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Juvenile-onset Systemic Lupus Erythematosus: Recent Advances in Pathogenesis and Treatment.

PURPOSE OF REVIEW: This review summarizes recent advances in understanding the pathogenesis and therapeutic landscape of juvenile-onset systemic lupus erythematosus (jSLE), with a focus on how emerging genetic and immunologic insights inform patient stratification, targeted treatments, and Treat-to-Target (T2T) approaches in pediatric care. RECENT FINDINGS: Studies of (ultra-)rare gene variants (e.g., affecting TLR7, UNC93B1, PLD4, PTPN2, BACH2) aided in understanding key pathogenic pathways, and allowed linking these to associated clinical phenotypes. Multi-ancestral genomic studies and cumu

Skin manifestations associated with systemic diseases - Part II.

The skin, by reflecting internal processes, externalizes what happens inside the body in many diseases. Thus, the skin, as an organ, extrapolates its functions of protection, barrier and signals the existence of systemic diseases, expanding the importance of the dermatologist beyond the skin surface. Thus, the dermatologist investigates diagnostic hypotheses for conditions related to all systems and refers patients to the appropriate specialty. Combined with examination by a trained eye, the skin, due to its easy access, is still the ideal place for performing biopsies, which often clarify the

A Case of Rowell Syndrome: Excellent Response to Oral Cyclosporine.

Lupus erythematosus is a multisystem disease which frequently involves the skin. There are several variants of cutaneous lupus, which are de … lupus erythematosus, erythema multiforme-like lesions, speckled pattern of ANA staining) and minor (chilblains, positive Anti-La or Anti-Ro antibodies, reactive rheumatoid factor); patients should present all three major criteria plus at least one minor criterion to be diagnosed with Rowell syndrome (4). First line treatment options for cutaneous lupus as we …

Rowell's Syndrome Triggered by Omeprazole.

Dear Editor, Rowell's syndrome is a rare disease, characterized by the appearance of erythema multiforme (EM)-like lesions in patients with … presence of anti-Ro and anti-La antibodies. As for the other minor criteria, RF was negative and no chilblains were found. Although there was a continuous time lapse (more than 1 year) between the initiation of omeprazole intake and the diagnosis of Rowell's syndrome, we suggest that the connection is probable. For instance, the latency differs depending on …

Evaluation of Effectiveness of Long-Term Therapy with Russian Rituximab Biosimilar (Acellbia) in Sjogren's Disease in Real Clinical Practice.

The aim to evaluate the effectiveness of long-term therapy with Russian rituximab (RTX) biosimilar in Sjögren's disease (SjD) in real-life clinical practice. MATERIALS AND METHODS : The retrospective study included 53 patients with SjD (Russian 2001 criteria and ACR/EULAR (American College of Rheumatology/European Alliance of Associations for Rheumatology) 2016 criteria), observed at the Nasonova Research Institute of Rheumatology from 2017 to 2024 and receiving long-term RTX therapy (Russian biosimilar Acellbia®, BIOCAD). The signs of clinical and laboratory activity of the disease, stomatolo

JAK-inhibitors. New players in the field of immune-mediated diseases, beyond rheumatoid arthritis.

Janus kinase (JAK)/signal transducers and activators of transcription (STATs) are a group of molecules associated with one of the major pathways through which many cytokines exert and integrate their function, and as such they are increasingly recognized as playing critical role in the pathogenesis subserving various immune-mediated diseases, including RA, PsA, SpAs, IBD, skin disorders (e.g. alopecia areata, atopic dermatitis), single-gene disorders like interferonopathies, and others. JAKs are the key initiating players of the JAK/STAT pathway. Upon binding of their respective effector molec

A phase 2, open-label, multicenter study of ixazomib plus lenalidomide and dexamethasone in adult Japanese patients with relapsed and/or refractory multiple myeloma.

BackgroundTOURMALINE-MM1 was a global study that demonstrated a significant improvement in progression-free survival with ixazomib plus lenalidomide and dexamethasone compared with placebo plus lenalidomide and dexamethasone, in patients with relapsed and/or refractory multiple myeloma. The current study was conducted to evaluate further the efficacy and safety of ixazomib plus lenalidomide and dexamethasone in Japanese patients.MethodsThis phase 2, open-label, single-arm, multicenter study enrolled patients aged ≥ 20 years with relapsed and/or refractory multiple myeloma at 16 sites in Japan.

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… TEAEs in the safety analysis set Ixazomib + LenDex N = 34 n (%) Patients with at least 1 AE 34 (100) Constipation 17 (50) Upper respiratory tract infection 16 (47) Diarrhea 14 (41) Rash 12 (35) Nasopharyngitis 11 (32) Nausea 10 (29) Platelet count decreased 9 (26) Influenza 8 (24) Neutropenia 8 (24) Chilblains … TEAEs occurring in ≥ 20% of patients were constipation (50%), upper respiratory tract infection (47%), diarrhea (41%), rash (35%), nasopharyngitis (32%), nausea (29%), platelet count decreased (26%), influenza (24%), neutropenia (24%), chilblains (21%), dysgeusia (21%), fall (21%) and peripheral sensory …

Extraglandular Manifestations of Sjögren’s Syndrome (SS): Dermatologic, Arthritic, Endocrine, Pulmonary, Cardiovascular, Gastroenterology, Renal, Urology, and Gynecologic Manifestations

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Skin manifestations associated with systemic diseases - Part I.

