-
RMD Open Jan 2024The hallmarks of the chronic inflammatory disease polymyalgia rheumatica (PMR) include pain, and morning stiffness in areas of the neck, shoulder and pelvic girdle. The...
OBJECTIVE
The hallmarks of the chronic inflammatory disease polymyalgia rheumatica (PMR) include pain, and morning stiffness in areas of the neck, shoulder and pelvic girdle. The human leucocyte antigen () gene was reported to be an important risk factor for PMR, but it has not been analysed precisely, especially in populations other than Europeans.
METHODS
Genotyping of and was performed in Japanese PMR patients (n=270) and controls (n=413). Associations between allele carrier and genotype frequencies were determined for PMR.
RESULTS
was associated with a predisposition to PMR (p=0.0006, =0.0193, OR 1.85, 95% CI 1.31 to 2.62). was associated with protection against PMR (p=1.46×10, =0.0004, OR 0.40, 95% CI 0.26 to 0.61). A shared epitope (SE) associated with PMR (p=3.07×10, OR 2.11, 95% CI 1.54 to 2.88). (p=0.0010, c=0.0140, OR 0.52, 95% CI 0.35 to 0.77) was associated with protection against PMR and (p=0.0009, c=0.0140, OR 1.82, 95% CI 1.28 to 2.58) was associated with predisposition to PMR. A gene dosage effect was observed for and , but not for SE or . Haplotype and logistic regression analyses suggested a protective effect for .
CONCLUSION
This study is the first to demonstrate predisposing associations of SE, and , and protective associations of and with PMR in Japanese patients. Our data indicate has predisposing and protective effects on the pathogenesis of PMR.
Topics: Humans; Epitopes; Giant Cell Arteritis; HLA Antigens; Japan; Pain; Polymyalgia Rheumatica; HLA-DR Antigens
PubMed: 38253597
DOI: 10.1136/rmdopen-2023-003897 -
Cureus Dec 2023Japanese spotted fever (JSF) poses a significant public health challenge, mainly due to its atypical presentation in specific demographics. This report details a unique...
Japanese spotted fever (JSF) poses a significant public health challenge, mainly due to its atypical presentation in specific demographics. This report details a unique case of JSF in an 89-year-old female who was admitted to a rural hospital exhibiting generalized pain and rapid cognitive decline but no rash. Initially misdiagnosed as polymyalgia rheumatica, her condition was complicated by thrombocytopenia and altered mental state, prompting consideration of tick-borne illnesses. Subsequent serological analysis confirmed JSF despite the absence of its hallmark rash. The patient's condition escalated to include bacteremia and aseptic meningitis. Treatment involved a regimen of minocycline and meropenem, along with endoscopic cauterization of a bleeding rectal ulcer. After treatment, the patient showed improvement and was transferred for rehabilitation. This case highlights the criticality of considering JSF in elderly patients within endemic areas, even when classic symptoms like erythema and petechiae are absent. It underscores the necessity for broad diagnostic perspectives, especially in atypical presentations, and the integration of comprehensive care approaches. The involvement of caregivers and relatives in early detection and seeking medical care promptly is crucial. The report illustrates the complexities in diagnosing and managing advanced JSF cases and stresses the importance of early serological testing and adaptive treatment strategies in managing such challenging cases.
PubMed: 38229818
DOI: 10.7759/cureus.50681 -
Journal of Family Medicine and Primary... Nov 2023Polymyalgia rheumatica (PMR) is an inflammatory rheumatic condition characterized by pain and stiffness around the shoulders and hip girdles, an elevated erythrocyte...
Polymyalgia rheumatica (PMR) is an inflammatory rheumatic condition characterized by pain and stiffness around the shoulders and hip girdles, an elevated erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) and a dramatic response to corticosteroids. It is usually seen in adults aged over 50 years; about 30% also have giant cell arteritis. Its etiology is unknown. A 72-year-old male received water vapor therapy, a novel, minimally invasive therapy for benign prostate hypertrophy (BPH). On postoperative day 1, he developed severe shoulder pain and weakness, with difficulty with lifting his arms above his head, and hip pain and weakness, with difficulty getting out of a bed or chair. Laboratory results showed elevated ESR and CRP, but a normal creatine kinase level. The patient received low-dose prednisone and had prompt symptom relief. This case illustrates that a diagnosis of PMR after water vapor therapy can be easily overlooked.
