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Biomedical subjects

Carlos García-Porrúa

Publications and source records attributed to Carlos García-Porrúa.

3 recordsLinked to original sources

Systemic vasculitides.

The vasculitides are a heterogeneous group of diseases that are characterized by blood vessel inflammation and necrosis. They have a wide spectrum of manifestations due to the involvement of arteries and other vessels of various sizes and locations. Classification criteria are useful in improving our understanding of the epidemiology of these conditions but they are not diagnostic criteria. In recent years a progressive increase in incidence has been reported. Both giant cell arteritis and Wegener's granulomatosis are more common in the Northern hemisphere. Environmental factors have been implicated in the pathogenesis of these syndromes. Recent studies in patients with large and small-sized vasculitides support a genetic influence in disease susceptibility. They have confirmed the association of giant cell arteritis and Henoch-Schönlein purpura with HLA-DRB1 alleles. Moreover, the polymorphisms of other genes, such as interleukin-1 receptor antagonist, seem to be implicated in disease severity in patients with cutaneous vasculitis.

Adult↗

Henoch-Schönlein purpura in children and adults: clinical differences in a defined population.

OBJECTIVE: To examine epidemiologic, clinical, and outcome differences between children and adults with Henoch-Schönlein purpura (HSP) in a well-defined population. PATIENTS AND METHODS: Retrospective study of unselected patients with HSP seen at the only referral hospital for the Lugo region of Northwest Spain between 1980 and 2000. Patients were classified according to the criteria proposed by Michel et al. Two well-differentiated age groups were established for comparison: children (under 14 years of age) and adults (over 20 years of age). Also, to assess possible differences in the outcome, only those patients with at least 1 year of follow-up were included in the study. RESULTS: Seventy-three children and 31 adults fulfilled the inclusion criteria described above. Unlike in children, HSP in adults was more common in males. While in children, HSP manifested more commonly in fall and winter, summer and winter were the most common seasons of onset in adults. The frequency of gastrointestinal manifestations was similar in both groups. However, during the course of the disease, 6 of the 31 adults (19%) had severe renal manifestations and another 4 (13%) renal insufficiency. In children, by contrast, the frequency of severe renal manifestations or renal insufficiency during the course of the disease was significantly reduced compared with adults. After 6 years' median follow-up in children, complete recovery was observed in most cases. However, after 5 years' median follow-up, almost 40% of adults had persistent hematuria and 3 of them (10%) renal insufficiency that required hemodialysis in 2 cases. CONCLUSIONS: HSP is generally benign and self-limited in children and more severe in adults.

Adolescent↗

Patients chosen for treatment with cyclosporine because of severe rheumatoid arthritis are more likely to carry HLA-DRB1 shared epitope alleles, and have earlier disease onset.

OBJECTIVE: To determine whether patients with rheumatoid arthritis (RA) selected for treatment with cyclosporin A (CSA) because of severe disease are more likely to carry HLA-DRB1 alleles encoding the conserved "shared epitope" (SE) sequence. METHODS: The majority of patients (n = 178) were currently being treated with methotrexate (MTX), either alone or in combination with chloroquine and/or CSA. In about 30% of patients, treatment with CSA had been initiated because of limited response to MTX or MTX and chloroquine. Patients were treated as clinically indicated without knowledge of their HLA-DRB1 status. HLA-DRB1 typing was by a reverse dot blot method. RESULTS: Patients that had been treated with CSA were significantly more likely to carry an SE allele than patients not treated with CSA (81.5% vs 60.5%; OR 2.9, p = 0.006). Patients with 2 SE alleles were the most likely to have been treated with CSA. Results were still significant after correction for age, sex, and disease duration in a logistic regression model. There was no association between rheumatoid factor positivity and requirement for CSA therapy. Examination of individual SE alleles by multiple logistic regression analysis indicated that the strongest association was with presence of HLA-DRB1**0401 (p = 0.004). The DRB1*0401/*0404 genotype provided the greatest risk of requiring CSA treatment. Patients selected for CSA treatment had developed RA at a significantly earlier age than those not requiring CSA (44.4 vs 51.3 yrs; p = 0.004). CONCLUSION: Patients requiring treatment with CSA because of severe RA were significantly more likely to carry an SE allele than patients not requiring such treatment. CSA treated patients were also more likely to have had earlier age of disease onset. These data provide further evidence that bearing the SE (particularly 2 alleles) is associated with development of severe RA.

Adult↗