Gender-related differences in systemic sclerosis

Hughes, Michael; Pauling, John D; Armstrong-James, Laura; Denton, Christopher P.; Galdas, Paul; Flurey, Caroline

doi:10.1016/j.autrev.2020.102494

Cited by 70 publications

(57 citation statements)

References 81 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The term TGGNB is used to refer to individuals who express their gender in ways that differ from the established cultural norms where gender is defined from sex assigned at birth. The social marginalisation of TGGNB individuals has a significant impact on their health and quality of life [130]. TGGNB people experience significantly high rates of poor physical and mental health [129].…”

Section: Sex and Gender In Researchmentioning

confidence: 99%

Sex and gender in interstitial lung diseases

et al. 2021

View full text Add to dashboard Cite

Sex and gender differences influence key domains of research, lung health, healthcare access and healthcare delivery. In interstitial lung diseases (ILDs), mouse models of pulmonary fibrosis are clearly influenced by sex hormones. Additionally, short telomeres, a biomarker of telomere regulation gene mutations, are impacted by sex, while heritability unexplained by genetic variation may be attributable to gendered environmental factors that drive epigenetic control. Diseases like idiopathic pulmonary fibrosis, hypersensitivity pneumonitis, occupational ILDs, connective tissue-associated ILDs and lymphangioleiomyomatosis have different prevalence and prognosis between men and women. These differences arise from a complex interplay between biological sex and sociocultural gender influencing genetics, epigenomic modifiers, hormones, immune function, response to treatment and interaction with healthcare systems. Much work remains to be done to systematically integrate sex and gender analysis into relevant domains of science and clinical care in ILD, from strategic considerations for establishing research priorities to guidelines for establishing best clinical practices. Accounting for sex and gender in ILD is essential to the practice of individualised, patient-centred medicine.

show abstract

Section: Sex and Gender In Researchmentioning

confidence: 99%

Sex and gender in interstitial lung diseases

et al. 2021

View full text Add to dashboard Cite

show abstract

“…Since anti-Scl-70 (anti-topo I) antibodies are considered to be associated with dcSSc ( 18 ) we also compared the proportion of memory B cell subsets in anti-topo I positive and negative patients, and found that the percentage of ASM B cells was significantly elevated in anti-topo I positive (median: 4.03, IQR: 2.24–4.49) compared to anti-topo I negative (median: 1.11, IQR: 0.82–1.97) SSc patients (p = 0.016) ( Figure 3B ). Male patients with SSc are reported to have a more severe disease ( 19 ), but even when only female patients were included in the analysis, the significant differences in association with dcSSc could still be observed (data not shown).…”

Section: Resultsmentioning

confidence: 96%

Increased Frequency of Activated Switched Memory B Cells and Its Association With the Presence of Pulmonary Fibrosis in Diffuse Cutaneous Systemic Sclerosis Patients

et al. 2021

View full text Add to dashboard Cite

Disease-associated, high-affinity pathological autoantibody production is a well-described consequence of immune dysregulation affecting B cells in systemic sclerosis (SSc), including the distribution of B-cell subsets. We have previously shown that the increased relative frequency of CD19+CD27+IgD− switched memory B cells is associated with the severe form of SSc. This study sought to analyze memory B cell subsets using an extended range of markers for further subdivision based on CD19, IgD, CD27, CD38 and CD95 phenotype, to define relationship between the alterations of memory B cell subsets and the clinical features of SSc. Peripheral blood samples were obtained from 21 SSc patients, including 14 diffuse (dcSSc) and 7 limited (lcSSc) cutaneous SSc patients, with disease duration of 2.7 ( ± 1.6) years. After purification of CD19+ B cells, multiparametric flow cytometry was performed and the frequencies of CD19+IgD−CD27−CD38+ double negative (DN) 1, CD19+IgDloCD27+CD38+ unswitched, CD19+IgD−CD27+CD38+CD95− resting switched and CD19+IgD−CD27+CD38−CD95+ activated switched memory (ASM) B cells were determined, and correlated with clinical features of SSc. The dcSSc patients had a higher frequency of ASM B cells (p = 0.028) compared to lcSSc patients. The percentage of ASM B cells was elevated in anti-Scl-70 (anti-topoisomerase I) antibody positive patients compared to negative patients (p = 0.016). Additionally, the frequency of ASM B cells was also increased in patients with pulmonary fibrosis (p = 0.003) suggesting that patients with severe form of SSc have higher ASM B cell ratios. Furthermore, the ratio of DN1 B cells was decreased (p = 0.029), while the level of anti-citrate synthase IgG natural autoantibody was elevated (p = 0.028) in patients with active disease. Our observations on the increase of ASM B cells in dcSSc and in patients with pulmonary fibrosis may point to the association of this alteration with the severe form of the disease. Functionally the correlation of ASM B cells as effector memory-plasma cell precursors with anti-topoisomerase I antibody positivity could reflect their contribution to pathological autoantibody production, whereas the decrease of memory precursor DN B cells and the increase of anti-citrate synthase IgG autoantibody may have potential significance in the assessment of disease activity.