PubMed: 38186798
DOI: 10.4103/jfmpc.jfmpc_676_23 -
Cureus Dec 2023We present a rare case of metastatic renal cell carcinoma in a patient who developed giant cell arteritis (GCA) after the administration of checkpoint inhibitor therapy...
We present a rare case of metastatic renal cell carcinoma in a patient who developed giant cell arteritis (GCA) after the administration of checkpoint inhibitor therapy with nivolumab and ipilimumab. The patient was initially treated with a combination of nivolumab and ipilimumab, showing a near-complete response with minimal side effects. However, after several cycles of checkpoint inhibitor therapy, the patient developed symptoms consistent with GCA, leading to a halt in the immunotherapy. This report highlights the complexity of managing the adverse effects of immunotherapeutic agents and the importance of a multidisciplinary approach in the management of complications such as GCA.
PubMed: 38186407
DOI: 10.7759/cureus.50121 -
Scientific Reports Jan 2024Polymyalgia rheumatica (PMR) is a chronic inflammatory disease characterized by arthralgia and myalgia of the shoulder and hip girdles, and fever. PMR is linked to...
Polymyalgia rheumatica (PMR) is a chronic inflammatory disease characterized by arthralgia and myalgia of the shoulder and hip girdles, and fever. PMR is linked to autoimmune diseases and autoinflammatory disorders. Exome sequencing has revealed the roles of rare variants in some diseases. Causative genes for monogenic autoinflammatory disorders might be candidate genes for the selective exome analysis of PMR. We investigated rare variants in the coding and boundary regions of candidate genes for PMR. Exome sequencing was performed to analyze deleterious rare variants in candidate genes, and the frequencies of the deleterious rare alleles in PMR were compared with those of Japanese population controls. Deleterious rare alleles in the NLRL12 gene were associated with PMR (P = 0.0069, Pc = 0.0415, odds ratio [OR] 4.49, 95% confidence interval [CI] 1.79-11.27). A multigene analysis demonstrated the deleterious rare allele frequency of the candidate genes for autoinflammatory disorders was also increased in PMR (P = 0.0016, OR 3.69, 95%CI 1.81-7.54). The deleterious rare allele frequencies of the candidate genes including NLRP12 were increased in PMR patients, showing links to autoinflammatory disorders in the pathogenesis of PMR.
Topics: Humans; Polymyalgia Rheumatica; Inflammasomes; Alleles; Giant Cell Arteritis; Gene Frequency; Intracellular Signaling Peptides and Proteins
PubMed: 38177227
DOI: 10.1038/s41598-024-51320-3 -
Nihon Ronen Igakkai Zasshi. Japanese... 2023Giant cell arteritis (GCA) is closely associated with polymyalgia rheumatica (PMR). We herein report an 82-year-old woman who developed GCA during PMR treatment. She...
Giant cell arteritis (GCA) is closely associated with polymyalgia rheumatica (PMR). We herein report an 82-year-old woman who developed GCA during PMR treatment. She initially presented with shoulder pain and was diagnosed with PMR based on elevated serum C-reactive protein (CRP) levels and bursitis detected in both shoulders on ultrasonography (US). Treatment was initiated with a daily dose of 15 mg prednisolone (PSL), which led to rapid symptom alleviation, and the dosage was tapered to 1 mg/day. One month later, she developed myalgia extending from the lumbar region to the thigh and tenderness in the left temporal region. However, no abnormalities in the temporal artery were observed on US. Although the PSL dose was increased to 2 mg for relapse of PMR, the symptoms did not improve. One week later, she developed occipital pain with an increased CRP level of 9 mg/dL. She was diagnosed with GCA based on the 1990 ACR Classification Criteria. Fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT) detected anomalous accumulations in the bilateral superficial temporal and vertebral arteries, but not in the larger vessels. We therefore diagnosed her with cranial-type GCA. At this time point, we repeated US and found a halo sign in the temporal artery. Although epithelioid and giant cells were not observed in the temporal artery biopsy, vascular inflammatory findings such as disruption of the internal elastic lamina and chronic inflammatory cell infiltration were noted. Symptoms improved immediately and CRP levels decreased after the PSL dose was increased to 30 mg daily. To mitigate the risk of steroid-induced diabetes, tocilizumab was introduced, and gradual tapering of PSL was implemented. In conclusion, we encountered a case of GCA that developed after PSL reduction during the course of PMR. PET/CT confirmed intracranial artery inflammation and facilitated a definitive diagnosis. Although PET/CT cannot be routinely performed for diagnose in Japan, we consider it useful as an adjunctive diagnostic tool.
