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Internal and Emergency Medicine Oct 2023To assess the rate of PMR who, during the follow-up, undergo a diagnostic shift as well as to assess which clinical, laboratory and US findings are associated to a...
Clinical, laboratory and ultrasonographic findings at baseline predict long-term outcome of polymyalgia rheumatica: a multicentric retrospective study : Polymyalgia rheumatica predicted by ultrasonographic findings polymyalgia rheumatica outcome predicted early by ultrasound.
To assess the rate of PMR who, during the follow-up, undergo a diagnostic shift as well as to assess which clinical, laboratory and US findings are associated to a diagnostic shift and predict the long-term evolution of PMR. All PMR followed-up for at least 12 months were included. According to the US procedures performed at diagnosis, patients were subdivided into four subgroups. Clinical data from follow-up visits at 12, 24, 48 and 60 months, including a diagnostic shift, the number of relapses and immunosuppressive and steroid treatment, were recorded. A total of 201 patients were included. During the follow-up, up to 60% had a change in diagnosis. Bilateral LHBT was associated with persistence in PMR diagnosis, whereas GH synovitis and RF positivity to a diagnostic shift. Patients undergoing diagnostic shift had a higher frequency of GH synovitis, shoulder PD, higher CRP, WBC, PLT and Hb and longer time to achieve remission, while those maintaining diagnosis had bilateral exudative LHBT and SA-SD bursitis, higher ESR, lower Hb and shorter time to remission. Cluster analysis identified a subgroup of older patients, with lower CRP, WBC, PLT and Hb, lower PD signal or peripheral synovitis who had a higher persistence in PMR diagnosis, suffered from more flares and took more GCs. Most PMR have their diagnosis changed during follow-up. The early use of the US is associated with a lower dosage of GCs. Patients with a definite subset of clinical, laboratory and US findings seem to be more prone to maintain the diagnosis of PMR.
Topics: Humans; Polymyalgia Rheumatica; Retrospective Studies; Giant Cell Arteritis; Ultrasonography; Synovitis
PubMed: 37498353
DOI: 10.1007/s11739-023-03373-x -
Medicine Nov 2023The clinical manifestations of Fabry disease affect the nerves, kidneys, heart, skin, gastrointestinal tract and eyes. Our aim is to familiarize people with the FD... (Review)
Review
RATIONALE
The clinical manifestations of Fabry disease affect the nerves, kidneys, heart, skin, gastrointestinal tract and eyes. Our aim is to familiarize people with the FD diagnostic process by reporting this case.
PATIENT CONCERNS
A 79-year-old-male patient presented with muscle pain and weakness in the extremities, also with an increasing erythrocyte sedimentation rate and C-reactive protein. Further examinations revealed that multiple organ involvement, such as rash, myocardial hypertrophy, peripheral neuropathy.
DIAGNOSES
Cardiac MR demonstrated hypertrophic cardiomyopathy, myocardial fibrosis and low myocardial T1 value. The patient was eventually diagnosed with Fabry disease through proteomics and genetic testing.
INTERVENTIONS
The treatment is enzyme replacement therapy (ERT). But this patient could not afford ERT and was given only general symptomatic treatment, pregabalin, and a gradual reduction in glucocorticoid.
OUTCOMES
The patient's symptoms of joint pain and muscle weakness reduced significantly, and ESR and CRP had decreased to normal.
LESSONS
FD is a rare disease and difficult to diagnose, but rare does not mean invisible. FD may present with signs and symptoms of rheumatic diseases. Rheumatologists should be aware and concerned about this disease.
Topics: Aged; Humans; Male; Enzyme Replacement Therapy; Fabry Disease; Giant Cell Arteritis; Heart; Kidney; Polymyalgia Rheumatica; Skin
PubMed: 37933054
DOI: 10.1097/MD.0000000000034630 -
Rheumatology (Oxford, England) Mar 2022To compare and validate the diagnostic accuracy of fluorodeoxyglucose (FDG)-PET/CT scores for PMR; and to explore their association with clinical factors. (Comparative Study)
Comparative Study
OBJECTIVES
To compare and validate the diagnostic accuracy of fluorodeoxyglucose (FDG)-PET/CT scores for PMR; and to explore their association with clinical factors.
