P051 Juvenile Behcet's disease

Background Behçet's disease is a rare systemic vasculitis in children. The most frequent manifestations are orogenitalaphthosis and uveitis. Neurological, digestive, vascular, joint, and skin involvement are possible. We aimed to describe the clinical, diagnostic criteria, and therapeutic features of pediatric-onset Behcet's disease Methods Study carried out based on files collected over 12 years (2007–2018) concerning patients under the age of 15 hospitalized in the pediatric department of CHU de Blida for Behcet disease Results For 12 years (2007–2018), we hospitalized five children for various reasons and whose diagnosis of Behcet's disease was retained. These are 03 boys (2 of the theme are brothers) and 02 girls. The average age at admission was 13.5 years (12–15 years), and the average duration of follow-up is 4.2 years (15 months-8 years). The mean age of onset of first symptoms was 11 years, and the meantime to diagnosis was 2 years (12 months-3 years) The main diagnostic criteria were: mouth ulcers in five patients (100%), genital ulcers in three patients (60%), ophthalmic manifestations in two patients (40%): one had bilateral uveitis, and the other had papillitis, the pathergy test was positive in 3 patients (60%). The other manifestations were as follows: polyarthralgia in 3 patients (60%), family history in one patient (20%). Neurological manifestations were present in 3 patients (60%): one child presented two episodes of aseptic meningitis and thrombosis deep cerebral venous; one child with diffuse cerebral demyelination lesions, and the third with HIC syndrome and massive lateral sinus thrombosis. Three children have a vascular involvement (60%): pulmonary embolism with thrombosis of the superior vena cava (20%), bilateral thrombosis of the jugular veins (20%), and thrombosis of the right ventricle complicated by pulmonary embolism (20%) Initial symptoms included recurrent aphthous stomatitis (100%), arthralgia (60%), prolonged fever (20%), behavioral disturbances and dysarthria (20%), weight loss (40%), and headache (60 %). The histocompatibility antigen (HLAB 51) was found in 2 children (40%). The treatment included corticosteroids for all patients (3 boluses of 1 g/m2/day of methylprednisolone then relayed by prednisone 1 mg/kg/day), azathioprine 2.5 mg/kg/day in 5 children (100%), and colchicine for 2 patients only one patient had received anticoagulant therapy (LMWH followed by VKA) Relapses were observed in a single patient (20%) and mortality in one patient (20%.) Conclusion Behcet's disease is a rare disease whose diagnosis remains difficult in children. However, early diagnosis and aggressive treatment are mandatory to avoid complications and functional sequelae.

Clinical Characteristics of Behçet Disease in an Egyptian Cohort

Aim of the work To study the clinical characteristics of Behcet’s disease (BD) in Egyptian patients. Patients and methods 150 BD patients were recruited from the Rheumatology and Internal medicine Department, Ain Shams University Hospitals. Demographic and clinical data were collected. Disease activity was assessed using Behcet Disease Current Activity Form (BDCAF). Results 150 BD patients were included in this study. Their ages were 34.05 ± 9.28 years (14 – 66 years) and with disease duration range (1–20 years). There were 116 males (77.3%)and 34 females (22.7%), with male to female ratio of 3.4:1. The BDCAF was 4.44 ± 1.97 (2–10). The frequency of the clinical manifestations was oral ulcers in 86.7%, followed by genital ulcers in 82.0%, ocular involvement in 68.7%, vascular affection in 58.7%, articular manifestations in 32.7%, pathergy test was positive in 28.0%, skin manifestations in 26.0% (pustular lesions, folicilitis,erythema nodosum and pyoderma gangrenosum), neurological in 25.3% in the form of (CNS infarction in 12.7%,vasculitis in 6.7%, Cerebral sinus thrombosis in 4.0% and Seizures in 2.0%) and gastrointestinal in 7.3% in the form of (nausea, vomiting and abdominal pain). Conclusion In Egypt, there is a male predominance in BD patients as other areas in worldwide countries. The clinical characteristics are comparable to other studies. However, in Egypt the gastrointestinal manifestations showed the lowest recorded frequency

Diagnosing neuro-Behçet’s disease

Behçet’s disease is a rare systemic vasculitis characterized by uveitis, recurrent oral and genital ulcers, firstly described by the Turkish dermatologist Hulusi Behçet. The etiology is unknown, although autoimmune mechanisms are described. There is no specific test for the diagnosis of Behçet’s disease. The International Criteria for Behçet’s Disease (ICBD) proposed a new set of diagnostic criteria including oral and genital aphthosis, skin, ocular and vascular manifestations, CNS involvement and positive pathergy test. The neurologic involvement could be classified in parenchymal neuro-Behçet’s and non-parenchymal neuro-Behçet’s disease. We report a case of a woman with a very impressive personal history. Over time, she had many specific neurological complications compatible with neuro-Behçet’s disease (NBD). The other general symptoms also suggested Behçet’s disease, according to ICBD.

