Positive Pathergy Test Research Articles

Diagnostic Sensitivity of Different Applications of Pathergy Test for Behçet's Disease.

This study aims to compare the sensitivity of the intradermal application of the pathergy test with the "three-step pathergy test". The study included 60 patients with Behçet's disease (BD) (23 males, 37 females; mean age 33.9±9.7 years; range, 11 to 57 years) and 100 patients with recurrent aphthous stomatitis as the control group (39 males, 61 females; mean age 32.4±12.1 years; range, 11 to 66 years). Simultaneous intradermal pathergy test and three-step pathergy test were applied to each patient. Twenty-six patients (43.3%) with BD had positive three-step pathergy test and 18 (30%) of them had positive intradermal pathergy test. A statistically significant relationship was found between the three-step pathergy test positivity and male sex while no relationship was detected between the disease activity and the positivity of the pathergy test. In the three-step pathergy test, pathergy positivity with intramuscular method was statistically significantly higher than with intravenous method. The three-step pathergy test in patients with BD was found to have higher sensitivity compared with the intradermal pathergy test. The intramuscularly applied pathergy test was the main cause of increase in sensitivity. Further studies are needed to develop new applications of pathergy test that would increase the sensitivity and which are also easy to apply.

Interleukin-17 mRNA expression and serum levels in Behçet’s disease

Behçet’s disease (BD) was considered a T-helper 1 (Th1)-mediated autoimmune disease, but with the introduction of Th17 cells, their role in the pathogenesis of BD was also addressed. Despite studies on IL-17 in BD, the prognostic value of this cytokine in BD is unclear. The aim of this study is to determine the IL-17 mRNA expression rate and serum levels in patients with BD and its correlation with clinical manifestations and activity of BD. Forty-six BD patients in the active phase of the disease and 70 healthy controls were recruited in this study. BD activity was measured by Behçet’s disease current activity form (BDCAF), Iranian Behçet’s disease dynamic activity measure (IBDDAM) and total inflammatory activity index (TIAI). The IL-17 mRNA expression and serum levels were significantly higher in the BD patients compared with the healthy controls. These parameters in the BD patients aged <25 at disease onset, positive pathergy test, and positive HLA-B5 and HA-B51 were significantly higher than the healthy controls (P < 0.05). The IL-17 serum level in the patients with active uveitis was lower than the patients with in-active uveitis. There was no association between other clinical manifestations of BD and these parameters. No significant correlation was found between BDCAF and IBDDAM with IL-17 mRNA expression and serum levels. However, TIAI had a significant and negative correlation with the serum levels of IL-17.

IL-38 serum levels in patients with Behcet's disease and the relationship with clinical features.

Behcet's disease (BD) is a chronic multisystem autoimmune disorder. Various cytokines take part in the pathogenesis of this disease. Interleukin (IL)-38, a new member of IL-1 cytokine family, has been reported to have anti-inflammatory properties; however, its role in BD has not been investigated yet. In this study, we aimed to examine the probable role of IL-38 in the clinical context of BD. A total of 81 patients with BD and 81 age- and sex-matched healthy subjects as controls were included in this study. The serum levels of IL-38 were measured in patients and controls sera using enzyme-linked immunosorbent assay. The relationship between the serum levels of IL-38 and clinical and laboratory characteristics of the patients were determined. IL-38 serum levels were significantly lower in patients in comparison with healthy controls at P = 0.003. We found significant differences between IL-38 levels in BD patients with positive and negative pathergy tests (P = 0.048) and patients with and without eye involvement (P = 0.046). Despite the absence of significant differences in serum levels between male and female patients, IL-38 levels were higher in female patients with a positive pathergy test (P = 0.048) and those patients with eye involvement (P = 0.046). As healthy controls showed higher IL-38 serum levels than patients, a protective anti-inflammatory role of IL-38 in BD is suggested. Together, these results suggest that the positive relationship between IL-38 serum levels and eye involvement that IL-38 may play a role in this clinical feature of the disease.

