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C Garcia-Porrua

Publications and source records attributed to C Garcia-Porrua.

At least 19 recordsLinked to original sources

Biopsy-negative giant cell arteritis: clinical spectrum and predictive factors for positive temporal artery biopsy.

OBJECTIVES: To examine the frequency and features of patients with biopsy-negative giant cell arteritis (GCA), establish differences with biopsy-proven GCA, and identify the optimal set of predictors for a positive temporal artery biopsy (TAB) in patients with GCA. METHODS: Retrospective study of an unselected population of patients with GCA diagnosed at the reference hospital for a defined population between 1981 and 1998. Patients were classified into biopsy-proven GCA if a TAB was positive for GCA, or biopsy-negative GCA if they fulfilled the American College of Rheumatology 1990 criteria for the classification of GCA (Hunder GG, et al Arthritis Rheum 1990; 33:1122-8) despite having a negative TAB. RESULTS: One hundred ninety Caucasian patients were diagnosed with GCA. Twenty-nine of them (15.3%) had a negative TAB. In these biopsy-negative patients, headache and polymyalgia rheumatica were frequent presenting symptoms. In contrast, jaw claudication, abnormal temporal artery on physical examination, and constitutional syndrome (asthenia, anorexia, and weight loss of 4 kg or more) were less common. They also had lower biologic markers of inflammation. The best predictive model of biopsy-proven GCA included a history of constitutional syndrome (OR = 6.1), an abnormal temporal artery on physical examination (OR = 3.2), and the presence of visual complications (OR = 4.9). CONCLUSIONS: In GCA, a subset of patients have a high likelihood of having a negative TAB. This subset seems to have less severe ischemic complications than that of biopsy-proven GCA. In patients without visual manifestations, abnormal temporal artery on examination or constitutional syndrome the risk of having an abnormal TAB is low.

Aged↗

Geoepidemiology of systemic vasculitis: comparison of the incidence in two regions of Europe.

OBJECTIVE: The aetiopathogenesis of the primary systemic vasculitides (PSV) is unknown but includes both environmental and genetic factors. The development of classification criteria/definitions for PSV allows comparison of the epidemiology between different regions. METHODS: The same methods and the American College of Rheumatology (1990) criteria or Chapel Hill definitions were used to compare the epidemiology of Wegener's granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, and polyarteritis nodosa in Norwich (east England population 413 500) and Lugo (northwest Spain population 204 100). Patients with PSV were identified between 1 January 1988 and 31 December 1998. RESULTS: Overall, the incidence of PSV in adults was almost equal in Norwich (18.9/million) and Spain (18.3/million). The incidence of Wegener's granulomatosis in Norwich (10.6/million) was greater than in Spain (4.9/million). There was a marked age-specific increase in incidence in Norwich with a peak age 65-74 years (52.9/million), but a virtually equal age distribution between ages 45 and 74 in Lugo (34.1/million). There was no significant increase with time in either population, or evidence of cyclical changes in incidence. CONCLUSION: These data support the suggestion that environmental factors may be important in the pathogenesis of PSV.

Adolescent↗

Epidemiology of biopsy proven giant cell arteritis in northwestern Spain: trend over an 18 year period.

OBJECTIVE: In Europe giant cell arteritis (GCA) is more common in Scandinavian countries than in southern regions. Epidemiological studies on GCA in other more distant countries have indicated a progressive increase in incidence. A regular cyclical pattern in incidence of GCA over 20 years has been reported in Olmsted County (Minnesota, USA). In contrast, no cyclical fluctuation has been recently reported in Sweden. To investigate further the epidemiology of GCA in southern Europe the trend in incidence and fluctuations of this vasculitis over 18 years in the Lugo region of northwestern Spain were examined. METHODS: A retrospective study of biopsy proven GCA diagnosed between 1 January 1981 and 31 December 1998 at a single hospital for a well defined population of almost 250 000 people. Annual incidence was calculated for the whole group of patients and for men and women separately. Monthly variations, annual peaks of incidence, and trend in the incidence of biopsy proven GCA with and without polymyalgia rheumatica (PMR) were also examined. RESULTS: One hundred and sixty one Lugo residents were diagnosed with biopsy proven GCA between 1981 and 1998. The average annual incidence for the population aged 50 and older was 10.24/100 000 (men 11.00/100 000, women 9.57/100 000). A progressive increase in the incidence in both men and women was seen. In men there was an annual increase of 8% (95% CI 4% to 13%; p<0.0001). In women the annual increase was 11% (95% CI 5% to 17%; p<0.0001). The overall annual increase for men and women was 10% (95% CI 6% to 14%; p<0.0001). No seasonal pattern or peaks in the incidence were seen. During the period 1981-94 GCA was more common in men than in women. In contrast, during the last years of study the increase in incidence was higher in women. In women the annual ratio of incidence of GCA with PMR/incidence of GCA without PMR was generally higher than 1. However, in men the annual ratio was initially 1 but decreased gradually, indicating a progressive decrease in the proportion of men with biopsy proven GCA associated with PMR. CONCLUSION: In northwestern Spain there has been a progressive increase in GCA incidence. As seen in other countries where GCA is more common, during the past few years the increase in incidence has been mainly due to a higher number of new cases in women.

