Introduction Les myopathies inflammatoires idiopathiques (MII) sont des maladies auto-immunes rares de pronostic variable. La corrélation directe entre les marqueurs inflammatoires systémiques et les caractéristiques histopathologiques musculaires demeure peu étudiée. Objectifs Cette étude vise à évaluer la relation entre les marqueurs inflammatoires systémiques (MIS) et les données quantitatives histopathologiques afin d’identifier des indicateurs systémiques fiables de la sévérité histopathologique des MII. Méthodes Cette étude rétrospective a inclus des patients atteints de MII diagnostiqués selon les critères ACR-EULAR 2017. Les caractéristiques histopathologiques (atrophie, nécrose, ischémie, régénération, fibrose, infiltrats inflammatoires) ont été évaluées selon un score prédéfini. Ces scores, les marqueurs immunohistochimiques (CD4, CD8, CD20, CD31, CD68, anti-MxA) et les quatre domaines histologiques (fibres, inflammation, vascularisation, tissu conjonctif) [1] ont été corrélés aux MIS (VS, CRP, protéines inflammatoires, lignées sanguines). Résultats Nous avons colligé 101 patients. La VS était significativement corrélée à la sévérité de l’atrophie des fibres musculaires (p<0,05). Une inflammation musculaire plus élevée était associée à des protéines totales augmentées (p=0,036), à une hyper-alpha-2-globulinémie (p=0,029), ainsi qu’à des ratios neutrophiles/lymphocytes (p<0,001) et plaquettes/lymphocytes élevés (p=0,007). Le nombre de neutrophiles corrélait à l’inflammation périvasculaire (p=0,017). Le score du domaine inflammatoire musculaire corrélait à la CRP, aux globules blancs, aux neutrophiles et aux lymphocytes. Discussion Les données de la littérature montrent que la CRP est étroitement liée à la sévérité inflammatoire dans les myosites de chevauchement. L’hyperneutrophilie reflète l’intensité et la distribution périvasculaire des infiltrats dans notre série. Plusieurs études confirment également le rôle central des neutrophiles, notamment via la formation de pièges extracellulaires dans les lésions musculaires des MII. Conclusion Les MIS, notamment les ratios NLR et PLR, constituent de bons indicateurs de la sévérité histologique. C’est la première étude corrélant l’ensemble des MIS à des scores histologiques standardisés, incluant tous les sous-types de MII.

Systemic sclerosis is an autoimmune disorder that affects both the skin and internal organs. Pericardial abnormalities in SSc may manifest as fibrous pericarditis, pericardial adhesions, or pericardial effusion, and rarely as pericardial tamponade or constrictive pericarditis. The present literature review reports the only eight cases of constrictive pericarditis that have been reported in the literature, highlighting the rarity of this severe complication. The present report also describes another case of constrictive pericarditis complicating systemic sclerosis in a 57-year-old woman. The onset of this complication happened during the patient's follow-up, leading to heart failure.

Background/Objectives Eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Strauss syndrome, is a rare systemic vasculitis often diagnosed late due to its heterogeneous presentation, leading to severe complications—particularly cardiac involvement, a major cause of morbidity and mortality. We developed EGPA-ML, an artificial intelligence (AI)-based tool using supervised machine learning (ML), to support early and accurate EGPA diagnosis, especially in non-specialized settings. Methods A retrospective cohort of patients evaluated for suspected vasculitis at Hedi Chaker Hospital, Sfax, Tunisia, from 1997 to 2023 (nearly three decades), provided 1,904 clinical, biological, and histological features. After data cleaning, standardization, and feature selection, 56 key features were retained. Patients were classified as {EGPA} or {NOT_EGPA} per the 2022 ACR/EULAR criteria, with expert consensus (κ = 0.85). Multiple supervised ML algorithms were evaluated via 10-fold cross-validation. The best model was integrated into EGPA-ML, a Java-based clinical decision support system. Performance was assessed on an independent dataset of n = 280 key features, with reference classification {EGPA}/{NOT_EGPA} validated by experts (κ = 0.89). Results On the test and evaluation dataset, EGPA-ML achieved a recall of 0.992, precision of 0.869, and F1-score of 0.926. Feature importance analysis identified asthma and eosinophil count as top predictors (36.5% each), followed by ANCA status, vascular purpura, and histological vasculitis. Conclusions EGPA-ML is a high-performance, interpretable, and adaptive tool based on supervised ML, supporting timely EGPA diagnosis. It represents a practical advancement for clinical decision-making in rare diseases, particularly in internal medicine, pulmonology, and cardiology.

