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  1. Article ; Online: The impact of hormones in autoimmune cutaneous diseases.

    Lopes Almeida Gomes, Lais / Werth, Adrienne J / Thomas, Preethi / Werth, Victoria P

    The Journal of dermatological treatment

    2024  Volume 35, Issue 1, Page(s) 2312241

    Abstract: Introduction: Dermatomyositis, systemic and cutaneous lupus erythematosus have a significantly higher prevalence in women than men, emphasizing the relevance of exploring the relationship between sex hormones and autoimmune skin diseases. This review ... ...

    Abstract Introduction: Dermatomyositis, systemic and cutaneous lupus erythematosus have a significantly higher prevalence in women than men, emphasizing the relevance of exploring the relationship between sex hormones and autoimmune skin diseases. This review analyzes the interplay between sex hormones and these two skin diseases.
    Materials and methods: We performed an extensive literature search using the PubMed database from July to August 2023. Search terms included 'contraceptives', 'pregnancy', 'hormone replacement', 'tamoxifen', and 'aromatase inhibitors'.
    Results and discussion: This comprehensive literature review shows that there remains considerable debate regarding the use of hormonal contraceptives and hormonal replacement therapy in individuals with autoimmune skin conditions. Nonetheless, it is well established that their use is contraindicated in patients with antiphospholipid syndrome or when antiphospholipid antibodies are positive. Individuals experiencing disease flares and uncontrolled symptoms should also avoid these interventions. Pregnancy planning should be timed to coincide with well-managed disease states to minimize obstetric and neonatal complications. Hormonal breast cancer treatment requires close skin monitoring.
    Conclusion: Pregnancy, menopause, contraceptive use, hormone replacement therapy, and breast cancer treatment drugs result in substantial shifts in hormone levels. Additionally, hormone levels are altered by aromatase inhibitors and anti-estrogen medications. These fluctuations can modulate mechanisms influencing autoimmune skin abnormalities.
    MeSH term(s) Pregnancy ; Male ; Infant, Newborn ; Humans ; Female ; Hormones ; Autoimmune Diseases/drug therapy ; Gonadal Steroid Hormones ; Menopause ; Breast Neoplasms ; Lupus Erythematosus, Systemic
    Chemical Substances Hormones ; Gonadal Steroid Hormones
    Language English
    Publishing date 2024-02-05
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 1036299-x
    ISSN 1471-1753 ; 0954-6634
    ISSN (online) 1471-1753
    ISSN 0954-6634
    DOI 10.1080/09546634.2024.2312241
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    Zs.A 2627: Show issues Location:
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  2. Article ; Online: Trial of intravenous immunoglobulin in dermatomyositis: a critically appraised research paper.

    Pandya, Rachita / Kleitsch, Julianne / Werth, Victoria P

    The British journal of dermatology

    2023  Volume 188, Issue 6, Page(s) 738–739

    MeSH term(s) Humans ; Dermatomyositis/drug therapy ; Immunoglobulins, Intravenous/therapeutic use
    Chemical Substances Immunoglobulins, Intravenous
    Language English
    Publishing date 2023-03-20
    Publishing country England
    Document type Clinical Trial ; Journal Article
    ZDB-ID 80076-4
    ISSN 1365-2133 ; 0007-0963
    ISSN (online) 1365-2133
    ISSN 0007-0963
    DOI 10.1093/bjd/ljad044
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  3. Article ; Online: Characterizing primary hospitalizations for systemic lupus erythematosus in the United States.

    Desai, Amar D / Werth, Victoria P / Elman, Scott A

    Rheumatology (Oxford, England)

    2023  Volume 62, Issue 12, Page(s) e335–e337

    MeSH term(s) Humans ; United States/epidemiology ; Hospitalization ; Lupus Erythematosus, Systemic/epidemiology
    Language English
    Publishing date 2023-06-12
    Publishing country England
    Document type Journal Article
    ZDB-ID 1464822-2
    ISSN 1462-0332 ; 1462-0324
    ISSN (online) 1462-0332
    ISSN 1462-0324
    DOI 10.1093/rheumatology/kead290
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  4. Article ; Online: Trial of Intravenous Immune Globulin in Dermatomyositis.