Age Factors↗

Seronegative rheumatoid arthritis in elderly and polymyalgia rheumatica have similar patterns of HLA association.

OBJECTIVE: To examine whether polymyalgia rheumatica (PMR) and late onset rheumatoid arthritis have identical or similar HLA-DRB1 genetic associations. METHODS: Seventy five PMR cases without evidence of giant cell arteritis were available for study. One hundred seven RA cases were investigated, of which 62 had disease onset after age 60 years. All cases were compared with 145 ethnically matched controls. All cases and controls originated from Lugo, NW Spain. HLA-DRB1 typing was performed on DNA samples using PCR based molecular methods. RESULTS: Early onset RA (< or =40 yrs) was strongly associated with DRB1*04 (DRB1*0401 and *0404). In contrast late onset RA (> or = 60 yrs) was not associated with DRB1*04 but was associated with DRB1*01. Stratification of late onset RA cases by rheumatoid factor status revealed that DRB1*01 was only increased in seropositive RA cases. Late onset seronegative RA cases exhibited increased frequency of DRB1*13/*14; this was also observed in PMR cases where coexistence of GCA had been excluded. CONCLUSION: These data indicate that (1) HLA associations with RA differ with respect to age at disease onset; and (2) seronegative late onset RA and "isolated" PMR have a similar HLA-DRB1 association and may have an identical etiological basis.

Adolescent↗

HLA-DRB1 alleles encoding an aspartic acid at position 70 protect against development of rheumatoid arthritis.

OBJECTIVE: To determine whether the association between rheumatoid arthritis (RA) and HLA-DRB1 is influenced by the amino acid residue encoded at position 70 (beta70) of the third hypervariable region (HVR3) of the HLA-DRbeta chain. METHODS: The frequencies of HLA-DRB1 alleles encoding different amino acid residues at beta70 were compared between patients with RA and controls in a population from the UK and in a confirmatory population from northwestern Spain. HLA-DRB1 typing was done by polymerase chain reaction methods on 476 clinic based patients with RA and 180 healthy controls from Staffordshire and Cheshire in the UK, and on 179 clinic patients and 145 controls from Lugo, Spain. Associations were investigated using chi-square analyses and regression analyses. The extended Mantel-Haenszel procedure was used for trend analysis. RESULTS: Carriage of 2 shared epitope (SE)+ alleles encoding a glutamine at beta70 (Q70SE+/Q70SE+) was associated with the greatest risk of RA in the UK and Spanish population (odds ratios 7.93 and 4.66, respectively), while possession of 2 SE- alleles encoding an aspartic acid at beta70 (D70SE-D70SE-) was associated with the lowest risk (OR 0.23 and 0.34, respectively). In individuals carrying one SE+ allele and an accompanying D70SE- allele there was no increased risk of developing RA [OR 0.93 (UK) and 1.30 (Spain)]. Possession of D70SE- was more strongly protective than possession of Q70SE. Analysis of trend indicated that the strength of association of different DRB1 genotypes with RA could be ranked in order (from Q70SE+/Q70SE+ to D70SE-/D70SE-) according to which amino acid residues were encoded at beta70, and whether or not they formed part of a SE sequence. The severity of radiographic damage could not be ranked in the same fashion. CONCLUSION: The amino acid residue at position 70 of the HVR3 in HLA-DRbeta molecules influences susceptibility to RA. The strength of the association of DRB1 genotypes with RA is dependent not only on SE status, but also on which amino acid residues are encoded at beta70 of the DRB1 alleles. Presence of an aspartic acid residue at beta70 protects against development of RA. However, the severity of erosive damage does not appear to be associated with the amino acid substitution at 1370.