Introduction: Cerebral sinus thrombosis is an uncommon neurologic condition caused by many etiologies. Behçet’s disease (BD) is one of the leading causes in Mediterraneen and Middle Eastern countries. The aim of our study was to summarize the clinical manifestation, the therapeutic strategies and the prognosis of cerebral sinus thrombosis of BD in southern Tunisia. Patients and methods: This is a descriptive study of patients followed for BD complicated with cerebral sinus thrombosis in the department of internal medicine of Hedi Chaker Hospital in Tunisia. The study was conducted during a period of 24 years (January 1996 to December 2021). Statistical study was performed with SPSS 2022. Results: 17 patients were enrolled with a mean age of 28 years and male predominance noted in 88% of cases. The most common mode of onset was subcute in 41% of cases. 53% of patients had intracranial hypertension. Transverse sinus and longitudinal sinus were the most affected in 59% and 47% of cases. Anticoagulation was the basis of the treatment in all patients. The specific treatment was based on colchicine in all patients, corticotherapy in 14 patients and cyclophosphamide was administered in four patients with ocular manifestation. Outcome was good in all patients with no relapses or death. Conclusion: In southern Tunisia cerebral sinus thrombosis in BD is predominant in male young patients. It has a subacute presentation dominated by intracranial hypertension and the prognosis is good under anticoagulation and etiological treatment.

AA amyloidosis is a rare complication of systemic lupus erythematosus (SLE). We report a 39-year-old woman with well-controlled SLE who developed nephrotic syndrome and progressive multi-organ involvement. Evaluation revealed elevated serum amyloid A levels, proteinuria, and increased cardiac biomarkers. Echocardiography suggested cardiac infiltration. Renal and labial salivary gland biopsies confirmed AA amyloid deposition by Congo red staining and immunohistochemistry. High-dose corticosteroid therapy led to partial clinical and biological improvement; however, the patient later developed gastrointestinal involvement and rapidly progressive clinical deterioration, resulting in death. This case highlights the diagnostic challenges of AA amyloidosis in SLE and emphasizes the importance of early recognition, systematic histological confirmation, and multidisciplinary management due to its poor prognosis.

Background: Deep vein thrombosis (DVT) of the lower limbs in the elderly is a major health problem, despite improved prophylaxis and diagnostic advances. It must be managed rapidly and effectively. The aim was to determine the main features and risk factors (RFs) of DVT in elderly patients admitted to internal medicine. Methods: This was a retrospective, descriptive study carried out in the internal medicine department of Hedi Chaker Hospital in Sfax, over a period of 18 years. Records of patients hospitalized in the department during this period were reviewed. Results: There were 102 cases, divided into 64 men (62.8%) and 38 women (37.2%), with an average age of 75.2 years. DVTs in the veins of the lower limbs were most often proximal (75%). In addition to advanced age, considered an independent RF for DVT, at least one RF for VTE was found in 54.6% of cases. Bed rest was the most frequent RF, noted in 44 cases (43.7%). A thrombogenic pathology predisposing to thrombosis was retained in 25 patients, i.e., 24.5% of cases. These were SAPL (11 cases), neoplasia (12 cases), Behçet’s disease (1 case), and hyper-homocysteinemia (2 cases). Conclusion: DVT in the elderly, a frequent pathology, poses above all a problem of etiological diagnosis. Identifying the RFs for recurrence in this population is important, as it enables appropriate prescription of anticoagulants. Such treatment is not without risk in elderly patients who are frequently polythematic, with a high excess risk of bleeding.

Background The multipositivity of myositis-specific antibodies (MSAs) is uncommon. Our study aimed to assess the prevalence and the clinical relevance of multiple positive MSAs in routine practice.Methods A 10 year single-center retrospective study (2015-2024) was conducted reviewing all samples analyzed with the Dot Myositis EUROLINE (Euroimmun Germany). Clinical data corresponding to samples with multiple positive MSAs was reviewed.Results Among 140 samples positive for at least 1 MSA, 22 patients (15.7%) were positive for at least 2 MSAs on the same sample. The diagnosis of idiopathic inflammatory myopathies (IIMs) was confirmed in only 6 patients (27.2%). The frequency of double-positive MSAs was 100% in IIM patients and 60% in non-IIM patients. No IIM patients and 40% of non-IIM patients (P = .037) had 3 or more positive MSAs. MSA titers were higher in IIM patients than in non-IIM patients (49 vs 32 AU, P = .33). The clinical IIM subtype joined the IIM subtype corresponding to the predominant MSA. We had no mixed features of either IIM subtype or added severe prognosis.Conclusion Detection of multiple MSAs is uncommon but possible using immunodot techniques. Taking into account MSA number, level of positivity, and clinical data helps in the interpretation of the results.

