Hormonal Dependence and Cancer in Systemic Lupus Erythematosus.

OBJECTIVE: To estimate the incidence and analyze any cancer-associated factors in patients with systemic lupus erythematosus (SLE), differentiating between hormone-sensitive (HS) and non-HS cancers. METHODS: This was a retrospective multicenter study of a patient cohort from the Systemic Lupus Erythematosus Registry of the Spanish Society of Rheumatology. Included were the first cancer post-SLE diagnosis, clinical and sociodemographic information, cumulative damage, severity, comorbidities, treatments, and refractoriness. Cancers were classified as HS (prostate, breast, endometrium, and ovarian) and non-HS (the remainder). The standardized incidence ratio (SIR) was calculated and logistic regression models were built. RESULTS: A total of 3,539 patients (90.4% women) were included, 154 of whom had cancer (91% female), and 44 had HS cancer (100% female). The cancer SIR was 1.37 (95% confidence interval [95% CI] 1.15-1.59), with higher values in women age <65 years (SIR 2.38 [95% CI 1.84-2.91]). The SIR in women with HS versus non-HS cancer was 1.02 (95% CI 0.13-1.91) and 1.93 (95% CI 0.98-2.89). In HS versus non-HS cancers, SLE diagnostic age (odds ratio [OR] 1.04 [P = 0.002] versus 1.04 [P = 0.019]), and period of disease evolution (OR 1.01 [P < 0.001] versus 1.00 [P = 0.029]) were associated with cancer. The Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (OR 1.27 [P = 0.022]) and angiotensin-converting enzyme (ACE) inhibitor prescriptions (OR 2.87 [P = 0.048]) were associated with non-HS cancers. CONCLUSION: Cancer incidence in patients with SLE was higher than in the Spanish population, particularly among young women. This increase might be due to non-HS cancers, which would be associated with SLE involving greater cumulative damage where more ACE inhibitors are prescribed.

Juvenile- and adult-onset systemic lupus erythematosus: a comparative study in a large cohort from the Spanish Society of Rheumatology Lupus Registry (RELESSER).

OBJECTIVES: We aimed to describe juvenile-onset systemic lupus erythematosus (jSLE) features and to establish its differences compared to adult-onset SLE (aSLE) from a large national database. METHODS: Data from patients (≥4 ACR criteria) included in Spanish Society of Rheumatology Lupus Registry (RELESSER) were analysed. Sociodemographic, clinical, serological, activity, treatment, cumulative damage, comorbidities and severity data were collected. Patients with disease onset <18 years were described and compared to those with disease onset ≥18 years. RESULTS: We reviewed 3,428 aSLE patients (89.6% women) and 484 jSLE patients (89.8% girls), 93% Caucasian (both groups). Mean age at diagnosis was 38.1±14 and 16.6±6.3 years (p<0.001) and mean age at the end of follow-up was 48.8±14.3 and 31.5±30 years (p<0.001), respectively. jSLE showed significantly more clinical (including lymphadenopathy, fever, malar rash, mucosal ulcers, pericarditis, pleuritis, Raynaud's phenomenon, lupus nephritis, recurrent nephritis, histologic nephritis changes, thrombocytopenia, haemolytic anaemia, thrombotic thrombocytopenic purpura, seizures, lupus headache and organic brain syndrome) and immunological (a-dsDNA and a-Sm antibodies, hypocomplementaemia) involvement than did aSLE, except for secondary Sjögren's syndrome, a-Ro antibodies, fibromyalgia and osteoporosis. jSLE also showed more SLE family history, longer diagnosis delay, higher SLEDAI and Katz scores, but lower Charlson scores than aSLE. Several specific domains were more frequently involved in SLICC/ACR DI in jSLE. jSLE patients more frequently underwent all SLE-related treatment and procedures, as well as dialysis and kidney transplantations. CONCLUSIONS: jSLE shares many clinical and serological features with aSLE. However, jSLE patients typically manifested more activity, severity, cumulative damage in certain areas, than their aSLE counterparts.

