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Background/Aims Behçet’s syndrome (BS) is a multi-systemic vasculitis highly prevalent in a broad range stretching from the Mediterranean region to eastern Asia. The vascular involvement, present in up to 40% of the cases, is characterised by a high tendency towards the venous system and thrombosis. Although pulmonary artery aneurysms (PAA) are considered to be rare (less than 5% in cohorts), they are a quasi-pathognomonic sign of BS and play a major role in patient’s morbidity and long-term mortality. Our work aims to depict the clinical profile, imaging features, therapeutic management, and outcomes of BS patients presenting with PAA. Methods We conducted a retrospective, descriptive and monocentric study including 531 BS patients admitted to the internal medicine department between 2000 and 2021. All patients fulfilled the International Study Group Criteria for Behçet’s disease. We enrolled all cases with documented PAA on CT angiography of the chest (CTA chest). Results Among 531 BS patients, 51 had PAA (10%). The mean age was 36 ± 11 [20-69] and the sex ratio (M:F) was 4:1. Cardiovascular risk factors comprised chronic tobacco consumption (13.5%) and high blood pressure (9.6%). PAA occurred after a median of 5.5 years following the disease onset. Patients were either asymptomatic (78%) or presented with hemoptysis (6%), dry cough (6%), chest pain (4%), and dyspnea (2%). On CTA chest, PAA were commonly multiple (78%), proximal (46%), and bilateral (29%). Concomitant arterial manifestations were observed in 25% of the patients, consisting of peripheral arteries aneurysms (17%), pulmonary artery thrombosis (7.7%), cerebral artery thrombosis (4%), and aortic aneurysms (2%). Venous thrombosis was also simultaneously found in 21% of the cases, observed in the superior (9.6%) and inferior vena cava (9.6%), cerebral veins (7.7%), and lower extremity veins (6%). On echocardiography, right intracardiac thrombosis was visualised (17%). The other extravascular involvements were mainly mucocutaneous (73%), ocular (46%), articular (29%), and neurological (10%). Our patients were treated with colchicine (100%) and an initial pulse of methylprednisolone (15mg/kg/day for three days), followed by oral prednisolone (1 mg/kg/day), gradually tapered (94%). The immunosuppressive therapy administered consisted of cyclophosphamide (38.5%), azathioprine (31%), and infliximab, a TNF alpha inhibitor (2%). Curative anticoagulation was prescribed in 21% of the cases. Only one patient underwent surgery. Relapse and death were recorded in respectively 15% and 6% of the cases. Conclusion PAA are a very unique feature of BS, with specific characteristics related to their inflammatory pathogenesis. We highly recommend evoking the diagnosis of BD in presence of PAA, especially in young male patients presenting with other vascular lesions and intracardiac thrombosis. The prompt initiation of anti-inflammatory and immunosuppressive therapy is of paramount importance to reduce the risk of massive hemorrhage from PAA rupture. Disclosure S. Chadli: None. H. Khibri: None. S. Fari Firdaous: None. N. Moatassim: None. W. Ammouri: None. M. Maamar: None. H. Harmouche: None. M. Adnaoui: None. Z. Tazi Mezalek: None.

