Polymyalgia rheumatica

doi:10.1016/j.berh.2004.06.003

Best Practice & Research Clinical Rheumatology

Volume 18, Issue 5, October 2004, Pages 705-722

https://doi.org/10.1016/j.berh.2004.06.003 Get rights and content

Abstract

Polymyalgia rheumatica (PMR) is an inflammatory condition of unknown etiology characterized by aching and stiffness in the shoulder and in the pelvic girdles and neck.

In the past, PMR was considered a manifestation of giant cell arteritis (GCA) or a variant of elderly-onset rheumatoid arthritis (EORA). The current diagnostic criteria for PMR were empirically formulated by clinical experts who had studied the disease extensively. Arthroscopic, radioisotopic and magnetic resonance imaging studies all have indicated the presence of a synovitis in proximal joints and periarticular structures. The synovitis is probably responsible for the musculoskeletal symptoms in PMR. The prominence assigned to the proximal symptoms has probably overshadowed the less well recognized and more variable distal musculoskeletal manifestations which are present in about half of the cases. A normal erythrocyte sedimentation rate does not exclude a diagnosis of PMR. C-reactive protein and interleukin-6 seem to be more sensitive indicators of disease activity both at diagnosis and during relapse/recurrence. Corticosteroids are the drugs of choice for treating PMR. A course of treatment of 1–2 years is often required. However, some patients have a chronic, relapsing course and require low doses of corticosteroids for several years. Large, multicenter, double-blind, placebo-controlled studies are required to define the role of methotrexate and anti-TNF-α agents as corticosteroid-sparing drugs in PMR.

Section snippets

Epidemiology and diagnostic criteria

Few population-based studies have evaluated the epidemiological aspects of PMR because of the lack of a diagnostic hallmark and universally accepted diagnostic and classification criteria. PMR is a common illness in certain populations, with a prevalence of one case for every 133 people over the age of 50 years.⁸ Like GCA, PMR is extremely rare in persons under 50. The incidence of PMR increases with age, with a peak in those 70–80 years of age. PMR is more frequent in females than in males in

Etiology, pathogenesis and pathology

PMR is probably a polygenic disease in which multiple environmental and genetic factors influence susceptibility and severity. The increased incidence at higher latitudes, in Scandinavian countries, and in the USA communities with a strong Scandinavian ethnic background (Table 1), as well as occasional familial cases, support the evidence for environmental and genetic causes.8., 9., 10., 11., 12., 13., 14., 15., 22.

A viral cause has been suspected but not confirmed in PMR and GCA. The sudden

Relationship between polymyalgia rheumatica and giant cell arteritis

Population-based studies of PMR have demonstrated the presence of biopsy-proven GCA in 16–21% of patients.8., 39. Conversely, symptoms of PMR have been observed in 40–60% of patients with GCA in clinical series.¹⁶

PMR may begin before, appear simultaneously with, or develop after GCA clinical manifestations. Prompt recognition of GCA is important in PMR patients because blindness may occur abruptly without premonitory symptoms. The overall risk of blindness in patients with ‘pure’ PMR was

Clinical manifestations

The combination of persistent pain (at least 1 month) with aching and morning stiffness in neck, shoulder and pelvic girdles lasting 30 minutes or more, plus evidence of systemic involvement—such as an erythrocyte sedimentation rate (ESR) elevated to 40 mm/hour or more—is strongly suggestive of PMR.1., 17., 18., 19. In patients with PMR the discomfort is bilateral; it involves the proximal limb and joint areas. The musculoskeletal pain is worse with movement of the affected area and usually

Laboratory

An ESR of at least 40 mm/hour is considered an important finding for the diagnosis of PMR. Some studies have reported that a normal ESR at diagnosis accounted for 7–20% of the patients with PMR.47., 48., 49., 50. C-reactive protein (CRP) is a sensitive and direct measure of the acute phase response as it is not influenced by the number or shape of erythrocytes, circulating immunoglobulins, hypercholesterolemia or renal function. CRP has been found to be a more sensitive indicator of disease

