Can we cure Behçet syndrome?

Behcet's syndrome (BS) is a multisystem syndrome that typically manifests as recurrent oral and genital ulcers, as well as other systemic manifestations. Few studies describing the characteristics of BS among Israeli patients have been published. To describe the characteristics of BS patients and to compare Jewish and Arab subpopulations. We retrospectively reviewed electronic medical records and extracted demographic, clinical, laboratory, and medication data for each patient. We compared the Jewish and Arabic BS patients. The cohort included 98 patients. Males constituted 49 (50%); mean age at the time of diagnosis was 29.9 years; 71 (72.4%) were Arab and 27 (27.6%) were Jewish. Oral ulcers were evident in 93 patients (94.9%) and genital ulcers in 54 (55.1%). Involvement of the skin, joints, eyes, gastrointestinal tract, and neurologic and vascular systems were demonstrated among 42 (42.9%), 57 (58.2%), 47 (48.0%), 8 (8.2%), 10 (10.2%), and 15 (15.3%), respectively. HLA B51 was positive in 24 of 37 (64.9%). Pathergy was positive in 8 of 12 (66.7%). Colchicine was used in 82 (83.7%), azathioprine 47 (48%), methotrexate 16 (16.3%), apremilast 10 (10.2%), cyclosporine-A 8 (8.2%), adalimumab 26 (26.5%), infliximab 12 (12.2%), cyclophosphamide 1 (1.0%), tocilizumab 2 (2.0%), and anti-coagulation 6 (6.1%). The Arab and Jewish subpopulations were significantly different regarding male proportion, 40 (56.3%) vs. 9 (33.3%), P = 0.042. BS is more common among Arabs in northern Israel, but no significant clinical or demographic differences were found except for a higher proportion of male patients among Arabs.

Background Behçet's Disease (BD) is characterized by oral and genital ulcers, arthritis, skin manifestations, uveitis, gastrointestinal tract, and central nervous system involvement. Although it is known to be more severe in men, there are studies in the literature with conflicting results regarding gender and the distribution of clinical findings. This study aimed to examine the relationship between clinical findings and gender in BD patients and to compare our results with the literature. Methods 506 patients diagnosed with Behçet's disease were included in the study. Demographic data, laboratory, and clinical findings of the patients were obtained retrospectively from hospital records. The distribution of clinical findings according to gender was evaluated. Results A total of 280 males (55.3%) and 226 females (44.7%) were included in the study. There was no significant difference between male and female patients regarding age at diagnosis (p=0.662). Genital ulcer (47.6% vs 52.4%, p=0.011), superficial thrombophlebitis (20.9% vs 79.1%, p=0.002), uveitis (33.7% vs 66.3%, p=0.02), deep vein thrombosis (22.5% vs 77.5%, p=0.00) and pulmonary artery aneurysm (11.1% vs 88.9%, p=0.046) were more common in males. There was no significant difference between the sexes in other clinical findings, HLA B5, and pathergy positivity. Conclusion Gender impacts the clinical manifestations of BD and should be considered in patient follow-up. However, it is a heterogeneous disease, other factors may certainly affect the emergence of clinical findings.