    Werth, Victoria P / Fiorentino, David F / Vleugels, Ruth Ann

    The New England journal of medicine

    2023  Volume 388, Issue 1, Page(s) 94

    MeSH term(s) Humans ; Immunoglobulins, Intravenous/therapeutic use ; Dermatomyositis/drug therapy ; Dermatomyositis/immunology
    Chemical Substances Immunoglobulins, Intravenous
    Language English
    Publishing date 2023-01-11
    Publishing country United States
    Document type Letter ; Comment
    ZDB-ID 207154-x
    ISSN 1533-4406 ; 0028-4793
    ISSN (online) 1533-4406
    ISSN 0028-4793
    DOI 10.1056/NEJMc2214285
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  5. Article: Editorial: Cutaneous lupus erythematosus landscape: pathophysiology, unmet needs, and related challenges in clinical practice. What is on the horizon?

    Merola, Joseph F / Nyberg, Filippa / Franchimont, Nathalie / Barbey, Catherine / Werth, Victoria P

    Frontiers in medicine

    2024  Volume 11, Page(s) 1373552

    Language English
    Publishing date 2024-02-27
    Publishing country Switzerland
    Document type Editorial
    ZDB-ID 2775999-4
    ISSN 2296-858X
    ISSN 2296-858X
    DOI 10.3389/fmed.2024.1373552
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  6. Article ; Online: Clinical characteristics and symptom progression of dermatomyositis subtypes: A retrospective analysis of a prospective database.

    Pandya, Rachita / Kleitsch, Julianne / Lim, Darosa / Werth, Victoria P

    Journal of the American Academy of Dermatology

    2024  

    Abstract: Background: Disease characteristics of classic dermatomyositis (DM) and clinically amyopathic DM (CADM) are well established, but there exists limited knowledge on the disease progression of these subtypes.: Objective: The objective of this study was ...

    Abstract Background: Disease characteristics of classic dermatomyositis (DM) and clinically amyopathic DM (CADM) are well established, but there exists limited knowledge on the disease progression of these subtypes.
    Objective: The objective of this study was to longitudinally track and characterize classic DM and CADM patients who experience changes in disease presentation.
    Methods: We conducted a retrospective review of prospectively collected data on 269 DM patients from a longitudinal database.
    Results: A total of 51% of the patients had classic DM and 49% had CADM. Forty percent of the classic DM patients became postmyopathic (PmDM). Median Cutaneous Dermatomyositis Disease Area and Severity Index activity (CDASI-A) score was lower in PmDM patients than in classic DM patients (13.0 vs 16.0), but 45% of the PmDM patients had CDASI-A scores > 14. Five percent of the CADM patients developed muscle involvement. Compared with CADM patients, those who developed muscle symptoms had milder skin disease before subtype conversion (median CDASI-A 12.0 vs 16.0) and at subtype conversion (median CDASI-A 9.0 vs 16.0).
    Limitations: This was a retrospective study conducted at a single tertiary-care dermatology clinic.
    Conclusions: Forty percent of the classic DM patients became PmDM. The majority continue with muscle disease, and many continue to have moderate/severe skin disease. CADM has a low risk of progressing to muscle disease, with the extent of skin disease as a potential predictive factor.
    Language English
    Publishing date 2024-02-09
    Publishing country United States
    Document type Journal Article
    ZDB-ID 603641-7
    ISSN 1097-6787 ; 0190-9622
    ISSN (online) 1097-6787
    ISSN 0190-9622
    DOI 10.1016/j.jaad.2024.02.007
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    Zs.A 1540: Show issues Location:
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  7. Article ; Online: Extracellular Vesicles in the Pathogenesis, Clinical Characterization, and Management of Dermatomyositis: A Narrative Review.

