HLA antigens in patients with giant cell arteritis and polymyalgia rheumatica

The frequency of 24 HLA antigens was determined in 43 patients with giant cell arteritis (25 of whom had polymyalgia rheumatica) and 12 others with polymyalgia rheumatica without evident arteritis. No haplotype was present in significantly increased frequency above controls in the total group nor when the patients were separated according to the presence or absence of polymyalgia rheumatica.     

Polymyalgia rheumatica and giant cell arteritis

Giant cell (temporal) arteritis and polymyalgia rheumatica are related conditions that primarily affect older persons. They are among the most common chronic inflammatory disorders in this age group. In recent years, several new insights have been gained about the clinical features of these illnesses as well as their underlying pathophysiology. In addition, a great deal of study is underway to find better ways of detecting, monitoring, and treating them. This article provides a general review of these conditions, including their diagnosis and treatment.     

Appearance of giant cell arteritis 6 years after the 1st manifestation of polymyalgia rheumatica

A case of polymyalgia rheumatica in a 70 year old woman is described. The patient was treated for 6 years with corticosteroids. Seven months after withdrawal of treatment, a severe relapse of polymyalgia rheumatica appeared which was connected with a histologically and immunohistochemically proved temporal arteritis. The problem of the duration of steroid treatment and the need for continued clinical as well as laboratory follow-up after withdrawal of therapy are discussed.     

Polymyalgia rheumatica in biopsy proven giant cell arteritis does not constitute a different subset but differs from isolated polymyalgia rheumatica

OBJECTIVE: To assess clinical and laboratory features that may be useful in differentiating isolated polymyalgia rheumatica (PMR) from PMR associated with biopsy proven giant cell arteritis (GCA); and in differentiating biopsy proven GCA associated with PMR from GCA without manifestations of PMR. METHODS: Clinical records of patients with PMR and biopsy proven GCA diagnosed at Hospital Xeral, Lugo, Spain from January 1987 through May 1997 were reviewed. Patients with a positive temporal artery biopsy were categorized into 2 different subgroups according to the presence or absence of associated PMR. The patients with biopsy proven GCA associated with PMR were compared with a group of patients with isolated PMR (not associated with GCA). RESULTS: From a total of 108 biopsy proven patients with GCA, 45 had associated PMR. Apart from a predominance of women and a longer delay to diagnosis, patients with PMR associated with GCA did not differ from the patients with GCA without PMR manifestations. In comparing patients with isolated PMR (n=117) with patients with PMR associated with GCA, we observed that PMR associated with GCA was a more severe disease, with significant abnormality in most laboratory variables, including constitutional syndrome, higher elevation of erythrocyte sedimentation rate and platelet counts, and lower values of hemoglobin. CONCLUSION: In both isolated PMR and PMR associated with GCA we observed a predominance of women. While there are no differences in the type of polymyalgia symptoms in patients with isolated PMR versus PMR associated with GCA, severe abnormalities associated with the inflammatory response in PMR may have prognostic value for more severe disease, which may be linked to the presence of GCA.     

Association of polymyalgia rheumatica and giant-cell arteritis with HLA-B8

Histocompatibility antigens were determined in 30 patients with temporal arteritis, 27 patients with polymyalgia rheumatica, and 216 normal blood donors. HLA-B8 was significantly more common in patients with polymyalgia rheumatica (59%) and temporal arteritis (50%) than in the controls (27%). The findings of HLA-A10 in 26% of the patients with polymyalgia rheumatica compared with only 10% of the controls may be associated with the suggested immunological pathogenesis of the condition.     

An atypical case of Horton's arteritis and associated polymyalgia rheumatica

The authors report a case of Horton's arteritis and associated rheumatic polymyalgia, which was complicated from a diagnostic point of view by the atypical clinical and biohumoral conditions. Both the subjective and objective symptoms were relatively unclear compared to laboratory and instrumental findings which pointed strongly to an autoimmune disorder. The coexistence of biohumoral data of a cholestatic hepatitic type, not common to other pathologies, although reported in the literature, led to further diagnostic difficulties. Diagnosis was finally confirmed by temporal artery biopsy.     

Giant cell arteritis and polymyalgia rheumatica can be differentiated by distinct patterns of HLA class II association

OBJECTIVE: To determine whether patients with polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) exhibit identical HLA class II associations. METHODS: A case-control association study was performed on a population sample from Lugo, in Northwestern Spain. DNA samples were available for 128 patients and 145 ethnically matched controls. Within the patient group 26 exhibited both PMR and GCA, 75 PMR alone, and 27 GCA alone. HLA-DRB1, DQA1, and DQB1 phenotypes were defined by molecular based techniques. RESULTS: HLA-DRB 1*0401 was associated with GCA regardless of PMR status, although this only reached statistical significance in the total GCA group. This was also seen for DRB 1*0101, *0102, although the association was less strong. Patients with PMR without GCA were not associated with DRB1*0401 or *0101, *0102, but exhibited a significant association with DRB1*13, *14. Nonsignificant increases in DQA1 and DQB1 phenotype frequencies appeared to reflect known patterns of linkage disequilibrium with the HLA-DRB1 alleles associated with GCA and PMR groups. An association was observed between the presence of the RA DRB1 shared epitope (SE) and GCA but not with PMR in the absence of GCA. This association was primarily accounted for by the presence of a single copy of the SE, and homozygosity for the SE did not confer additional risk. A high frequency of SE-bearing DRB1 alleles was observed in patients with GCA with jaw claudication or visual manifestations, although the sample size of these subgroups was small. CONCLUSION: PMR and GCA in a Northwestern Spanish population have distinct HLA class II associations. HLA is unlikely to account for the observed high level of overlap in these patients, and other etiological factors may be involved.     