Abstract Cyclin-dependent kinases 4/6 (CDK4/6) inhibitor is a standard-of-care for hormone receptor-positive/human epidermal growth factor receptor 2 negative metastases breast cancer (HR+/HER2- mBC), while substantial proportion of patients suffer from intrinsic resistance (IR), and the mechanism at the spatial level remains unclear. A total of 10 eligible HR+/HER2- mBC patients (age: median age 63, range 40-67 years) with liver metastases who underwent liver biopsies and received CDK4/6 inhibitor were included. The majority (N = 7, 70%) were of first-line treatment at metastatic disease settings and in combination with fulvestrant (N = 9, 90%). After a median follow-up of 22.2 months, four patients were determined as IR, with a median PFS of 4.2 months. We collected formalin-fixed paraffin-embedded samples from these patients and spatially profiled the expression of more than 570 proteins across tumor (panCK+), immune (CD45+), and cancer-associated fibroblast region (CAF, α-SMA+) regions, respectively using the GeoMx Digital Spatial Profiler (DSP) platform. The differentially expressed proteins (DEPs) were identified and GSEA analyses were performed. Elevated expression of MHC I proteins (HLA B7 and HLA E), extracellular matrix (ECM)-associated protein (FAK, Fibronectin, and Periostin), antioxidant-associated proteins (NQO1 and G6PD), and mucin-related proteins (Muc5AC and Muc1) were observed in tumor regions (all FDR < 0.05) among IR patients. Similarly, HLA B7, Fibronectin, MucAC5 and NQO1 were found to be overexpressed in CAF region. The proteins associated with the target of CDK4/6 inhibitor were down-regulated among IR patients [NF-kB p105, CK8 (phosphor S431), and Cyclin D1], suggesting that the IR could be partially explained by loss-of- drug-target. Meanwhile, downregulation of Caveolin-1 and CD63 were identified in CAF regions, while downregulation of HLA-DR and MHC II proteins were observed in immune regions. GSEA results showed that MHC I antigen presentation pathway was significantly upregulated in CAF regions (NES 1.87, FDR 0.016), while TGF-beta pathway and autophagy pathway were significantly downregulated in CAF regions (NES -1.77 [FDR 0.016] and -1.84 [FDR 0.040], respectively). These findings suggested that IR might be driven by the immune escape associated with CAFs. Our results showed that Bcl-2 expression in tumor region has the highest value in predicting IR (AUROC 0.989). Leveraging high-plex spatial proteomics data from HR+/HER2- mBC patients, the IR of CDK4/6 inhibitor might be associated with loss-of-target in tumor cells, immune escape driven by CAFs, and alterations in the ECM. Citation Format: Haoting Shi, Zheshen Han, Xue Wang, Yufei Wang, Chao Hu, Ruixin Pan, Xiaosong Chen, Qing Qu, Rong Cai, Kunwei Shen. Profiling the immune landscape of CDK4/6 inhibitor intrinsic resistance among metastatic HR+ breast cancer [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2025; Part 1 (Regular Abstracts); 2025 Apr 25-30; Chicago, IL. Philadelphia (PA): AACR; Cancer Res 2025;85(8_Suppl_1):Abstract nr 778.

Introduction. Psoriatic arthritis (PsA) is a complex autoimmune disease with genetic and immunological components influencing its pathogenesis. HLA antigens are critical in determining genetic predisposition and clinical variability. This study aims to explore HLA antigen diversity in PsA patients and its relationship to clinical variants. Material and methods. A cohort of 103 PsA patients, diagnosed according to CASPAR (2006) criteria, was studied. Patients were received treatment in rheumatology departments from 2005–2024. Two groups were formed: 76 patients with PsA and cutaneous psoriasis (Group I) and 27 without cutaneous manifestations (Group II). Each group was further subdivided into clinical variants: axial, oligoarticular, polyarticular, distal interphalangeal, and mutilans. Results. Significant correlations were identified between HLA antigens and PsA severity. Aggressive HLA antigens, including HLA-B27, B8, and B62, were associated with severe disease forms and high DAPSA scores (≥50), while protective antigens like HLA-A2 and A3 correlated with reduced activity (DAPSA <20). Group I exhibited HLA-B27/B62 and HLA-B27/A3 combinations linked to mixed articular and cutaneous involvement, whereas Group II had distinct profiles (e.g., HLA-B27/ B62, HLA-B27/B11). Factorial analysis highlighted the immunogenetic variability between clinical subtypes, emphasizing HLA antigens’ predictive and therapeutic relevance. Conclusions. HLA antigens significantly influence PsA severity and clinical diversity. Integrating genetic profiling into clinical practice offers promising opportunities for improving diagnostic precision, therapeutic outcomes, and patient quality of life.