Alleles↗

Polymorphism at codon 469 of the intercellular adhesion molecule-1 locus is associated with protection against severe gastrointestinal complications in Henoch-Schönlein purpura.

OBJECTIVE: Henoch-Schonlein purpura (HSP) is a small sized vasculitis affecting mainly children. Intercellular adhesion molecule-1 (ICAM-1) gene polymorphisms have recently been implicated in the susceptibility to some vasculitides. To further investigate the clinical implication of ICAM-1 polymorphisms in HSP, we examined their potential association and influence in the development of severe complications in an unselected series of patients with HSP. METHODS: Fifty-two patients, of which 41 were children, were diagnosed with HSP using classification criteria of Michel, et al at the Hospital Xeral-Calde (Lugo, Spain); 129 ethnically matched controls were included. Patients had at least one year of followup. Patients and controls were genotyped by allelic oligonucleotide techniques for ICAM-I polymorphism at codon 241 and 469. RESULTS: The frequency distribution of the alleles and genotypes for each ICAM-1 polymorphism did not show significant differences between HSP patients and controls. Also, no differences between patients with or without renal manifestations were found. However, the frequency of the codon 469 K/E genotype was significantly decreased in patients without severe gastrointestinal manifestations compared to those with them (22.29 vs 65%, OR 0.1, p = 0.02, after correction for age, sex, and disease duration). None of the 11 adults exhibited the R/G genotype at codon 241 compared with 7 of 41 children (OR 0.0, 95% CI 0.0-2.9, p = 0.14). Patients with the R/G genotype were associated with low incidence of renal manifestations and none developed permanent renal involvement (renal sequelae); however, this finding did not achieve statistical significance. CONCLUSION: ICAM-1 polymorphisms alone are not associated with development of HSP, but patients not carrying the codon 469 K/E genotype are at decreased risk of developing severe gastrointestinal complications. The R/G polymorphism at codon 241 may reduce the risk of renal sequelae in the development of HSP in adulthood.

Adolescent↗

HLA-DRB1*01 association with Henoch-Schönlein purpura in patients from northwest Spain.

OBJECTIVE: To examine the HLA-DRB1 phenotypes of patients with Henoch-Schönlein purpura (HSP) and determine if associations exist with disease susceptibility, clinical heterogeneity, or severe systemic complications. METHODS: A retrospective study was performed on an unselected population of patients from Northwest Spain with HSP classified according to proposed criteria. Patients were included in this study if they had at least one year of followup. Fifty Caucasian patients (25 women), 11 of them older than 20 years, were studied. Patients and ethnically matched controls were HLA-DRB1 genotyped from DNA using molecular based methods. RESULTS: During the course of the disease, renal manifestations, especially hematuria, and severe gastrointestinal (GI) manifestations (bowel angina or GI bleeding) were observed in more than 60% of the patients. Twenty percent of patients had persistent renal involvement (renal sequelae). Patients with HSP had a significantly higher frequency of the HLA-DRB1*01 phenotype compared to matched controls. The HLA-DRB1*07 phenotype was also significantly reduced compared with controls. Patients with severe GI manifestations or with persistent renal involvement did not exhibit any specific HLA-DRB1 association other than the underlying association with HLA-DRB1*01. CONCLUSION: HSP in a population from Northwest Spain is significantly associated with HLA-DRB1*01.

Adolescent↗

Lack of association between intercellular adhesion molecule-1 gene polymorphisms and giant cell arteritis.

OBJECTIVE: Studies have shown an association between HLA-DRB1*04 and giant cell arteritis (GCA). Intercellular adhesion molecule-1 (ICAM-1) gene polymorphisms were reported to contribute susceptibility to GCA in Italian patients where susceptibility to GCA is not associated with HLA-DRB1*04 alleles. ICAM-1 is also highly expressed within inflammatory infiltrates of the blood vessels of GCA patients. To investigate the clinical implications of ICAM-1 polymorphisms in GCA, we examined their potential association and influence in the development of visual ischemic complications in a series of patients with GCA from Northwest Spain where GCA susceptibility is associated with HLA-DRB1*04. METHODS: Fifty-eight biopsy proven GCA and 129 ethnically matched controls were studied. Patients and controls were genotyped for ICAM-1 polymorphism at codons 241 and 469 by PCR-RFLP. RESULTS: The distribution of the alleles and genotypes for each ICAM- polymorphism did not show significant differences between GCA patients and controls. Although visual manifestations were significantly more likely to occur in men than women (OR 5.2, p = 0.018), allele and genotype frequencies of ICAM-1 polymorphisms in patients with GCA were not associated with development of visual complications or anemia. Visual complications in GCA were primarily associated with carriage of an HLA-DRB1*04 allele. No evidence was found for interaction between HLA-DRB1*04 and ICAM-1 polymorphism. CONCLUSION: ICAM-1 polymorphisms are not genetic risk factors for the susceptibility and severity of GCA in Northwest Spain.

