Inflammatory Vasculitis Research Articles

Difficult-to-treat Takayasu arteritis: a case-based review.

Takayasu arteritis is a rare chronic inflammatory large vessel vasculitis which affects the aorta and its large branches. The diagnosis is based on the 2022 ACR/EULAR classification criteria for Takayasu arteritis. The management of this vasculitis is challenging. Although it is corticosteroid-responsive, relapses and disease progression are common. Thus, it is possible to resort to alternative conventional synthetic disease-modifying anti-rheumatic drugs and biologics, as second-line such as tumor necrosis factor-alpha inhibitors, tocilizumab, or JAK inhibitors as second-line agents is possible. Nevertheless, in some complex cases, the vasculitis remains active despite different proposed therapeutic lines, and a multitarget approach could induce sustained remission. We report herewith a case of 33-female patient with a refractory Takayasu arteritis which remained active after three different therapeutic lines with tocilizumab, then infliximab, then Upadacitinib. Finally, we consider a successful multitarget approach with a combination of infliximab, Upadacitinib, and methotrexate.

Cerebrovascular implications of takayasu arteritis: a review.

Takayasu arteritis (TA) is a rare, chronic, inflammatory large-vessel vasculitis that affects the aorta and its main branches, including the cerebrovascular system. This review analyzes current knowledge and patient outcomes concerning the cerebrovascular implications of TA. A literature search, with publications from 1994 to 2024, identified pertinent studies through PubMed. An illustrative case report details a 19-year-old female with Type 1 TA, illustrating the complex decision required in the absence of surgical or endovascular options. Our results offer a demographic analysis of 1,698 TA patients, highlighting a female predominance of 89.99% and a mean symptom onset at 33 years. The clinical spectrum of cerebrovascular involvement presented varied symptoms, most notably dizziness, with significant incidences of ischemic events and bilateral stenosis primarily affecting the carotid and subclavian arteries. The most common type of TA was Type V, affecting 40% of patients studied. Endovascular treatment had a 95% initial success rate, with a 67% restenosis rate. Surgical treatment was successful in 84% of cases, but 21% had notable post-operative complications. Similar to the endovascular population, those treated with stand-alone conservative therapy saw a 93% initial remission rate with 52% having relapsed. Assessing the disease activity of TA is crucial when planning vascular intervention due to its significant impact on treatment outcomes. Despite its greater initial invasiveness, surgical interventions showed lower restenosis rates compared to either endovascular interventions or standalone conservative management. We emphasize advancements in TA management and the pressing need for continued research into diagnostic and treatment protocols for improved patient outcomes.

Takayasu Arteritis in a Young Female Patient: A Case Report

Takayasu arteritis (TA) is a rare chronic inflammatory vasculitis that primarily affects large vessels like the aorta, mainly in women under 40, particularly of Asian descent. Its exact cause is unknown but is linked to cell-mediated inflammation, leading to complications such as occlusion and aneurysms. Symptoms vary widely, complicating diagnosis. Modern imaging techniques like computed tomography angiography (CTA) are crucial for early detection, which can significantly reduce complications. This case report highlights a 28-year-old woman with extensive aorto-arteritis, underscoring the importance of timely imaging and intervention in managing TA.

ThP8.12 - To do or not to do-Temporal artery Biopsy in Giant Cell arteritis

Abstract Aims Temporal artery biopsy (TAB) remains the gold-standard diagnostic test for giant cell arteritis (GCA). Our aim is to evaluate the correlation between initiation of corticosteroid therapy, timing of biopsy, and histological diagnosis of GCA through TAB. Moreover, analyse the usefulness of this practice. Methods Operative notes of TABs performed by a Vascular Surgery department at a teaching hospital between August 2021 and January 2023 were reviewed. We looked into the relationship between histological findings and the timing of corticosteroid treatment initiation prior to TAB. Results A total of eight patients had a TAB, with a male-to-female ratio of 2:6. The median age of the patients was 68-years-old (range 53-84). TABs were performed within 16 days of the referral (range 2-16). All were performed on elective theatre lists and none on emergency theatre lists (CEPOD). All patients had started corticosteroids by the time they had TAB, with a median duration of 11.5 days before TAB (range 2-16). The histopathology report was positive for active GCA in 1 case (12.5%) where the TAB was performed on day 3. In another performed on day 16, histopathology showed signs of healed inflammation (12.5%). In all other cases the report was negative (75%). All patients completed a full weaning course of steroids regardless of histological findings. Conclusion GCA is a systemic inflammatory vasculitis. Prompt initiation of high-dose corticosteroids is the mainstay management to reduce risks of ischaemic-related complications. Our work suggests the timing of biopsy is a key determinant of histological diagnosis of GCA.