Abstract The exploration of Behçet’s disease challenges the idea that “the oral cavity reflects systemic diseases.” This chronic systemic vasculitis, characterized by oral and genital ulcers, cutaneous lesions, and multi-organ involvement, poses diagnostic complexities. A 30-year-old male patient reported with Behçet’s syndrome, diagnosed via International Criteria for Behçet Disease 2014 and HLA B51 positivity, who achieved relief with Colchicine, highlighting the role of oral medicine.

AIDS (acquired immunodeficiency syndrome) is an infectious disease caused by the HIV virus (human immunodeficiency virus). The core of HIV treatment is antiretroviral therapy (ART). Abacavir, an antiretroviral drug used to treat HIV infections, is widely used in the treatment of HIV-supported infections. The active ingredient of the drug is not able to completely eradicate the infection, leading to a patient’s recovery, but it reduces the amount of virus in the body, keeping it at low levels. Pharmacogenetic tests are used in clinical practice to optimize the choice of medication or clinical management of the patient. Before starting treatment, it is necessary to perform a genetic exam to assess the presence of the HLA allele B57:01. Patients with the HLA B57:01 allele are therefore at increased risk of hypersensitivity to Abacavir. An adverse effect of abacavir is a hypersensitivity reaction, which can be severe and potentially life-threatening, and may limit treatment with the drug. The abacavir-induced hypersensitivity reaction was therefore associated with the presence of the class I allele of the major histocompatibility complex HLA-B*5701. Screening patients for HLA-B*57:01 before starting abacavir therapy reduces the incidence of hypersensitivity reactions. The study we carried out aims to evaluate the presence of the HLAB57:01 allele in the HIV-positive population present in our territory.

Background: Immunodeficiency 70 is a rare disease and there are only very few cases in the literature. Case History: Young boy in his early 20’s from Indian subcontinent presented with recurrent episodes of fever, oral ulcers, loss of weight for past 3 years. Four episodes (july 2019, april 2020, APRIL 2021, Jan 2022) of fever, each high grade associated with chills, rigor, nausea, myalgia, throat pain, holo-cranial headache, painful oral ulcers over the tongue and painless over lips. Initial episode of fever was associated with leucopenia (3200) and thrombocytopenia (122000), acute kidney injury (creatinie-3.0) and pyelonephritis, transaminitis. He received 2 weeks of parenteral antibiotics and improved. Subsequent episodes of fever is not associated with any localizing symptoms or signs. No history Suggestive of connective tissue disease. Family history was non contributory. Physical examination: He was febrile (101 degree F), pulse rate 100/minute, respiratory rate 18/minute, tongue ulceration in lateral border, no lymphadenopathy, no organomegaly, normal sytemic examination. Evaluation: The patient was evaluated for chronic infections, hematalogical malignancy, Behcet’s disease, auto inflammatory syndromes like Haplo insufficiency of A20. His complete blood count, electrolytes, renal function test, liver function test urine microscopy, blood culture were normal. CRP was elevated (58 mg/l). Tzanck smear from buccal mucosa was negative. CT thorax and abdomen showed no significant lymphadenopathy, organomegaly, evidence of infections or malignancy. In view of oral ulcers & fever Behcet’s disease was considered and HLA b51 was sent and came negative. Exome genome sequencing was done to diagnose immunodeficiency or autoinflammatory syndromes which came positive for immunodeficiency 70. Treatment: He was treated with colchicine and fever subsided and leucopenia improved Discussion: Immunodeficiency-70 (IMD70) is an autosomal dominant immunologic disorder characterized by severe cutaneous warts on the hands, feet, and face, suggesting increased susceptibility to human papillomavirus (HPV) infection. Affected individuals may also have recurrent bacterial infections like pneumonia, boils, sinusitis, as well as feature of autoinflammation, such as colitis, celiac disease, and retinal vasculitis. Laboratory studies show decreased CD4+ T cells and decreased CD19+ B cells; hypogammaglobulinemia, combined T cell and B cell immunodeficiency characterized by decreased CD4+ T cells, decreased CD19+ B cells, recurrent bacterial infections, and severe cutaneous warts on the hands, feet, and face that has material basis in heterozygous mutation in IVNS1ABP on chromosome 1q25.3.