METHODS
This retrospective study included 39 consecutive patients diagnosed with PMR and 19 PMR comparators. The final clinical diagnosis was established after 6 months follow-up. Patients underwent FDG-PET/CT prior to glucocorticoid treatment. Visual grading of FDG uptake was performed at 30 anatomic sites. Three FDG-PET/CT scores (the Leuven Score, two Besançon Scores) and two algorithms (the Saint-Etienne and Heidelberg Algorithms) were investigated. Receiver operating characteristic (ROC) analysis with area under the curve (AUC) was performed. Diagnostic accuracy was assessed at predefined cut-off points.
RESULTS
All three FDG-PET/CT scores showed high diagnostic accuracy for a clinical diagnosis of PMR in the ROC analysis (AUC 0.889-0.914). The Leuven Score provided a sensitivity of 89.7% and specificity of 84.2% at its predefined cut-off point. A simplified Leuven Score showed similar diagnostic accuracy to that of the original score. The Besançon Scores showed limited specificity at their predefined cut-off points (i.e. 47.4% and 63.2%), while ROC analysis suggested that substantially higher cut-off points are needed for these scores. The Heidelberg and Saint-Etienne Algorithms demonstrated high sensitivity, but lower specificity (i.e. 78.9% and 42.1%, respectively) for PMR. Female sex and presence of large-vessel vasculitis were associated with lower FDG-PET/CT scores in patients with PMR.
CONCLUSION
The Leuven Score showed the highest diagnostic utility for PMR. A modified, concise version of the Leuven Score provided similar diagnostic accuracy to that of the original score.
Topics: Adult; Aged; Aged, 80 and over; Case-Control Studies; Female; Fluorodeoxyglucose F18; Humans; Male; Middle Aged; Polymyalgia Rheumatica; Positron Emission Tomography Computed Tomography; Retrospective Studies
PubMed: 34117743
DOI: 10.1093/rheumatology/keab483 -
The American Journal of Case Reports Nov 2021BACKGROUND The differential diagnosis of generalized pain includes reactivity associated with bacterial and viral infections, autoimmune rheumatic disease, and...
BACKGROUND The differential diagnosis of generalized pain includes reactivity associated with bacterial and viral infections, autoimmune rheumatic disease, and orthopedic diseases. Obtaining a detailed medical history and establishing an accurate diagnosis are difficult in elderly patients with dementia. In addition, the differential diagnosis between polymyalgia rheumatica and pseudogout is often difficult. Thus, in our work, we examined the importance of interviewing the family of an elderly patient with dementia. CASE REPORT We report the case of an 88-year-old woman with dementia and a history of recurrent pseudogout who presented with a 12-day history of fever and generalized pain. Physical examination findings revealed warmth and swelling in the shoulder joints and right knee. Blood tests indicated increased inflammatory marker levels. The primary working impression was oligo-articular pseudogout. Based on family interview, the patient was seen to manifest atypical symptoms, including movement difficulty. Joint ultrasound findings showed inflammation of the left long head of the biceps attachment. Further, right knee arthrocentesis detected no calcium pyrophosphate crystals. After obtaining a detailed medical history from the patient's family and conducting other diagnostic tests, the patient was finally diagnosed with polymyalgia rheumatica, rather than oligo-articular pseudogout, with rapid improvement after undergoing low-dose prednisolone treatment. CONCLUSIONS Family interviews can be helpful for obtaining correct diagnosis in elderly patients with dementia.
Topics: Aged; Aged, 80 and over; Chondrocalcinosis; Dementia; Diagnosis, Differential; Female; Giant Cell Arteritis; Humans; Polymyalgia Rheumatica
PubMed: 34811343
DOI: 10.12659/AJCR.933926 -
Dermatology and Therapy Sep 2023Psoriasis (PsO) is currently regarded as a systemic inflammatory disease with a growing burden of post-diagnosis associated comorbidities. To determine the initial...
INTRODUCTION
Psoriasis (PsO) is currently regarded as a systemic inflammatory disease with a growing burden of post-diagnosis associated comorbidities. To determine the initial burden of comorbiditis we evaluated the comorbidome at PsO onset.
METHODS
In a matched case-control study, we extracted data on 57,228 patients and 125 morbidities from the Clalit Health Services Israeli insurance database. PsO cases were matched with control individuals by sex and age at enrolment. As pre-existing comorbidities, we considered all conditions already present in controls at the same age as the matched PsO case at the time of their diagnosis. To test for differences in the odds of comorbidities between the case and control groups, logistic regression analyses were run to calculate the odds ratio (OR) for each comorbidity, after which the comorbidome was graphically represented.