show abstract

“…Finally, 1 study showed male sex to be associated with severity of GI dysfunction ( 22 ). It is also important to note that the prevalence of SSc is 4- to 5-fold higher among female than male population ( 23 ). Age of presentation with SSc-associated pseudo-obstruction was ∼60 years in 2 prior studies ( 24 , 25 ).…”

Section: Discussionmentioning

confidence: 99%

Comparing Clinical, Imaging, and Physiological Correlates of Intestinal Pseudo-Obstruction: Systemic Sclerosis vs Amyloidosis and Paraneoplastic Syndrome

Pamarthy

Berumen

Breen‐Lyles

et al. 2020

Clinical and Translational Gastroenterology

View full text Add to dashboard Cite

INTRODUCTION: Intestinal pseudo-obstruction is characterized by impaired transit and luminal dilation in the absence of mechanical obstruction. Our study aims to describe the clinical, radiographic, and physiological findings in pseudo-obstruction associated with systemic sclerosis (SSc), amyloidosis, and paraneoplastic syndrome. METHODS: A retrospective cohort of patients evaluated at our institution between January 1, 2008, and August 1, 2018, was assembled. Clinical, imaging, and physiological characteristics were abstracted from electronic medical records. RESULTS: We identified 100 cases of pseudo-obstruction (55 SSc, 27 amyloidosis, and 18 paraneoplastic). Female population predominance was seen in SSc (71%) vs male population in amyloidosis (74%). Most common symptom was abdominal bloating in all 3 groups. Vomiting was more common in SSc than amyloidosis (73% vs 46%, P = 0.02). Diarrhea was more common in amyloidosis and SSc compared with paraneoplastic (81% and 67% vs 28%, P < 0.01). Weight loss (>5%) was more common in SSc compared with amyloidosis and paraneoplastic (78% vs 31% and 17%, P < 0.0001). Only small bowel dilation was seen in 79%, 40%, and 44% and only large bowel dilation in 2%, 44%, and 44% of patients in SSc, amyloidosis, and paraneoplastic, respectively. Five of 8 SSc patients had myopathic and 3 of 5 paraneoplastic had neuropathic involvement on gastroduodenal manometry. DISCUSSION: SSc-associated pseudo-obstruction demonstrates female population predominance and presents with vomiting, diarrhea, and weight loss. Amyloidosis-associated pseudo-obstruction shows male population predominance. Small bowel is more commonly involved than large bowel on both imaging and transit studies in SSc. Myopathic involvement was more common in SSc, contrary to neuropathic in paraneoplastic syndrome.

show abstract

Gender-related differences in systemic sclerosis

Cited by 70 publications

References 81 publications

Sex and gender in interstitial lung diseases

Sex and gender in interstitial lung diseases

Increased Frequency of Activated Switched Memory B Cells and Its Association With the Presence of Pulmonary Fibrosis in Diffuse Cutaneous Systemic Sclerosis Patients

Comparing Clinical, Imaging, and Physiological Correlates of Intestinal Pseudo-Obstruction: Systemic Sclerosis vs Amyloidosis and Paraneoplastic Syndrome

Contact Info

Product

Resources

About