    Ricco, Cristina / Eldaboush, Ahmed / Liu, Ming-Lin / Werth, Victoria P

    International journal of molecular sciences

    2024  Volume 25, Issue 4

    Abstract: Extracellular vesicles (EVs) are lipid-bilayer particles secreted from cells that primarily assist in cell-to-cell communication through the content of their cargo, such as proteins and RNA. EVs have been implicated in the pathogenesis of various ... ...

    Abstract Extracellular vesicles (EVs) are lipid-bilayer particles secreted from cells that primarily assist in cell-to-cell communication through the content of their cargo, such as proteins and RNA. EVs have been implicated in the pathogenesis of various autoimmune diseases, including dermatomyositis (DM), an inflammatory autoimmune disease characterized by distinct cutaneous manifestations, myopathy, and lung disease. We sought to review the role of EVs in DM and understand how they contribute to the pathogenesis and clinical characterization of the disease. We summarized the research progress on EVs in dermatomyositis based on recent publications. EV cargoes, such as double-stranded DNA, microRNA, and proteins, contribute to DM pathogenesis and mediate the proinflammatory response and cytokine release through signaling pathways such as the stimulator of interferon genes (STING) pathway. These nucleic acids and proteins have been proposed as disease-specific, stable biomarkers to monitor disease activity and responses to therapy. They also correlate with clinical parameters, inflammatory markers, and disease severity scores. Furthermore, some markers show an association with morbidities of DM, such as muscle weakness and interstitial lung disease. The continued study of EVs will help us to further elucidate our understanding of dermatomyositis.
    MeSH term(s) Humans ; Dermatomyositis/diagnosis ; Dermatomyositis/therapy ; Dermatomyositis/metabolism ; Extracellular Vesicles/metabolism ; MicroRNAs/genetics ; MicroRNAs/metabolism ; Lung Diseases, Interstitial/diagnosis ; Lung Diseases, Interstitial/etiology ; Lung Diseases, Interstitial/therapy ; Nucleic Acids/metabolism ; Proteins/metabolism ; Exosomes/metabolism
    Chemical Substances MicroRNAs ; Nucleic Acids ; Proteins
    Language English
    Publishing date 2024-02-06
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2019364-6
    ISSN 1422-0067 ; 1422-0067 ; 1661-6596
    ISSN (online) 1422-0067
    ISSN 1422-0067 ; 1661-6596
    DOI 10.3390/ijms25041967
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  8. Article: Subacute cutaneous lupus erythematosus versus discoid lupus erythematosus: A challenging diagnosis.

    Kleitsch, Julianne / Lim, Darosa / Pandya, Rachita / Werth, Victoria P

    JAAD case reports

    2023  Volume 41, Page(s) 93–97

    Language English
    Publishing date 2023-09-26
    Publishing country United States
    Document type Case Reports
    ZDB-ID 2834220-3
    ISSN 2352-5126
    ISSN 2352-5126
    DOI 10.1016/j.jdcr.2023.09.013
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  9. Article: Overlap of dermatomyositis and cutaneous lupus erythematosus: A case series.

    Pandya, Rachita / Lim, Darosa / Kleitsch, Julianne / Werth, Victoria P

    JAAD case reports

    2023  Volume 42, Page(s) 95–101

    Language English
    Publishing date 2023-10-14
    Publishing country United States
    Document type Case Reports
    ZDB-ID 2834220-3
    ISSN 2352-5126
    ISSN 2352-5126
    DOI 10.1016/j.jdcr.2023.10.002
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  10. Article: Evaluation of RNase therapy in systemic lupus erythematosus: a randomised phase 2a clinical trial of RSLV-132.

    Burge, Daniel J / Werth, Victoria P / Boackle, Susan A / Posada, James

    Lupus science & medicine

    2024  Volume 11, Issue 1

    Abstract: Background: Circulating, extracellular RNA is the primary trigger of type I interferon in systemic lupus erythematosus (SLE), and interferon is known to play a central pathogenic role in the disease. RSLV-132 is a catalytically active human RNase ... ...

