Current diagnosis and treatment of polymyalgia rheumatica

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.     

Drug therapies for polymyalgia rheumatica: a pharmacotherapeutic update

Introduction: Polymyalgia rheumatica (PMR), a common disease in individuals older than 50 in the western world, is characterized by bilateral inflammatory pain involving the shoulder girdle and less commonly the neck and pelvic girdle. The main goals of the currently available treatment are to induce remission and prevent relapse.

Areas covered: This review briefly presents the main epidemiological and clinical features of PMR and discusses in depth both its classical management as well as new therapies used in PMR.

Expert opinion: In general, patients with isolated PMR experience a rapid response (in less than seven days) to 12.5–25 mg/prednisone/day. Methotrexate is the conventional disease-modifying antirheumatic drug most commonly used for disease management, especially for relapses of the disease. However, this agent often yields a modest effect. Randomized controlled trials do not support the use of antitumor necrosis factor agents in PMR. Several case series and retrospective studies have highlighted the efficacy of the anti-interleukin-6 receptor antibody tocilizumab in PMR. However, controlled trials are needed to fully establish the efficacy of this biologic agent in PMR. The potential beneficial effect of the Janus-kinase inhibitors remains to be determined.     

The diagnosis and management of temporal arteritis

Temporal arteritis (TA), or giant cell arteritis, is a systemic autoimmune vasculitis affecting patients over 50 years of age. It can cause rapid, irreversible bilateral vision loss in older adults and is therefore considered an ophthalmological emergency. Many of the symptoms and signs of TA can be vague, non-specific and gradual in onset, often leading to a delayed or inaccurate diagnosis. As such, it is important for a wide variety of primary optometrists and health practitioners to maintain a robust understanding of the clinical presentation, key investigations and time-sensitive management of this disease, as early initiation of treatment for TA can be vision- and life-saving.     

Hidden coexisting pathology diagnosed after cervical surgery in patients with degenerative cervical myelopathy or myeloradiculopathy: A case series report

Many neurological disorders can present similar symptomatology to degenerative cervical myelopathy (DCM) or myeloradiculopathy (DCMR). Therefore, to avoid misdiagnosis, it is important to recognise the differential diagnosis, which has been well described in previous literature. Additionally, DCM or DCMR can also coexist with other diseases that overlap some of its clinical manifestations, which may be overlooked before cervical surgery. Nevertheless, few studies have addressed this clinical situation. In clinical practice, the diagnosis of coexisting disease with DCM or DCMR would be typically made when some symptoms persist without improvement after cervical surgery. To inform the patients of this possibility preoperatively and arrive at the early diagnosis during the postoperative period, some knowledge of the possible coexisting diseases would be necessary. In this report, we reviewed 230 patients who underwent surgery for DCM or DCMR in an academic centre to examine the prevalence and kind of underlying disease that was overlooked preoperatively. The coexisting diseases relevant to their baseline symptoms were diagnosed only after cervical surgery in three patients (1.3%) and included amyotrophic lateral sclerosis, lung cancer and polymyalgia rheumatica. The overlapping symptoms were gait difficulty, scapular pain and neck pain, respectively. Surgeons should recognise that the coexisting disease with DCM or DCMR may be overlooked before cervical surgery because of overlapping symptomatology, although its prevalence is not certainly high. Further, when the specific symptom persisted without improvement after surgery for DCM or DCMR, the patient should be comprehensively examined, considering diverse pathological conditions, not only neurological disorders.     

Tocilizumab controls bone turnover in early polymyalgia rheumatica

ObjectivesThis study explores changes in the bone homeostasis by testing the N-terminal collagen type I extension propeptide (PINP) marker for osteo-formation and the carboxy-terminal region of collagen type I (CTX-I) marker for osteo-resorption in patients taking tocilizumab for polymyalgia rheumatica (PMR).MethodsTwenty patients were included in the prospective open-label TENOR study (Clinicaltrials.gov NCT01713842) and received three monthly tocilizumab infusions, followed by corticosteroids starting at week (W) 12. PINP and CTX-I were tested at inclusion (W0), after tocilizumab but before steroid initiation (W12), at the end of the protocol (W24) and were compared to healthy controls. Information regarding disease activity, bone mineral density using scanographic bone attenuation correlation (SBAC), inflammatory parameters and interleukin (IL)-6 levels were collected during the follow-up of the patients.ResultsPMR patients were characterised by a reduction in bone mineral density and a higher level of CTX-I relative to healthy controls matched in age and sex at baseline. PINP levels increased at W12 (P < 0.001, versus W0) following tocilizumab introduction and CTX-I levels decreased at W24 and after steroid initiation (P = 0.001, versus W0). Such modifications explain the altered correlation observed between PINP and CTX-I at W0 (r = 0.255 at W0 versus r = 0.641 in healthy controls) and its correction after treatment (r = 0.760 at W12 and r = 0.767 at W24). Finally, greater changes in PINP were observed in patients whose circulating IL-6 levels decreased after tocilizumab therapy.ConclusionsControl of bone turnover, in part through the inhibition of the IL-6 axis, is observed during tocilizumab and subsequent steroid treatment of PMR.     