Neuro-behçet disease cases with parenchymal and non-parenchymal involvement

Neuro-Behçet’s Disease (NBD) is classified as parenchymal and non-parenchymal involvement. The diagnosis is made by the diagnosis of aphthous lesions, as well as by eye, skin findings, positive pathergy test and vascular lesions. In this article, we aimed to present three cases who presented to our clinic with different neurological complaints and were diagnosed with NBH as a result of clinical evaluations. Our first patient was diagnosed with NBH because of recurrent oral aphthae, genital scar, pathergy test and HLA B51 positivity, magnetic resonance (MR) involvement, and previous Behçet’s disease (BD). In our second case, non-parenchymal involvement was present and widespread cerebral venous thrombosis was detected. The development of gastrointestinal system bleeding with anticoagulant stimulation was associated with GIS involvement and was stimulatory in terms of possible complications of GI. In our last case, there were more than 3 oral aphthae per year, intermittent arthralgia complaints and parenchymal central nervous system involvement and HLA B51 was positive. We wanted to emphasize the need for detailed history and examination of BD in patients with headache, consciousness changes or acute neurological deficits and emphasize the importance of this disease which is common in our country.

The influence of HLA-B51 on clinical manifestations among Japanese patients with Behçet’s disease: A nationwide survey

Objectives: To scrutinize the influence of HLA-B51 to each clinical manifestation of patients with Behçet’s disease (BD) using a database of the Ministry of Health, Labour and Welfare of Japan.Methods: The database of newly registered patients with BD was obtained from the Japanese Ministry of Health, Labour and Welfare. Patients who met International Criteria for Behçet's Disease (ICBD) and had data for HLA-B51 were selected and analyzed.Results: Among the 3044 analyzable cases, 1334 (43.8%) were men and 1710 (56.2%) were women; the median age was 38 years (IQR 29–48). HLA-B51 was positive for 1334 (44.5%). Prevalence of selected manifestations was 98.5% for oral ulceration, 85.5% for skin lesion, 42.1% for ocular lesion, 69.1% for genital ulceration, and 29.0% for gastrointestinal symptom. HLA-B51-positive patients had higher risk for ocular lesion (OR 1.59, 95%CI: 1.37–1.84; p < .001) and lower risk for genital ulceration (OR 0.72, 95%CI: 0.62–0.84; p < .001) and gastrointestinal symptom (OR 0.65, 95%CI: 0.55–0.77; p < .001). No significant difference was observed for other organ involvement; oral ulceration, skin lesion, positive pathergy test, arthritis, epididymitis, vascular lesion, or neurological manifestation. Subgroup analyses revealed that HLA-B51 was not related to genital ulceration in the cases with an ICBD score of 6 or higher and that HLA-B51 tended to more largely affect the risk of three manifestations for men compared to that for women.Conclusion: HLA-B51 positive is a risk factor for ocular lesion and vice versa for genital ulceration and gastrointestinal symptoms in patients with Japanese BD.

Behçet's Disease from Rheumatology and Neurology Perspectives with Special Reference to Children

AbstractBehçet's disease (BD) is an idiopathic chronic relapsing multisystemic vascular inflammatory disease. Although it is generally seen in young adults with male predominance, the onset age of the disease is the pediatric age group in only 4 to 26% of all cases of BD. According to the International Study Group for Behçet's Disease Classification Criteria, a diagnosis of BD requires recurrent oral aphthous ulcerations with two of the following: genital ulcerations, skin lesions, eye lesions, or a positive pathergy test. In this article, we extensively reviewed the current literature with respect to the rheumatological and neurological findings of BD in childhood.

Lenalidomide: An Alternative Treatment for Refractory Behçet's Disease and Relapsing Polychondritis.