RESULTS
In this study we enrolled 28,614 PsO patients and 28,614 controls with an average age of 45.3 ± 19.6 years. At the time of diagnosis, PsO patients were more likely to be diagnosed with 2-4 comorbidities (28.8% vs 23.8%) and > 5 (19.6% vs 12.9%,). PsO patients' specific comorbidomes evidenced several pathological cores: autoimmune and inflammatory systemic diseases [i.e., hidradenitis suppurativa (OR 3.55, 95% CI 1.88-7.28) or polymyalgia rheumatica (OR 3.01 95% CI 1.96-4.77)], inflammatory bowel diseases [i.e., Crohn's disease (OR 2.99 95% CI 2.20-4.13)], pulmonary inflammatory diseases [i.e., chronic obstructive pulmonary disease (OR 1.81 95% CI 1.61-2.04)], hepatological diseases [i.e., cirrhosis (OR 2.00 95% CI 1.36-3.00)], endocrine diseases [dysthyroidisms (OR 1.82 95% CI 1.30-2.59)], mental disorders [i.e., depression (OR 1.72 95% CI 1.57-1.87)], and cardiovascular diseases (i.e., hypertension (OR 1.47 95% CI 1.41-1.53)].
CONCLUSION
The PsO-onset comorbidome may help health professionals plan more comprehensive patient management. By screening for these common PsO-linked conditions, early diagnosis and treatment may become more frequent, thus greatly benefiting patients on their medical journey.
PubMed: 37542678
DOI: 10.1007/s13555-023-00986-0 -
Internal Medicine (Tokyo, Japan) Mar 2022Polymyalgia rheumatica (PMR) is an inflammatory rheumatic disease characterized by stiffness and aching mainly in the shoulders, neck and hip girdles. The underlying...
Polymyalgia rheumatica (PMR) is an inflammatory rheumatic disease characterized by stiffness and aching mainly in the shoulders, neck and hip girdles. The underlying pathogenesis of PMR involves myeloid lineage activation with a high expression of pattern recognition receptors. In addition, vaccination against severe acute respiratory syndrome coronavirus 2 with mRNA-1273 functions as both an immunogen and intrinsic adjuvant. It leads to the activation of innate immunity, resulting in antibody production. We herein report the first case of PMR-like syndrome seven days after mRNA-1273 vaccination. Reassuringly, the symptoms, such as pain of the neck, shoulder girdle and pelvic girdle, as well as elevated inflammatory markers were resolved within a month without glucocorticoid or immunosuppressant administration.
Topics: 2019-nCoV Vaccine mRNA-1273; COVID-19; COVID-19 Vaccines; Diagnosis, Differential; Giant Cell Arteritis; Humans; Polymyalgia Rheumatica; SARS-CoV-2; Vaccination
PubMed: 34980802
DOI: 10.2169/internalmedicine.8829-21 -
Rheumatology (Oxford, England) Dec 2019Compared with conventional cancer therapies, the spectrum of toxicities observed with checkpoint inhibitors is unique and can affect any organ system. Arthralgia and... (Review)
Review
Compared with conventional cancer therapies, the spectrum of toxicities observed with checkpoint inhibitors is unique and can affect any organ system. Arthralgia and myalgia were by far the most commonly reported rheumatic immune-related adverse events in clinical trials, and there is now a growing number of case series and reports describing clinical features of de novo rheumatic immune-related adverse events, which will be the focus of this review. Some patients develop genuine classic rheumatic and musculoskeletal diseases, but a number of rheumatic immune-related adverse events mimic rheumatic and musculoskeletal diseases with atypical features, mainly polymyalgia rheumatica, rheumatoid arthritis and myositis, as well as several systemic conditions, including sicca syndrome, vasculitis, sarcoidosis, systemic sclerosis and lupus.
Topics: Antineoplastic Agents, Immunological; Arthritis, Rheumatoid; Diagnosis, Differential; Humans; Immunologic Factors; Immunotherapy; Myositis; Neoplasm Metastasis; Neoplasms; Polymyalgia Rheumatica; Rheumatic Diseases; Sjogren's Syndrome; Vasculitis
PubMed: 31816082
DOI: 10.1093/rheumatology/kez295 -
European Journal of Rheumatology Jan 2020Polymyalgia rheumatica (PMR) is the most common inflammatory rheumatological condition affecting individuals aged >50 years. There have been rare reports of PMR and... (Review)
Review
Polymyalgia rheumatica (PMR) is the most common inflammatory rheumatological condition affecting individuals aged >50 years. There have been rare reports of PMR and other vasculitides developing within 3 months of influenza vaccination. Influenza is a major public health issue associated with seasonal increased mortality and intensified health care service use. Annual vaccination is the most effective intervention to prevent influenza, especially in elderly individuals. We report a severe "flare" of PMR in a 70-year-old patient after receiving the adjuvanted trivalent influenza vaccine, as recommended by the Joint Committee on Vaccination and Immunisations for this age group in the UK National Health Service in 2018-2019. The adverse event (AE) could be interpreted as the newly described autoimmune/inflammatory syndrome induced by adjuvants (ASIA syndrome) as both PMR and ASIA display hyperactive immune responses. Caution is warranted in the use of vaccine adjuvants in patients with PMR with pre-existing imbalance of B and T cell homeostasis. Rare AEs are important to individuals, and personalized medicine means we should move away from "one size fits all" for vaccines, as well as for therapeutics.
