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Behçet's disease is a relapsing multisystem polysymptomatic disease with exacerbations and remissions defined by the presence of the major symptom, recurrent oral aphthous ulcers, plus two of the following: recurrent genital ulceration, eye lesions, skin lesions or a positive pathergy test. Mucocutaneous manifestations like oral and genital ulcers, and cutaneous lesions (papulopustular lesions, erythema nodosum-like lesions, cutaneous ulcers, superficial thrombophlebitis) are considered the "fingerprint" of the disease, being the most common and often the first signs to appear. Although the exact etiopathogenesis is still not known, genetic predisposition and environmental factors may influence and contribute to the development of this disease. Diagnosis is based on the International Study Group criteria. During the last years, this disease has been largely studied and new immunological data and treatment strategies have been postulated. Despite that, further studies and attention to new data are needed. [ABSTRACT FROM AUTHOR]

Introduction: Primary Sjogren syndrome (pSS) is considered a chronic inflammatory disease. The most common clinical feature of pSS is xerostomia and xerophthalmia, but rare complications can occur such as autoimmune cytopenias. Case Report: 50 old women with stablished pSS according ACR criteria: positive Anti-SSA/Ro and Schirmer's test < 5mm/5 minutes in both eyes presented to emergency ward with bruising, gingival hemorrhage and fatigue for the past week. She had a dubious medical history of allergy to prednisolone. The physical examination besides multiple bruising's was normal. Laboratory investigations revealed Haemoglobin 11.5 g/dL, white cell count 7200/L, platelet count 1000/L and Erythrocyte Sedimentation Rate 57mm/h. Previous blood analysis showed positive ANA (1:360 titer), and both SSA and SSB antibodies were strongly positive and C3/C4 were normal. Cryoglobulin screening was negative. Antibodies against double-stranded DNA (dsDNA), lupus anticoagulant (LA), anti-p2-glycoprotein-I and anticardiolipin antibodies were pending. She initiated treatment with IVIg 400mg/kg/day. Her platelet count responded, and within 72 hours, her platelet count had raised to 88 000 U/L. On day 4 she develops a thrombophlebitis in her right arm, and cephalic vein thrombosis in her left arm and starts empiric treatment with Vancomicine, cefalozine and enoxaparin. On day 5 she develops acute respiratory failure and hemoptysis. Her platelet count had dropped to 22 000 U/L and a Thorax CT-Scan showed "crazy paving pattern" suggestive of alveolar haemorrage. Intensive care with ventilation was required and she was treated with high-dose steroids. Platelet count normalized and our patient started azathioprine (AZA), after discharged from Intensive Care. Discussion: This clinical case presents with several challenges. Firstly, the emergence of commencing an effective treatment regarding the values of severe thrombocytopenia, on the other hand the therapeutic caution for the presumed allergy to corticosteroids. Immunosuppressive drugs, such as methotrexate (MTX), AZA, rituximab (RTX) and cyclophosphamide (CYP), have all been used with wavering degrees of success in cytopenias. With corticosteroids seeming to be the mainstay of treatment (1). Based on data available from RTX efficacy in autoimmune cytopenias, we can extrapolate that it may be useful in the treatment of cytopenias associated with pSS. Several weeks are expected before response, however the urgency of the case would not allow it (2). CYP, MTX and AZA have been used in cases of thrombocytopenia associated with pSS, however, CYP due to its adverse effects and MTX and AZA, due to its late response, were precluded (3). IVIG has been tried in agranulocytosis complicating pSS and is a first line treatment to immune thrombocytopenia. An increase in platelet count is typically expected within 24 to 48 hours (4,5). This was the treatment of choice. However, the onset of tromboflebites and thrombosis, poses the doubt of antiphospholipid antibody syndrome as antibodies results were still pending. The decision to start enoxaparin at platelet safe levels is made. Another complication yet occurred, alveolar haemorrage, with the patient ventilated the decision for high dose corticosteroids is finally made. Antibodies against dsDNA, LA, anti-β2-glycoprotein-I, anticardiolipin and heparin antibodies came out negative. Before discharge AZA was used as a corticosteroid sparing, due to its relative safety profile. The patient has had no further relapses for 2 years now. [ABSTRACT FROM AUTHOR]