Acute post-streptococcal polymyalgia: Two new cases with a review of the literature

Two patients with acute post-streptococcal polymyalgia are described with a review of the seven cases previously reported cases. The common features are sudden onset of muscular pain with fever usually after an acute upper respiratory tract infection. Antistreptolysin O titre and inflammatory indexes are increased and muscle enzymes are normalConclusion Acute post-streptococcal polymyalgia should be considered as a possible diagnosis in every child complaining acute polymyalgia.     

28例风湿性多肌痛和颞动脉炎随诊分析

目的评价28例风湿性多肌痛(PMR)和颞动脉炎(TA)患者治疗过程,分析影响激素治疗时间和复发的因素。方法回顾性分析1992年至2001年本院诊治的28例PMR和TA患者,其中22例患单纯性PMR,3例PMR合并TA,3例单纯性TA,全部患者均应用糖皮质激素。其中13例加用免疫抑制剂。平均治疗时间25.5±24.0个月。按患者对激素有无抵抗及治疗后有无复发分组,对临床资料分析比较并分析影响PMR和TA治疗及预后的因素。结果激素抵抗组治疗前血沉和外周血白细胞水平较无抵抗组明显升高(P<0.01);复发组激素减量速度较无复发组快(P<0.05)。结论治疗前血沉快和外周血白细胞高者易发生激素抵抗。激素减量过快易复发。对激素治疗抵抗和复发者,或PMR合并TA者应加用免疫抑制剂治疗。     

加味阳和汤配合糖皮质激素治疗风湿性多肌痛临床观察

目的观察加味阳和汤对糖皮质激素治疗风湿性多肌痛使用剂量的影响。方法风湿性多肌痛患者61例,随机分为2组,中药组（加味阳和汤加泼尼松组）32例,对照组（单用泼尼松组）29例,两组均给予泼尼松20mg／d,2周后根据病情酌情减量,中药组加服加味阳和汤煎剂,每天l剂,共治疗12用。结果两组对风湿性多肌痛活动性疗效：中药组32例临床缓解9例（28．1％）,显效15例（46．9％）,有效7例（21．9％）,无效l例（3．1％）,总有效率96．9％;对照组29例分别为3例（10．3％）,ll例（37．9％）,10例（34．5％）,5例（17．2％）及82．8％。两组比较,差异有显著性（U=2．109,P〈0．05）;中药组血沉比对照组下降快（t=2．957,P〈0．05）;中药组泼尼松用量比对照组用量小（t=10．23,P〈0．05）。结论加味阳和汤有助于缩短治疗风湿性多肌痛的疗程,减少糖皮质激素的剂量,提高疗效。     

Aortic aneurysm and dissection are not associated with an increased risk for giant cell arteritis/ polymyalgia rheumatica

It has recently been claimed that giant cell arteritis (GCA) is associated with a markedly increased risk of aortic aneurysm formation or rupture. In the present study, the opposite approach was taken, by looking for the incidence of GCA and polymyalgia rheumatica (PMR) in patients with aortic aneurysm, aortic dissection, or both (AA/D). The records of 315 consecutive patients admitted with the diagnosis of AA/D were reviewed. In addition, follow up information was obtained in 82 patients by examination in the outpatient clinic. After careful examination and assessment of clinical and laboratory data, it was found that none of the 82 patients who survived hospitalisation and were available for examination had GCA or PMR. Moreover, review of the retrospective data available from hospital records of the total consecutive 315 patients with AA/D failed to find any patient with a diagnosis of GCA/PMR. In conclusion, the present study did not find an increased prevalence of GCA/PMR among a cohort of Israeli patients with AA/D. Therefore, it is suggested that a thorough investigation aiming to diagnose GCA/PMR is not cost effective in most of the elderly patients presenting with AA/D.