Distinct features of youth-onset primary antiphospholipid syndrome.

BACKGROUND: Characteristics of primary APS (PAPS) in the youth population have never been studied. In contrast with children, pregnancy is genuinely relevant in the youth age, and understanding clinical characteristics of PAPS patients within this specific age stratum may also provide insights regarding the well-known risk of poor obstetric outcomes during the adolescence. OBJECTIVE: To evaluate clinical and laboratory characteristics of patients with youth-onset PAPS (15-24 years) and compare them with adult-onset PAPS (over 24 years old). METHODS: This was a cross-sectional study derived from two rheumatology outpatient clinics. Patients who fulfilled Sidney criteria and who were 15 years of age or older at disease onset were included. Secondary APS patients were excluded. We subdivided patients into two groups: youth- (15-24 years) and adult-onset (over 24 years) and compared them regarding demographic characteristics, criteria and non-criteria manifestations, cardiovascular risk factors, and aPL status. For the pregnancy outcomes analysis, ever-pregnant patients were divided in three groups: youth-onset, early adult-onset (25-34 years), and late adult-onset (35-49 years). RESULTS: A total of 250 consecutive PAPS patients were included. Groups had a comparable female and Caucasian distribution. We found a similar disease duration (14.0±7.9 vs 17.0±10.1 years, p = 0.079) and similar rates of thrombotic arterial (34.2% vs. 42.0%, p = 0.250) and venous events (69.7% vs. 69.5%, p = 0.975) between them. Skin ulcers were more frequent in the youth-onset group (17.1% vs. 4.0%, p = 0.001), whereas nephropathy was less common (1.3% vs. 8.0%, p = 0.039). No differences were observed for the other criteria and non-criteria manifestations. The adult-onset group presented more frequently with hypertension (p = 0.002), hyperlipidemia (p = 0.008), and smoking (p = 0.003). The youth-onset group presented a higher frequency of obstetric events as the first manifestation of PAPS (30.3% vs. 21.7%, p = 0.005), with worse pregnancy outcomes, namely, fetal death (58.5% vs. 46.4% vs. 24.1%, p = 0.012) and premature delivery (35.8% vs. 19.0% vs. 10.3%, p = 0.016). Of note, all groups had a comparable number of pregnancies (2.81±2.52 vs 2.74±2.07, p = 0.899). CONCLUSION: This study provides novel evidence that youth-onset PAPS presents a higher frequency of obstetric complications as its first manifestation, with an increased risk of fetal death and preterm delivery. Early recognition of this condition by obstetricians is essential to improve prognosis.

Videocapillaroscopic Findings in Patients With Systemic Lupus Erythematosus With or Without Jaccoud Arthropathy.

BACKGROUND/OBJECTIVE: Systemic lupus erythematosus (SLE) is an autoimmune disease that can present changes in blood vessels, which can be evaluated by periungual nailfold videocapillaroscopy (VCP). This technique is important for the diagnosis of systemic sclerosis and to identify individuals with Raynaud phenomenon at higher risk of developing systemic sclerosis. This study aims to describe the videocapillaroscopic profile of a series of SLE patients and to investigate if the VCP pattern is different among those with Jaccoud arthropathy (JA) compared with those without. METHODS: Between September 2014 and March 2015, the patients in this study underwent VCP, clinical evaluation, and laboratory tests. The capillaroscopic patterns were defined as minor, major, and scleroderma (SD). The presence of capillaroscopic findings, such as elongated capillaries, tortuosity, ectasia, prominent venous plexus, neoangiogenesis, hemorrhage, and megacapillaries, were also observed. Associations were calculated using the χ2, Fisher exact, or Student t test. RESULTS: In a population of 113 females with SLE (67 without JA and 46 with JA), at least 1 alteration was observed in VCP in 89.40% of them, among which "nonspecific changes" were the most prevalent. Minor changes were seen in 39 (58.2%) and 26 (56.5%), major changes in 21 (31.3%) and 11 (23.9%), and SD pattern in 2 (3.0%) and 3 (6.5%), in the patients without and with JA, respectively (p > 0.05). CONCLUSIONS: The majority of patients with SLE demonstrated changes in the VCP examination, but this tool did not allow discrimination between those with or without JA.

Surgery for Jaccoud Arthropathy: A Systematic Review.

INTRODUCTION: Jaccoud arthropathy (JA) is characterized by the presence of joint deformities similar to those seen in rheumatoid arthritis but generally with a "reversible" pattern. The etiopathogenic mechanisms are not known, and its therapeutical approach has been regarded as disappointing. The aim of the present study was to perform a systematic review of the literature on the scientific evidence of the surgical procedures for JA. METHODS: The MEDLINE, LILACS, and Scielo databases were searched using the following keywords: "systemic lupus erythematosus," "rheumatic fever," "Jaccoud arthropathy," "deforming arthropathy," "surgery," and their corresponding terms in Portuguese and Spanish. The search period was between 1966 and 2014. RESULTS: Only 7 articles fulfilled the inclusion criteria, and a total of 58 patients underwent surgical procedures for JA. Such studies were limited to small case series, there was no uniform definition of the outcome, and the follow-up time varied largely. CONCLUSIONS: There is no consensus on the best approach for the surgical procedures in patients with JA, who are the best candidates to undergo this, and when to indicate the procedure.