Giant Cell Arteritis: Oral Versus Intravenous Corticosteroids

This review summarizes the current literature on the use of oral versus intravenous steroids for giant cell arteritis. Giant cell arteritis is an immune-mediated vasculitis of medium to large sized arteries that affects individuals older than the age of fifty. Patients typically present with signs of vascular insufficiency of the extracranial arteries of the head and systemic inflammation. Steroids remain the backbone of therapy, but the dose, maintenance and route of administration remain debatable.     

A case of giant cell arteritis and polymyalgia rheumatica misdiagnosed as temporomandibular dysfunction

We report the case of a 83-year-old man who presented with bilateral scalp ulcerations, temporomandibular joint pain, anorexia, and generalized discomfort. The aspecific presentation and timing of the symptoms led to a referral delay of 117 days. A temporal artery biopsy confirmed the diagnosis of giant cell arteritis (GCA). FDG-PET showed increased uptake at the large vessels, shoulders, and hips, suggesting concomitant polymyalgia rheumatica. Initiation of methylprednisolone resulted in resolution of the muscle-related complaints and healing of the ulcerations. This report highlights the importance of prompt diagnosis and the rarity of bilateral scalp necrosis in GCA, with an incidence of <5%.     

Soluble Receptor Activator of Nuclear Factor-κB Ligand (sRANKL)/Osteoprotegerin Balance in Ageing and Age-Associated Diseases

Recently, novel members of the TNF/TNF receptor superfamily, receptor activator of nuclear factor- kappa B ligand (RANKL), its receptor RANK, and the decoy receptor osteoprotegerin (OPG), have been identified as paracrine mediators of both the immune system and bone functions. The balance of RANK/RANK-L and OPG is critical for osteoclastogenesis modulation and physiological bone remodeling. In order to evaluate whether RANKL/OPG balance is modified by ageing, we analyzed, by imunoassay, systemic levels of OPG and sRANKL in healthy elderly subjects (age range from 70 to over 90 years) and in patients affected by two age-related diseases, osteoarthritis (OA) and polymyalgia rheumatica (PMR), characterized by bone metabolism alteration and involvement of the immune system. We demonstrated that (a) plasma concentrations of OPG increased significantly with age; (b) conversely, sRANKL significantly declined in the group of subjects aged between 81 and 90 years, being similar to the young controls in the other age groups; (c) in OA and PMR, circulating OPG did not differ from plasma levels found in age-matched control groups, while sRANKL concentration was significantly increased compared to controls. Hence, in ageing, the sRANKL/OPG system appears to be modified, with prominent changes in circulating OPG levels; in OA and PMR, the sRANKL/OPG balance alteration was shown to be mainly due to the increase of plasma sRANKL concentration.     

三种自身抗体联合检测在老年起病类风湿关节炎诊断中的作用

目的 研究抗环瓜氨酸肽(cyclic citrulinated peptid,CCP)抗体、抗角蛋白抗体(anti-keratin antibody,AKA)和抗RA33抗体在老年起病的类风湿关节炎(EORA)中的临床意义,探讨3种自身抗体联合检测在EORA诊断中的作用. 方法 检测69例EORA、73例风湿性多肌痛(PMR)和65例老年骨关节炎(0A)患者体内3种抗体水平,抗CCP抗体和抗RA33抗体采用酶联免疫吸附试验法(ELISA),AKA采用间接免疫荧光法(IIF)检测. 结果 EORA患者3种抗体的敏感性和特异性分别为55.1%和94.3 0A、31.3%和91.5%、36.2%和95.4%,显著高于其他两组(P<0.05).3种抗体串联检测,EORA组的阳性率显著高于PMR组和OA组(均为P<0.05),3种抗体串联检测敏感性降低,但其特异性提高至100.0%,且有更高的阳性预测值. 结论 抗CCP抗体、AKA和抗RA33抗体在EORA患者中均可检出,抗CCP抗体有较高的敏感性和特异性,3种抗体联合检测有更高的特异性和阳性预测值;结合临床症状、影像学改变,3种抗体联合检测对EORA的诊断具有较高的临床应用价值.     

Tocilizumab for polymyalgia rheumatica: Report of two cases and review of the literature

Background

Glucocorticoids (GC) are the mainstay of treatment of polymyalgia rheumatica (PMR). However GC-related adverse events occur frequently, particularly in patients with relapsing disease. Several studies have demonstrated that IL-6 is a key player in the pathogenesis of PMR.

Objectives

To report 2 patients with PMR treated with the anti-IL-6 receptor monoclonal antibody tocilizumab (TCZ) and to review the published evidence on the efficacy and safety of TCZ in patients with PMR.

Methods

We treated 2 GC-naive patients with newly diagnosed pure PMR with monthly TCZ infusions (8 mg/kg body weight) for 6 months. Disease activity and drug tolerability were assessed clinically, by laboratory tests, and bilateral shoulder ultrasonography before starting the treatment and subsequently every month during TCZ therapy. We performed a systematic literature search (PubMed until July 2012) using the terms “tocilizumab,” “anti-IL-6-receptor,” “polymyalgia rheumatica,” “giant cell arteritis”, and “large-vessel vasculitis” to identify published reports of patients with PMR treated with TCZ.