Characterization of Patients With Lupus Nephritis Included in a Large Cohort From the Spanish Society of Rheumatology Registry of Patients With Systemic Lupus Erythematosus (RELESSER).

The aim of the study was to profile those patients included in the RELESSER registry with histologically proven renal involvement in order to better understand the current state of lupus nephritis (LN) in Spain. RELESSER-TRANS is a multicenter cross-sectional registry with an analytical component. Information was collected from the medical records of patients with systemic lupus erythematosus who were followed at participating rheumatology units. A total of 359 variables including demographic data, clinical manifestations, disease activity, severity, comorbidities, LN outcome, treatments, and mortality were recorded. Only patients with a histological confirmation of LN were included. We performed a descriptive analysis, chi-square or Student's t tests according to the type of variable and its relationship with LN. Odds ratio and confidence intervals were calculated by using simple logistic regression. LN was histologically confirmed in 1092/3575 patients (30.5%). Most patients were female (85.7%), Caucasian (90.2%), and the mean age at LN diagnosis was 28.4 ±â€Š12.7 years. The risk for LN development was higher in men (M/F:47.85/30.91%, P < 0.001), in younger individuals (P < 0.001), and in Hispanics (P = 0.03). Complete response to treatment was achieved in 68.3% of patients; 10.35% developed ESRD, which required a kidney transplant in 45% of such cases. The older the patient, the greater was the likelihood of complete response (P < 0.001). Recurrences were associated with persistent lupus activity at the time of the last visit (P < 0.001) and with ESRD (P < 0.001). Thrombotic microangiopathy was a risk factor for ESRD (P = 0.04), as for the necessity of dialysis (P = 0.01) or renal transplantation (P = 0.03). LN itself was a poor prognostic risk factor of mortality (OR 2.4 [1.81-3.22], P < 0.001). Patients receiving antimalarials had a significantly lower risk of developing LN (P < 0.001) and ESRD (P < 0.001), and responded better to specific treatments for LN (P = 0.014). More than two-thirds of the patients with LN from a wide European cohort achieved a complete response to treatment. The presence of positive anti-Sm antibodies was associated with a higher frequency of LN and a decreased rate of complete response to treatment. The use of antimalarials reduced both the risk of developing renal disease and its severity, and contributed to attaining a complete renal response.

Relationship between damage clustering and mortality in systemic lupus erythematosus in early and late stages of the disease: cluster analyses in a large cohort from the Spanish Society of Rheumatology Lupus Registry.

OBJECTIVES: To identify patterns (clusters) of damage manifestations within a large cohort of SLE patients and evaluate the potential association of these clusters with a higher risk of mortality. METHODS: This is a multicentre, descriptive, cross-sectional study of a cohort of 3656 SLE patients from the Spanish Society of Rheumatology Lupus Registry. Organ damage was ascertained using the Systemic Lupus International Collaborating Clinics Damage Index. Using cluster analysis, groups of patients with similar patterns of damage manifestations were identified. Then, overall clusters were compared as well as the subgroup of patients within every cluster with disease duration shorter than 5 years. RESULTS: Three damage clusters were identified. Cluster 1 (80.6% of patients) presented a lower amount of individuals with damage (23.2 vs 100% in clusters 2 and 3, P < 0.001). Cluster 2 (11.4% of patients) was characterized by musculoskeletal damage in all patients. Cluster 3 (8.0% of patients) was the only group with cardiovascular damage, and this was present in all patients. The overall mortality rate of patients in clusters 2 and 3 was higher than that in cluster 1 (P < 0.001 for both comparisons) and in patients with disease duration shorter than 5 years as well. CONCLUSION: In a large cohort of SLE patients, cardiovascular and musculoskeletal damage manifestations were the two dominant forms of damage to sort patients into clinically meaningful clusters. Both in early and late stages of the disease, there was a significant association of these clusters with an increased risk of mortality. Physicians should pay special attention to the early prevention of damage in these two systems.