Horton's giant cell arteritis

Giant cell arteritis (GCA) is a spontaneous vasculitic syndrome specifically involving the walls of medium and large arteries. While involvement of other arterial beds is occasionally identified, this syndrome is most frequently recognized when symptomatic involvement of the temporal arteries occurs. Vascular lesions are characterized by patchy granulomatous infiltrates composed of T cells, macrophages, histiocytes, and giant cells. A better prognosis depends on early recognition of the clinical symptoms and prompt treatment. Diagnosis was based on the 5 clinical criteria previously used by the American College of Rheumatology (1990): 1) age 50 years or older; 2) new localized headache; 3) temporal artery tenderness or decrease in temporal artery pulse; 4) erythrocyte sedimentation over 50 mm/ hour; 5) abnormal result on artery biopsy. Giant cell arteritis was considered a rare disease under age 50; however, it is now known to be an important and significant cause of morbidity and mortality in elderly people. Therefore early recognition and treatment with corticosteroid are very important. There is no general agreement concerning the initial dosage, 40-65 mg/day are commonly recommended. After a few months the majority of patients can be treated with a low maintenance dosage of prednisolone (5 to 7.5 mg/day). The mean duration of treatment is about 5 years. The literature is reviewed and the clinical implications of this disease are discussed.     

Choroidal nonperfusion in giant cell arteritis

A 68-year-old man had visual loss secondary to isolated choroidal nonperfusion as a clinical manifestation of giant cell arteritis. Ophthalmoscopy disclosed scattered yellow-white lesions at the level of the retinal pigment epithelium in the posterior pole of the right eye. Intravenous fluorescein angiography demonstrated marked delay in choroidal filling of the macula in the right eye. There was no ophthalmoscopic or angiographic evidence of anterior ischemic optic neuropathy or central retinal artery occlusion. After approximately 72 hours of intravenous corticosteroid therapy, the patient's visual acuity improved and repeat intravenous fluorescein angiography showed normal choroidal circulation. Isolated choroidal ischemia is a potential cause of reversible visual loss in patients with giant cell arteritis.     

Carotid occlusion mimicking giant cell arteritis

Hybrids of a ligand protein crosslinked to a DNA binding protein have been developed as gene delivery vehicles mediated by receptors. To identify the effect of the crosslinks between the ligand and DNA binding protein on gene expression caused by an internalized hybrid-DNA complex, we prepared two kinds of transferrin-poly-L-lysine (TF-PL) hybrids: one was crosslinked by probably cleavable disulfide bonds (TF-ss-PL) and the other was linked by a probably uncleavable Schiff's base (TF-Schiff-PL). The binding affinity of the hybrids to HeLa cells was not different. However, the expression of a reporter gene (for luciferase) bound to these hybrids in HeLa cells transfected with TF-Schiff-PL was greater than that of TF-ss-PL.     

Multiple deletions of the mitochondrial DNA in polymyalgia rheumatica

We analyzed the mitochondrial DNA of patients with polymyalgia rheumatica, a disease frequently associated with mitochondrial myopathy. In an attempt to study the deletions, we have developed a qualitative PCR method using a highly thermostable polymerase in order to amplify multiple mitochondrial DNA large fragments (up to 12 kb). PCR serves to observe both deleted and normal fractions of the mitochondrial DNA. We found multiple deletions of the mitochondrial DNA in all of the patient muscles. Although these muscles harbored many ragged red fibers, we found no point mutations of the tRNA(Leu)(UUR)) and the mutation at nucleotide position 8344 was not present.     

Large vessel vasculitis (giant cell arteritis, Takayasu arteritis)

Giant cell arteritis and Takayasu arteritis are separate but similar idiopathic diseases clinically characterized by constitutional symptoms, shared surrogate markers of systemic inflammation and indistinguishable granulomatous pan-arteritis of large vessels. This review emphasizes and analyses changing perceptions about the diseases. Recent series suggest that aortic involvement in giant cell arteritis may be more common than was previously appreciated. The case for and against inflammatory arthritis in giant cell arteritis is discussed. Ethnic new geographical variation in Takayasu arteritis-disease expression is reviewed. New philosophies of treatment are presented for both diseases. Prognosis in giant cell arteritis and its relationship to treatment is analysed. The utility of the laboratory for diagnosis and monitoring disease activity is appraised for each.