PubMed: 31922479
DOI: 10.5152/eurjrheum.2019.19152 -
Journal of Clinical Medicine Dec 2022Polymyalgia rheumatica (PMR) is an inflammatory rheumatism of the shoulder and pelvic girdles. In 16 to 21% of cases, PMR is associated with giant cell arteritis (GCA)... (Review)
Review
Polymyalgia rheumatica (PMR) is an inflammatory rheumatism of the shoulder and pelvic girdles. In 16 to 21% of cases, PMR is associated with giant cell arteritis (GCA) that can lead to severe vascular complications. Ruling out GCA in patients with PMR is currently a critical challenge for clinicians. Two GCA phenotypes can be distinguished: cranial GCA (C-GCA) and large vessel GCA (LV-GCA). C-GCA is usually suspected when cranial manifestations (temporal headaches, jaw claudication, scalp tenderness, or visual disturbances) occur. Isolated LV-GCA is more difficult to diagnose, due to the lack of specificity of clinical features which can be limited to constitutional symptoms and/or unexplained fever. Furthermore, many studies have demonstrated the existence-in varying proportions-of subclinical GCA in patients with apparently isolated PMR features. In PMR patients, the occurrence of clinical features of C-GCA (new onset temporal headaches, jaw claudication, or abnormality of temporal arteries) are highly predictive of C-GCA. Additionally, glucocorticoids' resistance occurring during follow-up of PMR patients, the occurrence of constitutional symptoms, or acute phase reactants elevation are suggestive of associated GCA. Research into the predictive biomarkers of GCA in PMR patients is critical for selecting PMR patients for whom imaging and/or temporal artery biopsy is necessary. To date, Angiopoietin-2 and MMP-3 are powerful for predicting GCA in PMR patients, but these results need to be confirmed in further cohorts. In this review, we discuss the diagnostic challenges of subclinical GCA in PMR patients and will review the predictive factors of GCA in PMR patients.
PubMed: 36556036
DOI: 10.3390/jcm11247412 -
International Journal of Environmental... Apr 2020In patients with neuromuscular disorder, only little data of myalgia frequency and characterization exists. To date, only a weak correlation between pain intensity and...
BACKGROUND
In patients with neuromuscular disorder, only little data of myalgia frequency and characterization exists. To date, only a weak correlation between pain intensity and pressure pain threshold has been found, and it remains enigmatic whether high pain intensity levels are equivalent to high pain sensitivity levels in neuromuscular disorders.
METHODS
30 sequential patients with suspected neuromuscular disorder and myalgia were analyzed with regard to myalgia characteristics and clinical findings, including symptoms of depression and anxiety and pain- threshold.
RESULTS
A neuromuscular disorder was diagnosed in 14/30 patients. Muscular pain fasciculation syndrome (MPFS) without evidence for myopathy or myositis was diagnosed in 10/30 patients and 6/30 patients were diagnosed with pure myalgia without evidence for a neuromuscular disorder (e.g., myopathy, myositis, MPFS, polymyalgia rheumatica). Highest median pain scores were found in patients with pure myalgia and polymyalgia rheumatica. Pressure pain threshold measurement showed a significant difference between patients and controls in the biceps brachii muscle.
CONCLUSION
Only a weak correlation between pain intensity and pressure pain threshold has been suggested, which is concordant with our results. The hypothesis that high pain intensity levels are equivalent to high pain sensitivity levels was not demonstrated.
Topics: Adult; Anoctamins; Female; Humans; Male; Middle Aged; Muscle, Skeletal; Muscular Diseases; Myalgia; Myositis; Pain Threshold; Young Adult
PubMed: 32268560
DOI: 10.3390/ijerph17072502