    Abstract Background: Circulating, extracellular RNA is the primary trigger of type I interferon in systemic lupus erythematosus (SLE), and interferon is known to play a central pathogenic role in the disease. RSLV-132 is a catalytically active human RNase molecule fused to human IgG1 Fc designed to digest RNA and thereby decrease the chronic inflammation associated with SLE. The drug was evaluated in a cohort of patients with SLE with moderate-severe cutaneous disease activity and the presence of RNA immune complexes. The primary objective of the study was the assessment of the impact of 13 doses of 10 mg/kg RSLV-132 over 6 months on the mean Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) score.
    Methods: Sixty-five patients meeting the entry criteria of a baseline CLASI score of 10 or greater and positivity of at least one of five autoantibodies to RNA-binding proteins (SM/RNP, SSA/Ro, SSB/La, Sm, RNP) were randomly assigned (2:1) to receive 13 doses of RSLV-132 10 mg/kg or placebo, respectively. Participants received study drug for 24 weeks on days 1, 8, 15, 29, 43, 57, 71, 85, 99, 113, 127, 141 and 155 with an end-of-treatment visit on day 169 and a follow-up visit at the end of the study on day 215. The primary objective was assessed on days 85 and 169. Secondary objectives included assessment of systemic disease activity using the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K), the British Isles Lupus Assessment Group 2004 Index and the Physician's Global Assessment. Data from these instruments were used to calculate the SLE Responder Index 4 (SRI-4) and the British Isles Lupus Assessment Group-based Composite Lupus Assessment (BICLA) scores.
    Results: The mean CLASI score change from baseline at day 169 was -5.7 (±7.0) in the placebo group and -6.2 (±8.5) in the RSLV-132 group. A subgroup of participants with moderate-severe systemic disease activity and high baseline SLEDAI scores (≥9) were analysed with respect to BICLA and SRI-4 responses. The RSLV-132 treated participants in the high SLEDAI subgroup had a greater percentage of BICLA responses (62% vs 44%) and SRI-4 responses (23% vs 11%) as compared with placebo. A second subgroup of participants with high baseline CLASI scores (≥21) were analysed with respect to BICLA and SRI-4 responses. The RSLV-132 treated participants in the high CLASI subgroup had a greater percentage of BICLA responses (28% vs 8%) and SRI-4 responses (39% vs 8%) as compared with placebo.
    Conclusions: Six months of RSLV-132 therapy consisting of a weekly loading dose of RSLV-132 for 1 month, followed by 5 months of biweekly administrations did not significantly improve the mean CLASI score relative to placebo in this cohort of patients with SLE. The study entry criteria selected patients with moderate-severe cutaneous disease activity and no minimum SLEDAI score, which resulted in a wide range of systemic disease activity from inactive to severe as measured by SLEDAI. When the participants with higher SLEDAI and CLASI scores were analysed, a trend towards clinical improvement favouring RSLV-132 was observed. The results warrant further evaluation of RSLV-132 in SLE and suggest that patients with more active systemic disease are most likely to benefit from RNase therapy.
    MeSH term(s) Humans ; Lupus Erythematosus, Systemic ; Antibodies, Monoclonal, Humanized/therapeutic use ; Treatment Outcome ; Ribonucleases/therapeutic use ; Immunoglobulin G/therapeutic use ; Lupus Erythematosus, Discoid/chemically induced ; Lupus Erythematosus, Discoid/drug therapy ; RNA/therapeutic use ; Recombinant Fusion Proteins
    Chemical Substances RSLV-132 (6RQ92PNH8Z) ; Antibodies, Monoclonal, Humanized ; Ribonucleases (EC 3.1.-) ; Immunoglobulin G ; RNA (63231-63-0) ; Recombinant Fusion Proteins
    Language English
    Publishing date 2024-02-07
    Publishing country England
    Document type Randomized Controlled Trial ; Clinical Trial, Phase II ; Journal Article
    ZDB-ID 2779620-6
    ISSN 2053-8790
    ISSN 2053-8790
    DOI 10.1136/lupus-2023-001113
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