IntroductionBehçet’s disease (BD) is a chronic systemic condition characterized by recurrent oral and genital ulcers, neurological and vascular manifestations, ocular and skin lesions, and a positive pathergy test. Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome is rare and consists of features of BD together with relapsing polychondritis. The treatment of this syndrome is similar that of BD and depends on disease expression and symptoms.Case reportWe report the case of a 50-year-old man diagnosed with BD with relapsing polychondritis (MAGIC syndrome) which had evolved over approximately 35 years, with exuberant and frequent oral and genital ulcers. Various treatments were unsuccessful and had adverse effects. Lenalidomide was initiated at a dose of 5 mg per day with remission of oral and genital ulcers and no haematological or adverse effects.ConclusionBD is a complex disorder with a variety of presentations. Its treatment can be challenging but sometimes off-label drugs, like lenalidomide, can be effective.LEARNING POINTSBehçet’s disease (BD) is a chronic systemic autoinflammatory disease whose treatment can be challenging and is dependent on disease expression and symptoms.Thalidomide can be effective for treating recurrent oral aphthae, but its use is limited by adverse effects, mostly sensory polyneuropathy.Lenalidomide is a more powerful thalidomide analogue with fewer adverse effects and may be effective for the treatment of BD.

Behсet's disease: clinical and gender associations

Objective: to compare the frequency of clinical manifestations of Behcet's disease (BD) in patients of both sexes in a Russian cohort.Patients and methods. Examinations were made in 425 patients (285 men and 140 women; mean age, 33.2±10.2 years; median duration of BD, 134.3 [60.0; 192.0] months (about 11 years) with a reliable diagnosis of the disease. 208 (48.9%) patients were ethnic residents of the North Caucasus. The activity of BD was assessed using the BD Current Activity Form; the disease severity was evaluated according to the classification proposed by Ch. Zouboulis.Results and discussion. In men with BD in the Russian cohort, the activity and severity of the disease were significantly higher and the HLA-B5(51)- antigen was more common. There was an association of male sex with thromboses in the deep cerebral veins and cerebral sinuses, damage to the eyes and skin (pseudopustulosis and pseudofolliculitis), genital ulcers, and a positive pathergy test. The findings are consistent with the results of studies of other patient cohorts: Iranian, German, Turkish ones, in which the men were more frequently detected to have damage to the vessels, skin and eyes.Conclusion. In the Russian patient cohort, the male sex is associated with high BD activity, severe organ damages, and HLA-B5(51)-antigen positivity, which is the basis for prescribing immunosuppressive therapy in men with early-stage BD.

Behcet’s disease in Iran: Analysis of 7641 cases

Objective: To analyze Behcet’s Disease (BD) in Iran, from 1975 to 2018, and compare to 35 large/small reports from other countries.Methods: Patients from all over Iran, when suspected, were sent to the BD Unit. The diagnosis was done by expert opinion. All data were recorded in the BD registry (updated in each follow-up). The data are given in percentage with 95% confidence Intervals.Results: The mean age at onset was 25.6 years. Standard deviation (SD) was 9.8. The mean disease duration was 11.7 years (SD: 8.9). Males were 55.8% (54.7–56.9), Females 44.2% (43.1–45.3), Oral Aphthosis (OA) 97.5% (97.1–97.9), genital aphthosis (GA) 64.4% (63.3–65.5), skin lesions 62.2% (61.1–63.3), ocular lesions 55.6% (54.5–56.7), Joint Manifestations 38.1% (37.0–39.2), Gastrointestinal 6.8% (6.2–7.4), Vascular 8.9% (8.3–9.5), neurological (central–peripheral) 3.9% (3.5–4.3), epididymitis 4.6% (4.1–5.1). Lab tests were positive pathergy test 50.4% (49.3–51.5), elevated ESR 51.1% (50.0–52.2), abnormal urinalysis 13.4% (12.6–14.2). The International Study Group (ISG, 1990) criteria and the International Criteria for Behcet’s Disease (ICBD, 2014) had respectively a sensitivity of 76.2% (75.2–77.2) and 96.6% (96.2–97.0). The specificity was 99.3% (99.1–99.5) and 97.3% (96.9–97.7). The accuracy was 86.4% (85.8–87.0) and 96.9% (96.6–97.2).Conclusion: The most frequent manifestations were OA, GA, skin manifestations, and ocular manifestations.

Association of genetic variations in PTPN2 and CD122 with ocular Behcet’s disease

BackgroundProtein tyrosine phosphatases (PTPs) play critical roles in human autoimmunity. Previous studies found that PTPN2 may be the key regulatory factor in the T-cell-mediated immune response. PTPN2 regulates the Janus...

