Are polymyalgia rheumatica and giant cell arteritis the same disease?

doi:10.1016/j.semarthrit.2003.09.008

Seminars in Arthritis and Rheumatism

Volume 33, Issue 5, April 2004, Pages 294-301

https://doi.org/10.1016/j.semarthrit.2003.09.008 Get rights and content

Abstract

Objective

To summarize the evidence about the relationship between polymyalgia rheumatica (PMR) and giant cell arteritis (GCA).

Methods

Review of relevant articles from the English-language literature.

Results

Epidemiologic studies suggest that PMR and GCA are closely related conditions affecting people over 50 years and frequently occurring in the same patient. PMR symptoms have been observed in 40 to 60 percent of GCA clinical series. Also, temporal artery biopsy may yield positive results for GCA in patients with isolated PMR. Conflicting HLA-DRB1 genotype results have been reported, and recent studies have shown that PMR and GCA have different expression of RANTES, TNFα microsatellite, and IL-6 promoter genetic polymorphisms. Search for a possible common infectious agent have yielded disappointing results. Although parvovirus B19 DNA is present in the artery wall of patients with GCA, this virus may be only an innocent bystander. Cytokine studies on a limited number of temporal artery biopsy specimens have shown that interferon-γ is produced in GCA and not in PMR, suggesting that this cytokine may be crucial to the development of overt vasculitis.

Conclusions

PMR and GCA frequently occur together but no definitive conclusions can be drawn about the nature of this association.

Section snippets

Methods

A systematic review of Medline database was performed to identify English-language articles related to the epidemiology, etiology, and pathogenesis of PMR and GCA. Articles were selected if they included both controlled and open studies on clinical series of patients who met the most commonly accepted criteria for the diagnosis of PMR 21, 22, 23, and of patients with positive temporal artery biopsy or who satisfied the ACR classification criteria for GCA (24).

Epidemiologic data

PMR and GCA are closely related conditions affecting people over 50 years and frequently occurring in the same patient. The incidence of both diseases increases after the age of 50 and peaks between 70 and 80 years of age 25, 26. The incidence of PMR appears relatively stable in recent years (27); in contrast, the incidence of GCA appears to have increased and autopsy studies suggest that GCA may be more common than clinically recognized (28). The 2 disorders have a very low prevalence rate in

Discussion

This review indicates that PMR and GCA frequently occur together for unclear reasons and no definitive conclusions can be drawn regarding the nature of this association. Different methodologic and diagnostic approaches have negatively influenced the epidemiologic data relatively to incidence, racial and ethnic differences, and concurrence of the 2 disorders. Only population-based studies in different countries may clarify these concerns. To avoid patient selection bias, these studies should be

Fabrizio Cantini, MD: Consultant, Director 2nd Divisione de Medicina, Unità Reumatologica, Ospedale di Prato, Italy