Rheumatologic manifestations in a cohort of patients with Vogt–Koyanagi–Harada disease

ABSTRACT Objectives Vogt–Koyanagi–Harada Disease (VKHD) is a systemic autoimmune disorder characterized by granulomatous panuveitis. Inflammatory rheumatic diseases (IRDs) are among the differential diagnosis of VKHD. However, current knowledge on the rheumatological aspects of VKHD is still limited. We aimed to investigate the prevalence of rheumatic conditions in VKHD patients. Methods VKHD patients were included in the study and they were reviewed in terms of the presence of any rheumatological manifestations. Results There were 18 patients with a female preponderance (83.3%, female). Inflammatory type of peripheral joint pain (11%) and sicca symptoms (33%) were the most common rheumatological findings. The frequency of spondyloarthritis-related features such as inflammatory back pain and HLA-B27 rate was not increased. None of the patients had radiographic sacroiliitis. Anti-nuclear antibody was positive in high titres nearly in 30% of the patients and three patients had antibodies against extractable nuclear antigens. Nailfold capillaroscopy was abnormal in about one-third of the patients. Pathergy test was negative in all cohorts. While angiotensin-converting enzyme was elevated in nearly 20% of the patients, there were no abnormalities on chest X-rays. Conclusion VKHD shares some features with IRDs. The common features were mostly suggestive of connective tissue disease rather than SpA or rheumatoid arthritis.

PSEUDOTUMORAL PRESENTATION OF NEURO-BEHÇET DISEASE: A CONFUSING DIAGNOSIS

A 35-year-old man was referred to the internal medicine department for acute hemiplegia with oral aphthous ulcers. The patient reported recurrent oral and genital ulcers more than 3 times a year. He also experienced one episode of peripheral thrombosis 1 year ago. The physical examination showed left hemiparesis with positive Babinski sign, hyperreflexia, and pseudofolliculitis in legs and trunk. The pathergy test was positive. Cerebral MRI showed a large mass hyperintense in T2 sequence in the regions of basal ganglia and corpus callosum with peripheral contrast enhancement and intensive edema around the lesion. The diagnosis of pseudotumoral neuro-Behçet disease was made on the basis of clinical history, the cerebral MRI result, and the exclusion of other tumoral and infectious diseases. The patient was treated with corticosteroids and cyclophosphamide with favorable outcome.

Mucocutaneous Manifestations of Behçet's Disease

Behçet's disease (BD) is a chronic, relapsing, systemic inflammatory disease with clinical features showing mucocutaneous lesions involving the ocular, articular, and further miscellaneous organs. Mucocutaneous manifestations, one of the most characteristic signs of BD, have been most commonly observed upon onset or at any disease stage and are exceptionally important in its diagnosis. Given the lack of specific diagnostic laboratory tests for BD, diagnosis has been based on clinical findings. All diagnostic criteria published have thus far relied heavily on mucocutaneous manifestations, particularly oral ulcers (OU), genital ulcers (GU), cutaneous lesions, and pathergy test positivity. Worldwide, OU, GU, cutaneous lesions, and ocular and articular manifestations have been the most common symptoms, with erythema nodosum (EN)-like lesions and papulopustular lesions being the most prevalent cutaneous manifestations. While majority of the patients worldwide have reported OU as the most frequent symptom upon disease onset, GU, and EN-like lesions have also been identified upon onset. Considering that mucocutaneous symptoms precede severe organ involvement in most patients, familiarity with such symptoms is imperative for early diagnosis and prevention of potentially serious organ involvement through appropriate management.

Diagnosis of Behçet’s disease: clinical characteristics, diagnostic criteria, and differential diagnoses

Background The diagnosis of Behçet disease (BD) is challenging in many cases. The purpose of this study was to describe the clinical characteristics of patients at a referral BD clinic. Methods In a retrospective study, we collected data from patients at a national referral Behçet clinic from November 2018–August 2019. A BD diagnosis was confirmed (BD group) or ruled out (Non-BD group), and the two groups were compared for differences. Results A total of 238 patients satisfied the inclusion criteria. Forty patients (16.8%) were finally diagnosed with BD. Ocular and genital lesions were significantly more prevalent in the BD group. A positive pathergy test and HLA-B51 were also significantly more common in BD. However, oral lesions, articular involvement, and gastrointestinal manifestations were similar between groups. Also, patients with BD were significantly more likely to have multi-organ (≥2 organ systems) involvement. Conclusions Being the first study to evaluate the clinical characteristics of patients who are visited at a referral BD clinic and are believed to have a high probability of Behçet, the results of this study are important from an epidemiological standpoint. Also, the findings of this study could be used by referral Behçet clinics, which evaluate and diagnose patients with a high pretest probability and atypical presentations of BD on a daily basis. The alternative diagnoses established in this study could be used as the list of the most common differential diagnoses for Behçet’s disease.

