Publications by authors named "Mathian A"
Objectives: Lymphadenopathy is a classical manifestation of systemic lupus erythematosus (SLE) flare, occurring in approximately half of patients during the course of the disease. Lymphadenopathy in SLE is frequently associated with fever. Microbial infection may play a role in SLE onset and flares.
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- Onconephrology is a growing field focused on improving care for patients dealing with both cancer and kidney diseases, emphasizing the need for detailed kidney histology data.
- A retrospective analysis of 154 cancer patients who underwent kidney biopsies showed high rates of complications like proteinuria and kidney injury, with significant histological findings such as interstitial fibrosis and tubular atrophy.
- The most common cause of kidney issues was found to be nephrotoxicity from anticancer drugs, with many patients having multiple diagnoses, indicating the complex nature of kidney damage in cancer treatment.
Because Systemic Lupus Erythematosus (SLE) is a rare disease, and due to the significant prognostic impact of early management, a diagnosis confirmed by a physician with experience in SLE is recommended, for example from an expert center. Once the diagnosis is confirmed, existing manifestations should be identified in particular, renal involvement by an assessment of proteinuria, disease activity and severity should be determined, potential complications anticipated, associated diseases searched for, and the patient's socioprofessional and family context noted. Therapeutic management of SLE includes patient education on recognizing symptoms, understanding disease progression as well as when they should seek medical advice.
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- Severe thrombotic antiphospholipid syndrome (APS) significantly affects critical organs and has not been thoroughly studied in ICU patients, particularly regarding hematological issues.
- A retrospective study in France tracked 134 APS patients in ICUs, revealing high rates of anemia (95%) and thrombocytopenia (93%), with low platelet counts linked to increased mortality.
- The findings highlight the importance of understanding the causes of thrombocytopenia in APS patients, as conditions like thrombotic microangiopathy (TMA), heparin-induced thrombocytopenia (HIT), and disseminated intravascular coagulation (DIC) are prevalent in this population.
Objective: Previous studies have provided evidence that the discontinuation of hydroxychloroquine (HCQ), and chloroquine (CQ), in patients with systemic lupus erythematosus (SLE) is associated with an increased risk of disease flares, with limited information on the level of disease activity at the time of HCQ/CQ discontinuation. Here we aimed to describe the risk of SLE flare after withdrawal of HCQ or CQ in patients with SLE in remission.
Methods: Case-control study (1:2) comparing the evolution of patients with SLE after HCQ/CQ withdrawal for antimalarial retinopathy (cases) with patients with SLE matched for sex, antimalarial treatment duration and age at SLE diagnosis, whose antimalarial treatment was continued throughout the entire follow-up period (controls).
Objectives: Vacuoles, E1 enzyme, X-linked, autoinflammatory and somatic (VEXAS) syndrome is an adult-onset autoinflammatory disease associated with somatic ubiquitin-like modifier-activating enzyme 1 (UBA1) mutations. We aimed to evaluate the efficacy and safety of targeted therapies.
Methods: Multicentre retrospective study including patients with genetically proven VEXAS syndrome who had received at least one targeted therapy.
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- Patients with X linked agammaglobulinemia and those receiving anti-CD20 monoclonal antibodies (mAbs) for immune-mediated inflammatory diseases (IMIDs) are at increased risk for severe enterovirus (EV) infections, particularly meningoencephalitis.
- A study collected data from nine original cases and 17 previously published cases, revealing a high occurrence of meningoencephalitis (81%) and a mortality rate of 27% among affected patients treated with multiple anti-CD20 mAbs.
- The findings suggest that clinicians should consider EV infections in IMID patients presenting unusual symptoms and recognize that anti-CD20 mAbs can impair B-cell responses to EV infections, potentially indicating
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- - Relapsing polychondritis is an autoimmune disease primarily affecting cartilage, causing inflammation and deformation through periods of flare-ups and remission.
- - The condition severely impacts auricular and nasal cartilage, and can also involve the tracheobronchial and cardiac areas, making diagnosis complex due to overlapping symptoms with other inflammatory diseases.
- - Recent advancements in diagnostic methods and treatments, including immunosuppressants and targeted therapies, have significantly improved the prognosis for patients with this condition.
Purpose Of The Review: Antiphospholipid syndrome (APS) is a rare systemic autoimmune disorder that can escalate into a 'thrombotic storm' called the catastrophic antiphospholipid syndrome (CAPS), frequently requiring ICU admission for multiple organ failure. This review aims to offer insight and recent evidence on critically-ill APS patients.
Recent Findings: The CAPS classification criteria define this condition as the involvement of at least three organs/systems/tissues within less than a week, caused by small vessel thrombosis, in patients with elevated antiphospholipid antibodies levels.