Update on Takayasu arteritis: Year in review 2024.

Takayasu arteritis is an uncommon systemic inflammatory large vessel vasculitis affecting women in their third and fourth decades frequently. The disease poses considerable morbidity and mortality owing to the involvement of the aorta and its major branches. Treatment comprises medical and vascular interventions, tailored to each patient. We review the high-impact publications of the year 2023 up to April 2024, which provide great insight into clinical, biomarker, imaging, pathogenetic, and therapeutic updates.

FAPI-PET-CT for quantification of the tissue response in rheumatic diseases

Fibroblast activation protein (FAP) is mainly found on the surface of activated fibroblasts but is not expressed on the surface of inactive fibroblasts. Selective FAP inhibitors (FAPI), which are coupled to a radioactive tracer, can be used to quantify profibrotic and proinflammatory fibroblasts in patients using FAPI positron emission tomography (PET) computed tomography (CT). Following initial applications in neoplastic diseases, FAPI-PET/CT is also increasingly being applied in rheumatological diseases. The first studies have shown that in patients with systemic sclerosis (SSc) FAPI accumulates in actively fibrotically remodeled pulmonary and myocardial areas, that a high FAPI accumulation is associated with the risk of short-term progression and that this accumulation in the lungs regresses after successful treatment. In cases of immunoglobulin 4 (IgG4)-associated diseases (IgG4 rheumatic disease, RD), the FAPI signal correlates with the histological accumulation of activated fibroblasts and a poorer response to treatment to inhibit inflammation. Fibroblasts in chronically inflamed tissue, such as patients with inflammatory joint diseases, vasculitis or myositis, also express FAP and can be quantified by FAPI-PET/CT. The treatment-induced change of the phenotype from a destructive IL-6+/MMP3+THY1+ fibroblast subtype to an inflammation inhibiting CD200+DKK3+ subtype can be mechanistically demonstrated using FAPI-PET/CT. These studies provide indications that FAPI-PET/CT enables quantification of the tissue response in patients with fibrosing and chronic inflammatory diseases and can be used for patient stratification; however, further studies are essential for validation of the use of FAPI-PET/CT as a molecular imaging marker.

Central Retinal Artery Occlusion Associated with Takayasu Arteritis.

Takayasu arteritis is a chronic inflammatory vasculitis with granulomatous panarteritis particularly impacting large vessels including the aorta and its branches, especially the subclavian arteries, with clinical manifestation dependent on the involved artery. Sequelae of the active disease vary, including stenosis, occlusions, or aneurysmal dilatations of the large vessels. The prevalence of Takayasu arteritis is higher in the Asian population and in Japan, but quite low in the United States, varying from 0.9-8.4 per million people. Ocular manifestations are rare and lead to a delay in diagnosis and appropriate treatment. Ocular manifestations include Takayasu retinopathy, anterior ischemic optic neuropathy (AION), retinal artery occlusion (RAO) and retinal vein occlusion (RVO). We present two cases in which central retinal artery occlusion (CRAO) was associated with Takayasu arteritis. CRAO is an ophthalmic emergency with an incidence of 1.9 per 100,000 person years in the United States; only 5% of cases are arteritic, which can be observed with inflammatory vasculitides secondary to the formation of immune deposits.

TAFRO Syndrome and COVID-19.

TAFRO syndrome is a systemic inflammatory disease characterized by thrombocytopenia and anasarca. It results from hyperinflammation and produces severe cytokine storms. Severe acute respiratory syndrome coronavirus 2, which led to the coronavirus disease 2019 (COVID-19) pandemic, also causes cytokine storms. COVID-19 was reported to be associated with various immune-related manifestations, including multisystem inflammatory syndrome, hemophagocytic syndrome, vasculitis, and immune thrombocytopenia. Although the pathogenesis and complications of COVID-19 have not been fully elucidated, the pathogeneses of excessive immunoreaction after COVID-19 and TAFRO syndrome both involve cytokine storms. Since the COVID-19 pandemic, there have been a few case reports about the onset of TAFRO syndrome after COVID-19 or COVID-19 vaccination. Castleman disease also presents with excessive cytokine production. We reviewed the literature about the association between TAFRO syndrome or Castleman disease and COVID-19 or vaccination against it. While the similarities and differences between the pathogeneses of TAFRO syndrome and COVID-19 have not been investigated previously, the cytokines and genetic factors associated with TAFRO syndrome and COVID-19 were reviewed by examining case reports. Investigation of TAFRO-like manifestations after COVID-19 or vaccination against COVID-19 may contribute to understanding the pathogenesis of TAFRO syndrome.

