Disease pattern in cranial and large-vessel giant cell arteritis.
OBJECTIVE: To identify variables that distinguish large-vessel giant cell arteritis (GCA) with subclavian/axillary/brachial artery involvement from cranial GCA. METHODS: Seventy-four case patients with subclavian/axillary GCA diagnosed by angiography and 74 control patients with temporal artery biopsy-proven GCA without large vessel involvement matched for the date of first diagnosis were identified. Pertinent initial symptoms, time delay until diagnosis, and clinical symptoms, as well as clinical and laboratory findings at the time of diagnosis, were recorded by retrospective chart review. Expression of cytokine messenger RNA in temporal artery tissue from patients with large-vessel and cranial GCA was determined by semiquantitative polymerase chain reaction analysis. Distribution of disease-associated HLA-DRB1 alleles in patients with aortic arch syndrome and cranial GCA was assessed. RESULTS: The clinical presentation distinguished patients with large-vessel GCA from those with classic cranial GCA. Upper extremity vascular insufficiency dominated the clinical presentation of patients with large-vessel GCA, whereas symptoms related to impaired cranial blood flow were infrequent. Temporal artery biopsy findings were negative in 42% of patients with large-vessel GCA. Polymyalgia rheumatica occurred with similar frequency in both patient groups. Large-vessel GCA was associated with higher concentrations of interleukin-2 gene transcripts in arterial tissue and overrepresentation of the HLA-DRB1*0404 allele, indicating differences in pathogenetic mechanisms. CONCLUSION: GCA is not a single entity but includes several variants of disease. Large-vessel GCA produces a distinct spectrum of clinical manifestations and often occurs without involvement of the cranial arteries. Large-vessel GCA requires a different approach to the diagnosis and probably also to treatment. (+info)
Cyclical etidronate increases bone density in the spine and hip of postmenopausal women receiving long term corticosteroid treatment. A double blind, randomised placebo controlled study.
OBJECTIVE: To study the effect of cyclic etidronate in secondary prevention of corticosteroid induced osteoporosis. METHODS: A double blind, randomised placebo controlled study comparing cyclic etidronate and placebo during two years in 37 postmenopausal women receiving long term corticosteroid treatment, mainly for polymyalgia rheumatica (40% of the patients) and rheumatoid arthritis (30%). Bone density was measured in the lumbar spine, femoral neck, and femoral trochanter. RESULTS: After two years of treatment there was a significant difference between the groups in mean per cent change from baseline in bone density in the spine in favour of etidronate (p = 0.003). The estimated treatment difference (mean (SD)) was 9.3 (2.1)%. Etidronate increased bone density in the spine (4.9 (2.1)%, p < 0.05) whereas the placebo group lost bone (-2.4 (1.6)%). At the femoral neck there was an estimated difference of 5.3 (2.6)% between the groups (etidronate: 3.6% (1.4)%, p < 0.05, placebo: -2.4 (2.1)%). The estimated difference at the trochanter was 8.2 (3.0) (etidronate: 9.0 (1.5)%, p < 0.0001, placebo: 0.5 (2.3)%). No significant bone loss occurred in the hip in placebo treated patients. CONCLUSIONS: Cyclic etidronate is an effective treatment for postmenopausal women receiving corticosteroid treatment and is well tolerated. (+info)
HLA-DRB1 alleles associated with polymyalgia rheumatica in northern Italy: correlation with disease severity.
