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).
Behçet's disease is a systemic vasculitis characterized by recurrent bipolar aphtosis and ophthalmic disorders. Cardiac involvement is rarely reported and could be associated to poor prognosis. Intracardiac thrombosis is exceptional and represents a therapeutic issue.
View Article and Find Full Text PDFIntroduction: Castleman's disease (CD), known as angiofollicular lymph node hyperplasia, is an uncommon condition. The two most common histological subtypes are hyaline vascular and plasma cell. We performed a retrospective analysis to define the clinic-pathological features and survival of CD, which is quite rare focusing on the particularities of our series with a review of the recent literature.
View Article and Find Full Text PDFMelkersson-Rosenthal syndrome is a rare condition of unknown etiology. It is characterized by a classical triad of symptoms: relapsing facial and lip swelling, facial palsy and a fissured tongue. We report the case of a 29-year-old female patient who presented with the above-mentioned symptoms of Melkersson-Rosenthal syndrome.
View Article and Find Full Text PDFAcquired hemophilia A (AHA) is a potentially life-threatening hemorrhagic disorder with many etiologies. We report the first case in the literature describing the association of AHA with adult-onset Still's disease (AOSD).
View Article and Find Full Text PDFUnlabelled: Rosai-Dorfman disease (RDD) is an uncommon lymphoproliferative disorder; RDD with oropharyngeal involvement is extremely rare, especially in adults. A 65-year-old woman with a complaint of progressive dyspnoea since 2016 presented with laryngeal involvement of RDD. A laryngoscopy examination revealed two solid, polypoid masses in the subglottic region, and a laryngeal biopsy concluded chronic inflammation without signs of malignancy.
View Article and Find Full Text PDFGuillain-Barré syndrome (GBS) and polymyositis (PM) are two rare autoimmune diseases, one of which affects the peripheral nervous system and other the muscle. We report the case of a young woman with no previous medical history who was hospitalized with an ascending paralysis associated with acute respiratory failure due to a GBS. The patient was treated with plasmapheresis with unfavorable outcome and permanent proximal muscular disability.
View Article and Find Full Text PDFMonaldi Arch Chest Dis
November 2022
One of the most common interstitial lung diseases in antisynthetase syndrome is nonspecific interstitial pneumonia (NSIP). A 49-year-old woman presented with slow progression exertional dyspnea, myalgia, and arthralgia. The radiological findings indicated an NSIP pattern.
View Article and Find Full Text PDFAgranulocytosis is a rare acute condition characterized by severe a < gft (neutropenia in which the neutrophils count is less than 100/mm. It can be classified into two categories, inherited, and acquired. Acquired agranulocytosis is not commonly caused by auto-immune diseases such as systemic lupus erythematosus (SLE).
View Article and Find Full Text PDFPulmonary artery aneurysm must be evoked in front of any hemoptysis in a patient with Behçet disease as it requires urgent immunosuppressive therapy and often surgery.
View Article and Find Full Text PDFJ Steroid Biochem Mol Biol
July 2022
Clinical, genetic, and medical evidence has shown the inflammatory vasculitis aspect of Behçet's Disease (BD). Whereas oxysterols are vital factors in inflammation and oxidative stress, it is still unknown whether they are involved in the pathophysiology of BD. The current study aims to explore the profile of oxysterols in plasma of BD patients.
View Article and Find Full Text PDFUnlabelled: Hughes-Stovin syndrome is a very rare condition with no defined diagnostic criteria. We present the case of a 26-year-old man who had haemoptysis revealing Hughes-Stovin syndrome. We will consider the aetiology, therapeutic and evolutionary aspects of this disease.
View Article and Find Full Text PDFDiffuse infiltrate lung diseases (DILDs) are frequent in patients with connective tissue diseases. They can be suggestive of connective tissue diseases or occur during follow-up. Antisynthetase syndrome (ASS) is a complex and heterogeneous connective tissue disease.
View Article and Find Full Text PDFSarcoidosis Vasc Diffuse Lung Dis
June 2021
Background: Sarcoidosis is a systemic granulomatous disease of unknown etiology. It affects mostly young adults. In the elderly, the presentation of this disease is different, often posing positive diagnosis problems.
View Article and Find Full Text PDFPure extra-thoracic sarcoidosis is rare and poorly characterized in the literature. Herein we present features of pure extra-thoracic sarcoidosis, in comparison with sarcoidosis with intra-thoracic involvement, in a population from the south of Tunisia. Retrospective, descriptive and comparative study of the patients presenting systemic sarcoidosis and attending the Department of Internal Medicine at the Hedi Chaker University Hospital, Sfax, Tunisia, for 21 years, from January 1996 to December 2016.
View Article and Find Full Text PDFHenoch-Schonlein purpura (HSP) is a small-vessel vasculitis secondary to IgA deposits. We conducted a retrospective study of 14 cases of HSP in adults based on EULAR/PRINTO/PRES classification criteria for HSP over a period of 18 years (1996 to 2014) in Department of Internal Medicine at the Hedi Chaker University Hospital, Sfax, South Tunisia. The purpose of our study was to highlight the epidemiological, clinical, therapeutic and evolutionary features of HSP in our adult patients.
View Article and Find Full Text PDFAdult-onset Still's disease (AOSD) has been recognized as a cause of fevers of unknown origin. Malignancies are the most important differential diagnoses of AOSD which has been rarely reported in association with cancer. The present paper undertakes the study of a 69-year-old Tunisian woman with AOSD according to the diagnostic criteria of Yamaguchi.
View Article and Find Full Text PDFIntroduction: The prognosis of dermatomyositis (DM)/ polymyositis (PM) in adults is partly related to their association with neoplasia. The aim of our study was to report the epidemiologic, clinical, paraclinical, therapeutic and evolutionary aspects of DM associated with malignancy in patients from Sfax, south eastern of Tunisia.
Methods: A retrospective cohort study of patients with DM or PM admitted in Dermatology and Internal Medicine Departments of Hedi Chaker University Hospital of Sfax between 1996 and 2015.
Background: Multiple sclerosis and other demyelinating processes are sometimes difficult to differentiate from the neurological involvement in autoimmune diseases. Distinguishing multiple sclerosis from other lesions due to autoimmune diseases is crucial to avoid unsuitable or delayed treatments.
Methods: Charts of 6 patients diagnosed with mimicking multiple sclerosis between 1996 and 2014 were retrospectively assessed.
Objective: The aim of this work was to establish human leukocyte antigen (HLA) class I and hereditary hemochromatosis gene (HFE) mutation associations with recurrent aphthous oral ulcers (RAOU) and Behçet disease (BD) in a cohort of Southern Tunisian patients.
Subjects And Methods: A total of 232 patients with RAOU and 123 healthy controls (HCs) were enrolled in this study. The patients were divided into 2 groups based on the presence (BD+: n = 62) or absence of BD (BD-, n = 170).
Tuberous sclerosis or tuberous sclerosis complex (TSC) is an autosomal dominant inherited neurocutaneous disorder that variably affects the brain, skin, kidneys, heart, and other organs. It is characterized by skin and renal lesions in addition to central and peripheral nervous system tumors, with neurological and psychiatric findings. We report such a rare case of tuberous sclerosis in a 25-year-old female who presented with abdominal pain and hypertension.
View Article and Find Full Text PDFA 48 years-old-woman was admitted for anectric cholestasis. A history of recurrent personal and familial epistaxis was noted. Biologic findings revealed iron deficiency anemia and moderate cholestasis.
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