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Introduction: Dermatomyositis (DM) is an idiopathic inflammatory myopathy, characterized by features of proximal skeletal muscle weakness, evidence of muscle inflammation and characteristic skin manifestations. Caucasian male 18 years, previously healthy, was referred to our department due to a history with 5 months of evolution of proximal muscular weakness (especially in upper limbs). The patient reported fatigue, difficulty climbing stairs and difficulty swallowing. Simultaneously mentioned height loss (10kg in 3 months), and photosensitive erythema over face, neck, dorsum of metacarpalphalangeal (MCF) and proximal interphalangeal (PIP) joints. In the physical examination it stands out periorbital heliotrope rash and Gottron's papules over the dorsal PIP and MCP. In the neurological examination, muscle strength was 3/5 proximal at upper limbs, Growers sign was present. No arthritis was identified. Blood analysis exhibited a discrete elevation of creatine phosphokinase: 181 U/L, aldolase: 7.8 U/L (Normal< 7,6). The inflammatory markers, antinuclear antibody, myositis-specific, and myositis-associated autoantibody were negative. Electromyography presented lesion of muscular fibers. The diagnosis of DM was made, and the patient started 60mg/day of prednisolone. A muscular biopsy confirmed the diagnosis of DM (muscle with lesions of inflammatory myopathy). During the following period, the patient showed clinical improvement (improvement of muscle strength was 5/5 proximal at upper limbs, Growers sign was negative, allowing reduction of dosage of prednisolone. After 4 months of treatment, the patient presented bilateral hearing loss. An MRI was made which revealed «presence of an expansive formation centered on the posterior wall of the oropharynx, obliterating the nasopharynx, exerting a mass effect on the adjacent structures». A biopsy of the lesion was executed by otorhinolaryngologist, and histology revealed EBV-associated non-keratinizing epidermoid carcinoma whose staging was T2N2M0. The patient started radiotherapy with chemotherapy with cisplatin 100mg/m2, remaining in treatment with clinical improvement. Conclusion: The association between malignancy and inflammatory myopathy has been supported by numerous epidemiologic studies, with the strongest association occurring in those with DM2. The clinical implications of this association, irrespective of the pathophysiologic mechanisms involved, are that it is imperative to screen for tumors in patients with DM, particularly if the symptoms do not respond to conventional immunosuppressive treatment. The types of malignancies vary and include not only hematologic malignancies such as lymphoma but also solid tumors such3. Ethnic backgrounds should be considered in screening, because cancer types vary according to race, with lung and nasopharyngeal cancers being more frequent in Asians than Caucasians. The authors highlight this case due to the rarity of the association between DM and nasopharyngeal cancer especially in a young Caucasian patient. [ABSTRACT FROM AUTHOR]

Background: Paget's disease is a chronic progressive metabolic bone disorder that can affect one or multiple sites, but with a predilection for the axial skeleton. The majority of patients with Paget disease of bone are asymptomatic and the diagnosis is usually made incidentally. The prevalence of the disease in Portugal is unknown. Objectives: To report and describe the presentation and clinical characteristics of a rarely diagnosed rheumatic disease. Methods: Retrospective study of patients with a diagnosis of Paget's disease with follow up in the rheumatology department in a tertiary center. The demographic data, clinical features, investigation results and treatment of the cases were analyzed from medical records. Results: We identified 7 patients with Paget's disease of the bone (3 women and 4 men; mean diagnosis age 59.1±7.0 years). Four patients had bone pain at the affected site and three had unspecific arthralgias and presented as an incidental finding during the diagnostic investigation. All but one had raised serum alkaline phosphatase level with normal serum calcium levels. The majority of the patients (71.4%, n=5) had a monostotic disease and the most commonly involved site was the pelvis. All of them had hypercaption of the radiopharmaceutical agent at bone scintigraphy and alterations that were suggestive of pagetic bones at the radiographs. All of them were treated with bisphosphonates (intravenous pamidronate (n=2), oral alendronate (n= 3), oral risedronate (n=1), intravenous zoledronate (n=1)). The two patients treated with one administration of intravenous pamidronate were then treated with zoledronate or alendronate. After one year of treatment, the serum alkaline phosphatase levels have decreased in all patients. None of the patients had related complications (musculoskeletal, neurologic, and cardiovascular problems). Conclusion: The patients included are mainly men with bone pain at the site involved. The majority was a monostotic disease. All of them were treated with bisphosphonates with good clinical results. [ABSTRACT FROM AUTHOR]

