Cytokines in giant-cell arteritis

Cytokines are small proteins that serve as chemical messengers between cells, regulating cell growth and differentiation, tissue repair and remodeling, and many aspects of the immune response. Cytokines are instrumental in determining the nature, magnitude, and duration of inflammatory reactions and, as such, represent ideal targets for interfering with pathogenic processes. In OCA and PMR, cytokines are encountered in two locations, the inflammatory infiltrates accumulating in the arterial wall and in the circulation. IL-6, a cytokine involved in stimulating acute-phase responses, is located upstream of many of the laboratory abnormalities considered helpful in diagnosing and managing GCA/PMR, including elevated ESR and CRP. IL-6 has the potential to be helpful in predicting disease severity and may allow for a tailoring of immunosuppressive therapy. There is evidence suggesting that IL-6 outperforms other chemical markers in detecting disease activity and could, therefore, have a role in monitoring treatment. Interesting pathogenic clues have been derived from studies of cytokines produced in the vascular lesions. IFN-gamma has emerged as a key regulator in determining the nature and direction of the inflammatory response. IFN-gamma appears to be critically involved in modulating the process of intimal hyperplasia, the most destructive consequence of vasculitis, and, as such, emerges as a prime target for novel therapeutic approaches.     

Polymyalgia rheumatica and giant-cell arteritis

The subjects of the study were 112 patients with rheumatic polymyalgia (RPM) and giant-cell arteritis (GSA). The study shows that RPM and GSA are complicated by cerebral flow disturbances and myocardial infarction. Therapy with prednisolone and non-steroid antiinflammatory drugs reduces the risk of these complications and lethal outcome in patients with RPM and GSA.     

Polymyalgia rheumatica and giant-cell temporal arteritis

The article is dedicated to the contemporary concept of the prevalence, etiology, and pathogenesis of polymyalgia rheumatica and Horton's disease. The author analyzes the features of the pain syndrome, arthritis, fever, and other manifestations of the disease. Taking into consideration the fact that polymyalgia rheumatica and Horton's disease are usually revealed too late, the author pays special attention to their diagnostics and differential diagnostics. An increased ESR is a feature of this disease. Polymyalgia rheumatica is treated with prednisolone. The stages of glucocorticoid therapy, the rules of adjustment and lowering prednisolone doses, combined therapy with glucocorticoids and cytostatics, the effectiveness of therapy, and the prognoses are described in detail.     

The numb chin syndrome as an early manifestation of giant-cell (temporal) arteritis: a case report

We describe a 70-year-old woman with a 2-month history of a numb chin and gradually increasing bilateral headache and malaise. Neurological examination disclosed chin hypoesthesia while investigations showed a normocytic anemia, ESR of 100, and CRP of 72. A CT brain scan, chest X-ray, and bone scan showed no evidence of malignancy. Temporal arteritis was suspected and prednisolone started with prompt resolution of the headache, chin hypoesthesia, ESR, and CRP. This case illustrates an unusual etiology of the numb chin syndrome, which in most occasions is associated with malignancy. Temporal arteritis should be borne in mind as a possible explanation for this as it is a treatable condition with potentially serious, life-threatening complications.     

Microheterogeneity of alpha 1-antichymotrypsin in the management of giant-cell arteritis and polymyalgia rheumatica

1. Using crossed immunoaffinity electrophoresis with free concanavalin A in the first dimension, we studied the glycan microheterogeneity of alpha 1-antichymotrypsin in sera from patients with giant-cell arteritis and/or polymyalgia rheumatica, and its variation in the serum of several of these patients during induction of disease remission by prednisone therapy and possible further recurrence of giant-cell arteritis and/or polymyalgia rheumatica. 2. In the serum of patients with active disease we observed increased proportions of concanavalin A nonreactive and concanavalin A weakly reactive fractions. The results were expressed as the ratio of concanavalin A non-reactive fraction plus concanavalin A weakly reactive fraction to concanavalin A reactive fraction, called R alpha 1-ACT. An R alpha 1-ACT higher than 1.8 (upper normal value) was found in 30/31 sera from patients with active disease (sensitivity 97%) and in 2/22 sera from patients with inactive disease (specificity 91%). 3. The erythrocyte sedimentation rate and the serum C-reactive protein level, common markers of biological inflammation, are usually elevated in active giant-cell arteritis and/or polymyalgia rheumatica. The two parameters are commonly used to guide the therapy during the course of the disease, but they have no specificity. An erythrocyte sedimentation rate of greater than 30 mm/h was found in 30/31 sera from patients with active disease (sensitivity 97%) and in 5/22 sera from patients with inactive disease (specificity 77%).(ABSTRACT TRUNCATED AT 250 WORDS)     