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Subclinical giant cell arteritis in polymyalgia rheumatica: Concurrent conditions or a common spectrum of inflammatory diseases?
2024, Autoimmunity Reviews
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.
Polymyalgia rheumatica
2023, The Lancet
Polymyalgia rheumatica is an inflammatory disease producing pain and stiffness, mainly in the shoulders and pelvic girdle, in people older than 50 years. Elevation of acute phase reactants is common due to the inflammatory nature of the disease. Since there are no specific diagnostic tests, diagnosis requires the exclusion of other diseases with similar presentations. Imaging has helped to identify the pathological substrate of polymyalgia rheumatica and it is increasingly used to support clinical diagnosis or to detect coexistent giant cell arteritis. Although polymyalgia rheumatica does not clearly impair survival or organ function, it can have a detrimental effect on quality of life. Glucocorticoids at 12·5–25·0 mg prednisone per day are effective in inducing remission in most individuals but, when tapered, relapses occur in 40–60% of those affected and side-effects are common. Assessment of disease activity can be difficult because pain related to common comorbidities such as osteoarthritis and tendinopathies, can return when glucocorticoids are reduced, and acute phase reactants are increased less during flares in individuals undergoing treatment or might increase for other reasons. The role of imaging in assessing disease activity is not yet completely defined. In the search for more efficient and safer therapies, tocilizumab and sarilumab have shown efficacy in randomised controlled trials and additional targeted therapies are emerging. However, judicious risk–benefit balance is essential in applying therapeutic innovations to people with polymyalgia rheumatica.
Neurological complications of giant cell arteritis: A study of 15 cases and a review of the literature
2023, Journal Francais d'Ophtalmologie
Giant cell arteritis (GCA) is considered a neuro-ophthalmologic emergency. New-onset headache in patients aged 50 years and above with elevated erythrocyte sedimentation rate should prompt evaluation for GCA.
Retrospective study of 15 patients presenting with GCA from 1991 to 2008 at the Mohamed V Military hospital at Rabat and Avicenne Military hospital of Marrakech.
Fifteen cases were recorded, with female predominance (male to female ratio 2:3) and a mean age of 63 years (range: 55–83 years). All patients (100%) presented with headache. The headache was isolated in 20% of cases and neuro-ophthalmic complications were found in 73% of cases. Biopsy was conclusive for GCA in 67% of cases and all of our patients were placed on steroids with spectacular improvement.
New-onset headache in patients aged 50 years and above should prompt evaluation for GCA. Steroids, especially during the acute phase, must be started urgently to avoid irreversible neurological impairment.
L’artérite à cellules géantes (ACG) est considérée comme une urgence neuro-ophtalmologique. Une céphalée d’apparition récente chez les patients âgés de 50 ans et présentant une vitesse de sédimentation élevée devrait inciter à rechercher une ACG.
Une étude rétrospective de 15 patients présentant une ACG, de 1991 à 2008 à l’hôpital militaire Mohamed V de Rabat et à l’hôpital militaire Avicenne de Marrakech.
Quinze cas ont été enregistrés avec une prédominance féminine (ratio homme/femme, 2:3), un âge moyen de 63 ans (extrêmes: 55 et 83 ans). Tous les patients (100%) ont présenté des céphalées. La céphalée était isolée dans 20 % des cas, et des complications neuro-ophtalmiques étaient retrouvées dans 73 % de nos patients. La biopsie a été concluante d’ACG dans 67 % des cas et tous nos patients ont été mis sous stéroïdes avec une amélioration spectaculaire.
L’apparition de nouvelles céphalées chez les patients âgés de plus de 50 ans devrait inciter à rechercher l’ACG. Les stéroïdes surtout pendant la phase aiguë doivent être commencés en urgence pour éviter une atteinte neurologique irréversible.
Treat to Target: A Valid Concept for Management of Polymyalgia Rheumatica and Giant Cell Arteritis?
2019, Rheumatic Disease Clinics of North America
Citation Excerpt :
GCA is the most frequent primary systemic vasculitis, and is characterized by inflammatory involvement of large vessels.13 From 40% to 60% of patients with GCA also have features of PMR,13,14 and in about 16% to 21% of patients with PMR, histologic features are detected on temporal artery biopsy that are consistent with GCA13,15; a further 10% to 30% of patients with PMR have evidence of large vessel vasculitis (LVV) when studied with fluorodeoxyglucose-positron emission tomography (FDG-PET). PMR and GCA are considered by some authors as different manifestations of the same disease and by others as different diseases within the same clinical spectrum.14
New developments in giant cell arteritis
2016, Survey of Ophthalmology
Citation Excerpt :
In PMR, vessel wall inflammation is incomplete, and systemic inflammation is combined with myalgia of the neck, shoulders, and pelvis girdle.171 The systemic inflammatory components of these two conditions may be indistinguishable,50 characterized by a production of large amounts of macrophage-derived inflammatory cytokines.28,78 These cytokines provide evidence of a common pathogenetic mechanism of vasculitis.
Giant cell arteritis (GCA) is a medium-to-large vessel vasculitis with potentially sight- and life- threatening complications. Our understanding of the pathogenesis, diagnosis, and treatment of GCA has advanced rapidly in recent times. The validity of using the American College of Rheumatology guidelines for diagnosis of GCA in a clinical setting has been robustly challenged. Erythrocyte sedimentation rate, an important marker of inflammation, is lowered by the use of statins and nonsteroidal anti-inflammatory drugs. Conversely, it may be falsely elevated with a low hematocrit. Despite the emergence of new diagnostic modalities, temporal artery biopsy remains the gold standard. Evidence suggests that shorter biopsy lengths and biopsies done weeks to months after initiation of steroid therapy are still useful. New imaging techniques such as positron emission tomography have shown that vascular inflammation in GCA is more widespread than originally thought. GCA, Takayasu arteritis, and polymyalgia rheumatica are no longer thought to exist as distinct entities and are more likely parts of a spectrum of disease. A range of immunosuppressive drugs have been used in conjunction with corticosteroids to treat GCA. In particular, interleukin-6 inhibitors are showing promise as a therapy.
Prevalence of ischemic complications in patients with giant cell arteritis presenting with apparently isolated polymyalgia rheumatica
2015, Seminars in Arthritis and Rheumatism
Citation Excerpt :
In these patients, the possibility of subclinical aortitis was considered during follow-up as part of the differential diagnosis of patients who were in apparent clinical remission (i.e., had no arteritic or polymyalgic symptoms) and had persistently elevated inflammatory markers and/or severe constitutional symptoms. The incidence of GCA in patients presenting with apparently isolated polymyalgia rheumatica is a subject of controversy in the literature [6–17,21–37]. When TABs are systematically performed in patients considered to have isolated PMR, vasculitic lesions may be found in 0−41% of patients [8,21–34] (Table 2).
To investigate the frequency and type of giant cell arteritis (GCA)-related ischemic complications in a series of patients with GCA who, for a substantial period of time (i.e., at least 3 mo), lacked vascular symptoms and presented with apparently isolated polymyalgia rheumatica (PMR).
Retrospective follow-up study of an unselected population of 167 patients with GCA diagnosed from 1985 to 2014.
In all, 18 patients (11%) developed GCA on a background of a prior history of PMR. They were diagnosed as having isolated PMR because they did not have clinical evidence of GCA at diagnosis and exhibited a prompt and complete response to low-dose steroid therapy. However, during the course of treatment, 17 patients later experienced an arteritic relapse with the development of typical craniofacial symptoms, and one patient developed signs of upper extremity vascular insufficiency, resulting in the diagnosis of large-vessel GCA. The median time to GCA diagnosis from the initiation of low-dose steroid therapy was 9 ± 14.4 mo (range: 3−39).
At the time of GCA diagnosis, severe ischemic complications were observed in 50% (9/18) of the patients. Of these patients 22% (4/18) were considered to have “true” occlusive disease (i.e., permanent visual loss, stroke, and/or limb claudication).
Late inflammation of the aorta and its branches occurred in 4 (22%) of the patients during long-term follow-up.
Patients with GCA presenting with apparently isolated PMR have a significant risk of developing transient or permanent disease-related ischemic complications; these complications occurred in 50% of the cases.