тромбозы при болезни Бехчета: от патогенеза к лечению

Введение. Болезнь Бехчета (ББ) – системный васкулит неизвестной этиологии, характеризующийся рецидивами язвенного процесса в ротовой полости и на гениталиях, частым поражением глаз и рядом проявлений, включающих патологию суставов, желудочно-кишечного тракта, нервной системы и сосудов. Цель исследования: определить частоту и локализацию сосудистых проявлений у больных с ББ, уточнить связь последних с полом, возрастом и другой клинической симптоматикой ББ. Материалы и методы. Обследовано 250 больных ББ, сосудистая патология выявлена у 63 (25,2%) пациентов. Возраст больных с сосудистыми проявлениями составил 34,8 ± 10,4 лет, соотношение мужчины:женщины – 3,5:1. Сосуды вовлекались в процесс чаще в течение первых трех лет болезни. Поражалось в основном венозное русло, доля больных с артериальной патологией составила 9,5%. Результаты. У пациентов с сосудистой патологией по сравнению с больными без тромбозов достоверно чаще выявлялись узловатая эритема (71% против 44%; р = 0,002) и эпидидимит (22,4 % против 10,9%; р = 0,04), а генерализованный увеит (17,4% против 32%; р = 0,002) и ангиит сетчатки (22% против 40,6%; р = 0,008) встречались достоверно реже. В системе коагуляции у больных ББ с и без сосудистой патологии достоверных различий не выявлено. Заключение. Сосудистые нарушения выявлены у ¼ больных (преимущественно мужчин молодого возраста), развивались чаще в первые три года болезни и ассоциировались с узловатой эритемой, эпидидимитом и положительным тестом патергии. Introduction. Behcet's disease (BB) is a systemic vasculitis of unknown etiology; it is characterized by recurrent aphthous stomatitis, genital ulcers, uveitis and other manifestations including arthritis, pathology of gastrointestinal tract, nervous system and blood vessels. The aim of study: to determine frequency and localization of vascular manifestations in patients with BB, to clarify the relationship of manifestations with sex, age and other clinical symptoms of BB. Materials and methods. We examined 250 patients with BB, vascular pathology was revealed in 63 (25,2%) patients. The age of patients with vascular manifestations was 34,8 ± 10,4 years, the ratio of men:women was 3,5:1. Vessels were involved in the process more often during the first three years of the disease. Mainly veins were affected, arterial pathology had 9,5% of patients. Results. In patients with vascular pathology erythema nodosum (71% vs. 44%, p = 0,002) and epididymitis (22,4% vs. 10.9%; p = 0,04) were significantly more frequent than in patients without thromboses, and panuveitis (17,4% vs. 32%; p = 0,002) and retinal vasculitis (22% vs. 40,6%; p = 0,008) were found significantly more rarely. There were no significant differences in coagulation system between patients with and without vascular pathology. Conclusion. Vascular disorders were detected in ¼ of patients (predominantly in men of young age), developed more often in the first three years of the disease and associated with erythema nodosum, epididymitis and a positive pathergy test.

Bladder Dysfunction in Behçet’s Disease

Behcet’s disease (BD) is a systemic vasculitis usually seen in young adults, which affects both venous and arterial vessels of the vascular system. Its etiology is still unknown. The patognomic symptoms are recurrent oral aphtae accompanies with at least two of these; positive pathergy test, skin lesions, recurrent genital ulcers and lesions in the eye. Urogenital involvement usually consists of genital ulcers, urethritis, epididymitis and recurrent cystitis. Bladder involvement is extremely rare and only several cases have been reported. The findings suggest that neurogenic bladder due to neuro-Behcet disease is characterized uro-dynamically by overactive detrusor in storage phase and detrusor sphincter dyssynergia in voiding phase 12. We aimed to present

The association of pathergy reaction and active clinical presentations of Behçet's disease.