肝原发性破骨细胞样巨细胞瘤临床病理观察

目的探讨肝原发性破骨细胞样巨细胞瘤(OGCT)的组织学来源、临床病理特征及生物学行为。方法对1例肝原发性OGCT的大体、组织学特征、免疫组化进行观察并复习相关文献。结果患者女性,41岁。右上腹痛1周。巨检:肿物大小8 cm×4.5 cm×3 cm,部分突出肝被膜;切面灰红色、质韧,部分出血、坏死及囊性变。镜检:肿物由大量的单核间质细胞及破骨细胞样巨细胞组成,出血、坏死较多。免疫组化:单核间质细胞及破骨细胞样巨细胞CD68和vimentin(+),而CK7、SMA、CK19、CK18、EMA、溶菌酶、Hep Par-1、AFP、CK20和CD34均(-),Ki-67增殖指数15%。结论肝原发性OGCT是一种非常罕见的肿瘤,其诊断和生物学行为应该结合临床病理特征、免疫组化及是否有转移等综合因素,首先要排除转移性肿瘤。     

以发热为首要症状的巨细胞动脉炎一例报道

<正>患者,女性,78岁,汉族,因"发热1个月余"于2018年8月14日入四川大学华西医院治疗。入院前1个多月,患者出现无明显诱因发热,每日下午体温升高,服用解热镇痛药后可缓解。自诉体温大多波动在37～38℃之间,最高体温38.1℃,伴有轻度咳嗽,咳少量白色黏痰,有夜间出汗、心慌等不适症状。无胸闷、胸痛,无腹痛、腹泻,无尿频、尿急、尿痛,无全身关节疼痛等症状。半个月前于外院住院,多次查血常规均提示血象正常,但红细胞及血红蛋白进行性下降,红细胞沉降率(erythrocyte sedimentation rate,ESR)明显高于正常,给予抗感染、对症支持等治疗后患者病情无好转,进一步行胸部CT、骨髓     

The application of bone cement to fill the surgical defect after resection of a giant-cell tumor: A case report

Background. A case of a large bone defect treated with bone cement filler is discussed, and the usefulness and advantages of this treatment method are assessed. Case report. A 25-year-old male was admitted to our hospital with an intra-articular pathological fracture of the lateral femoral condyle due to an underlying giant cell tumor (stage I according to the Enneking classification). The tumor was surgically resected, and the walls of the post-operative bone defect were treated with a burr. The defect was then filled with bone cement (PMMA). The tumor was found to be histologically benign. Post-surgical treatment included immobilization in a cylinder cast and no weight bearing for a period of 8 weeks. The follow-up included frequent check-ups and repeated radiographs and CT scans. The follow-up period was 7 years, during which time no clinical or radiological signs of tumor recurrence were found. There was visible osteosclerosis surrounding the bone cement filler, which is believed to be a prognostically favorable finding, associated with a low risk of recurrence. The functional outcome of treatment was assessed with the Knee Rating Score. The patient gained a knee score of 100 points and a functional score of 100 points. Conclusions. The use of bone cement in order to fill a bone defect after tumor excision proved to be an effective and safe method to manage a giant-cell tumor of the bone. This method provided good biomechanical circumstances and allowed for good follow-up, as it enabled easy detection of possible tumor recurrence.     

Polymyalgia rheumatica and temporal arteritis: a case presentation

Polymyalgia rheumatica (PMA) and temporal arteritis (TA) are common clinical syndromes that affect the elderly population. Both syndromes may include similar constitutional complaints, an increase in acute phase reactants, a rapid response to corticosteroids, and the presence of anteritis giant cells upon temporal artery biopsy. Differential diagnosis is one of exclusion. A case presentation is included to assist practitioners in recognizing presenting symptoms, identifying diagnostic testing, and proceeding with appropriate therapy and follow-up.     

类似主动脉缩窄的大动脉炎1例

病例女,18岁.8天前因焦虑出现持续性头部闷胀,伴胸闷、恶心呕吐胃内容物两次,就诊于当地卫生院,诊断为＂高血压病＂,后血压进行性升高,最高达180/70mmHg,转诊于当地市医院,经治疗血压控制在150/70mmHg.为明确高血压病因转入我院.     

Gene expression in giant-cell tumors

Malignant transformation is thought to be associated with changes in the expression of a number of genes, and this alteration in gene expression is considered critical to the development of the malignant phenotype. In this study, gene expression in 8 samples of giant-cell tumor (GCT) of bone, as well as in bone at the site of osteoarthritis and in a variety of normal tissues, was determined at Gene Logic Inc (Gaithersburg, Md) with the use of Affymetrix GeneChip U_133 arrays containing approximately 40,000 genes/expressed sequence tags (ESTs). Gene-expression analysis was performed with the use of the Gene Logic GeneExpress Software System. Differences in gene expression between GCTs and bone were observed. In addition, genes expressed uniquely in GCTs among these and 519 samples from 20 other tissue types were identified. Some of the genes that were found to be overexpressed in GCTs, such as tartrate-resistant acid phosphatase and the lysosomal H + -transporting ATPase, are also expressed by osteoclasts. Osteoprotegrin ligand (OPGL) was also selectively overexpressed in GCTs. The genes found to be overexpressed in GCTs appear to reflect the genetic profile of osteoclast-lineage cells and also the genetic profile of an osteoclastogenic environment. The genes identified in this study may play a role in the pathogenesis of GCTs, confirm the likely importance of OPGL in GCT pathogenesis, and may indicate other possible targets to which antitumor therapy could be directed.