Results

One of our patients responded well to TCZ, while the other patient required GC therapy after the 2nd TCZ infusion because of lack of appreciable clinical response. Both patients tolerated TCZ well. The review of the literature revealed 4 reports with a total of 9 patients who received TCZ for PMR. In 7 of these 9 patients, PMR was associated with giant cell arteritis. Including our patients, 5 patients received TCZ alone and 6 TCZ plus GC. A good response to TCZ treatment was observed in all patients reported in the literature without any major adverse events.

Conclusions

TCZ both as monotherapy and in association with GC appears to be mostly effective and safe to treat patients with PMR. However, larger controlled studies are required to confirm these favorable data.     

Elderly onset rheumatoid arthritis and polymyalgia rheumatica: ultrasonographic study of the glenohumeral joints

The glenohumeral joints of 32 patients (aged 60 or above) were examined using ultrasonography. Thirteen patients were suffering from characteristic polymyalgia rheumatica (PMR) symptoms. In contrast 19 other patients initially had similar complaints, but were diagnosed as having elderly onset rheumatoid arthritis (EORA) upon development of typical symptoms. Ultrasound examination revealed glenohumeral joint inflammation in 61% (8 out of 13) of the patients with PMR and 63.2% (12 out of 19) of the patients with EORA. These findings suggest that a subgroup of patients with PMR and EORA suffers from shoulder joint inflammation and this synovitis/bursitis/intraarticular effusion might play an important role in the understanding of their symptoms. We conclude that overlapping forms of PMR and a predominate rheumatoid factor negative subgroup of EORA might exist and should be further characterized. Received: 25 August 1997 / Accepted 9 January 1998     

CRANIAL ARTERITIS A TWENTY-YEAR REVIEW OF CASES

Thirty-five cases of cranial arteritis in an Australian population have been analysed. The female to male ratio was 2.2:l and the average age of onset was 71 years. Eighty-five percent of patients suffered headache and this was most frequently temporal or bitemporal. Polymyalgia rheumatica (40%), permanent visual loss (29%) and jaw claudication (26%) were common, six patients complained of diplopia and four gave a past history of thyroid disease. The temporal artery was clinically abnormal in only 69% of cases but biopsy was positive in 26 of 28 specimens examined. Sixty-seven percent of patients had ESR's greater than 60 mmlh. The average duration of prednisone therapy was 22 months. Side effects of steroids were observed in seven of 19 patients followed up. The importance of starting steroid therapy as soon as the condition is suspected clinically is emphasised by the case of one patient who lost vision while awaiting temporal artery biopsy.     

Repetitive 18F‐fluorodeoxyglucose positron emission tomography in giant cell arteritis: A prospective study of 35 patients

Objective

To study fluorodeoxyglucose (FDG) uptake in the different vascular beds and in the large joints of patients with giant cell arteritis (GCA) at diagnosis, during steroid treatment, and at relapse.

Methods

All consecutive patients admitted to our department with a diagnosis of GCA underwent FDG–positron emission tomography (PET) scan before treatment with methylprednisolone was started. PET scans were repeated at 3 and 6 months, if the initial PET scans showed vascular FDG uptake. PET scans were scored at 7 different vascular areas and a total vascular score (TVS) was calculated, ranging from 0 to 21.

Results

A total of 35 patients entered the study. At diagnosis, vascular FDG uptake was noted in 29 patients (83%), especially in the subclavian arteries (74%), but also in the aorta (>50%) and up to the femoral arteries (37%). TVS decreased from a mean ± SD score of 7.9 ± 5.5 at baseline to 2.4 ± 3.5 on repeat PET scan at 3 months (P < 0.0005), but did not further decrease at 6 months. The patients who relapsed had similar earlier decreases of TVS compared with those who did not relapse. FDG uptake in the shoulders at diagnosis correlated significantly with the presence of polymyalgia rheumatica (P = 0.005).

Conclusion

FDG uptake in the large vessels is a sensitive marker for GCA, which can involve the larger thoracic, abdominal, and peripheral arteries. Polymyalgia rheumatica symptoms in patients with GCA correlate with (peri)synovitis of the shoulders. Relapses of GCA cannot be predicted by results of former PET scintigraphies.

     

风湿性多肌痛86例回顾性临床分析

目的探讨风湿性多肌痛的临床特点及男女患者之间的差别。方法回顾性分析86例风湿性多肌痛患者的临床表现和治疗反应。结果女性患者表现为颈后肌和肩胛带肌痛的比例高于男性(P<0.05)。而男性患者出现高热、关节痛、体质量下降、血清C反应蛋白水平、白细胞计数升高以及免疫球蛋白升高的比例均高于女性患者(P<0.05)。然而,女性患者的复发率高于男性(P<0.05)。结论男性患者部分全身症状和炎性反应重于女性,女性患者较男性更易复发。