Fibromyalgia prevalence and related factors in a large registry of patients with systemic lupus erythematosus.

OBJECTIVES: The objective of this study is to determine the prevalence of fibromyalgia (FM) in systemic lupus erythematosus (SLE) patients and to study its relationship to depression and other SLE-related factors. METHODS: A cross-sectional data analysis from the RELESSER-Transversal Spanish Registry, which includes SLE patients in a national multicentre retrospective charts review, was performed. INCLUSION CRITERIA: patients who fulfilled ≥4 ACR 1997 SLE criteria. Main variables were disease duration, depression, sociodemographics, comorbidities, SLE activity symptoms, serological findings, therapies and different disease status indices. Statistical analyses included a descriptive, associative and logistic regression analyses. A literature review was performed. RESULTS: 3,591 SLE patients were included, 90.1% women, 34.6 years of age at diagnosis (SD 14.6 years) and 93.1% Caucasians. FM prevalence was 6.2%. SLE patients with disease duration >5 years showed more FM than those with duration <5 years: 6.9% vs. 4.0%, respectively (p<0.05). SLE-FM patients showed higher prevalence of depression compared to non-FM-SLE patients: 53.1% vs. 14.6%, respectively (p<0.001). After adjusting by risk factors, the OR (CI) of suffering depression in FM-SLE patients was 6.779 (4.770-9.636), p<0.001. The OR of having secondary Sjögren's 2.447 (1.662-3.604), p<0.001, photosensitivity 2.184 (1.431-3.334), p<0.001, and oral ulcers 1.436 (1.005-2.051), p=0.047. CONCLUSIONS: Prevalence of FM in Caucasian SLE patients was high compared to the general population, and was significantly higher in those in later stages of disease. SLE patients with depression showed a strong risk of developing FM. Photosensitivity, oral ulcers and secondary Sjögren's were the only SLE-related factors associated with FM.

Cardiovascular Events in Systemic Lupus Erythematosus: A Nationwide Study in Spain From the RELESSER Registry.

This article estimates the frequency of cardiovascular (CV) events that occurred after diagnosis in a large Spanish cohort of patients with systemic lupus erythematosus (SLE) and investigates the main risk factors for atherosclerosis. RELESSER is a nationwide multicenter, hospital-based registry of SLE patients. This is a cross-sectional study. Demographic and clinical variables, the presence of traditional risk factors, and CV events were collected. A CV event was defined as a myocardial infarction, angina, stroke, and/or peripheral artery disease. Multiple logistic regression analysis was performed to investigate the possible risk factors for atherosclerosis. From 2011 to 2012, 3658 SLE patients were enrolled. Of these, 374 (10.9%) patients suffered at least a CV event. In 269 (7.4%) patients, the CV events occurred after SLE diagnosis (86.2% women, median [interquartile range] age 54.9 years [43.2-66.1], and SLE duration of 212.0 months [120.8-289.0]). Strokes (5.7%) were the most frequent CV event, followed by ischemic heart disease (3.8%) and peripheral artery disease (2.2%). Multivariate analysis identified age (odds ratio [95% confidence interval], 1.03 [1.02-1.04]), hypertension (1.71 [1.20-2.44]), smoking (1.48 [1.06-2.07]), diabetes (2.2 [1.32-3.74]), dyslipidemia (2.18 [1.54-3.09]), neurolupus (2.42 [1.56-3.75]), valvulopathy (2.44 [1.34-4.26]), serositis (1.54 [1.09-2.18]), antiphospholipid antibodies (1.57 [1.13-2.17]), low complement (1.81 [1.12-2.93]), and azathioprine (1.47 [1.04-2.07]) as risk factors for CV events. We have confirmed that SLE patients suffer a high prevalence of premature CV disease. Both traditional and nontraditional risk factors contribute to this higher prevalence. Although it needs to be verified with future studies, our study also shows-for the first time-an association between diabetes and CV events in SLE patients.