Behçet disease (BD) is a rare disease in childhood and its uveitis may lead to blindness if not properly treated. We aim to describe a cohort of paediatric BD patients with uveitis. This is a multicentric retrospective study. Six paediatric rheumatology units in Italy were involved including children with a diagnosis of paediatric BD according to the International Criteria for BD Criteria and/or to the International Study Group Criteria for BD, or Paediatric BD classification criteria if they had uveitis. Demographic, laboratory and clinical data were collected and followed up to March 2023. Ocular characteristics and treatment response were assessed according to Standardization Uveitis Nomenclature. Among the 97 children with BD followed, 33 (34%) had uveitis (22 males, 66.7%). The median age at onset of BD and uveitis were, respectively, 11.5 years (2.5-17.1) and 11 years (3-17.3). Uveitis preceded BD diagnosis in 18 children (54.5%). Seventeen children had HLA B51 positivity (51.5%). Uveitis was bilateral in 25 (75.8%), and panuveitis in 16 (48.5%). All the patients received at least 1 systemic treatment for uveitis: 25 adalimumab, 2 tocilizumab, 1 abatacept, 3 infliximab, 4 azathioprine, 1 methotrexate and 1 corticosteroid. The remission was achieved with 30/35 treatments (85.7%) after a median time of 8 months (6-24). Six children had a relapse in therapy after the achievement of remission (20%). Fourteen patients stopped the therapy for persistent remission, but 5 relapsed (35.7%) after a median time of 9 months (range 1-48). Uveitis in BD is a sight-threatening condition, and it is more frequently a panuveitis. Biologic treatments seem to be often required to control ocular inflammation.

P215 Highly sensitized HLA B46 homozygous patient that makes antibodies to Bw4 and Bw6 public epitopes

e18023 Background: Undifferentiated Carcinoma of Nasopharyngeal Type (UCNT) is a rare and aggressive type of head and neck cancer related to the Epstein Barr virus (EBV), genetic (HLA B5 in Algeria) and environmental factors. It is the most frequent histological variant of ENT cancers. It has geographically selective epidemiologic features unrelated to external carcinogens, and is known to be radiosensitive and chemosensitive. Methods: Seventy-one patients with biopsy-proven UCNT presenting to a single oncology unit between 2015 and 2022 were assessed for their long-term response to treatment. The sex ratio of cases was 3M: 1F; thirty-one patients (43%) were under the age of 50; 11 (15%) under the age of 35; and among these, there were two 19 year old males. The initial median World Health Organization (WHO) performance status was 1.6. These patients with locoregional (LR), recurrent (REC) and/or metastatic (MTS) UCNT were treated with monthly cycles of platinum-based chemotherapy of cisplatin (CDDP) 75 mg/m2 IV or Carboplatine AUC-4 and Docetaxel 75 mg/m2 IV followed by fluorouracil (5FU) 1000 mg/m2/day continuous IV infusion days 1 to 4 (TPF) in locally advanced disease, and with cisplatin (CDDP) 75 mg/m2 day 1 or Carboplatine AUC-5; and fluorouracil (5FU) 1000 mg/m2/d continuous IV infusion days 1 to 4 (PF) in recurrent and/or metastatic cases. The choice of systemic therapy was individualized based on patient characteristics including performance status, goals of therapy. Results: Those LR or MTS patients who were still alive without evidence of disease after 36+ months were categorized as complete responders (CRs). A subgroup of CRs was identified who had failed prior chemotherapy. MTS patients with less than three distant osseous sites underwent additional radiation therapy. Conclusions: Encouraging results for this particular type of head and neck cancer confirm the chemosensitivity of UCNT, and the observation of long-term responders makes curability a real consideration.

