These are a good example of classification philosophy to reduce heterogeneity of positively classified cases by selecting the subset of the most typical manifestations.
Therefore, these criteria are not to be met in atypical cases of PMR. On the other hand, these have a potential to reduce the rate of false positive diagnosis Source : Ref Musculoskeletal ultrasound gains importance in rheumatology. PMR criteria integrated ultrasonographic evaluation into classification process for the first time in rheumatology Ultrasound criterion requires examining both shoulders for glenohumeral synovitis, bursitis or biceps tenosynovitis and hips for joint synovitis or trochanteric bursitis.
The intention for this is assessment of the symmetry of changes between inflammatory changes in both shoulders and between upper and lower limbs involvement. Each of these will score an additional point to the scoring algorithm. Final sum of five out of eight 6 from algorithm without ultrasound plus 2 from ultrasound examination points enables to classify a patient as PMR with 66 per cent sensitivity and 81 per cent specificity Ultrasound evaluation is relatively simple to perform as the findings of merely the presence of joint effusion, tenosynovitis or bursitis are sufficient.
However, these abnormalities are hardly specific for PMR. A short ultrasound examination of proximal joints usually fails to demonstrate differences between inflammatory joint diseases. A more detailed ultrasound assessment can demonstrate joints' erosions and extensive synovial proliferation of both small and large joints that are more typical for RA. Ultrasound can demonstrate degenerative or post-traumatic joint lesions as well as detect GCA overlap 40 Fig.
Ultrasound examination of glenohumeral joint from axillary approach revealing no significant effusion inside a joint capsule bottom of the picture. There are no pathognomonic antibodies or other PMR-specific markers discovered. Other acute phase markers fibrinogenemia, thrombocythemia and elevated IL-6, the latter correlates best with the disease activity 42 are also present. Anaemia of chronic disease type is common and is reversed shortly after CSs treatment initiation. Sometimes, slightly increased transaminases and alkaline phosphatase levels are present.
Sparse studies indicated a significantly higher occurrence of anti-phospholipid antibodies, but these have not been proved to be associated with ischaemic or thromboembolic complications 42 , 43 , 44 , The benefits and drawbacks of classification criteria sets must be duly considered before applying them for diagnosis.
However, ESR, rheumatoid factor and ultrasound changes still have only limited specificity. The drawbacks of all of the PMR criteria sets are their unsatisfactory sensitivity and specificity. They were also formulated in populations with a high PMR prevalence. If classification criteria are not met which usually takes place in atypical PMR , the disease should not be diagnosed hastly but only after excluding other causes of similar symptoms 23 , The need for considering PMR exclusions was underlined in the previous criteria 37 and is also found in the current guidelines The typical clinical picture of PMR requires only basic differential diagnostics.
The more atypical the clinical picture, the wider differential diagnostics is required. The differential diagnostics in countries with low PMR incidence requires considering the relatively higher number of PMR mimics. It was illustrated in a study from Turkey that 30 per cent of patients with final PMR diagnosis were hospitalized, 30 per cent were treated with antibiotics, and in 29, 22 and 19 per cent abdominal, chest and brain computed tomography CT , respectively, were performed.
Why do PMR-like manifestations mask the symptoms of other diseases? PMR pathogenesis is mediated by innate immunity. It triggers non-specific inflammatory reaction which is not unique for PMR Acute inflammatory response can mask more characteristic symptoms of a disease that are not reported by patients. For example, elderly onset RA may go with systemic inflammatory manifestations and large joint involvement that cause patient immobilization. Therefore, small joints inflammation is not reported by a patient whose main complaint is inability to get out of bed.
Further, serious, GCA-associated ischaemic manifestations such as double vision or jaw claudication that are typical prodromal symptoms of vision loss can be unreported by patients seeking medical advice because of much more disturbing manifestations of overlapping PMR.
Paraneoplastic syndromes that can be manifested long before an appearance of symptoms associated with tumour growth may also be misclassified as PMR Differentiation between PMR and seronegative, elderly onset RA affecting proximal joints is actually a common reason for diagnostic uncertainty.
It may also be a case if bilateral painful shoulder syndrome coexists with depression and elevated ESR. Ultrasound examination of the shoulder joints may be helpful in determining the cause of the pain. Diagnosing the sources of inflammatory reaction and mood disorders in the elderly may be demanding, requiring knowledge on geriatrics. Musculoskeletal symptoms resembling PMR may originate from myopathy due to hypo- or hyperthyroidism, CSs or statins use, amyloidosis; Addison's disease also adynamia suggesting depression and a good response to CSs 49 , Typical PMR age group is associated with a high risk of cancer.