Sarcoma epitelioide del ilíaco derecho imitando una sacroilitis / Epithelioid sarcoma of the right ilium mimicking sacroiliitis

El sarcoma epitelioide representa menos del 1% de los sarcomas de partes blandas, posee una elevada capacidad para invadir los linfáticos regionales y alta tasa de recurrencias. Las formas clásicas se localizan preferentemente en la porción distal de las extremidades y afectan sobre todo a varones jóvenes, mientras que las formas proximales, además de características histológicas diferentes, tienden a situarse en el tronco, la pelvis y la raíz de las extremidades. El desarrollo intraóseo de este sarcoma es excepcional. En este artículo describimos a un paciente de 47 años que consultó por dolor en la región sacroilíaca derecha imitando una sacroilitis. La tomografía computarizada pélvica objetivó una lesión osteolítica en la región posterosuperior del ilíaco derecho, de la que se obtuvieron muestras mediante biopsia percutánea. Los hallazgos histológicos fueron típicos de sarcoma epitelioide clásico. En nuestro conocimiento, este es el segundo caso descrito en la literatura en que esta neoplasia se desarrolla primariamente en el tejido óseo (AU)

Epithelioid sarcoma represents less than 1% of soft tissue sarcomas and has a high recurrence rate and strong ability to invade the regional lymphatic pathways. Classic epithelioid sarcoma has a predilection for young men and usually affects the distal extremities, while the proximal-type is characterized by different histological features and most commonly occurs in the proximal part of the extremities, trunk and pelvis. Intraosseous tumor development is rare. We describe a 47 year old patient with sacroiliac pain mimicking sacroiliitis. A computed tomography of the pelvis showed a destructive intraosseous lesion of the upper- right iliac. Percutaneous biopsy of the lesion was obtained and histological findings were typical of classic epithelioid sarcoma. To our knowledge, this is the second case of intraosseous epithelioid sarcoma described in the medical literature (AU)

Epithelioid sarcoma of the right ilium mimicking sacroiliitis.

Epithelioid sarcoma represents less than 1% of soft tissue sarcomas and has a high recurrence rate and strong ability to invade the regional lymphatic pathways. Classic epithelioid sarcoma has a predilection for young men and usually affects the distal extremities, while the proximal-type is characterized by different histological features and most commonly occurs in the proximal part of the extremities, trunk and pelvis. Intraosseous tumor development is rare. We describe a 47 year old patient with sacroiliac pain mimicking sacroiliitis. A computed tomography of the pelvis showed a destructive intraosseous lesion of the upper- right iliac. Percutaneous biopsy of the lesion was obtained and histological findings were typical of classic epithelioid sarcoma. To our knowledge, this is the second case of intraosseous epithelioid sarcoma described in the medical literature.

Artritis séptica como complicación de la artropatía neuropática del hombro asociada a siringomielia cervical / Septic arthritis complicating neuropathic shoulder due to cervical syringomyelia

Una de las principales causas de osteoartropatía neuropática del hombro es la siringomielia cervical. El dolor crónico y la tumefacción del hombro son las manifestaciones más frecuentes, pero ocasionalmente pueden desarrollarse lesiones destructivas osteoarticulares de rápida evolución (menos de 6 semanas), que plantean el diagnóstico diferencial con la artritis séptica y algunos tumores. Describimos 2 varones con artritis séptica sobre artropatía neuropática del hombro secundaria a siringomielia. Ambos cursaron con dolor agudo exacerbado por los movimientos activos y pasivos, malestar general y fiebre. El primero, un hombre de 39 años, presentó una artritis del hombro izquierdo por Staphylococcus aureus. El segundo, un varón de 59 años, sufrió una infección del hombro derecho por Staphylococcus epidermidis, microorganismo que también se aisló en los 3 hemocultivos. El clínico debe considerar la infección como una posible complicación en el curso evolutivo de la artropatía neuropática del hombro (AU)

