Development and initial validation of a damage index (DIAPS) in patients with thrombotic antiphospholipid syndrome (APS).

INTRODUCTION: In antiphospholipid syndrome (APS), certain principal manifestations are associated with a worse prognosis and organ damage. OBJECTIVE: The objective of this paper is to describe the development and initial content, criterion and construct validity of a disease-specific cumulative damage index in patients with thrombotic APS (DIAPS). METHODS: Through expert panel agreement, 47 items were considered to reflect damage in APS. This preliminary version of the DIAPS was submitted to four local and international clinical and research experts in APS who ranked each item according to severity. A Delphi exercise resulted in a final 37 item instrument. In the second phase, a cross-sectional study was conducted applying the DIAPS in patients included in a multicenter electronic registry of patients with APS. Quality of life related to health status was evaluated with the EuroQol for construct validation. An α Cronbach and correlation with the EuroQol scale were calculated with SPSS 20.0 (p < 0.05). RESULTS: We evaluated the DIAPS in 156 patients, 77% female, with a mean age at diagnosis 34.7 ± 5.5 years. A total of 69% had primary APS. Common comorbidities included obesity, depression and dyslipidemia. The most frequent manifestations resulting in sequelae were deep venous thrombosis and ischemic stroke. Blindness, retinal occlusive vessel disease, myocardial infarction, cardiac valve requiring replacement, mesenteric thrombosis, and renal insufficiency also occurred. Homogeneity: α Cronbach 0.619. DIAPS items correlated with EuroQol domains with the exception of pulmonary, renal, gastrointestinal, and endocrine systems. CONCLUSION: This study demonstrates content, criterion and construct validity of a new physician-reported instrument to assess the DIAPS. In addition, the DIAPS correlated with the EuroQol.

Vasculitis in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a complex heterogeneous autoimmune disease with a wide variety of clinical and serological manifestations that may affect any organ. Vasculitis prevalence in SLE is reported to be between 11% and 36%. A diverse clinical spectrum, due to inflammatory involvement of vessels of all sizes, is present. Even though cutaneous lesions, representing small vessel involvement, are the most frequent, medium and large vessel vasculitis may present with visceral affection, with life-threatening manifestations such as mesenteric vasculitis, pulmonary hemorrhage, or mononeuritis multiplex, with detrimental consequences. Early recognition and an appropriate treatment are crucial. Recent studies have shown that vasculitis in patients with SLE may present different clinical forms based on the organ involved and the size of the affected vessel. It is noteworthy that the episodes of vasculitis are not always accompanied by high disease activity. Recent articles on this topic have focused on new treatments for the control of vascular disease, such as biological therapies such as Rituximab and Belimumab, among others.

[Joint hypermobility syndrome]. / Síndrome de hipermovilidad articular.

Joint hypermobility syndrome is an inherited disorder with autosomal dominant pattern; is characterized by joint hyperlaxity and musculoskeletal pains. Thermal hypermobility refers to the increase in active or passive movements of joints based on their normal ranges. Joint hypermobility syndrome also has gastrointestinal symptoms, sleep disorders, fibromyalgia, psychological disorders, migraine headache, ophthalmic, autonomic, among others. To diagnose hypermobility syndrome, Brighton's criteria are generally accepted and published in 1998. This criteria also known as benign joint hypermobility syndrome. The term benign is used to distinguish it from other more severe conditions such as Ehler-Danlos (classic or vascular type), Marfan syndrome, and imperfect osteogenesis. Treatment with physiotherapy and pharmacological means help improve patients' quality of life.

El síndrome de hipermovilidad articular es un desorden hereditario con patrón autosómico dominante; se caracteriza por hiperlaxitud articular y dolores musculoesqueléticos. El término hipermovilidad se refiere al incremento en los movimientos activos o pasivos de las articulaciones con base en sus rangos normales. El síndrome de hipermovilidad articular presenta además síntomas gastrointestinales, trastornos de sueño, fibromialgia, trastornos sicológicos, cefalea migrañosa, oftálmicos, autonómicos, entre otros. Para diagnosticar el síndrome de hipermovilidad, en general son aceptados los criterios de Brighton, los cuales fueron publicados en 1998. También se le conoce como síndrome de hipermovilidad articular benigno. El término benigno se utiliza para distinguirlo de otras condiciones más severas como Ehler-Danlos (tipo clásico o vascular), síndrome de Marfan y osteogénesis imperfecta. El tratamiento con fisioterapia y medidas farmacológicas ayudan a mejorar la calidad de vida de los pacientes.