View all citing articles on Scopus

Fabrizio Cantini, MD: Consultant, Director 2nd Divisione de Medicina, Unità Reumatologica, Ospedale di Prato, Italy

Laura Niccoli, MD: Assistant, 2nd Divisione di Medicina, Unitè Reumatologica, Ospedale di Prato, Italy

Lara Storri, MD: Assistant, 2nd Divisione di Medicina, Unità Reumatologica, Ospedale di Prato, Italy

Carlotta Nannini, MD: Assistant, 2nd Divisione di Medicina, Unità Reumatologica, Ospedale di Prato, Italy

Ignazio Olivieri, MD: Consultant, Director Servizio di Reumatologia, Ospedale S.Carlo, Potenza, Italy

Angela Padula, MD: Assistant, Servizio di Reumatologia, Ospedale S.Carlo, Potenza, Italy

Luigi Boiardi, MD: Assistant, Divisione di Reumatologia, Arcispedale S. Maria Nuova, Reggio Emilia, Italy

Carlo Salvarani, MD: Consultant, Director, Divisione di Reumatologia, Arcispedale S. Maria Nuova, Reggio Emilia, Italy.

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Are polymyalgia rheumatica and giant cell arteritis the same disease?

Abstract

Objective

Methods

Results

Conclusions

Section snippets

Methods

Epidemiologic data

Discussion

Clin Rheum Dis

Semin Arthritis Rheum

Hum Immunol

Rev Med Intern

Am J Med

Rheum Dis Clin North Am

Giant cell arteritis, or arteritis of the aged

BMJ

Polymyalgia rheumaticaA manifestation of systemic giant cell arteritis

Ann Intern Med

Polymyalgia rheumatica and giant cell arteritis

N Engl J Med

Polymyalgia rheumaticaA disorder of extraarticular synovial structures?

J Rheumatol

Shoulder ultrasonography in the diagnosis of polymyalgia rheumaticaA case-control study

J Rheumatol

Distal musculoskeletal manifestations in polymyalgia rheumaticaa prospective follow-up study

Arthritis Rheum

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

Arthritis Rheum

Neurologic disease in biopsy-proven giant cell (temporal) arteritis

Neurology

Peripheral neuropathic syndromes in giant cell (temporal) arteritis

Neurology

Color duplex ultrasonography in the diagnosis of temporal arteritis

N Engl J Med

Illustrated histopathologic classification criteria for selected vasculitis syndromes

Arthritis Rheum

Immunohistologic and cytochemical studies of temporal arteritis

Arthritis Rheum

Musculoskeletal manifestations in a population-based cohort of patients with giant cell arteritis

Arthritis Rheum

Polymyalgia rheumatica and seronegative rheumatoid arthritis may be the same entity

J Rheumatol

The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis

Arthritis Rheum

Large artery involvement in giant cell (temporal) arteritis

Ann Intern Med

Imaging findings in extracranial (giant cell) temporal arteritis

Clin Exp Rheumatol

Giant cell arteritis, temporal arteritis, and polymyalgia rheumatica

Arthritis Rheum

The epidemiology of giant cell arteritis including temporal arteritis and polymyalgia rheumatica

Arthritis Rheum

An evaluation of criteria for polymyalgia rheumatica

Ann Rheum Dis

Polymyalgia rheumaticaA 10-year epidemiologic and clinical study

Ann Intern Med

The American College of Rheumatology 1990 criteria for the classification of giant cell (temporal) arteritis

Arthritis Rheum

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

Arthritis Rheum

The incidence of giant cell arteritis in Olmsted County, MinnesotaApparent fluctuations in a cyclic pattern

Ann Intern Med

Trends in the incidence of PMR over a 30 year period in Olmsted County, Minnesota, USA

J Rheumatol

An arteritis with special reference to polymyalgia arteritica

Acta Pathol Microbiol Scand

The epidemiology of giant cell arteritis

Arthritis Rheum

Giant cell arteritis and polymyalgia rheumatica in a region of FinlandAn epidemiologic, clinical and pathologic study, 1984–1988

J Rheumatol

Temporal arteritis in a northwestern area of SpainStudy of 57 biopsy proven patients

J Rheumatol

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

Clin Exp Rheumatol

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

J Rheumatol

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

Arthritis Rheum