Management of systemic sclerosis‐associated interstitial lung disease in the current era

Amjadi, Sogol; Roofeh, David; Namas, Rajaie; Khanna, Dinesh

doi:10.1111/1756-185x.13799

Cited by 5 publications

(4 citation statements)

References 21 publications

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“…It remains an urgent issue to improve clinical outcomes among patients with SSc-ILD. Although new anti-fibrosis drugs, autologous stem cell transplantation, and lung transplantation have been used for treatment, the overall prognosis is not optimistic ( 22 , 23 ). It still owns great challenge to explore effective and simple treatments.…”

Section: Discussionmentioning

confidence: 99%

Exome sequencing link mutation in RGPD4 with systemic sclerosis-associated interstitial lung disease and the low level of testosterone—an exploration study

et al. 2022

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BackgroundInterstitial lung disease (ILD) is the most common and potentially most devastating manifestation of SSc in pulmonary involvement. However, the mechanism for systemic sclerosis-associated ILD (SSc-ILD) is unclear. This work aims to explore the potential candidates for SSc-ILD upon whole exome sequencing (WES) and attempts to analyze the possible pathogenesis of SSc-ILD from the perspective of the genetic level.MaterialsVariants were confirmed by whole exome sequencing (WES), and SKAT analysis was employed to explore the most differential variants. Targeted variants were performed in biological functions, associated with clinical manifestations, and the probable change of downstream.ResultsBy WES and SKAT analysis of SSc with and without ILD, only the variants of RGPD4 achieved statistical power (P < 2.51 × 10-6, P-FDR = 0.025, OR = 15.95). A total of 20 rare functional variants (missense, truncating, splicing) were tested for the RGPD4 gene, and five truncating and damaging missense variants were identified. Carriers showed the older inclusion age (P = 0.02) and the higher frequency use of prednisone (P=0.02) compared to the non-carriers. Further analysis illustrated that carriers showed lower levels of TES in comparison to non-carriers but did not reach statistical difference (P = 0.08). In bivariate correlation analysis, we analyzed the relationship between the mutant status of RGPD4 and the levels of sex hormones after adjusting for age confounders. Only the level of TES showed a negative correlation with the mutant status (B = -0.509, P = 0.037).ConclusionThe variants of RGPD4 might contribute to the ILD development of SSc and might also be a causative factor of lower TES among SSc-ILD, which provided insight to a better understanding of pathobiology of SSc-ILD, and androgen hormone supplement might be a therapeutic target in this debilitating disease.

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Section: Discussionmentioning

confidence: 99%

Exome sequencing link mutation in RGPD4 with systemic sclerosis-associated interstitial lung disease and the low level of testosterone—an exploration study

et al. 2022

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“…Disease management has improved over the past decade 7,8 largely due to earlier detection which was facilitated by the 2013 release of classi cation criteria from the American College of Rheumatology and European League Against Rheumatism 9 . Currently, standard treatment primarily focuses on controlling or managing disease processes and relies on pharmacologic therapy with immunosuppressive, anti brotic, and anti-in ammatory medications 1,7,8 . Additional treatments include heterologous/autologous stem cell transplantation, organ transplantation, and surgery 1,10,11 .…”

Section: Introductionmentioning

confidence: 99%

“…However, scleroderma can produce life-threatening complications in multiple organs including the cardiovascular, gastro-intestinal, and renal systems 5,6 . Disease management has improved over the past decade 7,8 largely due to earlier detection which was facilitated by the 2013 release of classi cation criteria from the American College of Rheumatology and European League Against Rheumatism 9 . Currently, standard treatment primarily focuses on controlling or managing disease processes and relies on pharmacologic therapy with immunosuppressive, anti brotic, and anti-in ammatory medications 1,7,8 .…”

Section: Introductionmentioning

confidence: 99%

An Online Program of Seated Gentle Yoga & Yogic Breathing Improves Symptoms in Scleroderma: A Quality Improvement Study