Imaging

The presence of proximal synovitis in PMR has been proved using different techniques such as scintigraphy, MRI and ultrasonography (US).34., 35., 56. US and MRI studies of the shoulders have shown that subacromial-subdeltoid bursitis is the most frequent lesion, present in almost all of the patients with PMR (Figure 2A and B). A recent case-control study demonstrated that US and MRI were equally effective in confirming bilateral subacromial and subdeltoid bursitis in PMR.⁵⁶ The US evidence of

Treatment and course

Corticosteroids (CSs) are the drugs of choice for treating PMR. An initial dose of 10–20 mg/day of prednisone or equivalent is adequate, in most cases, in the absence of associated GCA. In some patients with mild disease a short course with non-steroidal anti-inflammatory drugs (NSAIDs) may be tried. If NSAIDs do not adequately control PMR clinical manifestations in 2–4 weeks, CS should be started. Usually, the response to CS is rapid, with complete or nearly complete resolution of symptoms

Polymyalgia rheumatica, RS3PE syndrome and elderly-onset rheumatoid arthritis (EORA)

Distal symptoms of PMR have been underestimated, and a diagnosis of RA or other syndromes is often made to explain these findings when they occur. The occurrence of peripheral arthritis, particularly in both hands, may create some difficulties in the differential diagnosis between PMR and elderly-onset rheumatoid arthritis (EORA). Pronounced symmetrical peripheral involvement, seropositivity, and the development of joint erosions and extra-articular manifestations differentiate RA from PMR.

Differential diagnosis

A wide variety of conditions, including other rheumatic diseases76., 77., may mimic the clinical picture of PMR.

Systemic lupus erythematosus (SLE) in the elderly may sometimes present as PMR.⁷⁸ In these cases, the presence of pleuritis or pericarditis—common in late-onset SLE—and hematological abnormalities such as leukopenia or thrombocytopenia should raise the suspicion of SLE. Because the frequency of positive antinuclear antibodies (ANA) increases with age, a positive ANA by itself does not

Summary

Polymyalgia rheumatica (PMR) is an inflammatory condition of unknown etiology characterized by aching and stiffness in the shoulder and in the pelvic girdles and neck. PMR is related to giant cell arteritis (GCA), both conditions sometimes occurring in the same patient.

PMR is a common illness in certain populations, with a prevalence of one case for every 133 people over the age of 50 years. Like GCA, PMR is extremely rare in persons under 50. The incidence of PMR increases with age, with a