Introduction: Over the past decades, Early Arthritis Clinics (EAC) have been created to identify early arthritis and institute appropriate treatment as soon as possible. In Rheumatoid Arthritis (RA) many studies show that ultrasonography (US) is superior to clinical exam for the detection of synovitis and has good correlation with clinical findings and markers of inflammation and can be used to improve the certainty of a diagnosis of RA. [1] However, few studies address the agreement between the US with the clinical examination in patients with early arthritis. Objective: To evaluate the agreement between clinical examination and US findings of metacarpophalangeal and proximal interphalangeal joints of patients with early arthritis Methods: Patients from the EAC of our department with suspect arthralgia were included. Patients were submitted to clinical evaluation by a rheumatologist to identify tender and swollen joints. They were then submitted to an US examination of metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints, by an experienced sonographer oblivious of the previous examination. Each joint was scored for the presence of synovial hypertrophy (SH) and Power Doppler (PD) signal. Based on OMERACT guidance, we defined synovitis as: ≥ grade 1 grey scale synovitis (hypoechoic SH regardless of the presence of effusion) and £ grade 1 power-Doppler. The diagnostic value of clinical evaluation was assessed through sensitivity, specificity, Negative predictive value (NPV) and Positive predictive value (PPV), assuming the US synovitis as gold standard. Clinical arthritis was defined by joint swelling. Cohen's kappa coefficient was used to analyse concordance between joint swelling appreciated by clinical exam and HS, PD and the presence of US synovitis. Kappa values < 0 were considered poor, 0-0.20 slight, 0.21-0.40 fair, 0.41-0.60 moderate, 0.61-0.80 good and 0.81-1.00 excellent. [2] Statistical significance was defined as p<0.05. Statistical analysis was performed using IBM SPSS Statistics, version 21.0. Results: 77 consecutive patients were included (53.2% female) with a mean age of 53.8±19.1 years. We evaluated 770 MCP and 770 PIP joints. The sensitivity and specificity of clinical examination in relation to US synovitis was respectively 71% and 60% for MCP and 54.5% and 43.9% for PIP. The NPV and PPV for MCP were 87.8% and 33.3% respectively, and for PIP were 85.3% and 13.9%. The level of agreement between joint swelling and HS, PD and the presence of synovitis is show on Table I. Conclusion: The clinical evaluation of MCP showed a better performance than clinical evaluation of IFP. The high NPV of clinical examination makes its suitable to be used to rule out MCP and PIP involvement in patients with early arthritis. The performance of the two assessment strategies on the same day may increase agreement and diagnostic certainty. [ABSTRACT FROM AUTHOR]

Introduction: Ultrasonography is an imaging technique that allows rheumatologists to visualise structural and inflammatory changes within a joint. The objective of this study was to assess the inter-reader reli bility of interpretation of inflammatory and destructive changes in a wide range of joints in patients with polyarthritis. Methods: This study was divided in two parts: 1) consensus process and 2) reliability exercise. For the first part, a written questionnaire was sent by email to 6 sonographers from 3 portuguese hospitals with the highest level of competence (EULAR competency assessment level 2). The questionnaire included 17 questions divided in two groups: 1) elementary components in Bmode and Doppler assessment (effusion, synovial hypertrophy (SH), power Doppler (PD), erosions and synovitis definition) and 2) approach at the joint level (the definition of which plan and recess will be assessed in each joint). The participants were asked to rate their level of agreement/disagreement for each statement using a 1-5 Likert scale (1=strongly disagree to 5-strongly agree). For the reliability exercise, video clips of US examinations of 40 joints (wrist, metacarpophalangeal (MCP) from 1 to 5, proximal interphalangeal (PIP) from 1 to 5, knee, tibiotarsal (TT) and metatarsophalangeal (MTP) joints from 1 to 5, elbow and shoulder) from each of 15 patients were collected (showing a multiplanar bilateral ultrasound approach). Each joint in each video was scored by individual ultrasonographers for the presence/absence of elementary components: effusion (Yes/No), SH (No/Grade 1 to 3), PD (No/Grade 1 to 3) and erosions (Yes/No). Inter-reader agreement analysis was assessed through Fleiss' kappa coefficient and classified according to Landis and Koch[8]: κ values < 0 were considered poor, 0-0.20 slight, 0.21-0.40 fair, 0.41-0.60 moderate, 0.61-0.80 good and 0.81-1.00 excellent. Statistical significance was defined as p<0.05. Statistical analysis was performed using STATA V.14. Results: Thirty-seven joints of the 600 joints were excluded due to dislocation of the joint or presence of objects (rings/catheters) and the videos of a total of 563 joints were analysed by the 6 ultrasound experts. Interreader agreement was superior for TT joints and inferior for wrist; the identification of the erosions had the better agreement in the elementary components (Table I). Conclusions: The reliability of interpretation of inflammatory and destructive changes using video clips was in general good to excellent and it was better for erosions and tibiotarsal joint (regarding elementary component and anatomical region, respectively). [ABSTRACT FROM AUTHOR]