Background/Aims Amyloidosis is a broad spectrum of heterogeneous diseases, characterized by the accumulation and extracellular deposit of misfolded proteins, resulting in organs’ thickening and dysfunction. Cardiac amyloidosis (CA) is common and represents an important key factor in patients’ prognosis. Our study aims to depict the profile of patients with CA, so as present their therapeutic management and outcomes. Methods We retrospectively carried out a descriptive study between 2015 and 2022, including all patients admitted to our department for systemic amyloidosis (SA) with confirmed cardiac involvement on echocardiography (TTE) and/or cardiac magnetic resonance (CMR). Results 31 patients were enrolled. The sex ratio (M/F) was 0.93 and the mean age was 62 ± 15 years (26-86). Past history included multiple myeloma (n = 7), monoclonal gammapathy of undetermined significance (n = 1), T lymphoma (n = 1), and systemic amyloidosis (n = 1). Cardiovascular risk factors comprised high blood pressure (25%), type 2 diabetes (15%), dyslipidemia (9%), and smoking (8%). Patients presented after a mean of 12 ± 4 months since the onset, with peripheral edema (68%) and other signs of right (53%) and global heart failure (19%). Concomitant extracardiac involvements were mainly renal (55%), mucocutaneous (20%), and neurological (16%). Electrocardiogram showed low-voltage (41%), poor R-wave progression (23%), atrial fibrillation (10%), atrioventricular block (6%), right bundle branch block (6%), left bundle branch block (6%), junctional tachycardia (6%), and left ventricular hypertrophy (LVH) (6%). On TTE, the features observed were the altered longitudinal global strain (93%) with a bull’s eye pattern (90%), LVH (90%), valvular and septal hypertrophy (88%), sparkling myocardium (65%), pericardial effusion (54%), diastolic dysfunction (37%), dilated cavities (35%), pulmonary hypertension (29%), and aortic stenosis (3%). Systolic function was mostly conserved (73%), at a mean of 53 ± 10%. The most recurrent signs on CMR were: late enhancement (90%), LVH (80%), and abnormalities in myocardium nulling (25%). Cardiac biomarkers included a Troponin of 0.35 ng/ml [0.13; 506] and BNP of 4895 pg/ml [1588; 13 224]. BS showed positive cardiac fixation (grade 2 or 3) in six cases, confirming ATTR-CA. Peripheral biopsies, serum and urine immunofixation, free light chains assays, and medullar investigations demonstrated AL in 23 patients. Two cases of AA were confirmed on immunohistochemistry, complicating sarcoidosis (n = 1) and ankylosing spondylitis (n = 1). Patients were treated with chemotherapy (n = 11), Tafamidis (n = 3), and Infliximab (n = 2). The outcomes documented were complete remission (n = 1), partial remission (n = 3), and death (n = 9) with a median survival of 7 months. Conclusion Our study highlights the grim prognosis still carried by CA patients. Although increased awareness is noticed among physicians, the disease is often discovered at a late stage or even overlooked. Further understanding of CA and prompt screening of at-risk patients are thus crucial for early diagnosis and management, in order to improve patients' outcomes. Disclosure S. Chadli: None. M. Maamar: None. H. Khibri: None. N. Moatassim: None. W. Ammouri: None. H. Harmouche: None. M. Adnaoui: None. Z. Tazi Mezalek: None.

Background/Aims Behçet’s disease (BD) is a relapsing vasculitis that can affect vessels of all sizes and kinds. Intracardiac thrombosis (ICT) is a rare but serious complication which often presents with vascular lesions. Yet, the link between these manifestations is not fully clarified. Our study aims to describe the profile, therapeutic management, and outcomes of BD patients with ICT. Secondly, it aims to shed some light on the relationship between ICT and vascular involvement in BD. Methods We retrospectively conducted a descriptive and monocentric study, including 531 Behçet’s patients admitted to our department between 2010 and 2022. All patients selected fulfilled the criteria of the International Study Group of Behçet’s Disease. Results Twelve patients were enrolled (2%). The sex ratio (M:F) was 9:1 and the mean age was 29 ± 6 years (20-41). ICT occurred as the revealing form of BD (50%), or after a median of 4 years since the disease onset (50%). The main symptoms were dyspnea (58%), chest pain (58%), hemoptysis (41%), and dry cough (33%). Concurrent BD flares were mainly mucocutaneous (80%). Inflammatory parameters were elevated with a median ESR of 48 mm/h and CRP of 52 mg/L. High levels of BNP and troponin were noted in 58% and 16% of the cases. Hemostasis parameters were within the normal range. On transthoracic echocardiogram, ICT was located in the right (100%), and left cavities (20%), with an extension to the inferior vena cava in half of the cases (50%). Multiple thrombi were visualized (n = 4). The associated cardiac lesions were tricuspid insufficiency (50%), pericardial effusion (41%), mitral insufficiency (16%), endomyocardial fibrosis (8%), myocarditis (8%), and ischemic coronaropathy (8%). Pulmonary hypertension (58%) and right dilatation (50%) were common. Concomitant pulmonary artery involvement was frequent (83%), consisting of thrombosis (70%) and aneurysms (50%). Six patients had a past history of lower extremity DVT, either as a single episode (n = 4) or recurrent episodes (n = 2). Vascular thrombosis was also found in the superior vena cava (n = 2), suprahepatic veins (n = 2), internal jugular vein (n = 1), pulmonary veins (n = 1), cerebral vein (n = 1), superior mesenteric vein (n = 1), and femoral artery (n = 1). Patients were treated with colchicine (100%), glucocorticoids (100%), and immunosuppressants (92%), mainly cyclophosphamide (n = 11), and a TNF-alpha inhibitor (n = 2). Six patients were under curative anticoagulation. A good response to therapy was observed in all patients. One case of relapse was reported. No deaths were recorded. Conclusion ICT usually occurs during the first years of BD onset in young male patients presenting with vascular lesions, especially of the pulmonary arteries and vena cava. We thus believe that, unlike common thrombotic diseases, ICT in BD is a direct consequence of the underlying vasculitis. The use of immunosuppressants, with or without anticoagulants, is generally associated with a favorable outcome. Disclosure S. Chadli: None. H. Khibri: None. S. Fari: None. N. Moatassim: None. W. Ammouri: None. M. Maamar: None. H. Harmouche: None. M. Adnaoui: None. Z. Tazi Mezalek: None.

