BackgroundMulticentric Castleman's disease (MCD) is a disorder characterized by polyclonal proliferation of B lymphocytes that is frequently associated with autoimmune manifestations and connective tissue diseases. MCD presents high levels of IL-6 and systemic symptoms such as fever, arthralgia, hepatosplenomegaly and serositis, so it is recommended to include MCD in the differential diagnosis of adult-onset Still's disease (AOSD). However, there are no studies comparing both groups of patients.ObjectivesTo compare the clinical and laboratory features at onset between patients with MCD and AOSD seen in a Madrid tertiary care hospital.MethodsWe performed a retrospective observational study in patients with diagnosis MCD and AOSD attended our center between January 1989 and December 2015. The variables included demographics, clinical manifestations, laboratory tests and Yamaguchi's criteria.ResultsA total of 34 patients were included, 17 with MCD and 17 with AOSD. The comparison of the characteristics of both groups is presented in the table. There were no differences in age, uration of disease (MCD 158,6 days and AOSD 250,5 days, p=0,3919), diagnostic delay (MCD 18,4 and AOSD 52,2, p=0,2711), arthritis, myalagias, pleuritis or macrophagic activation syndrome, but persistent fever, rash, arthralgia, pharyngitis and pericarditis were significantly more frequent in ESA, whereas male gender, human immunodeficiency virus (HIV) and/or human herpes virus 8 (HHV8) infection, hepatosplenomegaly and lymphadenopathy were significantly higher in MCD. In the laboratory test results, leukocytosis and hypertransaminasemia were significantly higher in AOSD. 52.9% of patients with MCD met 5 or more Yamaguchi's criteria for AOSD.Multicentric Castleman's disease (n=17)Adult-onset Still's disease (n=17)p-value Male:female ratio2.4:10.4:10.0163Age at diagnosis (mean ± SD)46.4±13.137.5±13.50.0598HIV infection35.2%0%0.0154HHV8 infection35.2%0%0.0154Persistent fever47.1%94.1%0.0078Rash29.4%64.7%0.0392Arthralgias35,3%88.2%0.0014Arthritis0%17.6%0.0696Pharyngitis0%41.2%0.0029Hepatosplenomegaly47.1%5.9%0.0065Pericarditis0%23.5%0.0332Pleuritis17.6%11.7%0.6217Lymphadenopathy100%17.6%0.0001Macrophage activation syndrome11.7%17.6%0.6282Leucocytes >10000/mm335.2%76.5%0.0156Increased transaminases0%58.8%0.0002Erythrosedimentation rate (mm/Hg)5277.50.1104C-reactive protein (md/dl)75.1109.50.3699Ferritin (ng/ml)24019260.1234ConclusionsPatients with MCD may present common systemic manifestations and laboratory abnormalities at onset with AOSD and up to 50% of them may fulfill Yamaguchi's criteria for this disease, so MCD should be taken into account in its differential diagnosis.Disclosure of InterestNone declared

BackgroundThymoma is the most common neoplasm originated from the thymus gland and accounting for 50% of anterior mediastinal tumors. Within its clinical manifestations are included the loss of self-tolerance and the development of autoimmunity.ObjectivesTo study the frequency of autoimmune diseases (AD) in patients with thymoma and to describe their clinical characteristics and outcome.MethodsWe performed a retrospective observational study of a cohort of patients diagnosed with thymoma and followed-up in our center between January 1985 and September 2016. The variables evaluated included demographics, thymoma characteristics, clinical and analytical manifestations of autoimmunity, treatment and outcome.ResultsA total of 83 patients were included, 56.6% of them women, with a mean age at diagnosis of the thymoma of 58.4±15.8 years (range: 16–94), 31.3% of which corresponded to type I The classification of Masaoka, 39.1% to II, 17.2% to III and 10.9% to IV. There were one or more AD associated in 41 cases (49.4%). The most frequent diagnoses were myasthenia gravis (19), systemic lupus erythematosus (SLE) (4), subacute cutaneous lupus erythematosus (1), Sjögren's syndrome (1), rheumatoid arthritis (1), spondyloarthritis (1), sarcoidosis (1), hemolytic anemia (2), pernicious anemia (1), aplastic anemia (1), cutaneous limited systemic sclerosis (1), urticaria-vasculitis, erythroblastopenia (1), recurrent pericarditis (1), thyroid disease (2) and lichen planus (1). The diagnosis of AD preceded to thymoma in 38.2% of cases and was later in the remaining cases. In 4 cases there was also a concomitant primary immunodeficiency (variable common immunodeficiency 3, CD4 immunodeficiency 1). The most frequently identified autoantibodies were anti-acetylcholine receptor (14/41, 34.1%), anti-striated muscle (3/41, 7.3%), ANA (11/41, 26.8%), (3/41, 7.3%), rheumatoid factor (3/41, 7.3%), anti-thyroid (3/41, 7.3%), antiphospholipids (2/41, 9%) and