Background/Objective The epidemiology of vasculitis is variable in different geographic areas, and this issue has not been approached in Brazil yet. The objective of this study was to assess the frequency of vasculitis in specialized centers in Brazil. Methods This cross-sectional study was performed in 9 vasculitis outpatient clinics from 6 different states mainly from the Southeast and the Northeast regions of Brazil between 2015 and 2017. Diagnosis and/or classification criteria for Behçet disease (BD), Takayasu arteritis (TA), giant cell arteritis (GCA), polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and cryoglobulinemic vasculitis (CryoVas) were used to include patients with at least 6 months of follow-up in this hospital-based survey. Results A total of 1233 patients with systemic vasculitis were included from the Southeast region. Behçet disease was the most frequent vasculitis (35.0%) followed by TA (26.4%), GPA (16.2%), PAN (5.8%), GCA (5.8%), EGPA (4.3%), MPA (3.4%), and CryoVas (3.0%). Up to 7.8% of vasculitis patients had a juvenile onset, and the frequency of vasculitides found in children and adolescents was as follows: TA (52.6%), BD (24.7%), GPA (12.4%), and PAN (10.3%). No cases of EGPA, MPA, and CryoVas were diagnosed before the age of 18 years. As a comparator, 103 vasculitis patients were included in the Northeast of Brazil where TA was found in 36.9% and BD in 31.1% of vasculitis cases. No GCA cases were found in the Northeast part of Brazil. Conclusions Similar to the epidemiology of vasculitis in Asia, BD and TA are the most frequent vasculitis in Southeastern Brazilian referral centers.

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Ultrasound examination is a reliable and noninvasive imaging method for the evaluation of joint involvement in SLE. Half of the patients with JA have ultrasonographic signs of joint inflammation, and these abnormalities may be found even in the absence of disease activity.

Objectives The objective of this paper is to perform an ultrasonography (US) analysis of hands and wrists in two groups of patients with systemic lupus erythematosus (SLE), with and without Jaccoud's arthropathy, matched by age and disease duration and to correlate them with levels of CXCL13 clinical features, laboratory tests and disease activity score. Methods Sixty-four patients with SLE were enrolled, 32 with and 32 without Jaccoud's arthropathy. Each patient underwent physical examination, laboratory tests (including CXCL13 by ELISA) and bilateral US. Synovial hypertrophy, tenosynovitis and erosions were evaluated according to a semiquantitative grading system with a 0-3 rating. US findings were correlated with serum levels of CXCL13, other serological parameters and disease activity index. Results Synovitis was found in 25/64 patients (39%) and tenosynovitis in 14/64 (22%). These findings were more frequent in SLE patients with Jaccoud's arthropathy, particularly tenosynovitis ( p = 0.002) and synovitis ( p = 0.01). Median serum level of CXCL13 was 20.16 pg/ml in the whole population (23.21 pg/ml in the Jaccoud's arthropathy group and 11.48 pg/ml in the group without). There was an association between the presence of disease activity and high level of CXCL13 ( p = 0.004). However, no association was found between high levels of CXCL13 and "arthritis" in SLEDAI, swollen joints on physical examination or synovitis on US. Conclusions US findings in joints of SLE patients with Jaccoud's arthropathy confirm that synovitis and tenosynovitis are common in these patients. In addition, serum level of CXCL13 is associated with disease activity in SLE but does not seem to be a biomarker for arthritis in these patients.