Background/Aims Behçet’s syndrome (BS) is a complex inflammatory disorder frequently observed in the so-called “Silk Road”. Venous thrombosis (VT) is common in BS and known to be a predictive factor of future severe manifestations of the disease. Our work aims to describe the clinical and imaging characteristics of VT in BS, in order to shed the light on its specific features for early diagnosis and management of these patients. Methods We conducted a retrospective, descriptive and monocentric study, including 200 BS patients admitted to our internal medicine department between 2014 and 2021. All patients fulfilled the International Study Group criteria for Behçet’s disease. Results VT was found in 62 patients (31%). The mean age was 29 ± 10.8 (18-61) and the sex ratio (M:F) was 7.85:1. Family history of BS was documented in 5% of the cases. VT was either the revealing manifestation of the syndrome (56%) or occurred after a median of 3.5 years (1; 6.5) following the disease onset. Four patients had superficial venous thrombosis (SVT) mostly affecting the superficial femoral vein (98%). All patients had deep vein thrombosis (DVT), visualised in the lower extremities (58%), superior vena cava (21%), inferior vena cava (16%), cerebral veins (13%), and suprahepatic veins (5%). Atypical sites of DVT occurred in the renal (n = 1), axillary (n = 1), central retinal (n = 1), and dorsal penile (n = 1) veins. DVT was observed in one vein (55%), two veins (27%), or multiple (3 to 7) veins (16%). Thrombi sizes ranged from 10 to 40 mm, being willingly extended (55%) and strongly adherent to the vessel walls (95%). Concomitant arterial lesions were found in 34% of the patients, mainly in the pulmonary arteries (57%). They consisted of aneurysms (52%), thrombosis (43%), stenosis (17%), and pseudoaneurysms (9%). Cardiac lesions were also observed (11%), including right intracardiac thrombosis (n = 4), coronary artery thrombosis (n = 2), pericarditis (n = 1), and myocarditis (n = 1). The other extravascular involvements associated were mucocutaneous (100%), ocular (42%), neurological (29%), articular (24%), and digestive (23%). Fever was reported in 4 cases. All patients were treated with colchicine and glucocorticoids. Immunosuppressive therapy was prescribed in 75% of the cases: cyclophosphamide (n = 31), azathioprine (n = 12), and TNF alpha inhibitor (n = 3). Curative anticoagulation was also administered (98%). Two patients underwent surgery: thrombectomy (n = 1) and embolectomy (n = 1). Relapse occurred in 27% of the patients. No death was recorded. Conclusion VT is widely considered to be the hallmark of BS as well as a major risk factor for future severe vascular and cardiac involvements. Due to its inflammatory pathogenesis, it has specific clinical and imaging features. Better knowledge of these characteristics could help early establish the diagnosis of BS, thus improving patient outcomes. Disclosure S. Chadli: None. H. Khibri: None. R. Benachour: None. S. Firdaous Fari: None. N. Moatassim: None. W. Ammouri: None. M. Maamar: None. H. Harmouche: None. M. Adnaoui: None. Z. Tazi Mezalek: None.

We suspect a mediastinal relapse of the cancerous pathology when a thymic hyperplasia occurs in patients with complete remission post chemotherapy. In this article, we report the case of a thymic hyperplasia that appeared six months after the end of chemotherapy in a 31-years-old patient followed for stage IV T-cell lymphoma treated with CHOEP chemotherapy. The PET scan (Positron Emission Tomography) objectified a complete metabolic remission with the disappearance of the thymic hyperplasia

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) accounts for lessthan 1% of all Lymphomas that manifest themselves with sub cutaneous nodes, SPTCL may be associated to a systemic disease. We report in this paper the first case described in our department of a 57 years old female patient followed up for 5 years for a Subcutaneous panniculitis-like T-cell lymphoma, treated with dermocorticosteroids after 4 years of therapeutic abstention and who developed a Systemic lupus erythematosus for which she was put under treatment with a very good evolution.