Aged↗

HLA-DRB1 associations in biopsy proven erythema nodosum.

OBJECTIVE: To examine the HLA-DRB1 associations of patients with erythema nodosum (EN), establish HLA-DRB1 differences among patients with idiopathic and secondary EN, and identify the HLA-DRB1 associations with specific conditions presenting with EN. METHODS: We conducted a retrospective study of 100 patients (83 women) with biopsy proven EN diagnosed for a defined population in Northwest Spain. Patients were classified into idiopathic or secondary if skin nodules occurred in the context of a well defined disease, or there was a precipitating event in close temporal relationship with the onset of EN. Patients and controls were HLA-DRB1 genotyped from DNA using molecular based methods. RESULTS: At the time of diagnosis no precipitating events or underlying diseases were identified in 35 cases and, due to this, they were considered to be idiopathic. Although strong associations do not appear to exist between HLA-DRB1 and unstratified EN, the idiopathic group showed a significantly lower frequency of HLA-DRB1*04 phenotype compared with controls. Among the patients with secondary EN, the patient subgroup with sarcoidosis exhibited a significantly increased frequency of HLA-DRB1* 13 compared with healthy controls. CONCLUSION: Idiopathic and some groups of secondary EN seem to have different HLA-DRB1 associations. These differences may have prognostic value in identifying patients with specific conditions associated with this syndrome.

Adolescent↗

The spectrum of conditions mimicking polymyalgia rheumatica in Northwestern Spain.

OBJECTIVE: To examine the spectrum and the main clinical data of patients presenting with polymyalgia symptoms who have conditions other than polymyalgia rheumatica (PMR) or PMR associated with giant cell arteritis (GCA) during a 10 year period in Northwestern Spain. METHODS: Clinical records of patients presenting with polymyalgia symptoms diagnosed at the Hospital Xeral-Calde Lugo from 1987 to 1996 were reviewed by rheumatology staff members. Patients were considered as having a condition suggestive of PMR if they met the following criteria: (1) Age > or =50 years at the onset of symptoms; (2) severe bilateral pain associated with morning stiffness for > 1 mo in at least 2 of 3 areas: neck, shoulder, and/or pelvic girdle; (3) erythrocyte sedimentation rate at the time of diagnosis > or =40 mm/h. Patients with pure PMR or with PMR associated with GCA were excluded from study. RESULTS: Twenty-three of the 208 patients (age 67.8 +/- 9.0 yrs) presenting with PMR symptoms were finally diagnosed as having conditions different from PMR and GCA. Men outnumbered women (61%). Malignancies and rheumatic diseases, especially seronegative symmetrical polyarthritis (SSP), were the most common entities. Elderly patients with solid malignancies had a poor response to low doses of prednisone. In patients with hematologic malignancies atypical symptoms of PMR such as lack of accentuation of symptoms with movement and a more diffuse continuous aching were observed. During followup 5 patients developed episodes of SSP (median duration 13 months, range 5 to 24), particularly in both hands, satisfying the American College of Rheumatology 1987 criteria for rheumatoid arthritis. However, arthritis responded promptly to corticosteroids with no disease progression. No cortical erosions or new episodes of PMR were seen in these patients after a followup of 6.8 +/- 2.6 years. With the exception of these 5 patients, duration of polymyalgia symptoms was not longer than 3 months from the onset of polymyalgia symptoms until a specific diagnosis was made. CONCLUSION: Polymyalgia symptoms are not uncommon as presenting manifestations of a wide spectrum of conditions. Special concern about the presence of diseases different from PMR or GCA must be considered in patients presenting with atypical symptoms of PMR. Also, the possibility of developing a SSP has to be considered during the followup of these patients.

Diagnosis, Differential↗

Hip pain.

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Adult↗