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BackgroundJoint involvement in systemic lupus erythematosus (SLE) is one of the earliest manifestation of the disease (1).Only 2–5% of the cases develop a deforming and non-erosive type of arthritis, known as Jaccoud Arthropathy (JA). Until now, there is no serum autoantibodies marker for it (2).The chemokine CXC ligand 13 protein (CXCL13) is one of the most potent B-cell chemo attractants and is constitutively expressed in the B-cell follicles of secondary lymphoid organs (3). Its seric level has been associated to the degree of synovitis in patients with rheumatoid arthritis as studied by ultrasonography (US).ObjectivesTo perform the first detailed US analysis of hands and wrists of SLE patients, with and without JA, and to correlate those findings with the levels of CXCL13,other clinical and laboratory features and disease activityMethods64 patients with SLE were included, being 32 with JA and 32 without JA paired by age and disease duration. The definition of JA was based on clinical criteria recently described by Santiago (4).Patients and controls underwent a high-resolution US exam of wrists and hands. Synovial hypertrophy,tenosynovitis and erosions were evaluated according a semi-quantitative grading system according definitions provided by OMERACT (5). Serum concentrations of CXCL13 were quantified in both groups utilizing a commercially available kit. Autoantibodies such as antinuclear antibody (ANA), anti-dsDNA,anti-Sm,anti-SSA, anti-SSB were also tested. US findings were correlated with seric levels of CXCL13, other serological parameters and SLEDAI score.ResultsIn the JA group, the mean age was 46.2 years and the mean duration of the disease was 17.3 years. Synovitis on US was found in 25 patients and tenosynovitis in 14. All of these findings were more frequent in SLE with JA, particularly tenosynovitis with difference statistically significant (p=0.002). In JA patients the median levels of CXCL13 was 23.21 pg/ml as compared to 11.48 pg/ml in SLE without JA group (p=0.08). There was an association between tenosynovitis and higher levels of CXCL13 in the JA group (p=0.026). Patients with active disease were more common in the JA group (p=0.004) and had increased serum levels of CXCL13 compared to patients with disease inactive (p=0.008).ConclusionsIn conclusion, the present study is one of a few to describe US findings in SLE patients with JA and it confirms that synovitis and tenosynovitis are common features in the majority of these patients. In addition, CXCL13 may be regarded as a biomarker for tendon inflammation in JA.References Messuti LZ, A. Joint Involvement in SLE: Controversy of RHUPUS. INTERNATIONAL TRENDS IN IMMUNITY. 2014;2(4):155–61.Galvao V, Atta AM, et al. Profile of autoantibodies in Jaccoud's arthropathy. Joint Bone Spine. 2009 Jul;76(4):356–60.Zheng B, Ozen Z, et al. CXCL13 neutralization reduces the severity of collagen-induced arthritis. Arthritis Rheum. 2005 Feb;52(2):620–6.Santiago MB. Jaccoud's arthropathy: proper classification criteria and treatment are still needed. Rheumato...

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CLINICAL IMAGE Gestational gigantomastia in systemic lupus erythematosus 885 infectious mastitis, she was administered cephalexin (2 g/d) for 10 days, without improvement.On physical examination, at 15 weeks of pregnancy, she had large breasts with a peau d'orange aspect (FIGurE 1), without nodules or nipple discharge, and a chest diameter of 133 cm. The rest of the physical examination was unremarkable. The levels of estradiol, luteinizing hormone, progesterone, prolactin, testosterone, thyrotropin, and T 4 were within the reference ranges. Breast ultrasound showed diffuse thickening of the skin and thickening of the subcutaneous tissue without evidence of organized collection. The patient underwent a deep biopsy of the breasts. Histopathological findings were pseudoangiomatous stromal hyperplasia, apocrine metaplasia, and A 33 -year -old woman was diagnosed with systemic lupus erythematosus (SLE) 9 years earlier, based on clinical findings of photosensitive malar rash, alopecia, arthritis, and the presence of autoantibodies, such as antinuclear antibodies with a titer of 1:160 and a speckled pattern, as well as anti -double stranded DNA, anti-ribonucleoprotein, and anti-Sjögren syndrome A antibodies.The patient was regularly using hydroxychloroquine (400 mg/d), with no signs of disease activity or other comorbidities. She became pregnant, and after 10 weeks, she noticed a rapid diffuse, bilateral, and symmetrical enlargement of her breasts, associated with local pain and hyperemia. Her previous pregnancy 10 years earlier was uneventful. With a presumptive diagnosis of