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La polimialgia reumática es una enfermedad inflamatoria crónica común en la población geriátrica. Su cuadro clínico se caracteriza por dolor en la cintura escapular, región cervical y caderas, asociado frecuentemente a rigidez de estas áreas articulares posterior a periodos de reposo. El diagnóstico de esta patología es clínico y debe hacerse posterior a descartar otras entidades como artritis reumatoide o espondiloartropatía de aparición tardía. Hasta el momento, no hay criterios de clasificación estandarizados y aceptados, por lo que recientemente se desarrollaron unos criterios provisionales por parte del Colegio Americano de Reumatología y de la Liga Europea Contra el Reumatismo. La polimialgia reumática se asocia hasta en un 30% de los pacientes con arteritis de células gigantes. El principal tratamiento es con dosis bajas de glucocorticoides, con lo cual los pacientes presentan rápida mejoría sintomática.
Polymyalgia rheumatica is an inflammatory disease common in the geriatric population. The clinical profile is characterized by pain, mainly in the shoulder girdle, hip and cervical region. The diagnosis of this disease is clinical, and should be made to rule out another diseases such as late onset rheumatoid arthritis and spondyloarthritis. Although no standardized classification criteria have been accepted, although there are some, yet to be evaluated, provisional classification criteria that have recently been developed by the American College of Rheumatology and European League Against Rheumatism. The polymyalgia rheumatica is associated with up to 30% of patients with giant cell arteritis. The main treatment is low-dose glucocorticoids, with which patients have rapid symptomatic improvement.
Diagnostic and therapeutic updates of "rhizomelic pseudopolyarthritis"
2014, Revue du Rhumatisme (Edition Francaise)
L’approche diagnostique et thérapeutique de la pseudo-polyarthrite rhizomélique a considérablement évolué ces dernières années. Il est apparu nécessaire de démembrer cette affection trop souvent considérée comme une maladie et non comme un syndrome. Les « critères diagnostiques » utilisés depuis des décennies ont montré leurs insuffisances, justifiant la proposition d’un nouveau concept pathologique élargi : la « PPR syndrome » et une proposition de démarche diagnostique et thérapeutique standardisée visant à éliminer les affections dont la présentation initiale est trompeuse afin d’arriver à la constitution d’un petit noyau restreint de patients que l’on dénommera « PPR maladie ». Des critères récents (pour la « PPR syndrome » et pour la « PPR maladie ») ont été proposés pour ces patients, ils doivent encore être validés. Nous commenterons la démarche diagnostique et l’approche thérapeutique proposées et leurs critères. Nous passerons volontairement sous silence, dans cette revue, les travaux portant sur les modèles physiopathologiques, les mécanismes inflammatoires, la génétique, etc. qui ont été réalisés sur des populations hétérogènes de patients ne validant pas les nouveaux critères. Il ressort donc que la PPR est un « mode d’entrée » dans un rhumatisme inflammatoire chez un sujet de plus de 50 ans et non pas (le plus souvent) une maladie en soi.
Current diagnosis and treatment of polymyalgia rheumatica
2014, Joint Bone Spine
Citation Excerpt :
Nevertheless, the most recent data do not support routine temporal artery biopsy in patients without symptoms suggesting temporal artery inflammation [3–5]. Glucocorticoid therapy remains the first-line treatment for PMR [6,9,37]. Current recommendations support an initial dosage in all patients of 15 mg/day for 3 weeks (regardless of body weight).
The diagnostic and therapeutic strategies for polymyalgia rheumatica (PMR) have changed substantially in recent years. Rather than a single disease entity, PMR has emerged as a syndrome produced by a variety of conditions. The diagnostic criteria that have been used for several decades are inadequate. These facts support a new and broader pathological concept, polymyalgic syndrome, and a standardized diagnostic and therapeutic approach designed to rule out diseases with misleading presentations and to identify the limited number of patients with polymyalgic syndrome who have PMR. Criteria for both polymyalgic syndrome and PMR were developed recently but remain to be validated. These criteria are discussed, as well as the suggested diagnostic approach and treatment strategy. In contrast, studies on pathophysiological models, inflammatory mechanisms, and genetic factors are not considered herein, as they were conducted in heterogeneous populations of patients who did not meet the new criteria. Current data indicate that polymyalgic syndrome is a mode of onset of inflammatory joint disease in individuals older than 50 years of age and not (in most cases) a disease entity.
Connective Tissue Diseases, Vasculitis, and the Nervous System
2014, Aminoff's Neurology and General Medicine: Fifth Edition