FRI0491 IS THERE A RELATIONSHIP BETWEEN VOGT-KOYANAGI-HARADA AND INFLAMMATORY RHEUMATOLOGICAL DISEASES

Background:Vogt-Koyanagi-Harada Disease (VKHD) is a systemic autoimmune disease characterized by bilateral granulomatous panuveitis associated with systemic symptoms, including neurological, dermatological and audiovestibular systems. Due to its systemic nature, it may accompany with other autoimmune conditions. However, there is a considerably limited number of reports on the association of VKHD and rheumatologic diseases.Objectives:To investigate the relationship between VKHD and inflammatory rheumatological diseases.Methods:Patients who had bilateral granulomatous uveitis and fulfilled the 2001 revised diagnostic criteria for VKHD were included in our study. All patients were systematically reviewed in terms of the presence of any rheumatological manifestations including connective tissue diseases, spondyloarthritis (SpA), vasculitis, Behcet’s disease and sarcoidosis.Results:Demographic findings: There were fifteen patients in the study (86,7%,female), the mean age at diagnosis was 31,2 ± 11,1 years.Comorbidities: Six patients (4 hashimoto thyroiditis, 2 diabetes mellitus) had comorbid diseases.Rheumatological findings: Mechanical back pain in 4 patients, 1 patient had morning stiffness without any other SpA related features; 2 patients had inflammatory arthralgias in small joints, 4 patients had sicca symptoms, 1 patient had arthritis in knee joint, 3 patients had oral aphthae and 1 patient had photosensitivity.Laboratory tests and autoantibodies: The acute phase reactants were within normal ranges. The mean CRP value at the time of diagnosis was 2,7 ± 3,2 mg/L and ESR was 14,4 ± 9,2 mm/h. Two (15,3%) out of 13 patients had high serum ACE levels. RF, anti-CCP and anti-dsDNA were negative in all patients. ANA was positive (>1/160 titers) in 4 patients (28,6%) and 3 patients had a titer above 1/320. Anti-ENA profile was positive in 2 patients with anti-SS-B and anti-histone components. MPO-ANCA was positive in one patient.HLA test: HLA-B27 was negative in all patients. HLA-B51 and B18 were positive in 2 patients.Radiographic findings: One patient had heel enthesitis on X-ray, 4 patients had bilateral grade 1 and one patient had unilateral grade 2 sacroiliitis. None of them fulfilled the Modified New York criteria for radiographic sacroiliitis. Hand X-rays of all patients were normal. One patient had reticular density on chest X-ray.Pathergy: The pathergy test was negative in all patients.Capilleroscopy: Four patients had pathological capilleroscopy findings (3 patient tortuous loops, 1 patient tortuous loops and microhemorrhages).Conclusion:This study suggests that; 1) inflammatory arthralgias and sicca symptoms were the most common rheumatological findings, 2) the frequency of SpA related features were not increased in VKHD, 3) increased autoantibody frequency, particularly in high titers of ANA could be seen in VKHD possibly reflecting the autoimmune nature of the disease, 4) even though there were signs of rheumatic diseases, none of the patients were grouped into any rheumatologic diagnostic classification.Demographic findings and rheumatological manifestations in VKHD patientsVKHD patients (n=15)Females,n (%)13 (86,7)Age,yrs (mean ± std)34,6 ± 12,6Age of diagnosis, yrs (mean±std)31,2 ± 11,1CRP baseline (mean±std)2,7 ± 3,2 mg/LESR baseline (mean±std)14,4 ± 9,2 mm/HHigh ACE levels,n(%)2/13 (15,3)RF positivity,n(%)0/14 (0)Anti-CCP positivity,n (%)0/6 (0)ANA positivity,n(%)4/14 (28,6)ANA pattern,(n)Homogeneous (2)Nuclear (1)Homogeneous speckled (1)Anti-dsDNA positivity,n(%)0/11 (0)Anti-ENA profile positivity,n(%)2/14 (14,2)ANCA positivity,n(%)1/12 (8,3)Pelvis X-Ray abnormality,n(%)Sacroiliitis (n)5/12 (41,7)Bilateral grade 1 (4)Unilateral grade 2 (1)Capilleroscopy abnormality,n(%)4/9 (44,5)Pathergy,n (%)0/12 (0)Disclosure of Interests:None declared