ObjectivesThe pathergy skin test is a hypersensitivity reaction to a prick skin trauma caused by a pin or a needle, which is considered as a specific presentation in Behçet’s disease (BD) and the precise mechanism of this test is not well elucidated. This study was designed to evaluate the association of pathergy reaction (PR) with the active clinical manifestations of BD patients, to assess the clinical importance of PR.Materials and methodsThis was a cohort study on 1675 BD patients who fulfilled the entry criteria based on the International Criteria for Behçet’s Disease (ICBD) from 1975 to 2011. The patients were divided into two groups; the pathergy positive group included 841 patients (50.2%) and the pathergy negative group 834 patients (49.8%). The active mucocutaneous and systemic disease manifestations were analyzed according to the presence of the PR. The odds ratio and 95% confidence interval (95% CI) were calculated for each item.ResultsIn this study, 841 patients (50.2%) had a positive pathergy test. Patient’s mean age and mean disease duration were similar in the two groups (pathergy positive and pathergy negative patients). There was no association between positive pathergy test and age of patients, or disease duration. The PR was associated with male gender (p = 0.013), oral aphthosis (p < 0.001), pseudofolliculitis (p < 0.001), anterior uveitis (p = 0.001) and posterior uveitis (p = 0.028).ConclusionsThe presence of PR was associated with male gender, as well as some of mucocutaneous manifestations and uveitis in adult patients. There was no association with retinitis and vascular involvements. PR isn’t associated with the severity of the disease.

Successful treatment of Behçet’s uveitis with Tocilizumab

A 33-year-old man with history of lymphoma was misdiagnosed as multiple sclerosis (MS) 18months previously, developed intermediate uveitis, unresponsive to interferon, and corticosteroids. A diagnosis of Behçet’s disease (BD) was made on the basis of recurrent orogenital ulceration, erythema nodosum, and a positive pathergy test. The patient was started on Tocilizumab and experienced an improvement in visual acuity and intraocular inflammation over the following 2weeks. In patients with BD-related uveitis, Tocilizumab can be an effective alternative to anti-tumor necrosis factor alpha medications.

The IL-8 Gene Polymorphisms in Behçet’s Disease Observed in Denizli Province of Turkey

ABSTRACTBehçet’s disease is a multisystemic inflammatory disorder as a triad of symptoms including recurrent oral and genital aphthous ulceration and uveitis with unknown pathogenesis. IL-8, a proinflammatory cytokine, has been found increased in the active stage of BD. DNA samples were obtained from 88 patients with BD and 112 healthy control subjects in Denizli province of Turkey. All genotyping experiments of SNPs in IL-8 gene were performed using polymerase chain reaction-restriction fragment polymorphism. We found that IL-8 −845 T > C and −738 T > A sites are non-polymorphic. There were no differences in the polymorphisms of IL-8 +396 G/T, +781 C/T, and +1633 C/T sites except IL-8 −251 T > A in between patients and healthy controls. Analysis of IL-8 polymorphisms indicates that the distribution of frequencies seems to be associated with −251 T > A and gender, −251 T > A and erythema nodosum, −251 T > A and ocular involvement, +781 C > T and erythema nodosum, +396 G > T and pathergy positivity, and +1633 C > T and papulopustular lesion. We demonstrated that the frequencies of IL-8 haplotypes were significantly different with BD patients than control group. We found that the distribution of IL-8 haplotypes was significantly different with genital ulcers, ocular involvement, positive pathergy test, erythema nodosum, papulopustular lesions, and arthritis with BD patients than healthy control individuals. Our study suggests that IL-8 gene polymorphisms may affect susceptibility to BD and increase the risk of developing disease. In order to confirm and assess the association of IL-8 and other cytokine gene polymorphisms in the pathophysiology of BD, large cohort studies are needed.