Background: Long-term hydroxychloroquine (HCQ) or chloroquine (CQ) intake causes retinal toxicity in 0.3-8 % of patients with rheumatic diseases. Numerous risk factors have been described, eg, daily dose by weight, treatment duration, chronic kidney disease, concurrent tamoxifen therapy and pre-existing retinal or macular disease.
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- The study investigates the characteristics of systemic lupus erythematosus (SLE) in patients also living with human immunodeficiency virus (HIV) and compares these characteristics to those in SLE patients without HIV.
- Researchers analyzed data from 13 SLE patients with HIV, noting a median diagnosis age of 43, with 77% being women, and identifying common symptoms such as joint pain and skin issues.
- The findings reveal that SLE patients with HIV exhibit more kidney problems and autoimmune blood issues compared to those without HIV, highlighting a rare but significant relationship between these conditions.
Article Synopsis
- Cutaneous lupus erythematosus (CLE) can occur on its own or alongside systemic lupus erythematosus (SLE), where lab tests show specific abnormalities.
- This study aimed to compare isolated CLE to CLE with lab-confirmed SLE and to validate a scoring system predicting the risk of developing severe SLE (sSLE).
- Results indicated that patients with CLE and laboratory SLE had a significantly higher risk of progressing to sSLE compared to those with isolated CLE, though the study had limitations due to its small, single-center patient sample.
Objectives: Sarcoidosis is a multisystemic granulomatosis diagnosed mainly in young adults.18F-fluorodeoxyglucose (18F-FDG) positron emission tomography-computed tomography (PET-CT) is useful in sarcoidosis cases to search for a biopsiable site or assess disease activity.18F-FDG PET-CT can reveal bone hypermetabolism in sarcoidosis patients, even in the absence of osteoarticular symptoms.
View Article and Find Full Text PDFAntiphospholipid syndrome (APS) is a chronic autoimmune disease involving vascular thrombosis and/or obstetric morbidity and persistent antibodies to phospholipids or certain phospholipid-associated proteins. It is a rare condition in adults and even rarer in children. The diagnosis of APS can be facilitated by the use of classification criteria based on a combination of clinical and biological features.
View Article and Find Full Text PDFBackground: Patients with systemic lupus erythematosus (SLE) exhibit a high risk for cardiovascular diseases (CVD) which is not fully explained by the classical Framingham risk factors. SLE is characterized by major metabolic alterations which can contribute to the elevated prevalence of CVD.
Methods: A comprehensive analysis of the circulating metabolome and lipidome was conducted in a large cohort of 211 women with SLE who underwent a multi-detector computed tomography scan for quantification of coronary artery calcium (CAC), a robust predictor of coronary heart disease (CHD).
Glucocorticoids (GCs) remain a cornerstone of the treatment of Systemic Lupus Erythematosus (SLE). Numerous studies have emphasized the risk of damage accrual in SLE patient treated with GC, but currently, it is not possible to dissociate favorable and undesirable effects of GCs because their underlying mechanisms are entangled at the molecular level. Here, we review whether available data suggest that it is possible, feasible and desirable to taper and discontinue GC treatment in SLE.
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- Fulminant myocarditis is a serious condition that requires accurate diagnosis, typically through a myocardial biopsy, but the procedure carries risks, and its effectiveness for treatment is unclear.
- A study at a French ICU examined patients who were on mechanical circulatory support (MCS) and underwent biopsy, finding that while the diagnosis of definite myocarditis increased significantly after biopsy, the actual change in treatment was minimal.
- The study concluded that the risks associated with myocardial biopsy may outweigh its benefits in these vulnerable patients, suggesting a need for more caution in deciding to perform the procedure.
The improved understanding of the molecular basis of innate immunity have led to the identification of type I interferons (IFNs), particularly IFN-α, as central mediators in the pathogenesis of several Immune-mediated inflammatory diseases (IMIDs) such as systemic lupus erythematosus (SLE), systemic sclerosis, inflammatory myositis and Sjögren's syndrome. Here, we review the main data regarding the opportunity to target type I IFNs for the treatment of IMIDs. Type I IFNs and their downstream pathways can be targeted pharmacologically in several manners.
View Article and Find Full Text PDFKidney dysfunction due to AA amyloidosis in a morbidly obese female.
Clin Nephrol Case Stud
July 2023
Kidneys are commonly involved in systemic amyloidosis. Systemic AA amyloidosis is known to be associated with states of chronic inflammation such as autoimmune conditions, chronic infections, and malignancies. Obesity is increasingly recognized to be a risk factor for low-grade, chronic inflammation.
View Article and Find Full Text PDFIntroduction: Systemic lupus erythematosus (SLE) is non-organ specific autoimmune disease with mainly skin, joint, and kidney involvement. SLE-related acute lung disease (ALD) is rare, poorly investigated and can lead to acute respiratory failure. We conducted a retrospective study aiming to describe clinical features, treatments and outcome of SLE-related APD.
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