Objective. To determine the features of the distribution of antigens of the major histocompatibility complex in children of preschool and primary school age with atopic diseases.
 Materials and methods. Under observation there were children with a moderate course of the diseases such as atopic dermatitis, atopic dermatitis with concomitant persistent allergic rhinitis, independent persistent allergic rhinitis, atopic bronchial asthma. The patients were identified class I and class II antigens of HLA-complex.
 Results. The studies have shown that patients with atopic dermatitis had a high incidence of HLA B15, DRB1*13 and DQB1*0602-8: in patients suffering from atopic dermatitis with concomitant persistent allergic rhinitis HLA B12, B13 and DQB1 * 01; in patients with independent persistent allergic rhinitis HLA A10 and DQB1 * 201; in patients with atopic bronchial asthma HLA B18.
 Conclusions. Representation of these antigens of the HLA complex in the tissues was associated with an increase in the risk of atopic diseases by 2.34.6 times.

BackgroundThe clinical findings of Behçet’s disease (BD) differ according to the country and race investigated. The most important genetic factor known in the pathogenesis of BD is HLA-B51, and this positivity is high in countries on the “Silk Road” where BD is as frequent as it is in Turkey. Although the positivity of HLA B51 is proven to be high in Turkey, there are no studies in the area of the western Black sea demonstrating its relation to the demographic. We aimed to investigate the association of HLA-B51 positivity in Turkish patients diagnosed as having BD and the relationship between the demographic and clinical findings of the patients.ResultsIn this descriptive, cross-sectional study, a convenience sample of adults with BD was obtained from an outpatient clinic of a university hospital in Turkey between January 2018 and January 2022. Patients were diagnosed as having BD according to the criteria of the International BD Study Group, and the patients’ sociodemographic and clinical characteristics were recorded retrospectively. Demographic data and the frequency of clinical findings were compared between patients who were HLA-B51-positive and HLA-B51-negative. Sixty patients (55.6%) were HLA-B51-positive. Oral ulceration, genital ulceration, thrombophlebitis, and family history of BD were found to be higher in patients who were HLA-B51-positive. Erythema nodosum, papulopustular eruption, pathergy positivity, arthritis, and ocular involvement were less frequent in patients with HLA-B51 positivity. However, there were no statistically significant differences according to the frequency of clinical findings between the HLA-B51-positive and HLA-B51-negative groups.ConclusionsHLA B51 positivity is not diagnostic of BD; however, it may affect clinical phenotypes. Although oral and genital ulcerations, thrombophlebitis, and positive family history of BD were found to be common in patients with HLA-B51 positivity, this relationship could not reach statistical significance.

Introduction:Behçet’s Disease (BD) is a systemic vasculitis, highly prevalent in Eastern Asia to Mediterranean countries. Iran is among the countries with the highest prevalence of BD, and previous studies in different countries have shown a broad range of clinical manifestations of the disease. The present study is conducted to evaluate the prevalence of the clinical manifestations of BD in patients referring to rheumatology clinics of two distinct referral hospitals in Tehran and Zanjan, Iran.Methods:In this retrospective, cross-sectional study, the medical records of patients with BD were reviewed, and age at onset, sex, the delay between the onset of symptoms and diagnosis, clinical manifestations, HLA B27, HLA B51, HLA B5, haematuria, proteinuria, leukocyturia, Erythrocyte Sedimentation Rate (ESR), and pathergy phenomenon were included in the study. The collected data were analysed by χ2 test using SPSS 23.Results:A total of 188 patients (Male/female ratio = 1.47) were included in the study with mean ± SD age at onset of 27.98 ± 10.47 years and a mean ± SD of delay between the onset of symptom and diagnosis of 5.70 ± 7.16 years. The most common clinical manifestation was mucosal involvement (85.1%), followed by the ocular lesion (55.3%) and skin manifestations (44.7%). The Pathergy phenomenon was observed in 98 patients (52.1%). Moreover, 45.2% had positive HLA B5, followed by HLA B51 (35.1%) and HLA B27 (12.2%).Conclusion:This study demonstrated that male/female ratio and mean age at onset were comparable to the results of previous studies in Iran. Significant associations between HLAB5 and clinical manifestations underline the pivotal role of genetic factors in BD.