Adult onset Still disease (AOSD) is a rare systemic auto inflammatory disease of unknown origin. It is characterized by its clinical and biological polymorphism. Eliminating differential diagnoses is one of the most important steps when the diagnosis of adult Still’s disease is suggested, given the absence of clinical or laboratory signs to support the diagnosis; adult Still’s disease is a so-called “diagnosis of exclusion”. We conducted a retrospective study to describe clinical and laboratory features, treatment, course, and complications of AOSD in 65 Tunisian patients and to compare them to the literature. All patients responded to Yamaguchi criteria. There were 52% women (80%) and 13 men (20%). The mean age at diagnosis were 36.5 years old (range: 16-70 years). Fever was constant, associated with an altered general condition in 27 patients (41.5%). The other signs were polyarthritis (63.8%), skin involvement (83.1%) with a typic rash in 66.3%, throat sore (33.8%), lymphadenopathy (31.3%), splenomegaly (25%), hepatic involvement (50%) and pericarditis (18.5%). The inflammatory biological syndrome was constant. Leukocytosis greater than 10,000 Elt/mm³ was described in 87.3% of cases with polynuclear neutrophil > 80% in 63.6%. Hyperserotonemia was observed in 56 patients (96.5%). Treatment was based on corticosteroids, as a first line treatment, and methotrexate as a second line treatment. In terms of disease course, 68.3% of cases evolved to the systemic form and 31.7% of them to the chronic articular form. AOSD is a relatively benign disease. It can be life-threatening due to its severe systemic damage and functionally damaging due to its destructive joint damage.

Background. Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are rare demyelinating disorders most often linked to the rapid correction of hyponatremia, malnutrition, or chronic alcoholism. Their occurrence in autoimmune diseases, particularly systemic lupus erythematosus (SLE), is exceptionally rare. Case presentation. We report the case of a 54-year-old woman who developed both CPM and EPM as the initial manifestations of SLE. She presented with inflammatory arthralgia, lower limb edema, and acute neuropsychiatric symptoms. Laboratory investigations revealed nephrotic-range proteinuria, positive antinuclear antibodies (ANA), positive anti-DNA antibodies, and low complement levels. Magnetic resonance imaging (MRI) demonstrated characteristic findings of both pontine and extrapontine myelinolysis. After ruling out other potential etiologies, she was treated with corticosteroids and cyclophosphamide, leading to both clinical and radiological improvement. Conclusion. This case highlights the rare but significant neurological manifestation of SLE. Early recognition and prompt immunosuppressive treatment can result in favorable outcomes, emphasizing the importance of considering CPM and EPM in SLE patients presenting with neuropsychiatric symptoms.

Hereditary haemorrhagic telangiectasia is a rare disease characterized by cutaneo-mucous and visceral arteriovenous malformations. Cardiac involvement is uncommon and was presented primarily by hyper-output heart failure. Hemorrhagic pericardial effusion, although is extremely rare, can occur during HHT. We report the case of a 48-years-old woman which was hospitalized in 2015 in internal medicine department, Sfax, Tunisia in 2015 for anicteric cholestasis. She noticed a personnel and familial history of recurrent epistaxis. Biologic findings revealed anemia and moderate cholestasis. Viral investigations and immunologic tests were negative. Abdominal tomography showed multiple arterio-venous shunts of the liver. Liver involvement due to Rendu Osler Weber disease was retained. She was treated by ferrous iron, but she was lost to follow up. She was presented in February 2021, with severe anemia (5 g/dL). Physical examination revealed signs of global heart failure. Biological investigations found anemia, inflammatory biological syndrome, cytolysis and cholestasis. Heart ultrasound revealed an abundant pericardial effusion. Only 500cc of hemorrhagic fluid could be aspirated before the needle became blocked. Unfortunately, one week after, re-accumulation of pericardial fluid and worsening occurred. She underwent a partial surgical pericardial excision with pleuropericardial opening. Analysis of the fluid ruled out any infectious cause of this effusion. Histological examination confirmed the vascular dysplasia with signs of hemorrhage and inflammation. The patient was discharged 1 month after surgery with no other bleeding episodes. For her anemia, she received a transfusion of red blood cells. Then, the patient was treated by iron treatment.

Après un premier épisode de thrombose veineuse profonde non provoquée, le risque de récidive persiste pendant de nombreuses années.Un traitement anticoagulant à long terme prévient la récidive des thromboses veineuses mais est associé à un risque hémorragique majeur.Comme les plaquettes jouent également un rôle dans l'initiation et la formation du thrombus dans la maladie veineuse thrombo-embolique (MVTE), les anti-agrégants plaquettaires peuvent également jouer un rôle dans le traitement et la prévention de cette maladie.Cette mise au point résume les données de la littérature sur l'effet de l'aspirine dans la prévention des récidives de la thrombose veineuse profonde.