Behçet's Disease: A Retrospective Study of 30 Cases from China

Background: Behcet's disease is a chronic systemic inflammatory vasculitis with multi-organ involvement. The etiology remains unknown and despite the genetic correlation with HLA-B51, it is thought that certain environmental triggers such as viral and bacterial infections are necessary for the development of this disease. The purpose of this case series is to retrospectively study clinical characteristics of Behcet's disease and analyze the efficacy of a standardized treatment regimen. Methods: We analyzed 30 patients, aged 20-50 years old, with Behcet's disease in terms of clinical characteristics and treatment outcomes. Patient selection was based on the presence of recurrent oral ulcers plus at least two additional findings, including recurrent genital ulceration, ocular involvement, skin lesions, or positive pathergy test. All patients were placed on a standardized treatment regimen based on individual disease characteristics. Results: In our study, ocular involvement occurred exclusively in male patients with the disease involving predominantly young to middle aged individuals (20-40 years old). No pathognomonic clinical and laboratory findings were specific to the disease, thus often delaying the diagnosis. Treatment regimens consisted of combination therapy with immunosuppressive and anti-inflammatory medications. Treatment was divided into two phases: management of acute disease and maintenance therapy with all patients achieving clinical remission after completing treatment. Conclusion: Behcet's disease is a multifactorial disease involving many possible mechanisms, both genetic and environmental. The mainstay of treatment is immunosuppression and anti-inflammatory medication. Our standardized treatment regimen was based on a therapeutic ladder and proved to be effective with all 30 patients in our study.

Adult Behcet's disease in Iran: analysis of 6075 patients.

This is an analysis of adult Behcet's disease (BD) in Iran, from the Iran Registry of 7187 BD patients, gathered from 1975 to 2014, among which 6075 were adults (84.5%). Patients were seen by a multidisciplinary team of experts. The diagnosis was by expert opinion, not by any specific classification/diagnosis criteria. However, 96.8% of them were classified by the International Criteria for Behcet's Disease (ICBD). Adult BDs were patients who had their first manifestation at the age of 16 or later. Males constituted 56% (male/female ratio 1.3/1). The mean age at onset was 28.3 ± 8.7, mean duration 10.8 ± 8.2, and mean follow-up 5.0 ± 6.3. Oral aphthosis was seen in 97.5%, genital aphthosis 65.7%, skin manifestations 64.6% (pseudofolliculitis 53.2%, erythema nodosum 23.9%), ocular manifestations 58.1% (anterior uveitis 41.1%, posterior uveitis 45%, retinal vasculitis 33.6%, cataract 24.4%), joint manifestations 39.4% (arthralgia 18.9%, monoarthritis 9.1%, oligoarthritis 17.8%, ankyloing spondylitis 2%), gastrointestinal manifestations 7% (gastroduodenitis 2.3%, peptic ulcer 1.2%, diarrhea 2.1%, rectorrhagia 1.0%, abdominal pain-nausea 1.8%), neurological manifestations 10.6% (central 3.7%, peripheral 0.3%, headache 7.6%), vessel involvement (large vessel 1.7% with large vein thrombosis 1.1% and arterial involvement 0.7%, phlebitis 6.6%, superficial phlebitis 2.3%), epididymitis 4.6%, pulmonary manifestations 1% and cardiac manifestations 0.6%. Positive pathergy test was seen in 52.3%, human leukocyte antigen (HLA)-B5 in 54%, HLA-B51 in 48.9%, and high erythrocyte sedimentation rate in 52.8% of patients. By International Study Group (ISG) criteria 77.9% were classified, compared to ICBD revised criteria with 96.9%. The specificity of ISG was 99.2% and ICBD 97.2%. Results are near the nationwide surveys from Japan, China, Korea and Germany.

Investigation of Pathergy In Patients With Fibromyalgia

Aim: Recent studies showed increased axon reflex flare reaction to mechanical and chemical stimuli associated with neurogenic inflammation in patients with fibromyalgia (FM). Pathergy test is identified as a non-specific hypersensitivity reaction to minimal trauma. The purpose of this study was to investigate whether patients with FM had a positive pathergy test or not. Methods: Forty-six patients who met the 1990 American College of Rheumatology (ACR) criteria for the classification of FM and fifty-one healthy control subjects (HCs) were included in this study. Twenty-eight patients with Behcet’s disease were included as disease control for Pathergy test. The Fibromyalgia Impact Questionnaire (FIQ) was used for assessment of functional status in patients with FM. The Nottingham Health Profile was used for assessment of quality of life in all subjects. Results: There was no significant difference in demographic characteristics between the three groups (p > 0.05). NHP-pain, NHP-physical mobility, NHP-energy, NHP-emotional reaction, NHP-sleep and NHP-total scores were significantly higher in patients with FM compared to HCs (p < 0.001). Neither patients with FM nor HCs had a positive pathergy test. Conclusion: There are some pathophysiological changes in the skin biopsies of patients with FM; however, these changes are not accompanied by a positive pathergy test. Key Words: Fibromyalgia, Pathergy test

Characterization of the major histocompatibility complex locus association with Behçet's disease in Iran.