Three young males with Hugh-Stovin’s syndrome presented with cough, haemoptysis, fever, raised inflammatory markers, and pulmonary artery aneurysm. Only one had recurrent oral ulcers suggestive of Behcet’s disease, and none were HLA B51 positive. All responded well to immunosuppression but eventually needed either an endovascular procedure or surgery.

Introduction - Behcet disease is an auto-inflammatory systemic vasculitis. It usually comprises a triad of recurrent oral and genital ulcerations, ocular manifestations. Case Report- A18 years old lady presented with recurrent oral ulceration since 1 year which was causing difficulty in chewing food and burning sensation in oral cavity. Out of high grade of clinical suspicion, due to the recurrent nature of ulcers and rapid resolution by steroids, HLA profiling was done, and HLA B5 came out to be positive and the patient was diagnosed as Behcet disease. Conclusion- Behcet disease has a multi-systemic involvement. The diagnosis of Behcet disease requires a very high threshold of clinical suspicion to appropriately manage the disease. Key words: Behcet Disease, HLA B5, Aphthous ulcer, Oral steroids

Sarcoidosis is a multisystem granulomatous disorder. Its exact cause is unknown, but it is believed that an external agent may cause the characteristic immune reaction in genetically susceptible individuals. There is therefore general recognition that genetic vulnerability to sarcoidosis is one of the potential risk factors. HLA is encoded by genes in the major histocompatibility complex on chromosome 6. These surface cells are important in presentation of antigen and play a key part in the body's immune response to external antigens. Various HLA subtypes are more common in people with sarcoidosis than in those without. Variances in vulnerability, presentation,progression and prognosis have been related to different HLA phenotypes. HLA genes offer information into the factors driving sarcoidosis and prognosticating tools. However, in Africa, including South Africa (SA), there are no data on HLA types in relation to sarcoidosis. To determine HLA class I and II associations in SA sarcoidosis patients. Phenotype frequencies of HLA-A, B and C and DQB1 and DRB1 were calculated for 51 consecutive patients with biopsy-proven sarcoidosis attending the respiratory clinic at Charlotte Maxeke Johannesburg Academic Hospital and 63 controls, who were potential organ donors. The frequencies of the tested HLA loci were determined by direct counting. The significance of the associations between the various loci tested for and the presence or absence of sarcoidosis was estimated from 2 × 2 tables using the χ2 test. Of the 51 patients, 70.6% were female. The mean age was 44.6 years. Analysis of HLA class I and class II phenotypes in sarcoidosis patients revealed a significant association with HLA-B15, C4, C7, C12, C15, C16, C17, DQ3, DR8 and DR11. In addition, a significant negative (protective) association with HLA A9, A28, B12, B17 and DR2 was observed. This HLA study in SA patients suggests that genetic factors play a role in the causation of sarcoidosis. Some HLA subtypes have a significant association with sarcoidosis in SA patients, while other subtypes may be protective. The study supported the association of HLA antigens with sarcoidosis and implies that there is a genetic predisposition to sarcoidosis in the SA population.

