen desconocido (FOD) continúa siendo un tema de análisis para el médico internista. A pesar de los notables avances en las técnicas diagnósticas a nivel de estudios infectológicos, ténicas de imagen e inmunológicas, el porcentaje de casos en los cuales no se llega al diagnóstico continúa siendo elevado. Por esta razón el buen criterio clínico a la hora de decidir los estudios indicados para cada caso según los planteos más adecuados no ha perdido vigencia.

Fiebre de origen desconocido en colangenopatías y vasculitis / Fever of unknown origen in collagen diseases and vasculitis

La fiebre de origen desconocido (FOD) continúa siendo un tema de análisis para el médico internista. A pesar de los notables avances en las técnicas diagnósticas a nivel de estudios infectológicos, ténicas de imagen e inmunológicas, el porcentaje de casos en los cuales no se llega al diagnóstico continúa siendo elevado. Por esta razón el buen criterio clínico a la hora de decidir los estudios indicados para cada caso según los planteos más adecuados no ha perdido vigencia. (AU)

The role of anticyclic citrullinated peptide antibodies in the differential diagnosis of elderly-onset rheumatoid arthritis and polymyalgia rheumatica.

There are clinical difficulties to differentiate elderly-onset rheumatoid arthritis (EORA) patients from those with polymyalgia rheumatica (PMR), especially when dealing with EORA-like PMR-onset, seronegative EORA, and PMR with peripheral synovitis, which constitute the subgroups presenting the greatest difficulties. Serum samples were obtained from two groups of patients, one with EORA diagnosis and another with a PMR diagnosis. Anticyclic citrullinated peptide (anti-CCP) antibodies (enzyme-linked immunosorbent assay method) and rheumatoid factor (RF; latex technique) were determined. Of the 16 EORA patients, 9 presented anti-CCP antibodies, 4 of whom tested positive for RF. Of the 12 EORA patients who remained negative to RF, 5 were positive for anti-CCP antibodies. Eight of the EORA patients started with polymyalgic symptoms. Three of these patients showed positive titles of anti-CCP antibodies with negative RF. All PMR patients presented negative anti-CCP antibodies, except one with weak positive titles, and all were negative for RF. Of 15 patients with PMR, 7 presented oligoarticular synovitis at the onset. After a mean follow-up of 3 months, two patients developed RA. When evaluating them for RF and anti-CCP antibodies, one tested negative, while the other was positive for both antibodies. We observed a tendency to higher values of anti-CCP antibodies in patients with extraarticular manifestations, radiological damage, and disease-modifying antirheumatic drugs. When compared to the PMR group, EORA patients presented positive anticitrulline antibodies at the beginning of the disease in a statistically significant amount. One third of the seronegative EORA patients presented positive anti-CCP antibodies at the onset.

Polimialgia reumática como presentación de mieloma múltiple y amiloidosis / Polymialgia rheumatica as a presentation of multiple mieloma and amyloidosis

La polimialgia reumática es una entidad con características clínicas definidas que puede ser el inicio o estar asociada a otras entidades. Aunque no se asocia a neoplasia, puede ser su expresión inicial. Informamos acerca de un paciente que presentó el cuadro inicial de polimialgia reumática como manifestación de otra enfermedad, el mieloma múltiple asociado a amiloidosis; discutimos las características clínicas de estas entidades y revisamos la literatura disponible con respecto a estas entidades y su asociación

Cytidine deaminase in polymyalgia rheumatica and elderly onset rheumatoid arthritis.

Serum cytidine deaminase (CD) as a marker of inflammatory disease was assessed in 44 patients and 47 controls to differentiate polymyalgia rheumatica (PMR) from elderly onset rheumatoid arthritis (EORA). The patients were divided into four groups: PMR with and without synovitis and seropositive and seronegative EORA. No statistically significant differences were found when serum CD levels of seropositive EORA patients were compared with serum CD of PMR patients without synovitis, neither when serum CD levels of all PMR patients were compared with a seronegative EORA group, nor when serum CD levels of PMR patients with synovitis were compared with those with EORA. Nevertheless, statistically significant differences were detected between EORA's serum CD levels and the control group (p=0.023). This difference was 10% when comparing CD levels of PMR patients with the control group (p=0.070). We did not demonstrate that serum CD levels could be a useful tool to differentiate PMR from EORA, but these findings could nevertheless reflect the presence of an inflammatory disease.

[Polymyalgia rheumatica revisited]. / Actualización en polimialgia reumática.

Polymyalgia rheumatica is an inflammatory disorder that usually affects persons over the age of 50 causing proximal muscle pain and stiffness, and an elevated erythrocyte sedimentation rate. Although increasingly recognized in this age group, it remains a diagnosis of exclusion and although several diagnostic criteria have been proposed, none has been clearly accepted. While polymyalgia rheumatica is associated with giant cell arteritis, obtaining a temporal artery biopsy is not recommended in patients with polymyalgia rheumatica without symptoms of giant cell arteritis. Early diagnosis and low dose corticosteroid therapy improve patients' clinical features and functional status. Treatment usually lasts between 12 and 24 months and the majority of patients manage to discontinue treatment completely.

Actualizacion en polimialgia reumática / Polymyalgia rheumatica revisited

Polymyalgia rheumatica is an inflammatory disorder that usually affects persons over the age of 50 causing proximal muscle pain and stiffness, and an elevated erythrocyte sedimentation rate. Although increasingly recognized in this age group, it remains a diagnosis of exclusion and although several diagnostic criteria have been proposed, none has been clearly accepted. While polymyalgia rheumatica is associated with giant cell arteritis, obtaining a temporal artery biopsy is not recommended in patients with polymyalgia rheumatica without symptoms of giant cell arteritis. Early diagnosis and low dose corticosteroid therapy improve patients clinical features and functional status. Treatment usually lasts between 12 and 24 months and the majority of patients manage to discontinue treatment completely (AU)