Background: biosimilars of biotechnological agents represent an important opportunity to increase accessibility to these medications. Clinicians still maintain reservations regarding the similarity of their efficacy and safety in practice. Aim: to evaluate the clinical consequences of a nonmedical switch of etanercept (ETN) original to biosimi - lar in a clinical practice setting. Methods: the study included all patients aged 18+ treated in a Tertiary Rheumatology Department with original ETN who were switched to his biosimilar following a decison by the hospital administration, accepted by rheumatologists. Patients were informed of the switch prior to its occurrence, in simple terms underlining the similarity in terms of regulatory issues and scientific evidence. Disease activity, adverse events and adaptation to the drug delivery system were eva - luated at each visit. Disease activity at baseline (time of switch), 3 and 6 months after was compared using Paired samples T-test or Wilcoxon test as adequate. A p£0.05 was considered statistically significant. Continuous variables are presented as means and categori - cal variables as proportions. Results: from 98 patients treated with original ETN in our department, 89 were switched to his biosimilar. The remaining ones maintained the treatment with the reference biological product for several reasons. Eight patients were excluded from this analysis due to poor adherence to treatment (n=4) and early interruption of treatment [n=4: due to surgery (n=1), respiratory infection (n=1), suspected allergic reaction to biosimilar (n=1) and own initiative (n=1)]. Of the remaining 81 patients (58% female, mean age 56.2±12.1 years), 38.3% had RA, 40.7% SpA, 18.5% PsA and 2.5% JIA. Disease activity was stable over the follow up in patients with RA, PsA and SpA as no statistically significant differences were observed in acute phase reactants, patient or physician global assessment between the three time points. Minor adverse events were reported by 2 patients (pain and local cutaneous reaction), 2 reported sense of disease exacerbation in the first three months that was not confirmed by clinical and analytical evaluation and 2 patients reported minor infections. Good adaptation to the drug delivery instrument was reported by 93.8% of patients (n=76). Conclusion: the switch from ETN to his biosimilar in this group of patients followed in routine care did not affect the overall efficacy and safety of treatment. We did not observe a significant impact of nocebo effect. [ABSTRACT FROM AUTHOR]

Ibandronate is considered to be a safe drug, and the few reported side-effects in the digestive system are usually related to the upper gastrointestinal tract. Large bowel lesions associated to ibandronate use in humans have not been reported. We describe the case of a 52-year-old female with two episodes of lower abdominal pain after the intake of oral ibandronate. The second pain episode was followed by hematochezia with endoscopic and histological evidence of sigmoid ischemia. No other possible causes of colonic ischemia were found. After a short hospital admission, there was a complete clinical, endoscopic and histological recovery. Ibandronate was withdrawn and the patient reported no further gastrointestinal complaints. This is the first reported case of colon bleeding related to the use of Ibandronate. [ABSTRACT FROM AUTHOR]