Manifestations of PMR may also resemble paraneoplastic syndromes. However, PMR frequently starts suddenly and manifests more dynamically. Spontaneous remission, which can occur in PMR, is unusual for cancer. Attempts should be made to minimize this period by differential diagnostics and careful observation of atypical PMR cases Some of the PMR symptoms fever, night sweats and joint pain may suggest systemic lupus erythematosus or other autoimmune diseases and infectious diseases, including endocarditis or tuberculosis.
Focus on musculoskeletal pain can mask the endogenous or reactive depression being the real cause of deterioration of patient's state. Due to PMR and GCA overlap, physical examination of PMR patients should encompass temporal arteries for tenderness, loss of pulsation and large arteries analogically to Takayasu arteritis upper and lower limbs intermittent claudication, differences in blood pressure between both limbs, presence of vascular bruits.
Treatment-resistant PMR indicates a special need for imaging of large arteries for overlapping vasculitis. It may include ultrasound examination of temporal and large axillary, sub-clavian, common carotid arteries by a specialist experienced in differentiating vascular wall inflammation from arteriosclerosis, as well as assessment of the aorta and its branches with contrasted computed tomography CT , magnetic resonance imaging MRI or positron emission tomography PET with CT 52 , Lack of GCA manifestations at the time of PMR diagnosis should not stop the awareness of developing vasculitis during follow up.
PMR patients should be educated to immediately seek medical advice in case of vision disturbance double vision, transient ischaemic attacks , jaw claudication or scalp tenderness. Rapid and spectacular improvement shortly after CSs introduction enables concluding on a cause based on an observed response to the treatment.
Therefore, PMR patients are much pleased shortly after treatment initiation and grateful to their doctors Diagnosis ex juvantibus was included in Jones and Hazleman's criteria New, PMR classification criteria do not include a good response to CSs in the diagnostic process It raised some discussion as this criterion is widely used in a daily practice It was argued that treatment response was non-specific and difficult to define feature.
Indeed, elderly onset RA or other inflammatory conditions also respond well to CSs. However, this response would be weak in OA and transient if applied in infection or neoplasm. In these cases review of the initial PMR diagnosis is needed. It is not a mistake to reassess the initial diagnosis and change it accordingly. About 10 per cent of patients initially diagnosed with PMR are later reclassified as having elderly onset RA For that purpose, careful monitoring of patients is needed.
PMR patients require regular medical check-ups. Diagnosing ex juvantibus may also be made eagerly because it does not require an effort of time-consuming and expensive procedures. Based on our own experience, PMR is easy to overdiagnose. Establishing rational PMR diagnosis illustrates a challenge to resist fashion and wishful thinking in medicine. Adherence to the PMR classification criteria could be beneficial in preventing overdiagnosis because these do not include response to therapy.
At least as long as there are no better disease markers. Lack of specific biomarkers of PMR is problematic and research is needed. Up to now, the diagnosis remains clinical. There are many clinical subtleties to be considered. However, differential diagnosis encompasses diseases with bad prognosis; therefore, PMR overdiagnosis can be detrimental.
Conflicts of Interest: None. National Center for Biotechnology Information , U. Indian J Med Res. Marcin Milchert and Marek Brzosko. Author information Article notes Copyright and License information Disclaimer. Reprint requests: Dr.
Received Feb This article has been cited by other articles in PMC. Abstract Polymyalgia rheumatica PMR is a unique disease of elderly people, traditionally diagnosed based on a clinical picture. Key words: Classification criteria, corticosteroids, diagnosis, musculoskeletal, polymyalgia rheumatica. Introduction Polymyalgia rheumatica PMR is an auto-inflammatory rheumatic disease of people over 50 years, presenting with pain and stiffness in the neck, shoulder and hip girdles 1.
Table I Differences in treatment strategy underlining the need for identifying concomitant giant cell arteritis GCA in patients with polymyalgia rheumatic PMR. Open in a separate window. How to Diagnose Polymyalgia Rheumatica? Musculoskeletal manifestations It is difficult to find PMR case without bilateral pain and stiffness of muscles and joints of neck, shoulder and hip girdles.
Manifestations associated with inflammatory response or deterioration of general state It is surprising in PMR patient as to how intense inflammatory reaction in elderly may manifest. It is governed by the peer review system and all original papers are subject to internal assessment and external reviews.
The journal accepts submissions of articles in English and in Spanish languages. The Impact Factor measures the average number of citations received in a particular year by papers published in the journal during the two preceding years. SRJ is a prestige metric based on the idea that not all citations are the same. SJR uses a similar algorithm as the Google page rank; it provides a quantitative and qualitative measure of the journal's impact.
SNIP measures contextual citation impact by wighting citations based on the total number of citations in a subject field. Polymyalgia rheumatica PMR is an inflammatory rheumatic disease that commonly affects individuals older than 50 years old. It is characterised by pain and morning stiffness in the shoulder and pelvic girdle.