One of the main causes of neuropathic osteoarthropathy of the shoulder is cervical syringomyelia. Chronic pain and swelling of the shoulder are the most frequent manifestations, but it occasionally can develop rapid osteoarticular destructive lesions (in less than six weeks), which raise the diagnostic possibility of septic arthritis and some tumours. We present the report of two men with septic arthritis of the shoulder associated with neuropathic arthropathy secondary to syringomyelia. Both patients presented with sudden shoulder pain exacerbated by either passive or active joint movements, malaise and fever. The first patient, a 39-year-old man, suffered left shoulder arthritis due to Staphylococcus aureus. The second patient, a 59-year-old man presented with right shoulder arthritis caused by to Staphylococcus epidermidis. The last microorganism also was isolated in three blood cultures (AU)

[Septic arthritis complicating neuropathic shoulder due to cervical syringomyelia]. / Artritis séptica como complicación de la artropatía neuropática del hombro asociada a siringomielia cervical.

One of the main causes of neuropathic osteoarthropathy of the shoulder is cervical syringomyelia. Chronic pain and swelling of the shoulder are the most frequent manifestations, but it occasionally can develop rapid osteoarticular destructive lesions (in less than six weeks), which raise the diagnostic possibility of septic arthritis and some tumours We present the report of two men with septic arthritis of the shoulder associated with neuropathic arthropathy secondary to syringomyelia. Both patients presented with sudden shoulder pain exacerbated by either passive or active joint movements, malaise and fever. The first patient, a 39-year-old man, suffered left shoulder arthritis due to Staphylococcus aureus. The second patient, a 59-year-old man presented with right shoulder arthritis caused by to Staphylococcus epidermidis. The last microorganism also was isolated in three blood cultures. Infection should certainly be considered as a possible complication of the natural history of the neuropathic shoulder.

Osteoartropatía hipertrófica primaria (paquidermoperiostosis). Aportación de 2 casos familiares y revisión de la literatura / Primary hypertrophic osteoarthropathy (pachydermoperiostosis). Report of two familial cases and literature review

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[Primary hypertrophic osteoarthropathy (pachydermoperiostosis). Report of two familial cases and literature review]. / Osteoartropatía hipertrófica primaria (paquidermoperiostosis). Aportación de 2 casos familiares y revisión de la literatura.

The primary hypertrophic osteoarthropathy (pachydermoperiostosis) is a hereditary disease characterized by skin thickening (pachydermia), finger clubbing, and proliferation of periosteum (periostitis) with subperiosteal new bone formation. We describe the cases of two brothers of 30 and 24 years, who consulted due to bone pain, arthralgia and oligoarthritis. Pachydermia, hyperhidrosis, seborrhea, digital clubbing, periostosis and non-inflammatory effusions of the knees. The first had been diagnosed with juvenile idiopathic arthritis at age 15, while the youngest also presented with a thoracic scoliosis, hypertrophic gastritis, iron deficiency anemia and glucose intolerance by pancreatic endocrine dysfunction. In both patients, symptoms were controlled satisfactorily with etoricoxib (90mg/day) and risedronate (35mg/week).

Artritis por Staphylococcus epidermidis sobre prótesis de rodilla en una mujer de 82 años con gota secundaria a diuréticos / No disponible

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[Clinical activity in outpatient rheumatology clinics in Galicia (Spain)]. / Actividad asistencial en las consultas externas de las unidades de reumatología de Galicia.

