Docetaxel-Induced Systemic Sclerosis with Internal Organ Involvement Masquerading as Congestive Heart Failure

Systemic sclerosis, or scleroderma, is a complex medical disorder characterized by limited or diffuse skin thickening with frequent involvement of internal organs such as lungs, gastrointestinal tract, or kidneys. Docetaxel is a chemotherapeutic agent which has been associated with cutaneous side effects. An uncommon cutaneous side effect of docetaxel is scleroderma-like skin changes that extend from limited to diffuse cutaneous systemic sclerosis. Several case reports have been published regarding the association of docetaxel and systemic sclerosis. However, those reports demonstrated the association between docetaxel and scleroderma-like skin changes without internal organ involvement. Here, we report a case of systemic sclerosis with pulmonary arterial hypertension and a microangiopathic kidney involvement induced by docetaxel chemotherapy. After an exhaustive literature review, this could be the first case of docetaxel-induced systemic sclerosis involving internal organs.

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Health Assessment Questionnaire-Disability Index (HAQ-DI) use in modelling disease progression in diffuse cutaneous systemic sclerosis: an analysis from the EUSTAR database

10.21203/rs.3.rs-38370/v2 ◽

2020 ◽

Cited By ~ 1

Author(s):

Yannick Allanore ◽

Sylvie Bozzi ◽

Augustin Terlinden ◽

Doerte Huscher ◽

Caroline Amand ◽

...

Keyword(s):

Systemic Sclerosis ◽

Disease Progression ◽

Health Assessment Questionnaire ◽

Multivariable Analysis ◽

Health Assessment ◽

Organ Involvement ◽

Risk Of Death ◽

Diffuse Cutaneous Systemic Sclerosis ◽

Assessment Questionnaire ◽

Questionnaire Disability

Abstract Background: Patients with diffuse cutaneous systemic sclerosis (dcSSc) have a poor prognosis. The importance of monitoring subjective measures of functioning and disability, such as the Health Assessment Questionnaire-Disability Index (HAQ-DI), is important as dcSSc is rated by patients as worse than diabetes or hemodialysis for quality of life impairment. This European Scleroderma Trials and Research (EUSTAR) database analysis was undertaken to examine the importance of impaired functionality in dcSSc prognosis. The primary objectives were to identify predictors of death and HAQ-DI score progression over 1 year. HAQ-DI score, major advanced organ involvement, and death rate were also used to develop a comprehensive model to predict lifetime dcSSc progression. Methods: This was an observational, longitudinal study in patients with dcSSc registered in EUSTAR. Death and HAQ-DI scores were, respectively, analyzed by Cox regression and linear regression analyses in relation to baseline covariates. A microsimulation Markov model was developed to estimate/predict natural progression of dcSSc over a patient’s lifetime.Results: The analysis included dcSSc patients with (N = 690) and without (N = 4132) HAQ-DI score assessments from the EUSTAR database. Baseline HAQ-DI score, corticosteroid treatment and major advanced organ involvement were predictive of death on multivariable analysis; a 1-point increase in baseline HAQ-DI score multiplied the risk of death by 2.7 (p < 0.001) and multiple advanced major organ involvement multiplied the risk of death by 2.8 (p < 0.05). Multivariable analysis showed that baseline modified Rodnan Skin Score (mRSS) and baseline HAQ-DI score were associated with HAQ-DI score progression at 1 year (p < 0.05), but there was no association between baseline organ involvement and HAQ-DI score progression at 1 year. HAQ-DI score, major advanced organ involvement, and death were successfully used to model long-term disease progression in dcSSc.Conclusions: HAQ-DI score and major advanced organ involvement were comparable predictors of mortality risk in dcSSc. Baseline mRSS and baseline HAQ-DI score were predictive of HAQ-DI score progression at 1 year, indicating a correlation between these endpoints in monitoring disease progression. It is hoped that this EUSTAR analysis may change physician perception about the importance of the HAQ-DI score in dcSSc.