Balasubramanian,

Blackwelder,

Wahlquist

et al. 2024

Preprint

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Importance Dry mouth is a common symptom experienced by scleroderma patients. Gentle yoga & yogic breathing improves salivation and other symptoms through parasympathetic dominance and may help scleroderma patients. Objective To conduct a pilot, feasibility study of an online Gentle Yoga and Yogic Breathing (GYYB) intervention for scleroderma patients during COVID-19 pandemic. Design, Setting, and participants A 60-minute GYYB exercise session was designed with consideration for the limitations of the scleroderma population and advertised in online communities. There were 99 participants registered over a seven week period. The sessions were led by certified Yoga Therapist weekly on Saturdays from 11 AM to 12 PM eastern time for seven weeks during COVID-19 pandemic (from 4/4/2020 to 5/16/2020) via the Zoom online teleconferencing tool. After each session participants had the opportunity to voluntarily complete feedback questionnaires about the session using Google Forms® designed for this purpose. Results Of 99 people that attended the sessions, 45 participants responded to the questionnaires. Over half (57.8%) respondents stated they would “Definitely” return to another session and participant satisfaction with the intervention was high (mean [SD] score 9.1 (1.3) on a scale of 1 [“Didn’t Like It at All”] to 10 [“Loved It”]). The majority reported liking both the yoga and breathing activities (75.6%) and 22.2% preferred the breathing activities. The majority (75.6%) found participation using computer or mobile phone “Very Easy”, with one participant reporting that it was “Very Difficult”. Most participants found doing yoga once daily (61.4%) for 30 minutes (56.6%) was most feasible. After one session, the majority (64.3%) reported they “Felt Better”, and no adverse effects were reported by any participant during the study. Conclusion and Relevance These data provide the first published evidence that an online yoga intervention may help address the need for low-cost, effective, non-pharmacological interventions to help people with scleroderma manage secondary symptoms and potentially improve their QoL.

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“…Its severity is clinically heterogeneous 6,7 and may be defined by its impact on lung physiology measured by pulmonary function testing. These measures have been used to predict SSc-ILD outcomes [8][9][10][11] , develop treatment algorithms [12][13][14] , and are used as outcome measures in SSc-ILD randomized clinical trials (RCTs) [15][16][17] .…”

Section: Introductionmentioning

confidence: 99%

Outcome measurement instrument selection for lung physiology in systemic sclerosis associated interstitial lung disease: A systematic review using the OMERACT filter 2.1 process

Roofeh

Barratt

Wells

et al. 2021

Seminars in Arthritis and Rheumatism

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Management of systemic sclerosis‐associated interstitial lung disease in the current era

Cited by 5 publications

References 21 publications

Exome sequencing link mutation in RGPD4 with systemic sclerosis-associated interstitial lung disease and the low level of testosterone—an exploration study

Exome sequencing link mutation in RGPD4 with systemic sclerosis-associated interstitial lung disease and the low level of testosterone—an exploration study

An Online Program of Seated Gentle Yoga & Yogic Breathing Improves Symptoms in Scleroderma: A Quality Improvement Study

Outcome measurement instrument selection for lung physiology in systemic sclerosis associated interstitial lung disease: A systematic review using the OMERACT filter 2.1 process

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Management of systemic sclerosis‐associated interstitial lung disease in the current era

Cited by 5 publications

References 21 publications

Exome sequencing link mutation in RGPD4 with systemic sclerosis-associated interstitial lung disease and the low level of testosterone—an exploration study

Exome sequencing link mutation in RGPD4 with systemic sclerosis-associated interstitial lung disease and the low level of testosterone—an exploration study

An Online Program of Seated Gentle Yoga &amp; Yogic Breathing Improves Symptoms in Scleroderma: A Quality Improvement Study

Outcome measurement instrument selection for lung physiology in systemic sclerosis associated interstitial lung disease: A systematic review using the OMERACT filter 2.1 process

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An Online Program of Seated Gentle Yoga & Yogic Breathing Improves Symptoms in Scleroderma: A Quality Improvement Study