The inflammatory systemic autoimmune diseases can affect the peripheral and central nervous systems in diverse ways. In this chapter, the neurologic presentation of connective tissue disorders and vasculitis is discussed. In addition, the differential diagnosis of central and peripheral nervous system syndromes in relation to these disorders as well as their treatments are presented so that clinicians can make rapid therapeutic and treatment decisions when confronted with these problems. The bibliography is largely confined to papers published in the last 20 years, but references to the earlier literature can be found in this chapter as it appeared in the previous edition of this book.
Polymyalgia rheumatica in 2011
2012, Best Practice and Research: Clinical Rheumatology
Polymyalgia Rheumatica (PMR) is an inflammatory rheumatic disease that commonly affects individuals over 50 years of age, characterised by pain and morning stiffness of the shoulder and pelvic girdle. PMR can present as ‘isolated’ form or may be associated with giant cell arteritis. The progress of imaging techniques has helped in understanding different clinical patterns: subclinical vasculitis can occur in at least one-third of PMR patients, causing ischaemic complications. It is considered a polygenic disease and environmental factors may play a role in its pathogenesis, such as viral or bacterial triggers, both in the ‘wide’ form or assembled with adjuvants in vaccines. The response to steroid therapy is generally dramatic and side effects may occur, as well as the development of glucocorticoid resistance. The optimisation of therapy may require steroid-sparing agents as well as modified-release prednisone as ‘nighttime’ replacement therapy.
Polymyalgia rheumatica: a descriptive study in Medellin, Colombia
2011, Revista Colombiana de Reumatologia
la polimialgia reumática (PMR) es una enfermedad inflamatoria que afecta a mayores de 50 años, caracterizada por dolor y rigidez en región cervical, cinturas escapular y pélvica y por una rápida respuesta a los glucocorticoides. Hasta donde sabemos, no hay estudios clínicos sobre PMR en nuestra población. Algunas enfermedades reumatológicas de comienzo tardío como artritis reumatoide (AR), lupus eritematoso sistémico (LES), espondiloartropatías y arteritis de células gigantes (ACG) pueden manifestarse con hallazgos de PMR. Igualmente, neoplasias hematológicas y algunos tumores sólidos pueden presentarse con un síndrome polimiálgico.
analizar las características clínicas, de laboratorio y terapéuticas de pacientes de Medellín con PMR entre 1998 y 2011.
estudio descriptivo transversal. Se revisaron historias clínicas de pacientes con PMR y al menos 1 visita de seguimiento. Se analizaron variables sociales, demográficas, clínicas, de laboratorio y terapéuticas.
se evaluaron sesenta y nueve pacientes (79,7% mujeres), 68 (98,6%) de los cuales tuvieron dolor y/o rigidez en cintura escapular y 62 (89,8%) en cintura pélvica. La velocidad de sedimentación globular (VSG) fue igual o mayor a 40 mm/h en 43 (62,3%) pacientes y la proteína C reactiva (PCR) igual o mayor a 0,8 mg/dl en 56 (81,2%). El 76.9% de los pacientes recibieron prednisolona en dosis = 15 mg/día; 95,7% respondieron en los 3 primeros meses de tratamiento, 94,2% presentaron remisión (84,6% en los 6 primeros meses) y 39,1% tuvieron recaída (77,8% en el primer año de seguimiento). Los pacientes con VSG elevada tuvieron menor respuesta en el primer mes. Durante el seguimiento, 5 pacientes desarrollaron artritis reumatoide y 2 ACG.
los hallazgos encontrados aportan mayor información sobre las características de los pacientes con PMR en nuestra población y confirman la rápida respuesta al tratamiento con dosis bajas a moderadas de esteroides y menor respuesta inicial en pacientes con VSG elevada. Su diseño y tamaño de muestra son insuficientes para definir asociaciones estadísticamente significativas.
Polymyalgia rheumatic is a syndrome that affects people over 50 years, characterized by pain and stiffness of shoulder and pelvic girdle. Its behavior in our population is unknown. It may be a manifestation of rheumatoid arthritis, systemic lupus erythematous, and neoplasm or could be associated with giant cells arteritis and it is a common indication for steroid usage.
To analyze clinical, laboratory and treatment features of patients with PMR in Medellín between 1998–2011.
Descriptive cross-sectional study. We reviewed medical records of patients with PMR with at least 1 follow-up visit. We analyzed social, demographic, clinical, laboratory and therapeutic variables.
The records of 68 patients were assessed, 79.7% women. Pain and stiffness in shoulder and pelvic girdle were major symptoms. 62.3% had erythrocyte sedimentation rate equal to or greater than 40 mm/h and 81.2% had C reactive protein equal to or greater than 0.8 mg/dl. 76.9% received doses of prednisolone of 15 mg/day or below. 95.7% responded within the first 3 months of treatment, 94.2% showed remission (84.6% within the first 6 months), 39.1% relapsed (most within the first year of monitoring). Those with elevated ESR had a lower response in the first month. Five patients developed rheumatoid arthritis and two patients giant cells arteritis.
These findings add more information about the clinical characteristics of patients with PMR. They allow suggesting early responses to treatment with low to moderate doses of steroids and a lower initial response in patients with elevated ESR. Design and sample size are insufficient to identify statistically significant associations.