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Development of Two Types of Skin Cancer in a Patient with Systemic Sclerosis: a Case Report and Overview of the Literature

Case Reports in Oncological Medicine ◽

10.1155/2021/6628671 ◽

2021 ◽

Vol 2021 ◽

pp. 1-5

Author(s):

Firdevs Ulutaş ◽

Erdem Çomut ◽

Veli Çobankara

Keyword(s):

Systemic Sclerosis ◽

Skin Cancer ◽

Cell Carcinoma ◽

Case Reports ◽

Pulmonary Function Tests ◽

Case Series ◽

Skin Neoplasms ◽

Skin Cancers ◽

Nonspecific Interstitial Pneumonia ◽

Diffuse Cutaneous Systemic Sclerosis

Systemic sclerosis (SSc) is an uncommon rheumatic disease in which the underlying main histopathologic feature is a thickening of the skin due to excessive accumulation of collagen in the extracellular tissue. Fibrogenesis, chronic inflammation, and ulceration may eventually promote skin neoplasms. Although nonmelanoma skin cancer (NMSC) is the most frequent type, there have been restricted case reports and case series with skin cancers in SSc patients in the literature. Herein, we describe a 78-year-old woman diagnosed with diffuse cutaneous systemic sclerosis thirteen years ago and associated nonspecific interstitial pneumonia that was successfully treated with high cumulative doses of cyclophosphamide. She developed basal cell carcinoma and squamous cell carcinoma of the skin in the follow-up. She is still on rituximab treatment with stable interstitial lung disease as indicated by pulmonary function tests and high-resolution chest computed tomography. To our knowledge and a literature search, this is the first reported patient with SSc with two types of skin cancer. In this review, we also aimed to emphasize the relationship between SSc and skin cancer, and possible risk factors for SSc-related skin cancer.

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Pembrolizumab induced scleroderma

Discussion of Clinical Cases ◽

10.5430/dcc.v6n4p6 ◽

2020 ◽

Vol 6 (4) ◽

pp. 6

Author(s):

Reem Alkilany ◽

Stanley Ballou

Keyword(s):

Lung Cancer ◽

Vascular Dysfunction ◽

Case Reports ◽

Severe Disease ◽

Connective Tissue Disorder ◽

Multiple Organ ◽

Organ Involvement ◽

White Female ◽

Multisystem Disease ◽

First Case

Systemic sclerosis (SSc) is a rare connective tissue disorder. It is a chronic multisystem disease characterized by vascular dysfunction and progressive fibrosis of tissue, with skin hardening and thickening (known as scleroderma) being the hallmark of the disease. It tends to affect females more than males and has a higher prevalence in African American population with earlier onset and more severe disease. While scleroderma can be a manifestation of conditions other than SSC, the presence of skin thickening of the fingers, extending proximally to metacarpophalangeal joints is sufficient to classify a patient as having SSc. SSc treatment is challenging given the heterogeneity of the disease, multiple organ involvement, different subtypes and poorly understood etiology and pathogenesis. Yet, systemic immunosuppressive therapy is often the treatment of choice. Here we present a 60-year-old white female who developed skin thickening of her fingers extending to the forearms and of her proximal thighs after being treated with pembrolizumab for metastatic non-small cell lung cancer. It was difficult to determine internal organ involvement given her history of metastatic lung cancer, but scleroderma specific autoantibodies were negative. Her symptoms improved after treatment with methotrexate and stopping pembrolizumab. This is one of the first case reports of scleroderma secondary to pembrolizumab.

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Increased expression of S100A8 and S100A9 in patients with diffuse cutaneous systemic sclerosis. A correlation with organ involvement and immunological abnormalities

Clinical Rheumatology ◽

10.1007/s10067-013-2305-4 ◽

2013 ◽

Vol 32 (10) ◽

pp. 1501-1510 ◽

Cited By ~ 22

Author(s):

Xue Xu ◽

Wen-yu Wu ◽

Wen-zheng Tu ◽

Hai-yan Chu ◽

Xiao-xia Zhu ◽

...

Keyword(s):

Systemic Sclerosis ◽

Organ Involvement ◽

Diffuse Cutaneous Systemic Sclerosis ◽

Immunological Abnormalities

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Endothelin Receptor Antagonists for the Treatment of Raynaud's Phenomenon and Digital Ulcers in Systemic Sclerosis

International Journal of Rheumatology ◽

10.1155/2011/201787 ◽

2011 ◽

Vol 2011 ◽

pp. 1-7 ◽

Cited By ~ 13

Author(s):

Kait Arefiev ◽

David F. Fiorentino ◽

Lorinda Chung

Keyword(s):

Systemic Sclerosis ◽

Case Reports ◽

Raynaud’S Phenomenon ◽

Raynaud's Phenomenon ◽

Digital Ulcers ◽

Endothelin Receptor Antagonists ◽

Endothelin Receptor ◽

Significant Morbidity ◽

Receptor Antagonists ◽

Internal Organs

Systemic sclerosis is a connective tissue disease characterized by fibrosis of the skin, internal organs, and widespread vasculopathy. Raynaud's phenomenon and digital ulcers are vascular manifestations of this disease and cause significant morbidity. Current treatments are only moderately effective in reducing the severity of Raynaud's in a portion of patients and typically do not lead to substantial benefit in terms of the healing or prevention of digital ulcers. Several studies have evaluated the efficacy of targeting the vasoconstrictor endothelin-1 for the treatment of systemic sclerosis-associated vascular disease. The purpose of this paper is to summarize the published studies and case reports evaluating the efficacy of endothelin receptor antagonists in the treatment of Raynaud's phenomenon and digital ulcers associated with systemic sclerosis.