IntroductionThe aim of this study was to characterize the association of human leukocyte antigen (HLA) B alleles and major histocompatibility complex (MHC) single nucleotide polymorphisms (SNPs) with Behçet’s disease (BD) in an Iranian dataset.MethodsThe association of three SNPs in the MHC region previously identified as the most associated in high-density genotyping studies was tested in a case–control study on 973 BD patients and 825 controls from Iran, and the association of HLA-B alleles was tested in a subset of 681 patients and 414 controls.ResultsWe found that HLA-B*51 (P = 4.11 × 10−41, OR [95% CI] = 4.63[3.66-5.85]) and B*15 confer risk for BD (P = 2.83 × 10−2, OR [95% CI] = 1.75[1.08-2.84]) in Iranian, and in B*51 negative individuals, only the B*15 allele is significantly associated with BD (P = 2.51 × 10−3, OR [95% CI] = 2.40[1.37-4.20]). rs76546355, formerly known as rs116799036, located between HLA-B and MICA (MHC class I polypeptide-related sequence A), demonstrated the same level of association with BD as HLA-B*51 (Padj = 1.78 × 10−46, OR [95% CI] = 5.46[4.21-7.09], and Padj = 8.34 × 10−48, OR [95% CI] = 5.44[4.20-7.05], respectively) in the HLA-B allelotyped subset, while rs2848713 was less associated (Padj = 7.14 × 10−35, OR [95% CI] = 3.73[2.97-4.69]) and rs9260997 was not associated (Padj = 1.00 × 10−1). Additionally, we found that B*51 genotype-phenotype correlations do not survive Bonferroni correction, while carriers of the rs76546355 risk allele predominate in BD cases with genital ulcers, positive pathergy test and positive BD family history (2.31 × 10−4 ≤ P ≤ 1.59 × 10−3).ConclusionsWe found that the HLA-B*51 allele and the rs76546355/rs116799036 MHC SNP are independent genetic risk factors for BD in Iranian, and that positivity for the rs76546355/rs116799036 risk allele, but not for B*51, does correlate with specific demographic characteristics or clinical manifestations in BD patients.Electronic supplementary materialThe online version of this article (doi:10.1186/s13075-015-0585-6) contains supplementary material, which is available to authorized users.

Pulmonary manifestations in a group of patients with Behçet’s disease

Behcet’s disease (BD) is a multisystem vasculitis and pulmonary involvement in BD is reported to indicate a poor prognosis and high mortality. We aimed to study the manifestations of pulmonary involvement in a group of patients with BD and to study the correlation of pulmonary involvement with other clinical manifestations. Our study included 15 patients with BD admitted to Cairo University Hospital, 14 men (93.3%) and one woman (6.7%), mean age 30.06 ± 9.8 years. All patients fulfilled the diagnostic criteria published by the International Study Group for Behcet’s Disease in 1990. All patients were subjected to both plain chest radiography and a helical computed tomography study of the chest. Pulmonary manifestations were present in 11 patients, 10 men (90.9%) and one woman (9.1%). The main pulmonary and constitutional symptoms were as follows: dyspnea (81.8%), cough (63.6%), expectoration (36.4%), chest pain (54.5%), hemoptysis (36.4%), massive hemoptysis (9.1%), fever (36.4%), and weight loss (63.6%). In the 11 patients with pulmonary manifestations, analyses of both vascular and parenchymal lung lesions in helical computed tomography scan indicated the following: pulmonary artery aneurysm occurred in five patients (45.4%), pulmonary nodules in three patients (27.2%), pleural effusion in three patients (27.2%), pulmonary infarction in one patient (9.1%), and pneumonitis in one patient (9.1%). Pulmonary involvement was associated significantly with a positive pathergy test, erythema nodosum, and gastrointestinal manifestations. A high frequency of pulmonary artery aneurysm was observed in our patients with pulmonary BD. Patients with pulmonary BD have higher frequencies of skin and gastrointestinal manifestations.