Hiperplasia nodular regenerativa hepática en una paciente con inmunodeficiencia variable común / Nodular regenerative hyperplasia of the liver in a patient with common variable immunodeficiency

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Síndrome antifosfolípido en pacientes con bacteriemia por Salmonella enteritidis / Antiphospholipid syndrome in a patient with Salmonella enteritidis bacteremia

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[Clinical characteristics of familial rheumatoid arthritis in Spain. A study of 73 families. Spanish Consortium for Rheumatoid Arthritis (CEAR) and European Consortium for Familial Rheumatoid Arthritis (ECRAF)]. / Características clínicas de la artritis familiar en España. Estudio de 73 familias. Consorcio Español para la Artritis Reumatoide (CEAR) y el European Consortium for Rheumatoid Arthritis Familiar (ECRAF).

OBJECTIVE: To describe clinical characteristics and the homogeneity of disease expression between involved members in multicase Spanish rheumatoid arthritis (RA) families. PATIENTS AND METHODS: 73 families with two or more siblings with RA were found, with a total of 149 patients distributed in 79 pairs (70 sib pairs, and 3 sib trios). Demographic, clinical and radiological characteristics were recorded in a standard questionnaire. RESULTS: Clinical characteristics were similar to those of sporadic RA with a high frequency of women (78%), positive rheumatoid factor (RF) (86%), erosions (89%) and a 25% of the patients having extraarticular disease. The most important variable in disease severity was disease duration. The concordance between family members of the same age and calendar year of disease onset, and the pattern of disease expression, was not higher than expected, showing that the disease is heterogenic. CONCLUSION: Environmental factors seem to be more important in RA susceptibility. Clinical characteristics of familiar RA in Spain do not seem to be different from sporadic RA, although differences were found in disease expression within families that may be due to variation of genetic or environmental factors, responsible for the susceptibility and disease duration.

[High prevalence of vitamin D deficiency in postmenopausal women at a rheumatology office in Madrid. Evaluation of 2 vitamin D prescription regimens]. / Alta prevalencia de deficiencia de vitamina D en mujeres posmenopáusicas de una consulta reumatológica en Madrid. Evaluación de dos pautas de prescripción de vitamina D.

BACKGROUND: Vitamin D deficiency has been frequently observed in the elderly population in Europe. However few information is available about the vitamin D status in postmenopausal women in the Mediterranean countries. The aim of this study was to evaluate the vitamin D status assessed by serum 25(OH)D3 (calcidiol) in postmenopausal women who attended a Rheumatology practice in Madrid area, and to evaluate calcidiol serum levels through one year after two forms of vitamin D administration. PATIENTS AND METHODS: Calcidiol serum levels were measured in 171 postmenopausal women (111 with osteoporosis and 60 without osteoporosis). 82 women with calcidiol serum levels < 10 ng/ml were distributed in two groups: Group I received 800 U/day of vitamin D3 associated with calcium (1 g/day) and group II, one dose of 80,000 U vitamin D orally as calcidiol and latter a daily dose of 800 U vitamin D3 plus 1 g calcium. Calcidiol serum levels were measured by RIA in both groups at basal condition and after three, six and twelve months under treatment. RESULTS: Three cut-offs were considered: 10, 15 and 20 ng/ml of calcidiol. Percentages of postmenopausal women with vitamin D deficiency for such cut-offs were: 35.3%, 64.1% and 87.1%, respectively. After three months of treatment, women from group II showed calcidiol serum levels higher than group I. At six and twelve months calcidiol serum levels were similar in both groups. CONCLUSIONS: A high prevalence of vitamin D deficiency was observed in a group of postmenopausal women who attended a rheumatology practice in Madrid area. Both forms of vitamin D administration seem not sufficient to maintain the adequate calcidiol serum levels in postmenopausal deficient women. A dose of 80,000 U of calcidiol twice a year should be considered.

Pamidronate and biochemical markers of bone turnover.

We have examined the response of different biochemical bone turnover markers to intravenous pamidronate administration (15 mg for 5 days) in 14 patients with Paget's disease, on days 8, 15 and 30 after pamidronate treatment. Urinary parameters of bone resorption, free pyridinolines (Pyr) and hydroxyproline (OHP), as well as serum tartrate-resistant acid phosphatase (TRAP) were measured. Two serum biochemical osteoblastic markers, alkaline phosphatase (AP) and osteocalcin (OC), were also analysed. In addition, ionic calcium (Ca2+) was measured in blood, and parathyroid hormone and calcitriol were measured in serum. All the biochemical markers of bone resorption tested decreased throughout the study. TRAP levels decreased slowly, meanwhile Pyr decreased maximally, below OHP values on day 8. However, the latter were lowest and were lower than those of Pyr on days 15 and 30. AP serum values also decreased during the study. Conversely, OC serum levels increased on days 8 and 15, decreasing to baseline levels on day 30. Ca2+ blood levels decreased while PTH plasma levels increased at all times during the period studied. Calcitriol serum levels increased on day 15. In conclusion, intravenous pamidronate administration was found to modify several biochemical parameters of bone turnover, including Pyr. Moreover, the changes in these parameters were different in intensity and "time course" during the study.