Thromboangiitis obliterans: a review

Buerger's disease was initially described by von Winiwarter in 1879 where he described it as thromboangiitis obliterans (TAO), detailing the case of spontaneous gangrene secondary to intimal proliferation. In 1908, Leo Buerger published a complete pathological description based on the amputation of a group of people who later suffered short-term gangrene. Buerger's disease or TAO is defined as a non-atherosclerotic inflammatory vasculitis that affects the small and medium vessels (including arteries and veins) of the lower and upper extremities. Although there are hypotheses about its etiopathogenesis such as the association with exposure to tobacco, few biomedical investigations have been carried out, so its direct causality remains unknown. Diagnosis continues to be a challenge, since the findings tend to be non-specific or inconsistent with suspicion, which is why it is currently based on ruling out other causes such as atherosclerosis and vasculopathies. With current evidence, there are many types of treatments, both pharmacological and non-pharmacological, with the cessation of tobacco consumption having greater evidence of results. It is essential to strengthen basic research as well as clinical trials to standardize management in this type of patient, since it is a disease with a high impact on quality of life.

Psoriasiform Drug Eruption Associated with Carbamazepine

Behçet’s disease (BD) is a chronic systemic inflammatory vasculitis of unknown etiology characterized by recurrent episodes of oral aphthous ulcers, genital ulcers, skin lesions, ocular lesions, and other manifestations. This disease affects many organs and systems and shows a wide range of clinical manifestations. The prevalence of anxiety, depression, and general psychiatric symptoms is higher among patients with BD compared with healthy individuals. However, syndromes such as psychosis appear to be less frequent. Therefore, we present a case of BD complicated by schizophrenia-like symptoms.

Carotid artery ultrasonography and shear wave elastography in Takayasu's arteritis: a comparative analysis with diabetes mellitus.

Takayasu's arteritis (TAK) is a chronic inflammatory large vessel vasculitis with a grim prognosis due to the excessive risk for cardiovascular (CV) diseases. Its diagnosis relies on radiographic imaging and its differentiation particularly from atherosclerosis could be challenging. Hypothesising that vascular morphology observed in TAK would be comparable to that found in type 2 diabetes mellitus (T2DM), a prototype for advanced atherosclerosis, we compared two disease groups using carotid artery B mode US and shear wave elastography (SWE). A total of 72 patients with TAK (63F/9M; mean age: 42.7± 10.0 years) and 74 patients with T2DM (65F/9M; mean age: 50.2± 7.1 years) were studied. Intima-media thickness (IMT), outer diameter and arterial stiffness as assessed by SWE values were measured on the common carotid artery (CCA) and atherosclerotic plaques were recorded. Clinical characteristics, CV risk factors and previous history of CV diseases were determined. Framingham risk score was calculated. Patients with TAK exhibited significantly lower atherosclerotic risk but higher systolic blood pressure (BP) levels compared to those with T2DM. The mean values of CCA IMT, outer diameter, and stiffness were significantly elevated among patients with TAK compared to those with T2DM. Carotid artery plaques were evenly distributed between the study groups, but their anatomical localisation and composition differed significantly. While coronary artery disease (CAD) was more prevalent among T2DM patients, cerebrovascular diseases were more frequent among TAK patients. Our study revealed distinctive vascular alterations and atherosclerotic changes when compared to advanced atherosclerosis associated with T2DM. Apart from these, higher levels of systolic BP and significantly different distribution of CV diseases between TAK and T2DM also suggest that TAK should be handled with distinct assessment strategies than that employed in conventional atherosclerotic conditions.

