Risk, predictors, and clinical characteristics of lymphoma development in primary Sjögren's syndrome.

OBJECTIVE: To assess the risk and predictors of lymphoma development in a large cohort of patients with primary Sjögren's syndrome (pSS). METHODS: Cox-regression analyses were used to study the predictive value of clinical and laboratory findings at pSS diagnosis, and Kaplan-Meier survival curves to compare survival probability between patients who developed lymphoma and the total cohort. Expected risk for lymphoma was calculated by comparison with the background population. RESULTS: Eleven (4.5%) from 244 patients developed a non-Hodgkin lymphoma (NHL). Diffuse large B-cell and mucosa-associated lymphoid tissue lymphomas occurred at a similar frequency. Three (27.3%) patients died: 2 due to transformation from mucosa-associated lymphoid tissue to diffuse large B-cell. Purpura (HR 8.04, 95% confidence interval [CI] 2.33-27.67), parotidomegaly (HR 6.75, 95%CI 1.89-23.99), anemia (HR 3.43, 95%CI 1.04-11.35), leukopenia (HR 8.70, 95%CI 2.38-31.82), lymphocytopenia (HR 16.47, 95%CI 3.45-78.67), hypergammaglobulinemia (HR 4.06, 95%CI 1.06-15.58), low C3 (HR 36.65, 95%CI 10.65-126.18), and low C4 (HR 39.70, 95%CI 8.85-126.18) levels at pSS diagnosis were significant predictors of NHL development, but only hypocomplementemia and lymphocytopenia were independent risk factors. Hypocomplementemia was related to earlier development of NHL and higher mortality. The cumulative risk of developing lymphoma ranged from 3.4% in the first 5 years to 9.8% at 15 years. Standardized incidence ratio (95%CI) for NHL development was 15.6 (95%CI 8.7-28.2). CONCLUSIONS: Patients with pSS have a 16-fold increased risk of developing lymphoma. This risk increases with time. Hypocomplementemia and lymphocytopenia at pSS diagnosis are the strongest predictors. Survival is clearly reduced in patients with hypocomplementemia. Indolent lymphomas tend to evolve over time toward a more aggressive histologic type.

Stroke and multi-infarct dementia as presenting symptoms of giant cell arteritis: report of 7 cases and review of the literature.

Cerebrovascular accidents (CVAs) and multi-infarct dementia have rarely been reported as presenting symptoms of giant cell arteritis (GCA), although 3%-4% of patients with GCA may present with CVAs during the course of the disease. We describe 7 patients with biopsy-proven GCA who presented with stroke or multi-infarct dementia. Most of them had other symptoms of GCA when the disease began that were misdiagnosed or not noticed. The internal carotid arteries were involved in 4 patients and the vertebrobasilar arteries in 3, with bilateral vertebral artery occlusion in 1. Small cerebral infarction foci on cranial computed tomography (CT) scan and magnetic resonance imaging (MRI) were found in 5 cases, and cerebellar infarction, in 2. MR angiography showed intracranial arteritis in 4 cases. Treatment with glucocorticoids and adjunctive antiplatelet or anticoagulant therapy was given in all cases, with neurologic improvement in 5. Two patients died. Necropsy demonstrated generalized GCA involving the medium and small cerebral vessels in 1 case. Central nervous system involvement is a rare complication in GCA but is important to recognize, as it can be reversible if diagnosed and treated promptly. Suspicion should arise in elderly patients suffering from strokes with a quickly progressing stepwise course and associated headache, fever, or inflammatory syndrome. In these cases, temporal artery biopsy should be performed without delay. Early diagnosis of GCA and immediate initiation of corticosteroid treatment may prevent progressive deterioration and death. Additional antiplatelet or anticoagulant therapy should be evaluated according to the individual risk and benefit to the patient under care.

Fármacos antifactor de necrosis tumoral en las vasculitis sistémicas / Anti-TNF alpha agents in vasculitis

No disponible

Paraneoplastic vasculitis in patients with solid tumors: report of 15 cases.