anticentromere (1/41, 2.4%). In the comparison of patients with and without associated AD, no significant differences were found regarding age, sex or Masaoka classification. There were 6 deaths, 4 in group with associated AD and 2 in the group without AD, but without significant difference (p=0.3797).ConclusionsIn the analyzed population of patients with thymoma of our center, almost half of them developed AD, which in a major group preceded the diagnosis of neoplasia. The spectrum of autoimmunity associated with thymoma was quite broad, including organ-specific AD such as myasthenia gravis (which is most frequently described in the literature) and autoimmune cytopenias, but also to systemic AD, the most common being SLE. The autoimmunity study should be included in the assessment of the patient with thymoma as it could contribute to the early diagnosis of associated AD.Disclosure of InterestNone declared

BackgroundJaccoud arthropathy (JA) is a chronic non-erosive reversible joint disorder commonly associated with systemic lupus erythematosus (SLE), and occurs in roughly 5% of all cases. Some studies suggest different profiles of clinical and immunological features between SLE patients with or without JA.ObjectivesTo compare the profile of clinical and serological manifestations of patients with SLE with and without JA followed in a tertiary care hospital of Madrid.MethodsWe performed a retrospective observational study of a cohort of patients diagnosed with SLE (4 or more ACR criteria) between June 1977 and December 2015. The variables evaluated included demographics, clinical, analytical and radiological manifestations. The definition of JA was based on the presence of clinical criteria (reversible joint deformities) and absence of radiographic erosions typical of rheumatoid arthritis. Statistical analysis was performed using SPSS software version 21.0.ResultsWe included 108 patients representing a sample of 24% of the total number of patients with SLE treated at our center during that period. The majority of patients were women (89.8%), mean age at diagnosis was 30±12.29 years (range: 7–75) and duration of disease was 127 months (range: 2–411). Thirteen patients (12.03%) had findings compatible with JA. There were no significant differences in age, sex or race, but the duration of disease was higher in JA patients (190 vs. 118.2 months, p=0.0299). There were significant differences in the presence of malar rash (p=0.0009), photosensitivity (p=0.0050), oral ulcers (p=0.0032) and pericarditis (p=0.000001), which were more frequent in patients without JA, but arthritis, nephritis, pleuritis, seizures, psychosis, Raynaud's phenomenon and antiphospholipid syndrome had a similar distribution between both groups. Among the immunological features, no significant difference was found in relation to hemolytic anemia, lymphopenia, thrombocytopenia, ANA, anti-ENA, anti-DNA, anticardiolipin, anti-β2 glycoprotein I and lupus anticoagulant, but leukopenia was also more frequent in patients without JA (p=0.0041).ConclusionsIn the analyzed sample of patients in our center JA was a relatively frequent finding and was associated with a longer duration of the disease. It was not possible to corroborate other JA associations suggested in previous studies such as a lower frequency of lupus nephritis or major secondary antiphospholipid syndrome, probably due to limited sample size, but there are also other studies that do not demonstrate significant differences in relation to clinical and serological findings in patients with SLE with JA with respect to those who do not present it. Studies involving a larger number of patients with JA associated with SLE are required in order to better identify these differences, so a multicenter initiative could be of great help.Disclosure of InterestNone declared

virus infection was present in 29%. 25% were treated with tenofovir plus protease inhibitors, and 47% with tenofovir plus a non-nucleoside reverse transcriptase inhibitor. The mean value of BMD in lumbar spine (LS) was 0.93 g/cm 2 (range: 0.84-1.02) and in femoral neck (FN) 0.78 g/cm 2 (range: 0.69-0.86). For the comparison with the ESOSVAL cohort the worst value of T-score in either LS or FN was chosen and patients were classified according to WHO definitions (osteoporosis ≤-2.5, osteopenia-1 to-2.5); the results are presented in the table. Only the data for the 50-64 and 65-74 years groups were compared because the number of older HIV patients in our center was small. Significant differences were found between the categories of osteoporosis in men in the 65-74 years old group, and that of osteopenia in women in the 55-64 years old group.