P030 Multiple autoantibody formation post COVID-19 infection

Abstract Background/Aims COVID-19 has been linked to the formation of a number of autoantibodies. This case is an important reminder to clinicians interpreting autoantibody results in conjunction with clinical manifestations. A previously fit 51 year old gentleman, with no prior history of autoimmune disease required admission with respiratory symptoms in April 2020 consistent with COVID-19 infection. His acute respiratory illness was complicated by empyema and left-sided pneumothorax requiring left pleural decortication and adhesiolysis under (VATS). Persistent respiratory symptoms and fatigue lead to investigations for long COVID. Positive antibodies detected were dsDNA 166IU/ml, Proteinase 3 antibodies 20IU/ml, Myeloperoxidase antibodies 32IU/ml, IgG anticardiolipin antibodies 90IU/ml and Glomerular basement membrane antibodies 351 IU/ml. Negative autoantibodies included ANA, RF, aCCP, b2GPI, smooth muscle, AMA and myositis related antibodies. Methods There were no clinical signs or symptoms of inflammatory arthropathy, vasculitis, thrombosis or connective tissue disease. Other investigations included a CT thorax showing no new lung lesions, unremarkable urine dipstick, normal urine ACR, normal US abdomen, CT sinuses and MRI brain. Results Trial course of prednisolone was given therapeutically due to long COVID symptoms. There was no significant impact on symptoms/ autoantibody titres thus steroids were discontinued. The patient was followed closely by the rheumatology team for two years. During that time, the autoantibody levels gradually decreased, and he did not develop clinical signs of autoimmune disease. His long COVID symptoms persisted, and he remains under the long COVID clinical team. Conclusion COVID-19 infection is known to cause an immune response. There have been published cases of positive ANA, U1-snRNP anti-SS-B/La, ANCA, anti-phospholipid and anti-SRP antibody formation following COVID-19 infection. The link between the pathophysiology of COVID-19, autoantibody formation and the role of autoimmunity is still unclear. This man's case was unusual in the number and variety of autoantibodies formed post COVID, this has not been reported in the literature to date. Some of the autoantibodies detected are highly specific for certain conditions, for example anti-GBM antibodies have a high specificity for Goodpasture’s syndrome, thus this case also illustrates the need to interpret positive autoantibodies in the context of clinical findings and history. Disclosure S. Islam: None. N. Erb: None.

VAERS Vasculitis Adverse Events Retrospective Study: Etiology Model of Immune Complexes Activating Fc Receptors in Kawasaki Disease and Multisystem Inflammatory Syndromes.

Vasculitis diseases include Kawasaki disease (KD), Kawasaki disease shock syndrome (KDSS), Multisystem Inflammatory Syndrome (MIS), Henoch-Schönlein purpura (HS), or IgA vasculitis, and additional vasculitis diseases. These diseases are often preceded by infections or immunizations. Disease incidence rates are higher in children than in adults. These diseases have been extensively studied, but understanding of the disease etiology remains to be established. Many studies have failed to demonstrate an association between vasculitis diseases and vaccination; this study examines possible associations. Herein, the Vaccine Adverse Event Reporting System (VAERS) database is retrospectively examined for associations between vasculitis diseases and immunizations. For some vaccines, the number of rare cases of KD, MIS, and HS are higher than the background rates. These rare cases are predicted to occur in individuals with (1) genetic risk factors with (2) antibody titer levels above the primary immune response level. Herein, the model of humoral immune response antibodies bound to antigens (pathogen or vaccine) creating immune complexes is proposed. These immune complexes are proposed to bind Fc receptors on immune cells and platelets, resulting in cell activation and the release of inflammatory molecules including histamine and serotonin. Immune complexes and inflammatory molecules including serotonin and histamine likely trigger vasculitis. Elevated serotonin and possibly histamine drive initial vasoconstrictions, disrupting blood flow. Increased blood flow pressure from cardiac capillary vasoconstrictions is predicted to trigger coronary artery aneurysms (CAA) or lesions (CAL) in some patients. For KDSS and MIS patients, these cardiac capillary vasoconstrictions are predicted to result in ischemia followed by ventricular dysfunction. Ongoing ischemia can result in long-term cardiac damage. Cases associated with pathogens are likely to have persistent infections triggering disease onset. The proposed model of immune complexes driving disease initial disease etiology by Fc receptor activation of immune cells and platelets, resulting in elevated histamine and serotonin levels, is testable and is consistent with disease symptoms and current treatments.