Topics: Humans; Female; Aged, 80 and over; Giant Cell Arteritis; Polymyalgia Rheumatica; Prednisolone; Positron Emission Tomography Computed Tomography; Drug Tapering; Inflammation
PubMed: 38171762
DOI: 10.3143/geriatrics.60.440 -
Clinical Nephrology. Case Studies 2023We present two atypical cases of calciphylaxis presenting with ocular ischemic pathology - both without the hallmark cutaneous manifestations - to raise awareness of...
PURPOSE
We present two atypical cases of calciphylaxis presenting with ocular ischemic pathology - both without the hallmark cutaneous manifestations - to raise awareness of this rare yet highly disabling condition.
OBSERVATIONS
We report two cases of ophthalmic calciphylaxis presenting as (1) anterior ischemic optic neuropathy (AION) and cilioretinal artery occlusion in a 76-year-old woman with pre-dialysis kidney failure, and (2) AION with contralateral central retinal artery occlusion (CRAO) in a 44-year-old man on hemodialysis.
CONCLUSION AND IMPORTANCE
These cases highlight the need for judicious clinical suspicion of calciphylaxis in patients with kidney failure, presenting with microvascular ischemic ophthalmic pathology such as AION or CRAO. Confirmation with temporal artery biopsy is essential to direct targeted individualized multi-disciplinary treatment of calciphylaxis and avoid unnecessary steroid exposure in cases masquerading as giant cell arteritis (GCA).
PubMed: 38169875
DOI: 10.5414/CNCS111088 -
International Medical Case Reports... 2023Polymyalgia rheumatica (PMR) is a systemic inflammatory disease of the elderly population that increases in incidence as age advances. It is characterised by the sudden...
Polymyalgia rheumatica (PMR) is a systemic inflammatory disease of the elderly population that increases in incidence as age advances. It is characterised by the sudden or sub-acute onset of symptoms affecting the shoulder and pelvic girdles, often accompanied by constitutional symptoms. Due to the lack of consensual diagnostic criteria and specific laboratory or radiological investigations for PMR, its diagnosis can be very challenging, particularly because it can be mimicked or masked by other geriatric syndromes. PMR responds well to glucocorticoid treatment, but if left untreated, can lead to morbidity and poor quality of life. We present the case of an 87-year-old male who presented with a one-week history of localised pain in the left hip joint, later involving the contralateral hip. Previously able to ambulate unaided, his mobility was now severely impaired. Due to his Alzheimer's dementia and multiple comorbid geriatric conditions, extensive investigations were undertaken before a diagnosis of atypical PMR was reached. Treatment with a low dose of prednisolone led to a full recovery. This case highlights the inconsistency between an atypical presentation and the classic presentation of PMR and draws attention to the possibility of missed diagnosis in older, frail patients. Atypical symptomatology on top of cognitive impairment and language barriers can be easily overlooked and left untreated and could lead to severe adverse outcomes. Accurate diagnosis is crucial, as PMR is readily diagnosed, but the treatment with glucocorticoids, though generally straightforward, can pose challenges, particularly when dealing with polypharmacy and multiple coexisting health conditions.
PubMed: 38163043
DOI: 10.2147/IMCRJ.S440486 -
Joint Bone Spine May 2024We aimed to describe the following in patients with polymyalgia rheumatica (PMR): (1) real-world glucocorticoid (GC) therapy, (2) improvement in inflammatory parameters...