Introduction: Recognition of advanced Rheumatoid Arthritis (RA) is usually facilitated by the presence of chronic deformities such as ulnar deviation, metacarpals radial shift and interosseous atrophy. Although being considered typical, these deformities are not pathognomonic of RA and other conditions must be ruled out, especially when the clinical picture is not clear. The authors present 2 clinical cases of RA-like hand deformities. Case 1: A 73-year-old female was referred from the Neurology Department because of suspicion of RA. The patient complained of polyarthralgias involving shoulders, elbows, wrists, PIP, hips, and knees. She also complained of mechanical low back pain and muscle stiffness lasting all day long. She had been diagnosed with Parkinson Disease (PD) 25 years ago and was being treated with ropinirole 8 mg per day and carbidopa/levodopa 25/250mg (6 times per day). Rheumatologic examination revealed the presence of Heberden's and Bouchard's nodes, interosseous atrophy, metacarpals radial shift, reducible ulnar deviation, and kyphosis but no signs of arthritis were present. Rheumatoid factor (RF), anti-citrullinated protein antibody (ACPA) and acute phase markers were negative. Spine radiographs showed old fractures of dorsal and lumbar spine. Proximal interphalangeal (PIP) and distal interphalangeal joint space narrowing with subchondral sclerosis and osteophytosis were seen on hands radiographs. Diagnosis of multifocal osteoarthritis and osteoporosis were made and the patient was started on pain relievers, vitamin D, calcium supplementation and bisphosphonate. The patient was kept on regular follow up, but arthritis was never observed. Case 2: A 73-year-old female presented with a 10-year history of mechanical polyarthralgias (affecting shoulders, elbows, wrists, PIP, hips, knees, and feet) with 30 minutes of morning stiffness. She had an 11-year history of PD, under carbidopa/levodopa 25/100mg (3 times per day), and osteoporosis (with several lumbar osteoporotic fractures). On rheumatologic examination, stood out a reducible ulnar deviation of the left hand and a marked kyphosis. There were no clinical signs of arthritis. RF and ACPA were negative and acute phase markers were normal. Radiographs of the hands and feet showed radiographic generalized osteopenia, without joint space narrowing, erosions or any other typical feature of RA. The patient was diagnosed with osteoporosis and treatment with pain relievers, bisphosphonate and calcium and vitamin D supplementation was started. The patient was kept on regular follow-up, once again, without any signs of arthritis. Conclusion: Both patients showed chronic hand deformities suggestive of advanced RA. However, no clinical evidence of the disease was found in neither case. Interestingly both patients have been suffering from long-standing PD, with osteoporosis. The deformities observed in both these patients hands are named striatal hands and have been reported in 10% of patients with untreated, advanced PD1. Unlike RA, local signs of joint involvement, such as pain, tenderness, heat and swelling are absent in striatal hands. Also, RA tends to occur bilaterally and deformaties are not reducible, whereas focal dystonia before PD tends to be unilateral, with tremor, rigidity, and bradykinesia developing on the ipsilateral side. [ABSTRACT FROM AUTHOR]

Background: patients with rheumatic diseases are at a higher risk for infections associated to the underlying disease and immunosuppressive therapy. This fact leads to an increased morbidity and/or mortality. Effective vaccination is essential for the prevention of a significant number of these infections, namely influenza and pneumococcal vaccination. Vaccination rates in this subgroup of patients are relatively low and given the increased risk of infections, vaccination seems to be important for patients with rheumatic diseases. Objective: to characterize the vaccination status in a cohort of portuguese rheumatic disease patients under immunosuppressive treatment. Methods: cross-sectional study in a tertiary rheumatology department. Convenience sampling was used for data collection from all 1086 patients aged3 18 years registered in rheuma.pt with at least one appointment in the last year. We apply a questionnaire (on the rheumatology appointment or by phone) between january and december 2018 asking about influenza and pneumococcal vaccination status, reasons for unvaccinated status, who suggest vaccination and associated complications to the procedure. Influenza vaccination was considered completed if the patient was vaccina ted in the last year and pneumococcal vaccination was considered completed according to the national recommendations. Categorical variables were described using absolute and relative frequencies and continuous data using mean and standard deviation. Statistical significance was defined as p<0.05. Statistical analysis was performed using IBM SPSS Statistics, version 21.0. Results: we include 432 patients (75.2% female, mean age 57.9±13.9 years); 60.2% (n=260) had RA, 15.3% (n=66) SpA, 13% (n=56) SLE, 7.6% (n=33) PsA, 1.4% (n=6) JIA and 2.5% (n=11) vasculitis. The majority of the patients was treated with at least one cDMARD at any point of the disease (83.8%, n=362, missing n=24) being 85,4% (n=309) in the last year and 46.8% (n=202, missing n=14) with biologic treatment. Vaccination rate and the reasons for non-vaccination status are shown in Table I. Two patients reported flu episodes after influenza vaccination. A significant association was found between biologic treatment and influenza and pneumococcal vaccination rate (p£0.002) but no with cDMARDs and between age and influenza vaccination but no with pneumococcal vaccination (higher influenza vaccination rate in the patients < 65 years; p=0.002). Vaccines were mostly prescribed by rheumatologists (41,9%) and general practitioner (18,8%). Conclusion: in our cohort, and despite current recommendations, vaccination rates are still low among patients with autoimune diseases. Comparing the three vaccines, vaccination rate was higher for influenza vaccine and the main reason stated by unvaccinated patients was their own belief that vaccination was useless. A greater effort is required to improve these results. [ABSTRACT FROM AUTHOR]