This disease can occur as an isolated phenomenon or in association with giant cell arteritis. Here we describe a case of nephrotic syndrome in a patient with PMR with no evidence of malignant disease. The patient remained independent for performing activities of daily living and suffered no cognitive deterioration.
The patient reported only a slight decrease in the rhythm of diuresis and one occasion of nicturia. A blood work analysis revealed: creatinine: 0. Haemogram and clotting tests results were normal. Viral serology for hepatitis B and C and human immunodeficiency virus was negative.
In the immunological study, antinuclear antibodies and anti-neutrophil cytoplasmic antibodies were negative, with normal complement. An electrophoresis analysis using a blood sample revealed a decrease in albumin with no signs of monoclonal peaks. Tumour markers carcinoembryonic antigen [CEA], Ca, alpha-fetoprotein, and prostate specific antigen [PSA] were all within normal values.
A hour urine protein analysis revealed 9. Urine electrophoresis also ruled out the presence of monoclonal peaks, with a negative Bence-Jones proteinuria test. Several months 6—10 months on average after the fall, none of these five patients had a new relapse.
No significant differences were found when we compared age, sex, and the cumulative dose of GC at the time of the fall between the group of patients with PMR relapse and the group of patients without.
The possibility of PMR relapse being realised immediately after a fall should be kept in mind in daily practice, especially when typical manifestations reappear immediately after a fall and other diagnostic hypotheses have been carefully excluded. If our monocentric data are confirmed by multicentric data, the assessment of the risk of falls through specific scales should be an integral part of the visit of all PMR patients.
Polymyalgia rheumatica PMR can be considered the most frequent inflammatory rheumatic disease in persons older than 70 years [ 1 , 2 ]. Its diagnosis is based on recognition of a clinical syndrome consisting of pain and stiffness in the shoulder and pelvic girdle and morning stiffness lasting at least 45 minutes, as underlined in all diagnostic criteria proposed since [ 3 , 4 ]. In most cases the erythrocyte sedimentation rate ESR and serum C-reactive protein CRP values are elevated, but the possibility that the clinical manifestations of PMR can be associated with normal values of ESR has been highlighted since by Ellis and Ralston [ 5 ].
Low-dose glucocorticosteroids GC are an effective treatment resulting in a striking improvement of symptoms and reduction of inflammatory indices but the rates and timing of response are not the same for all patients [ 6 ]. The relapse occurs mostly between 6 and 12 months after diagnosis. Some of these have a repeated relapsing course with GC therapy for several years and sometimes for a lifetime [ 8 ].
The role of external factors in inducing PMR relapse has been poorly investigated [ 8 ]. We present a case-series of five PMR patients who visited our gerontorheumatological outpatient clinics in the last two years, who presented a relapse immediately after a fall. The day after, she felt bilateral shoulder and neck pain associated with fever and morning stiffness lasting two hours.
She had gone to the hospital emergency, where bone fractures were excluded. She was advised to take a non-steroidal anti-inflammatory drug twice a day. We visited her two days later in our rheumatological outpatient clinic. An year-old Caucasian woman affected by PMR was in remission with 2. She fell and had a right ankle fracture. The next day she woke up unable to raise her arms, with violent neck pain and stiffness lasting about three hours.
Accompanied to the hospital, a left wrist fracture of Colles was diagnosed. The day after, the patient was unable to get up from the bed due to violent girdle pain; he complained of morning stiffness lasting about 45 minutes. Accompanied in hospital, fractures were excluded and hospitalisation was recommended for observation. The day after, she was unable to get out of bed due to violent pains located at the back and shoulders.
After a neurological examination and a TAC of the neck and skull, an injection of non-steroidal anti-inflammatory drug was made without any benefit. The rheumatologist diagnosed a PMS relapse and recommended that the prednisone dosage be increase to PMR-AS score was After seven days the prednisone dose was reduced to 10 mg.
An year-old Caucasian woman in PMR remission with 2. The next morning, when we visited her, despite having taken an analgesic tablet in addition to 2. The left knee was not swollen. In the early s, some investigators highlighted that an altered adrenal responsiveness to the adrenocorticotrophic hormone ACTH stimulation was present in untreated PMR patients and they hypothesised that PMR could be considered as a disease of hypothalamic-pituitary-adrenal HPA axis. A mechanism of immunity stimulation with small bleeding due to a fall could represent another hypothetical pathogenetic mechanism.
In the first half of 20 th century, so-called autohaemotherapy was recommended. It is possible that a similar mechanism associated with small extravasation of blood contributed to enhanced inflammatory response in PMR patients, and relapse is secondary to this. As is well known, approximately half of PMR patients experience a relapse.
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