OBJECTIVE: To examine the association of HLA-DRB1 alleles with polymyalgia rheumatica (PMR) in a Mediterranean country and to explore the role of HLA-DRB1 genes in determining disease severity. METHODS: A five year prospective follow up study of 92 consecutive PMR patients diagnosed by the secondary referral centre of rheumatology of Reggio Emilia, Italy was conducted. HLA-DRB1 alleles were determined in the 92 patients, in 29 DR4 positive rheumatoid arthritis (RA) patients, and in 148 controls from the same geographical area by polymerase chain reaction amplification and oligonucleotide hybridisation. RESULTS: No significant differences were observed in the frequencies of HLA-DRB1 types and in the expression of HLA-DRB 70-74 shared motif between PMR and controls. The frequency of the patients with double dose of epitope was low and not significantly different in PMR and in controls. No significant differences in the distribution of HLA-DR4 subtypes were observed between DR4+ PMR, DR+ RA, and DR4+ controls. Results of the univariate analysis indicated that an erythrocyte sedimentation rate (ESR) at diagnosis > 72 mm 1st h, the presence of HLA-DR1, DR10, rheumatoid epitope, and the type of rheumatoid epitope were significant risk factors associated with relapse/recurrence. Cox proportional hazards modelling identified two variables that independently increased the risk of relapse/recurrence: ESR at diagnosis > 72 mm 1st h (RR=1.5) and type 2 (encoded by a non-DR4 allele) rheumatoid epitope (RR=2.7). CONCLUSION: These data from a Mediterranean country showed no association of rheumatoid epitope with PMR in northern Italian patients. A high ESR at diagnosis and the presence of rheumatoid epitope encoded by a non-DR4 allele are independent valuable markers of disease severity. (+info)
Biopsy proven and biopsy negative temporal arteritis: differences in clinical spectrum at the onset of the disease. Groupe de Recherche sur l'Arterite a Cellules Geantes.
OBJECTIVES: To assess the clinical features of biopsy proven and negative biopsy temporal arteritis at the time of diagnosis and during a three year follow up. METHODS: Newly diagnosed cases of giant cell arteritis were included in a prospective, multicentre study. Initial clinical and biological features, season of diagnosis, and cardiovascular events occurring during the follow up were recorded. Biopsy proven and negative biopsy cases were compared. RESULTS: Two hundred and seven biopsy proven, and 85 negative biopsy cases were included from 1991 to 1997. Fifty eight per cent of the biopsy proven cases, compared with 39.29% of the negative biopsy cases, were diagnosed during the autumn or winter (p = 0.003). Visual problems (31.5%, v 19.1%, p = 0.031), blindness (9.7% v 2.38%, p = 0.033), jaw claudication (40.8%, v 28.243%, p = 0.044), and temporal artery palpation abnormalities (61.3% v 29.5%, p = 7.10(-7)) were more frequent in the biopsy proven than in the negative biopsy group. Less specific symptoms, such as headache (82.5% v 92. 9%, p = 0.021), or associated polymyalgia rheumatica (40.1% v 65.9%, p = 9 x 10(-5)) were more prevalent in the negative biopsy cases. Biological markers of inflammation were significantly more increased in the biopsy proven group. All cases of blindness occurring after treatment belonged to the biopsy proven group. CONCLUSION: Biopsy proven cases seem to be more severe than biopsy negative cases at the time of diagnosis and during follow up. Seasonal difference at diagnosis may suggest a different aetiological pattern. (+info)
Giant cell arteritis and polymyalgia rheumatica: are pregnancies a protective factor? A prospective, multicentre case-control study. GRACG (Groupe de Recherche sur l'Arterite a Cellules Geantes).
OBJECTIVE: To assess the potential role of allo-immunization, either by former pregnancies, or by a history of blood transfusion, in the pathogenesis of giant cell arteritis and polymyalgia rheumatica. METHODS: Two hundred and eighty-five incident female cases and 186 age-matched, population-based female controls were prospectively included in a multicentre case-control study. RESULTS: The number of pregnancies was significantly lower in cases than in controls (nulliparous: 21.55% vs 12.90%; > or =4 pregnancies: 16.25% vs 27.42%; Wilcoxon rank sum test: P = 0.0019) in biopsy-proven or negative temporal arteritis and, to a lesser extent, in polymyalgia rheumatica. No difference was found for history of blood transfusion. Pregnancies remained negatively associated with the disease in a multivariate analysis including cardiovascular risk factors such as smoking or a pre-existing peripheral vascular disease. CONCLUSION: Former pregnancies are not a risk factor for giant cell arteritis. Pregnancies may be protective thanks to an effect of the associated hyperoestrogenic state against alterations of the artery wall, as suggested in animal models. (+info)
Bronchiolitis obliterans organizing pneumonia associated with polymyalgia rheumatica.