OBJECTIVES: To analyze patients attending rheumatology units of the public health service in Galicia (Spain). PATIENTS AND METHODS: We performed a multicenter study. Outpatients from all rheumatology clinics of the Galician health service were included over a 1-week period. RESULTS: A total of 1,357 outpatients were studied (422 new patients and 935 follow-up patients). The mean ages were 55.1±15.6 and 56.9 ± 16.7 years, respectively. Among new patients, 22.5% had inflammatory disease, and 77.5% had noninflammatory disorders. The main source of patient referral was primary care. The overall kappa index in the analysis of agreement between the diagnosis of the referring physician and that of the rheumatologist was 0.8 (95% CI: 0.73-0.86). Agreement was appreciably lower in the group with inflammatory diseases. Among follow-up patients, those with a diagnosis of inflammatory disorders represented 63.4% of the total. This group showed a higher score in the HAQ test and had a greater frequency of patients with permanent work disability. Overall, patients with inflammatory disease required longer consultations than the remaining patients, and their discharge rate was considerably lower. CONCLUSIONS: In rheumatology outpatient clinics, patients with inflammatory diseases are more numerous, spend longer in consultations, have greater disability, are more likely to show permanent work disability, and tend to accumulate with the passage of time. Agreement was high in the diagnosis of noninflammatory diseases, contrasting with the high referral rate.

Análisis comparativo de las consultas externas de reumatología y cirugía ortopédica-traumatología en un área sanitaria gallega / Comparative analysis of the out patient consultories of rheumatology and orthopedic surgery-traumatology in a Gallician health care area

Objetivo: Analizar comparativamente las características de las consultas externas de los servicios de reumatología (REU) y cirugía ortopédica-traumatología (COT) dentro de un área sanitaria. Pacientes y métodos: POVISA es el centro de referencia de un sector de población del Servicio Gallego de Salud (SERGAS) de 127.000 habitantes. El servicio de documentación clínica revisó retrospectivamente las historias de los enfermos adultos (edad > 14 años) atendidos por primera vez en las consultas externas de COT y REU desde el 1 de enero hasta el 31 de marzo de 1999, registrando el origen de la derivación (atención primaria, atención especializada o servicio de urgencias), los procedimientos quirúrgico-instrumentales requeridos y codificando los diagnósticos consignados por el especialista (reumatólogo o traumatólogo). Resultados: Se atendieron un total de 1.983 pacientes, 1.403 (70,7 por ciento) en COT y 580 (29,3 por ciento) en REU, en su mayoría derivados desde atención primaria (92 por ciento en REU; 95 por ciento en COT). Hubo un claro predominio femenino en ambas especialidades: COT (60 por ciento), REU (77 por ciento). Se constataron uno o más diagnósticos en 496 (85,5 por ciento) de los pacientes de REU frente a 901 (64,2 por ciento) de los enfermos atendidos en COT, siendo el promedio de diagnósticos por paciente de 1,4 (REU) frente a 0,6 (COT) (p < 0,0001). Las artrosis (COT: 28,6 por ciento; REU: 63,3 por ciento) y los reumatismos de partes blandas (COT: 27,9 por ciento; REU: 26,2 por ciento) fueron los procesos más frecuentes, seguidos de las enfermedades del disco intervertebral y las anomalías biomecánicas de la columna: 21,5 por ciento (REU) y 8 por ciento (COT). Los traumatismos fueron registrados como causa de consulta en 81/1.403 casos (5,8 por ciento) y en 169/1.403 (12,9 por ciento) fue preciso algún procedimiento instrumental o quirúrgico. De los 50 enfermos derivados desde urgencias a COT, se diagnosticó artrosis o reumatismos de partes blandas en 19 (38 por ciento). En REU se recogieron procesos reumáticos inflamatorios en 54/496 pacientes (10,9 por ciento). Conclusiones: Existe una notable superposición asistencial entre los servicios de REU y COT. Para ambos, las enfermedades médicas del aparato locomotor, sobre todo artrosis y reumatismos de partes blandas, constituyen el principal motivo de consulta. El superior rendimiento diagnóstico observado en REU puede explicarse por las características de la especialidad, más afines con estos procesos. La asistencia en las enfermedades del aparato locomotor no parece adecuadamente estructurada; se precisa una mayor presencia de reumatólogos y la aplicación de protocolos de derivación que permitan optimizar y complementar la actividad de COT y REU (AU)

Mujer de 63 años con dolor referido al tobillo izquierdo / Sixty-three-years-old woman with left ankle pain

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