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Patient perception of disease burden in diffuse cutaneous systemic sclerosis

Journal of Scleroderma and Related Disorders ◽

10.1177/2397198319866615 ◽

2019 ◽

Vol 5 (1) ◽

pp. 66-76 ◽

Cited By ~ 1

Author(s):

Dinesh Khanna ◽

Yannick Allanore ◽

Christopher P Denton ◽

Marco Matucci-Cerinic ◽

Janet Pope ◽

...

Keyword(s):

Systemic Sclerosis ◽

Emotional Support ◽

Support Services ◽

Disease Burden ◽

Healthcare Professionals ◽

Unmet Need ◽

The United States ◽

Skin Changes ◽

Diffuse Cutaneous Systemic Sclerosis ◽

The Impact

Purpose: Systemic sclerosis is a rare multi-organ autoimmune rheumatic disease, resulting in progressive fibrosis of the skin/internal organs. This study aimed to understand the impact of diffuse cutaneous systemic sclerosis symptoms and disease burden from the patient’s perspective. Methods: This was a mixed methodology, market research study involving ethnography, structured interviews, video diaries, and patient tasks. Patients had been diagnosed with diffuse cutaneous systemic sclerosis for ⩾ 6 months and were recruited via healthcare professionals or patient associations (France, Italy, the United Kingdom, and the United States). Patients filmed short (~15 min) daily video diaries about their lives over 7 days and participated in ethnographic sessions, patient tasks, and structured video interviews. In Germany and Spain, patients participated in 60-min telephone interviews. Results: Twenty-three patients (mean age: 54 years; 83% women; minimum disease duration: 6 months) participated in the study. Time to diagnosis was prolonged, as patients overlooked their symptoms and some healthcare professionals attributed symptoms to other causes. Patients rarely received additional information or support services at diagnosis. Importantly, although patients were aware of the seriousness of organ involvement, they reported that skin changes, pain, and fatigue impaired their ability to perform routine tasks. Patients had a high prescription treatment burden (mean: 10 tablets/day; up to >25 tablets/day) with additional non-prescription medication taken for other comorbidities. Treatment discontinuation was common due to side effects. Patients experienced diffuse cutaneous systemic sclerosis as a loss of independence and self-esteem. Moreover, patients tended to have small support networks, and emotional support services were not offered as standard care. Conclusion: Patients with diffuse cutaneous systemic sclerosis had high treatment and disease burdens, with skin changes, pain, and fatigue profoundly affecting their lives. There is an unmet need for patient information at the time of diagnosis and emotional support services throughout the patient’s journey with diffuse cutaneous systemic sclerosis. Based on the results of this study, we provide recommendations for improving diffuse cutaneous systemic sclerosis care.

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Health Assessment Questionnaire-Disability Index (HAQ-DI) use in modelling disease progression in diffuse cutaneous systemic sclerosis: an analysis from the EUSTAR database

Arthritis Research & Therapy ◽

10.1186/s13075-020-02329-2 ◽

2020 ◽

Vol 22 (1) ◽

Author(s):

Yannick Allanore ◽

◽

Sylvie Bozzi ◽

Augustin Terlinden ◽

Doerte Huscher ◽

...

Keyword(s):