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BackgroundMycobacterium tuberculosis is an organism responsible for causing chronic granulomatous inflammation leading to active or latent tuberculosis (TB). Both pulmonary and extrapulmonary infections; including the skin, the eye, the cardiovascular, genitourinary and gastrointestinal systems can occur. Within the eye, it can cause global disease ranging from anterior or posterior uveitis to retinal TB. Within this abstract we demonstrate a case of chronic panuveitis with a diagnosis of ocular TB.MethodologyRetrospective chart reviewResultsWe reviewed a 9-year-old- male in the rheumatology outpatient clinic who was on treatment for ocular TB with methotrexate 2.5 mg once a week, prednisone 5 mg once a day, maxitrol eye drops once a day and prednisolone eye drops twice a day.He presented with a red, painless left eye with deteriorating vision 2 years ago. Functional enquiry was unremarkable. He was noted to have a fixed dilated left pupil at 3-4mm but other examinations were normal. Extensive investigations were carried out, including an Interferon-gamma release assay (IGRA)- which was positive. Toxoplasma antibody test, Treponema pallidum Haemagglutination test, and chest X-ray were all negative. He was then started on the above-mentioned therapies with marked improvement.A decision on increasing the methotrexate dose if steroid tapering showed no improvement was undertaken and a plan on reassessing the patient with ANA, ACE and HLA B51 was made, to ensure complete workup.Discussion and conclusionIntra or extra ocular TB (OTB) manifestations may result as a direct inoculation of the organism in ocular fluid or as a systemic immune response initiated by an infection from a distant site. There is a myriad of ocular manifestations of OTB which make forming a clinical diagnostic criterion difficult. The gold standard for diagnosis is an ocular fluid culture showing organisms but the yield may be low and the risk of damage to surrounding structures may be high. PCR tests done on the sample may yield clinical information but non ocular tests, such as the IGRA and tuberculin tests may also be employed. Therapy depends on active vs latent disease and mainly involves anti-inflammatory agents and anti-TBs.Ocular TB may lead to sudden and irreversible blindness with high socioeconomic implications and hence, an index of suspicion in areas with a high disease burden should be kept as diagnosis poses a challenge.

IntroductionBehçet's disease is a chronic, relapsing, multisystem vasculitis. The diagnosis is essentially clinical, due to the absence of specific biological criteria, which remains very difficult to establish in pediatric age because of often insidious or atypical disease onset. The association of Pseudo Inflammatory Non-Specific Tumors (PTINS) and Behcet's disease (BD) is exceptionally reported. We report the case of a 12-year-old boy with Behçet's disease who presented with an inflammatory pseudotumor during the course of his disease.Methods and resultsA 12-year-old boy, from Batna (Algeria), with no relevant past medical history, admitted in 2010 for the management of a venous thrombosis of the right lower limb without any obvious cause and which responded well to anticoagulant treatment.A year later, the child was readmitted for polymorphic clinical signs made up of joint damage (inflammatory arthralgia of the large joints), digestive (glairo-bloody diarrhea) and ocular (decreased visual acuity) and skin and mucous membranes (oral-genital apthosis and erythema nodosum of the 4 limbs).Lab work-up showed significant inflammatory syndrome with erythrocyte sedimentation rate (ESR) of 100 mm at the first h, C-reactive protein (CRP) up to 400 mg/l and thrombocytosis (700 000–900 000 elements/mm2). HLA B51 was negative.The patient was treated with Colchicine, Steroids, and Aspirin with partial response. Subsequently, a treatment with azathioprine was started, with clinical improvement (despite occasional digestive crises) but with persistent biologic inflammation.In 2012 the patient presented with a unilateral decrease in visual acuity. A CT scan was performed showing an intracranial tumor compressing the right optic nerve with all the criteria of a malignant tumor (rupture of the cortex, increase in volume on 2 successive CT scans). A transsphenoidal biopsy of the mass revealed a non-specific inflammation. Biological treatment with anti-TNF alpha (Infliximab) was associated with a spectacular tumor regression, allowing retrospectively the diagnosis of an inflammatory granulomatous lesion related to Behçet's disease rather than a malignant tumor. The outcomes were favorable with clinical and biological improvement for the first time since the onset of the disease.ConclusionBehcet is rare in the pediatric age group and it is difficult to diagnose. Nonspecific inflammatory pseudotumors can be associated with Behçet's disease and constitute a real diagnostic and therapeutic challenge.Disclosure of InterestNone declared