OBJECTIVE
We aimed to describe the following in patients with polymyalgia rheumatica (PMR): (1) real-world glucocorticoid (GC) therapy, (2) improvement in inflammatory parameters associated with disease activity (C-reactive protein [CRP] level and erythrocyte sedimentation rate [ESR]), and (3) incidence of GC-related adverse events (AEs).
METHODS
A cohort study was conducted using a Japanese electronic medical records database. We included newly diagnosed PMR patients aged≥50years with baseline CRP levels≥10mg/L and/or ESR>30mm/h and an initial GC dose of≥5mg/day. The outcomes were GC dose, inflammatory parameters, and GC-related AEs.
RESULTS
A total of 373 PMR patients (mean age, 77.3 years) were analyzed. The median initial GC dose was 15.0mg/day, which gradually decreased to 3.5mg/day by week 52. The median cumulative GC dose at week 52 was 2455.0mg. The median CRP level on day 0 was 64.3mg/L, which decreased during weeks 4-52 (1.4-3.2mg/L). At week 52, 39.0% of patients had a CRP level>3.0mg/L. The cumulative incidence of GC-related AEs at week 52 was 49.0% for osteoporosis, 30.2% for diabetes, 14.9% for hypertension, 12.2% for peptic ulcer, 11.3% for dyslipidemia, 2.9% for glaucoma, and 4.3% for serious infection. The incidence of osteoporosis and diabetes increased with the GC dose.
CONCLUSION
The incidence of GC-related AEs was associated with the GC dose in PMR patients. Further research is required to identify treatment strategies that can effectively control PMR disease activity while minimizing GC use.
Topics: Humans; Polymyalgia Rheumatica; Male; Female; Aged; Glucocorticoids; Middle Aged; Treatment Outcome; Cohort Studies; Aged, 80 and over; Japan; C-Reactive Protein; Retrospective Studies; Blood Sedimentation; Dose-Response Relationship, Drug; Severity of Illness Index
PubMed: 38143016
DOI: 10.1016/j.jbspin.2023.105680 -
Journal of Clinical Medicine Dec 2023The potential role of the COVID-19 vaccine and infection to induce autoimmunity is currently underestimated despite the literature emphasizing arthralgia as a common...
Post-COVID-19 and Post-COVID-19 Vaccine Arthritis, Polymyalgia Rheumatica and Horton's Arteritis: A Single-Center Assessment of Clinical, Serological, Genetic, and Ultrasonographic Biomarkers.
The potential role of the COVID-19 vaccine and infection to induce autoimmunity is currently underestimated despite the literature emphasizing arthralgia as a common adverse event. We aimed to study the impact of rheumatological complications post-COVID-19 (PC) and post-COVID-19 vaccine (PCV), comparing undifferentiated arthritis (UA) to Polymyalgia Rheumatica, Horton's Arteritis (PMR-HA) and isolated arthritis to UA with "connective-like" accompanying symptoms. We retrospectively included 109 patients with at least 6 months of follow-up, analyzing serum biomarkers, joint ultrasound (US), lung HRCT, DLCO, and HLA haplotypes. There were 87 UA patients showing increased gastrointestinal and lung involvement ( = 0.021 and = 0.012), higher anti-spike protein IgG levels ( = 0.003), and anti-SARS-CoV-2 IgG positivity ( = 0.003). Among them, 66 cases progressed to ACR-EULAR 2010 early arthritis after 3 months, whereas PMR-HA patients were more commonly PCV (81.8%, = 0.008), demonstrating higher CRP ( = 0.007) and ESR ( = 0.006) levels, a lower rate of ANA positivity ( = 0.005), and a higher remission rate after six months ( = 0.050). In UA patients, the prevalent HLA was DRB1*11 and C*07 (36.8% and 42.1%). Serum calprotectin, interleukin-6, and C*07 ( = 0.021, 0.041, 0.018) seemed more specific for isolated UA. Conversely, "connective-like" arthritis showed poorer DLCO ( = 0.041) and more frequent US synovitis ( = 0.041). In conclusion, UA is a frequent common PC and PCV complication and may persist over time when compared to PMR-HA.
PubMed: 38137631
DOI: 10.3390/jcm12247563