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6Polymyalgia rheumatica

Abstract

Section snippets

Epidemiology and diagnostic criteria

Etiology, pathogenesis and pathology

Relationship between polymyalgia rheumatica and giant cell arteritis

Clinical manifestations

Laboratory

Imaging

Treatment and course

Polymyalgia rheumatica, RS3PE syndrome and elderly-onset rheumatoid arthritis (EORA)

Differential diagnosis

Summary

Polymyalgia rheumatica and giant cell arteritis

New England Journal of Medicine

Senil rheumatic gout

British Medical Journal

Myalgic syndrome with constitutional effects: polymyalgia rheumatica

Annals of the Rheumatic Diseases

Prognosis in the anarthritic rheumatoid syndrome

British Medical Journal

Distal extremity swelling with pitting edema in polymyalgia rheumatica. Report of nineteen cases

Arthritis and Rheumatism

Polymyalgia rheumatica: a disorder of extraarticular synovial structures

Journal of Rheumatology

Giant cell arteritis, or arteritis of the aged

British Medical Journal

Epidemiology of polymyalgia rheumatica in Olmsted County, Minnesota, 1970–1991

Arthritis and Rheumatisms

The incidence of polymyalgia rheumatica and temporal arteritis in the country of Aust Agder, south Norway: a prospective study 1987–94

Journal of Rheumatology

Giant cell arteritis, temporal arteritis and polymyalgia rheumatica in a Danish county

Arthritis and Rheumatism

High incidence of polymyalgia rheumatica and giant cell arteritis in a Swedish community

Clinical and Experimental Rheumatology

Epidemiology and mortality in 220 patients with polymyalgia rheumatica

British Journal of Rheumatology

Synchronous variations of the incidence of temporal arteritis and polymyalgia rheumatica in different regions of Denmark; association with epidemics of Mycoplasma pneumoniae infection

Journal of Rheumatology

The spectrum of polymyalgia rheumatica in northwestern Spain: incidence and analysis of variables associated with relapse in a 10 year study

Journal of Rheumatology

Epidemiologic and immunogenetic aspects of polymyalgia rheumatica and giant cell arteritis in Northern Italy

Arthritis and Rheumatism

The incidence of giant cell arteritis in Olmsted County Minnesota: apparent fluctuations in a cyclic pattern

Annals of Internal Medicine

Polymyalgia rheumatica: a 10-year epidemiologic and clinical study

Annals of Internal Medicine

Long-term follow-up of polymyalgia rheumatica: evidence for synovitis

Seminars in Arthritis and Rheumatism

An evaluation of criteria for polymyalgia rheumatica

Annals of the Rheumatic Diseases

The sensitivity and specificity of reduced CD8 lymphocyte levels in the diagnosis of polymyalgia rheumatica/giant cell arteritis

Clinical and Experimental Rheumatology

CD8 lymphocyte subsets in active polymyalgia rheumatica: comparison with elderly-onset and adult rheumatoid arthritis and influence of prednisone therapy

British Journal of Rheumatology

Familial aggregation of polymyalgia rheumatica and giant cell arteritis

Arthritis and Rheumatism

Increased prevalence of antibodies to adenovirus and respiratory syncytial virus in polymyalgia rheumatica

Clinical and Experimental Rheumatology

Giant cell arteritis, polymyalgia Rheumatica, and viral hypotheses: a multicenter, prospective case-control study. Groupe de Recherche sur l'Arterite a Cellules geantes

Journal of Rheumatology

Lack of association between infection at onset of polymyalgia rheumatica

Journal of Rheumatology

Polymyalgia rheumatica is associated with both HLA-DRB1*0401 and DRB1*0404

British Journal of Rheumatology

HLA-DRB1 alleles associated with polymyalgia rheumatica in northern Italy: correlation with disease severity

Annals of the Rheumatic Diseases

Intercellular adhesion molecule 1 gene polymorphisms in polymyalgia rheumatica/giant cell arteritis: association with disease risk and severity

Journal of Rheumatology

Association of giant cell arteritis and polymyalgia rheumatica with different tumor necrosis factor microsatellite polymorphisms

Arthritis and Rheumatism

RANTES gene polymorphism in polymyalgia rheumatica, giant cell arteritis and rheumatoid arthritis

Clinical and Experimental Rheumatology

Interleukin-1 cluster and tumor necrosis factor-alpha gene polymorphisms in polymyalgia rheumatica

Clinical and Experimental Rheumatology

Adrenal gland hypofunction in active polymyalgia rheumatica. Effect of glucocorticoid treatment on adrenal hormones and interleukin-6

Journal of Rheumatology

Leukocyte infiltration in synovial tissue from the shoulder of patients with polymyalgia rheumatica. Quantitative analysis and influence of corticosteroid treatment

Arthritis and Rheumatism

Joint imaging in polymyalgia rheumatica

6
Polymyalgia rheumatica

Polymyalgia rheumatica is associated with both HLA-DRB10401 and DRB10404