OBJECTIVE: To review all cases of concurrent vasculitis and solid tumors diagnosed at our Department over a 15-year period and explore evidence that would support the notion of vasculitis being a true paraneoplastic syndrome. METHODS: We reviewed the records of all patients diagnosed with vasculitis and solid tumors within 12 months of each other and prospectively followed until death or our report. We analyzed the main features and outcome of vasculitis in this setting. We also reviewed all cases published in the French-English literature. RESULTS: Fifteen patients (9 men and 6 women) in whom both vasculitis and solid tumor occurred within the same 12 months were identified. Mean age was 72.5 years (range 58-84). In 7 cases the diagnosis of vasculitis antedated that of cancer, in 6 both processes were synchronously diagnosed, and in 2 vasculitis appeared after cancer diagnosis. The most common vasculitis was cutaneous leukocytoclastic vasculitis (n = 9). Other vasculitides included Henoch-Shönlein purpura (n = 2), polyarteritis nodosa (n = 1), and giant cell arteritis (n = 3). The commonest malignancies were carcinomas of urinary organs (40%), lung (26.7%), and gastrointestinal tract (26.7%). The median followup was 28.4 months (range 1-96). Thirteen of the 15 patients demonstrated concordance of disease activity and treatment response for both cancer and vasculitis. Vasculitis flared heralding tumor recurrence or progression in 7 (46.6%) cases. CONCLUSION: In our patients, resolution of vasculitis following effective treatment of the putatively linked malignancy, and recurrence of vasculitis heralding tumor recurrence or progression, provide strong evidence for vasculitis being a true paraneoplastic syndrome. Chronic or persistent vasculitis with poor response to usually effective therapy, especially in elderly patients, should raise questions about underlying malignancy.

Síndrome de Sjögren primario: características clínicas e inmunológicas de 114 pacientes / Primary Sjögren's syndrome: clinical and immunological characteristics of 114 patients

FUNDAMENTO: Analizar las características clínicas, evolutivas e inmunológicas de una serie de 114 pacientes diagnosticados de síndrome de Sjögren primario. Evaluar las diferencias clínicas en función de los criterios diagnósticos utilizados y la asociación del síndrome de Sjögren primario con procesos linfoproliferativos. PACIENTES Y MÉTODO: Se incluyeron 114 pacientes (108 mujeres y 6 varones) diagnosticados de síndrome de Sjögren primario. Todos cumplían los criterios diagnósticos propuestos por el Grupo de Estudio de la Comunidad Europea, y 76 cumplían los criterios de San Diego. RESULTADOS: La edad media de los pacientes fue de 51 años, con un tiempo medio de seguimiento clínico de 7,3 años. El síntoma más frecuente de inicio de la enfermedad (70 por ciento) fue la xerostomía/xeroftalmía (síndrome seco). Entre las manifestaciones extraglandulares, se observó afección articular en un 42 por ciento de los pacientes, neurológica en un 35 por ciento, pulmonar en el 21 por ciento y hepática en el 13 por ciento. Once pacientes (9 por ciento) presentaron fenómenos vasculíticos y 3 (2 por ciento), un proceso linfoproliferativo. No se apreciaron diferencias estadísticamente significativas en los síntomas de inicio de la enfermedad, en la frecuencia de afección glandular y extraglandular, ni en la gravedad de la enfermedad entre los pacientes diagnosticados según los criterios europeos frente a los de San Diego. La presencia del virus de la hepatitis C (VHC) se asoció de forma significativa con la vasculitis (p < 0,001; odds ratio [OR]: 20,6; intervalo de confianza [IC] del 95 por ciento, 3,2-129) y con procesos linfoproliferativos (p < 0,001). CONCLUSIONES: El curso clínico del síndrome de Sjögren no varía en función de los criterios diagnósticos. Existe asociación entre la vasculitis y los procesos linfoproliferativos con el VHC, lo que confiere unas características diferenciales a este subgrupo de pacientes (AU)