BackgroundAromatase inhibitors (AI) been related to an increased risk of bone loss and fractures in women receiving these drugs as adjuvant treatment, but few studies have assessed the impact of prior chemotherapy (CT) on bone mineral density (BMD) loss associated to AI.ObjectivesTo assess the impact of CT prior to the initiation of AI on BMD in postmenopausal patients with breast cancer (BC) seen at a Spanish tertiary care hospital.MethodsWe perform a longitudinal study in patients who received AI after initial CT (CT group) or as adjuvant therapy without prior CT (non-CT group) followed up for 12 months. BMD was assessed by DXA in lumbar spine (LS) and femoral neck (FN) at baseline and after 12 months of AI treatment following the usual protocol of our center, with in vitro coefficient of variation of 1% in both locations and estimated minimal significant change (MSC) of 0.0223 g/cm2 in LS and 0.0288 g/cm2 in FN. Demographics, neoplastic disease data, and osteoporosis risk factors were also collected.Results69 patients (CT group 39, non-CT group 30) attended at our center between August 2011 and December 2014 were included. Mean age at diagnosis was 59.9±7.7 years, most of them have BC stages I-II (84%). Most frequent AI in both groups was letrozole (95%). Baseline characteristics were similar, except for age at diagnosis that was significantly higher in the non-CT group, these data are presented in the table. Mean BMD at the start of AI was significantly lower in LS in the CT group (0.7793 g/cm2) than in the non-CT group (0.8483 g/cm2) (p=0.018), but no difference in FN (CT 0.6764 g/cm2 and non-CT 0.7077 g/cm2, p=0.123). A significant difference in LS (CT 0.7685 g/cm2, non-CT 0.8397 g/cm2, p=0.003) was found in the comparison of BMD means between the two groups at 12 months but not in FN (CT 0.6598 g/cm2, non-CT 0.6689 g/cm2, p=0.369). After 12 months of treatment with AI, mean BMD change in the CT group in LS was -0.0107 g/cm2 (95% confidence interval [CI] -0.0269, +0.0055, p=0.189) and in FN -0.0165 g/cm2 (95% CI: -0.0339, +0.0009, p=0.063), while in the non-CT group the means changes were in LS -0.0085 g/cm2 (95% CI -0.0416, +0.0244, p=0.599) and FN -0.0388 g/cm2 (95% CI -0.0707, -0.0068, p=0.019). During the study period there was a fracture in each group (CT 2.6%, non-CT 3.3%).CT group (n=39)Non-CT group (n=30)p-value Age at diagnosis, years (mean ± SD)58.2±7.362±7.90.042*Age of menarche, years (mean ± SD)12.5±1.212.5±1.10.980Age of menopause, years (mean ± SD)48±4.248.9±4.10.394Age >65 years13 (33%)9 (30%)0.284Body mass index (mean ± SD)25.8±627.6±4.30.184Osteopenia/osteoporosis before AI35 (89%)26 (86%)0.692Smoking5 (13%)2 (6%)0.401Previous fracture2 (5%)2 (7%)0.786Family history of fracture2 (5%)2 (7%)0.786Calcium + Vitamin D Supplements26 (67%)14 (47%)0.095Radiotherapy27 (69%)23 (77%)0.493Prior tamoxifen5 (13%)3 (10%)0.717Bisphosphonates2 (5%)1 (3%)0,717ConclusionsOur results do not demonstrate that CT prior to AI treatment significantly decreased BMD during the first year. Mean change in both LS and ...