The association of bronchiolitis obliterans organizing pneumonia (BOOP) with polymyalgia rheumatica is rare, and only one case has previously been described. This study reports on the case of an 80 yr-old male who presented with malaise, nonproductive cough and exertional dyspnoea for several weeks, along with a history of bilateral shoulder and pelvic girdle pain of several months' duration. The chest radiograph revealed a pneumonic infiltrate in the right lower lobe, which was unresponsive to antibiotics. Bronchoscopy, bronchoalveolar lavage and a transbronchial lung biopsy established the diagnosis of BOOP. The patient improved consistently on steroids. As in other connective diseases, organizing pneumonia may be one of the early manifestations of polymyalgia rheumatica. (+info)
Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) syndrome: a prospective follow up and magnetic resonance imaging study.
OBJECTIVE: To determine the clinical characteristics of patients with "pure" remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) syndrome, and to investigate its relation with polymyalgia rheumatica (PMR). Magnetic resonance imaging (MRI) was used to describe the anatomical structures affected by inflammation in pure RS3PE syndrome. METHODS: A prospective follow up study of 23 consecutive patients with pure RS3PE syndrome and 177 consecutive patients with PMR diagnosed over a five year period in two Italian secondary referral centres of rheumatology. Hands or feet MRI, or both, was performed at diagnosis in 7 of 23 patients. RESULTS: At inspection evidence of hand and/or foot tenosynovitis was present in all the 23 patients with pure RS3PE syndrome. Twenty one (12%) patients with PMR associated distal extremity swelling with pitting oedema. No significant differences in the sex, age at onset of disease, acute phase reactant values at diagnosis, frequency of peripheral synovitis and carpal tunnel syndrome and frequency of HLA-B7 antigen were present between patients with pure RS3PE and PMR. In both conditions no patient under 50 was observed, the disease frequency increased significantly with age and the highest frequency was present in the age group 70-79 years. Clinical symptoms for both conditions responded promptly to corticosteroids and no patient developed rheumatoid arthritis during the follow up. However, the patients with pure RS3PE syndrome were characterised by shorter duration of treatment, lower cumulative corticosteroid dose and lower frequency of systemic signs/symptoms and relapse/recurrence. Hands and feet MRI showed evidence of tenosynovitis in five patients and joint synovitis in three patients. CONCLUSION: The similarities of demographic, clinical, and MRI findings between RS3PE syndrome and PMR and the concurrence of the two syndromes suggest that these conditions may be part of the same disease and that the diagnostic labels of PMR and RS3PE syndrome may not indicate a real difference. The presence of distal oedema seems to indicate a better prognosis. (+info)
New arguments for a vasculitic nature of polymyalgia rheumatica using positron emission tomography.
OBJECTIVE: To study the possible contribution of fluorodeoxyglucose (FDG)-positron emission tomography (PET) in the diagnosis of giant cell arteritis and polymyalgia rheumatica. METHODS: A consecutive case series consisting of five patients with polymyalgia rheumatica, six patients with temporal arteritis and 23 age-matched patients with other inflammatory conditions were evaluated with FDG-PET. Studies were performed before therapy with steroids was started. RESULTS: A total of 4/6 patients with giant cell arteritis and 4/5 patients with polymyalgia had increased FDG uptake in their thoracic vessels, compared to 1/23 controls (P < 0.001). Increased vascular FDG uptake in the upper legs was seen in 8/11 patients with giant cell arteritis or polymyalgia compared to 8/23 control patients (P < 0.05), and in the lower legs in 6/11 patients compared to 6/23 controls (P = not significant). CONCLUSIONS: FDG-PET scan is the first non-invasive technique which may indicate large-vessel vasculitis and which can show its extension throughout the body. It strongly suggests that polymyalgia rheumatica is a form of vasculitis. (+info)