Erosive azotemic osteoarthropathy of the hands in chronic ambulatory peritoneal dialysis and hemodialysis.

OBJECTIVE: We studied the prevalence and significance of erosive azotemic osteoarthropathy (EAO) and its relationship with other osteoarticular abnormalities of dialysis-associated arthropathy (DAA). METHODS: 112 patients undergoing maintenance dialysis were studied: 63 hemodialysis (HD) and 49 continuous ambulatory peritoneal dialysis (CAPD). X-ray of the hands, shoulders, pelvis and cervical spine were examined for destructive spondyloarthropathy (SDA), bone cysts (BC), EAO and subperiosteal resorption. Beta 2-microglobulin (beta 2-m) and PTH were also measured. RESULTS: Fifteen patients (13%) had EAO, usually in several joints of the hands, DIPs being the most frequently affected. Both patients on HD and those on CAPD had EAO, although the prevalence was higher in the HD group, 12 (19%) vs. 3 (6%). Patients with EAO were older (p < 0.05) and had more carpal tunnel syndrome (CTS) (p < 0.05) and BC (p < 0.01). Only 3 out of 15 patients with EAO had severe secondary hyperparathyroidism (sHPTH) (PTH > 500), while 9/15 had neither radiologic nor laboratory evidence of sHPTH. No differences were found regarding the duration of dialysis, or beta 2-m or PTH level. CONCLUSION: EAO is not related to sHPTH and should be included within the spectrum of the clinical manifestations of DAA. Due to its location and radiologic picture, it is possible that etiologic factors leading to primary osteoarthritis may play a role in the development and evolution of EAO.

Calcitonin gene-related peptide II, substance P and vasoactive intestinal peptide in plasma and synovial fluid from patients with inflammatory joint disease.

Immunoreactive plasma and synovial fluid concentrations of calcitonin gene-related peptide II (CGRP II), substance P and vasoactive intestinal peptide (VIP) were measured in patients with osteoarthritis, gout and rheumatoid arthritis. Significantly higher levels of CGRP II and substance P-like immunoreactivity levels in synovial fluid were found in gout as well as CGRP II, substance P and VIP-like immunoreactivities in rheumatoid arthritis when compared to those in osteoarthritis. Plasma CGRP II, substance P and VIP-like immunoreactivity levels showed no significant differences among patients in the three different groups of arthritis. Our results suggest that these neuropeptides released from peripheral nerve endings into the synovial cavity probably play a pathogenic role in human joint inflammation.

McArdle's disease and gout.

We report the first case of McArdle's disease (muscle phosphorylase deficiency) and tophaceous gout. To examine the contribution of adenine nucleotide degradation to the disturbance of uric acid metabolism, we labeled the adenine nucleotide pool with [8-14C]adenine, and measured plasma and urine purines following vigorous exercise tests. Plasma and urinary hypoxanthine and xanthine concentrations and the specific radioactivity of urinary purines increased markedly, but plasma urate levels and uric acid excretion were not substantially modified. We suggest that, in this patient, the association of McArcle's disease with gout is coincidental.

Rheumatic manifestations in 556 patients with human immunodeficiency virus infection.

We studied in retrospect the rheumatic manifestations of 556 patients with human immunodeficiency virus (HIV) infection. Eighty percent were men. Eighty-six percent were intravenous drug abusers (IVDAs), 9% homosexual, 3% partners of high-risk persons having the infection, 0.4% hemophiliacs, and 2% had no known risk factors. We found rheumatic disorders in 63 (11%) patients. The most frequent findings were myalgias and/or arthralgias (4.5%; one patient had an inflammatory myopathy), skeletal infections (3.6%), and arthralgias (1.6%). Reiter's syndrome and seronegative arthritis were present only in 0.5%, and HIV-associated arthritis and vasculitis in 0.4%, respectively. Skeletal infections were caused predominantly by Staphylococcus aureus (60%) and Candida albicans (20%). All these patients were IV drug abusers whose clinical features were similar to those previously described in skeletal infections of non-HIV-infected IVDAs. Comparing these data with other studies composed primarily of homosexual men where Reiter's syndrome is the predominant rheumatic disorder, we conclude that the type of rheumatic complaint is more related to the risk factors than to HIV itself.

Female gout. Clinical spectrum and uric acid metabolism.

We reviewed the clinical features and uric acid metabolism in 37 female patients with gout. In 32 female patients (86%), gout was diagnosed after menopause. Among the five premenopausal patients, four had renal insufficiency and one had superactivity of phosphoribosylpyrophosphate synthetase. More than 50% of the female patients had osteoarthritis, hypertension, or renal insufficiency or were treated with diuretics. Comparison with 220 male patients with gout showed that female patients developed gout significantly later, more frequently had associated diseases, and more often were receiving diuretics, whereas significantly more male than female patients had alcoholism. The articular features of gout were similar in both groups. However, the prevalence of tophi was higher and its localization different in female than in male patients. Female patients with gout had a higher mean serum urate concentration and a lower mean urinary uric acid excretion than did male patients with gout. These differences were significant and independent of the effects of age, renal insufficiency, alcoholism, or previous diuretic intake. Renal underexcretion of uric acid appears to be more severe in female than in male patients with gout.