THE EFFECTIVENESS OF INTRAVITREAL ANTI-VEGF INJECTION IN EALES' DISEASE: A CASE REPORT

Introduction : Eales’ Disease (ED) is an idiopathic inflammatory retinal vasculitis, affecting retinal veins of small and large caliber that results in extraretinal neovascularization with vitreous hemorrhage. The etiopathogenesis of ED is poorly understood. However, many studies have attributed inflammation as a response to mycobacterium tuberculosis. Treatment of this disease can be done with corticosteroids, anti-VEGF therapy, laser photocoagulation, and vitreoretinal surgery.
 Case Illustration : We reported a patient, a 31-year-old woman with complaints of blurred vision and floaters in both eyes. An initial examination showed the visual acuity of the RE was 0,1F1 and the LE was 0,1. Examination of the posterior segment found perivascular exudate and peripheral retinal neovascularization. We performed an OCT examination on both eyes and found that there was extensive macular edema, especially in the RE. Patient was diagnosed with ED and received steroidsas an initial treatment. But there was no improvement in both eyes. In addition, patient received two anti-VEGF injections in the RE and one injection in the LE and showed good improvement. After that injection, the posterior segment showed very minimal lesions on the macula and repeated OCT results showed normal central macular thickness.
 Discussion : Anti-VEGF may also play a role in the treatment and management of ED. This is because, besides inflammation, there is an increased expression of VEGF in vitreous patients with ED. Several case reported there is significant improvement in anti-VEGF injection in the management of patients with ED.
 Conclusion : Intravitreal anti-VEGF injection shows good results in ED that did not improve with steroid treatment.

Halo sign on temporal artery ultrasound versus temporal artery biopsy for giant cell arteritis.

Halo sign on temporal artery ultrasound versus temporal artery biopsy for giant cell arteritis.

Treatment refractory acute necrotizing myelitis after COVID-19 vaccine injection: a case report

Introduction and importance: Post-vaccination myelitis is a rare and debilitating clinical situation. There are few reports of post-COVID-19 infection and vaccination neurological sequela. Case presentation: A 69-year-old lady was admitted to the emergency department due to weakness and hypoesthesia in her hands 1 week after the Sinopharm vaccine injection. MRI showed a cervicothoracic cord haemorrhagic lesion that deteriorated within 48 h. The clinical course was refractory to conservative treatments. She underwent an emergency cervical laminectomy as a salvage treatment. Intraoperative samples were in favour of acute necrotizing myelitis. Discussion: In the review of the literature, the inflammatory storm, vasculitis, and many unknown etiologies are deemed to be the possible causes of encephalopathy and myelitis after COVID-19 infection and vaccination. There are few cases of post-COVID-19 myelitis and hematomyelia, but this case was the first report of post-vaccination necrotizing myelitis. Conclusion: Post-vaccination necrotizing myelitis is a lethal medical situation requiring intensive and emergent neurosurgical vigilance. Early clinical diagnosis in the beginning and full neurosurgical-neurological treatment armamentarium options are cornerstones of treatment paradigms. Salvage treatment options such as extensive laminectomy may play a life-saving role in treatment refractory cases of acute necrotizing myelitis.

Unilateral acute posterior multifocal placoid pigment epitheliopathy (APMPPE) with delayed contralateral eye involvement

BackgroundAcute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare presumed inflammatory chorioretinopathy characterized by creamy, yellow-white placoid lesions at the level of the retinal pigment epithelium (RPE). Unilateral cases often have fellow eye involvement within days to a few weeks. This report details a rare case of delayed contralateral APMPPE, in which unilateral lesion resolution was followed by contralateral eye involvement 31 months later.Case presentationA 38-year-old woman presented with three days of blurry vision and photopsias in the right eye (OD). She endorsed a viral GI illness one month prior. Visual acuity was 20/25 -2 OD and 20/20 -1 in the left eye (OS). Examination revealed creamy, yellow-white placoid lesions in the posterior pole. Fluorescein angiography (FA) was notable for early hypofluorescence and late hyperfluorescence of the lesions, consistent with APMPPE. MRI and MRA brain were negative for cerebral vasculitis. She was treated with oral prednisone with complete resolution of her symptoms, vision, and lesion regression. She then presented 31 months later, with blurry vision OS and similar new creamy, yellow-white placoid lesions in the posterior pole OS. She endorsed receiving an influenza vaccine one month prior. FA again was notable for early hypofluorescence. She was diagnosed with APMPPE, this time involving the left eye, and was once again started on oral steroids with complete resolution. She denied any neurologic symptoms.ConclusionsAPMPPE is an inflammatory vasculitis of the choroid, leading to hypoperfusion and ischemic injury of the RPE with subsequent lesion formation. APMPPE may be preceded by a viral prodrome or vaccination, both of which were seen in this case. Choroidal inflammation seen in APMPPE is therefore thought to stem from immune-mediated processes. Unilateral cases often have fellow eye involvement within days to a few weeks. Single eye involvement with delayed contralateral presentation, as seen in our patient, is rare. This case demonstrates that lesion resolution in one eye can be followed by contralateral eye involvement up to 31 months later, highlighting the importance of routine ophthalmic monitoring for patients with unilateral APMPPE.