Pernicious anaemia (PA) is an autoimmune disease of multifactorial aetiology involving environmental and immunological factors. It is the most common cause of cobalamin deficiency anaemia worldwide. The disease is a macrocytic anaemia caused by a vitamin B12 deficiency, which, in turn, is the result of intrinsic factor deficiency, a protein that binds avidly to dietary vitamin B12 and promotes its transport to the terminal ileum for absorption. Despite the advances in understanding the pathogenesis and molecular biology, diagnosis of PA is still challenging for clinicians because of its complexity, diverse clinical presentations, and the limitations of the available diagnostic tools for the evaluation of cobalamin status and the presence of chronic autoimmune atrophic gastritis. Asymptomatic autoimmune gastritis, a chronic inflammatory disease of the gastric mucosa, precedes the onset of corpus atrophy by 10–20 years. Diagnostic dilemmas could occur when patients with PA present with spuriously normal or high cobalamin levels, normocytic or microcytic anaemia, nonanaemic macrocytosis, autoimmune haemolytic anaemia, pseudo-thrombotic microangiopathy, hyperhomocysteinemia-associated thromboembolism, pseudoleukemia, bone marrow failure, and neurologic manifestations without anaemia or macrocytosis. Other autoimmune disorders, especially thyroid disease, Type 1 diabetes mellitus, and vitiligo, are also commonly associated with PA. The present review focusses on novel aspects regarding the pathogenesis, clinical presentation, and the diagnostic approach of PA; the true usefulness of serum vitamin B12 levels; and the risk of adenocarcinoma and gastric carcinoids as well as their treatment and monitoring strategies.

Behçet disease is a multi-systemic complex vasculitis with unknown etiology characterized by different clinical involvements, including mucocutaneous, ocular, vascular, articular, neurological, and gastrointestinal manifestations. Growing evidence supports that different phenotypes, characterized by clusters of co-existing involvements, can be distinguished. Namely, the vascular phenotype identifies a specific group of patients who suffer from recurrent inflammatory thrombosis and arterial involvement. Vascular disease develops in up to 40% with a definite male preponderance and is usually an early manifestation. Venous involvement is significantly more common than arterial disease, and lower extremity deep vein thrombosis is its most frequent manifestation. Arterial disease involves mostly pulmonary arteries and aorta and manifests mainly in the form of aneurysms. Glucocorticoids and immunosuppressants are the recommended first-line treatments in vasculo-Behçet. Furthermore, controlled trials are still needed to assess the role of adding anticoagulation to the treatment regimen, with an accent on new oral anticoagulants. Treatment with anti-TNF alpha agents seems promising, but the management strategies are not clear yet.

Background & objectives: Rickettsioses are zoonoses transmitted to humans by arthropods. They are due to strict intracellular bacteria belonging to the family Rickettsiaceae. Our purpose is to present the clinical and paraclinical characteristics of 14 new cases diagnosed in Al-Hoceima region, Morocco; indeed, the patients associated a diagnosis of Mediterranean Spotted Fever (MSF). Results: The average age of patients was 55 years. The patients were hospitalized for infectious syndrome, renal deficiency, pneumonia, and suspected meningitis. All cases had a general papular rash with palmo-plantar involvement, 12 out of 14 patients showed an escarotic spot, while neurological disorders were observed in 2 patients. Ophthalmic involvement was represented by retinal vasculitis in a single patient. Thrombocytopenia and cytolysis were constant in all patients. Renal deficiency was found in 3 cases and 2 cases had interstitial syndrome. The serology was positive in only one patient. All cases had been treated with doxycycline 200mg/day for 7 days with a good improvement of the clinical and biological symptoms. Interpretation & conclusion: The frequency of MSF in Morocco is not completely elucidated. A good knowledge of the clinical form allows an early diagnosis in order to institute an effective treatment.

Diffuse peritoneal leiomyomatosis is a benign peritoneal tumor which develops from smoother muscular fibers. It is a rare entity in females undergoing hormonal effects. This pathological entity is exceptional in male patients. Hence, we report a unique case of diffuse peritoneal leiomyomatosis that occurred in a male patient without hormonal stimulations. The etiopathology and genesis are not completely elucidated. Besides, the clinical symptoms are not specific. The positive diagnosis is based on sets of imaging argument. The anatomicopathological studies allow confirming the diagnosis and reject any tumoral origin. The evolution of the pathology is often favorable. The malignant transformation is exceptional. The treatment is based on abolishing any of the hormonal stimulations. The surgical exeresis is indicated in case of higher peritoneal mass. In case of recurrence or progression, luteinizing hormone-releasing hormone analogs or surgical castrations are indicated.