The skin demonstrates what is happening in the body in many diseases, as it reflects some internal processes on the surface. In this sense, skin as an organ, goes beyond its protective and barrier functions, as it provides clues for the identification of some systemic diseases. The dermatologist then raises diagnostic hypotheses for conditions related to all systems and refers them to the appropriate specialty. With easy access to examination by trained eyes and biopsies, the skin can present specific or non specific alterations on histopathology. In the first case this combination establishes

Case report: Single infusion of combined anti-CD20 and anti-CD38 monoclonal antibodies in pediatric refractory lupus nephritis.

Lupus nephritis (LN), present in 30%-50% of systemic lupus erythematosus (SLE) patients, often necessitates standard immunosuppressive therapy (glucocorticoids, MMF, CYC) as suggested by the European League Against Rheumatism/European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) and Kidney Disease Improving Global Outcomes (KDIGO) guidelines. However, a subset of subjects remains refractory. Recent findings suggested the efficacy of targeting CD38-long-lived plasma cells in LN and SLE refractory to standard treatment. However, previous experiences were limite

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… 1600] | 1105 | 438 | 726 | 1185 | 513 | 720 IgA (mg/dl) [n.v. 70-400] | 93 | 107 | 69 | 113 | 94 | 149 IgM (mg/dl) [n.v. 40-230] | 25 | 12 | 16 | 98 | 32 | 53 Patient 2 Patient 2 is a 22-year-old man who was diagnosed with SLE at 15 years of age after presenting with fever, arthritis, malar rash, chilblains …

Current Therapeutic Options for the Main Monogenic Autoinflammatory Diseases and PFAPA Syndrome: Evidence-Based Approach and Proposal of a Practical Guide.

Monogenic autoinflammatory diseases are rare conditions caused by genetic abnormalities affecting the innate immunity. Previous therapeutic strategies had been mainly based on results from retrospective studies and physicians' experience. However, during the last years, the significant improvement in their genetic and pathogenic knowledge has been accompanied by a remarkable progress in their management. The relatively recent identification of the inflammasome as the crucial pathogenic mechanism causing an aberrant production of interleukin 1β (IL-1β) in the most frequent monogenic autoinflamm

COVID-19 signalome: Pathways for SARS-CoV-2 infection and impact on COVID-19 associated comorbidity.

The COVID-19 pandemic has been the focus of research the past two years. The major breakthrough was made by discovering pathways related to SARS-CoV-2 infection through cellular interaction by angiotensin-converting enzyme (ACE2) and cytokine storm. The presence of ACE2 in lungs, intestines, cardiovascular tissues, brain, kidneys, liver, and eyes shows that SARS-CoV-2 may have targeted these organs to further activate intracellular signalling pathways that lead to cytokine release syndrome. It has also been reported that SARS-CoV-2 can hijack coatomer protein-I (COPI) for S protein retrograde

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… Similar changes are also observed in autoimmune disorders, such as familiar chilblain lupus, an interferonopathy correlated to increased IFN type I production. … The same process has been proven to cause familial chilblains and other autoimmune diseases like lupus for development of autoimmune diseases when type I IFNs are incorrectly activated in response to self-antigens or nucleic acid accumulation [ 101 ]. …

Abstracts from the ISSAID 2021 Periodic Congress : Virtual. May - Dec 2021

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Proceedings of the 27th European Paediatric Rheumatology Congress (PReS 2021) : Virtual. 19-21 September 2021

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Harnessing the Therapeutic Potential of Capsaicin and Its Analogues in Pain and Other Diseases.

Capsaicin is the most predominant and naturally occurring alkamide found in Capsicum fruits. Since its discovery in the 19th century, the therapeutic roles of capsaicin have been well characterized. The potential applications of capsaicin range from food flavorings to therapeutics. Indeed, capsaicin and few of its analogues have featured in clinical research covered by more than a thousand patents. Previous records suggest pleiotropic pharmacological activities of capsaicin such as an analgesic, anti-obesity, anti-pruritic, anti-inflammatory, anti-apoptotic, anti-cancer, anti-oxidant, and neur

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… Tincture of capsaicin as a remedy for chilblains and toothache. Dublin Free Press. 1850;1:95–96. 20. Buchheim R. Fructus capsici. J. Am. Pharm. Assoc. 1873;22:106. 21. Cui M., Gosu V., Basith S., Hong S., Choi S. …

Ход работы сессии 28th Европейский конгресс педиатрической ремуматологии (ПСР) 2022) Прага, Чешская Республика. 20-23 сентябрь 2022.

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Ход работы сессии 25th Европейский конгресс педиатрической ремуматологии (ПСР) 2018) Лиссабон, Португалия. 5-8 сентябрь 2018

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Ход работы сессии 29th Европейский конгресс педиатрии.

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Neutrophil extracellular traps in homeostasis and disease.

Neutrophil extracellular traps (NETs), crucial in immune defense mechanisms, are renowned for their propensity to expel decondensed chromatin embedded with inflammatory proteins. Our comprehension of NETs in pathogen clearance, immune regulation and disease pathogenesis, has grown significantly in recent years. NETs are not only pivotal in the context of infections but also exhibit significant involvement in sterile inflammation. Evidence suggests that excessive accumulation of NETs can result in vessel occlusion, tissue damage, and prolonged inflammatory responses, thereby contributing to the