Association of Kawasaki disease with urbanization level and family characteristics in Taiwan: A nested case-control study using national-level data.

Kawasaki disease (KD) is an inflammatory vasculitis disorder of unknown etiology. It is a rare but fatal disease and the leading cause of acquired coronary heart disease in children under the age of 5 years. We examined the association of KD with the demographics of family members, parents' characteristics, and perinatal factors in Taiwanese children. This nested case-control study used data from Taiwan's Health and Welfare Data Science Center and initially included children born in Taiwan between January 1, 2006, and December 31, 2015 (n = 1,939,449); the children were observed for KD development before the age of 5 years (n = 7870). The control group consisted of children without KD who were matched with each KD case by sex and birth date at a ratio of 8:1. The odds ratio (ORs) of the aforementioned associations were estimated using conditional logistic regression. The risk of KD decreased in children with younger parents [<25 years; younger maternal age, OR = 0.72, 95% confidence interval (CI), 0.66-0.79; younger paternal age, OR = 0.68, 95% CI, 0.59-0.78], lower socioeconomic status, more than 2 siblings (OR = 0.80, 95% CI, 0.73-0.89), and siblings with a history of KD (OR = 4.39, 95% CI, 3.29-5.86). Children living in suburban (OR = 0.95, 95% CI, 0.90-1.00) and rural (OR = 0.81, 95%CI, 0.74-0.90) areas exhibited a lower risk of KD than children living in urban areas. In conclusion, a higher incidence rate of KD was observed in children aged <5 years who had an urban lifestyle, had siblings with KD, were born to older mothers, and belonged to high-income and smaller families. Parental allergic or autoimmune diseases were not associated with the risk of KD.

Human Leukocyte Antigen-B51 Association and Behcet’s Disease in Saudi Arabia

Introduction: Behcet’s disease (BD) is a chronic systemic inflammatory vasculitis of unknown etiology, characterized by recurrent oral ulcers, genital ulcers, skin lesions, ocular lesions, and other systemic manifestations. The pathogenesis of BD is unclear. Multiple studies have suggested close correlation between BD and human leukocyte antigen-B51 (HLA-B51) as a genetic factor. In our study, we aim to identify the association of HLA-B51 as a risk factor and its association with the multiple clinical signs and symptoms of Saudi BD patients in the kingdom of Saudi Arabia. Materials and Methods: A retrospective study was conducted at a tertiary treatment center in the rheumatology subspecialty clinics, Riyadh, Saudi Arabia, from 2013 to 2018. The study was conducted by searching the hospital database for BD and HLA-B51. Results: HLA-B51 positivity was observed in 20 of the 38 patients in our sample, accounting for about a quarter of the total (52.6%). Conclusion: Our retrospective study found that HLA-B51 was positive in 52.6% of Saudi patients attending a single tertiary care center. However, a larger multicenter study is required to validate these observational data.

Myocardial Infarction During Behcet's Disease: A Case Report

Behcet's disease is a multi-systemic inflammatory vasculitis of unknown etiology. Its diagnosis is based on a clinical score (international criteria’s) including oral and genital aphthosis, neurological, ophthalmological, and vascular involvement. Cardiac involvement is a rare complication and can affect all three tunics. Coronary involvement is exceptional and can be life threatening. We report the observation of a 38-year-old man suffering from Behcet's disease who presented a myocardial infarction related to the occlusion of the proximal anterior interventricular artery.