Systemic Sclerosis ◽

Disease Progression ◽

Health Assessment Questionnaire ◽

Multivariable Analysis ◽

Health Assessment ◽

Organ Involvement ◽

Risk Of Death ◽

Diffuse Cutaneous Systemic Sclerosis ◽

Assessment Questionnaire ◽

Questionnaire Disability

Abstract Background Patients with diffuse cutaneous systemic sclerosis (dcSSc) have a poor prognosis. The importance of monitoring subjective measures of functioning and disability, such as the Health Assessment Questionnaire-Disability Index (HAQ-DI), is important as dcSSc is rated by patients as worse than diabetes or hemodialysis for quality of life impairment. This European Scleroderma Trials and Research (EUSTAR) database analysis was undertaken to examine the importance of impaired functionality in dcSSc prognosis. The primary objectives were to identify predictors of death and HAQ-DI score progression over 1 year. HAQ-DI score, major advanced organ involvement, and death rate were also used to develop a comprehensive model to predict lifetime dcSSc progression. Methods This was an observational, longitudinal study in patients with dcSSc registered in EUSTAR. Death and HAQ-DI scores were, respectively, analyzed by Cox regression and linear regression analyses in relation to baseline covariates. A microsimulation Markov model was developed to estimate/predict natural progression of dcSSc over a patient’s lifetime. Results The analysis included dcSSc patients with (N = 690) and without (N = 4132) HAQ-DI score assessments from the EUSTAR database. Baseline HAQ-DI score, corticosteroid treatment, and major advanced organ involvement were predictive of death on multivariable analysis; a 1-point increase in baseline HAQ-DI score multiplied the risk of death by 2.7 (p < 0.001) and multiple advanced major organ involvement multiplied the risk of death by 2.8 (p < 0.05). Multivariable analysis showed that baseline modified Rodnan Skin Score (mRSS) and baseline HAQ-DI score were associated with HAQ-DI score progression at 1 year (p < 0.05), but there was no association between baseline organ involvement and HAQ-DI score progression at 1 year. HAQ-DI score, major advanced organ involvement, and death were successfully used to model long-term disease progression in dcSSc. Conclusions HAQ-DI score and major advanced organ involvement were comparable predictors of mortality risk in dcSSc. Baseline mRSS and baseline HAQ-DI score were predictive of HAQ-DI score progression at 1 year, indicating a correlation between these endpoints in monitoring disease progression. It is hoped that this EUSTAR analysis may change physician perception about the importance of the HAQ-DI score in dcSSc.

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Health Assessment Questionnaire-Disability Index (HAQ-DI) use in modelling disease progression in diffuse cutaneous systemic sclerosis: an analysis from the EUSTAR database

10.21203/rs.3.rs-38370/v1 ◽

2020 ◽

Author(s):

Yannick Allanore ◽

Sylvie Bozzi ◽

Augustin Terlinden ◽

Doerte Huscher ◽

Caroline Amand ◽

...

Keyword(s):

Systemic Sclerosis ◽

Disease Progression ◽

Health Assessment Questionnaire ◽

Multivariable Analysis ◽

Health Assessment ◽

Organ Involvement ◽

Risk Of Death ◽

Diffuse Cutaneous Systemic Sclerosis ◽

Assessment Questionnaire ◽

Questionnaire Disability

Abstract Background: Patients with diffuse cutaneous systemic sclerosis (dcSSc) have a poor prognosis. The importance of monitoring subjective measures of functioning and disability, such as the Health Assessment Questionnaire-Disability Index (HAQ-DI), is important as dcSSc is rated by patients as worse than diabetes or hemodialysis for quality of life impairment. This European Scleroderma Trials and Research (EUSTAR) database analysis was undertaken to examine the importance of impaired functionality in dcSSc prognosis. The primary objectives were to identify predictors of death and HAQ-DI score progression over 1 year. HAQ-DI score, major advanced organ involvement, and death rate were also used to develop a comprehensive model to predict lifetime dcSSc progression.Methods: This was an observational, longitudinal study in patients with dcSSc registered in EUSTAR. Death and HAQ-DI scores were, respectively, analyzed by Cox regression and linear regression analyses in relation to baseline covariates. A Markov model was developed to estimate/predict natural progression of dcSSc over a patient’s lifetime.Results: The analysis included dcSSc patients with (N = 690) and without (N = 4132) HAQ-DI score assessments from the EUSTAR database. Baseline HAQ-DI score, corticosteroid treatment and major advanced organ involvement were predictive of death on multivariable analysis; a 1-point increase in baseline HAQ-DI score multiplied the risk of death by 2.7 (p < 0.001) and multiple advanced major organ involvement multiplied the risk of death by 2.8 (p < 0.05). Multivariable analysis showed that baseline modified Rodnan Skin Score (mRSS) and baseline HAQ-DI score were associated with HAQ-DI score progression at 1 year (p < 0.05), but there was no association between baseline organ involvement and HAQ-DI score progression at 1 year. HAQ-DI score, major advanced organ involvement, and death were successfully used to model long-term disease progression in dcSSc.Conclusions: HAQ-DI score and major advanced organ involvement were comparable predictors of mortality risk in dcSSc. Baseline mRSS and baseline HAQ-DI score were predictive of HAQ-DI score progression at 1 year, indicating a correlation between these endpoints in monitoring disease progression. It is hoped that this EUSTAR analysis may change physician perception about the importance of the HAQ-DI score in dcSSc.

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