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La spondylarthrite ankylosante est un rhumatisme inflammatoire chronique,qui fait partie des groupes de spondyloarthrites, au cours duquel les signes généraux comme la fièvre et l'amaigrissement sont peu importants. La maladie de Still de l'adulte est une affection systémique rare, qui reste un diagnostic d'élimination, et qui associe classiquement une fièvre élevée hectique, une éruption cutanée, des arthrites, et des manifestations systémiques diverses. Peu de cas de spondylarthrites ankylosantes présentés avec un tableau de la maladie de Still de l'adulte ont été décrits dans la littérature. Nous rapportons un nouveau cas d'un patient de 31ans suivi pour spondylarthrite ankylosante et qui se présente avec une fièvre au long cours et des signes clinico-biologiques entrant dans le cadre de la maladie de Still de l'adulte. Un éventuel lien physiopathologique entre les deux pathologies peut être évoqué, même si leur survenue simultanée est rarement rapportée dans la littérature.

Intravascular large B-cell lymphoma (IVLBCL) remains a diagnostic challenge, because of non-specific findings on clinical, laboratory, and imaging studies. We present a case in which 18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET)/computed tomography was particularly useful to suspect the diagnosis, to detect unexpected locations, to guide contributive biopsy, and to assess the response to treatment. In case of initial negative results, FDG-PET should be repeated in the course of clinical evolution. In the presence of neurological or hormonal symptoms without brain magnetic resonance imaging abnormality, FDG-PET brain slices could depict additional pituitary and/or brain hypermetabolisms. We discuss the potential interests of FDG-PET in IVLBCL by a literature review.

Intravascular large B-cell lymphoma (IVLBCL) remains a diagnostic challenge, because of non-specific findings on clinical, laboratory, and imaging studies. We present a case in which 18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET)/computed tomography was particularly useful to suspect the diagnosis, to detect unexpected locations, to guide contributive biopsy, and to assess the response to treatment. In case of initial negative results, FDG-PET should be repeated in the course of clinical evolution. In the presence of neurological or hormonal symptoms without brain magnetic resonance imaging abnormality, FDG-PET brain slices could depict additional pituitary and/or brain hypermetabolisms. We discuss the potential interests of FDG-PET in IVLBCL by a literature review.

Introduction Subacute combined degeneration of the spinal cord is a rare cause of demyelination of the dorsal and lateral columns of the spinal cord and is a neurological complication of vitamin B12 deficiency. Subacute combined degeneration without anemia or macrocytosis is rare. Case presentation We present a case of cobalamin deficiency in a 29-year-old Moroccan woman who presented with subacute combined degeneration without evidence of anemia or macrocytosis. Magnetic resonance imaging of the spinal cord demonstrated abnormal hyperintense signal changes on T2-weighted imaging of the posterior and lateral columns from the medulla oblongata to the thoracic spine. A diagnosis of subacute combined degeneration of the spinal cord was considered and confirmed by low serum cobalamin. The patient was treated with vitamin B12 supplements and showed improvement in her clinical symptoms. Conclusion Physicians should diagnose subacute combined degeneration in patients early by having a high index of suspicion and using diagnostic tools such as magnetic resonance imaging.

Antiphospholipid syndrome (APS) is an autoimmune disorder with combination of at least 1 clinical and 1 laboratory criterion as defined by the SAPPORO statement. Clinical criteria result from vascular thrombosis that can affect artery, venous, or small vessel in any tissue or organ. Arterial stenosis is a rare lesion involved in APS, affecting mainly renal or intracranial arteries. We reported a case of a 33-year-old woman with abdominal angina and high blood pressure (BP). Imaging showed tight, not calcified, and hypodense stenosis of mesenteric superior artery and left renal artery, and a thrombosis of the celiac trunk. Treatment was digestive rest followed by angioplasty and stenting of mesenteric and renal artery, anticoagulation, antiplatelet, and statin therapy. Normal BP and digestive function were obtained postoperatively. Biological tests showed a positive lupus anticoagulant at diagnosis and at 12 weeks, which allowed us to make the diagnosis of APS. Physiopathology of stenosis in APS remains unclear but suggests arterial wall partial thrombosis, accelerated atherosclerosis, and/or proliferation of smooth muscle cells. We recommend screening of arterial stenosis in patients with APS and arterial symptoms, and inversely, searching for APS in young patients with atypical arterial stenosis to allow optimal therapy.