Depression

Depression involves symptoms that may include sadness, loss of interest or pleasure, feelings of guilt or low self-esteem, poor concentration, and disturbed sleep or appetite. A study of 345 patients with SSc enrolled in a Canadian registry reported that the prevalence of major depressive disorder (MDD) was 4% for the past 30 days, 11% for the past 12 months, and 23% for lifetime. A French study of 50 hospitalized patients with SSc and 50 patients with SSc who attended a patient organization meeting found that 19% had current MDD and 56% had lifetime MDD, and rates were higher in hospitalized (28% current) versus nonhospitalized (10% current) patients. Depression is substantially more common in patients with SSc than in the general population and may be more prevalent than in other rheumatic diseases. However, many patients with SSc and other chronic diseases who meet criteria for MDD at a given time point do not meet criteria consistently. In the Canadian sample, only 3 of 12 patients with SSc who had a current major depressive episode at baseline met diagnostic criteria 1 month later. Some episodes may be time limited and may resolve without targeted intervention or treatment. Others may reflect ongoing moderate symptoms that only variably meet criteria for formal diagnosis. Many patients with SSc describe ongoing emotional distress from the burden of living with the disease, but differentiate this qualitatively from what they consider clinical depression. Cross sectionally, factors associated with symptoms of depression in SSc include greater overall disease burden, which may involve degree of gastrointestinal involvement; breathing problems; skin involvement; and tender joints.

Anxiety and Fear

Anxiety can be a normal reaction to stress; however, it may also lead to mental health problems when experienced in excess. To date, only 1 study documented the prevalence of anxiety disorders among patients with SSc. In that study, 49% of 50 hospitalized patients and 50 patients who attended a patient meeting had at least 1 current anxiety disorder, and 64% met criteria for at least 1 anxiety disorder in their lifetimes. Social anxiety and generalized anxiety disorder were the most common. There was no difference in prevalence between hospitalized and nonhospitalized patients.

The course of SSc is highly unpredictable and patients may perceive the future as uncertain. For patients, worry about the future, including fear of disease progression, fear of becoming physically disabled, and fear of being dependent on others, is an important source of stress. Because SSc is unpredictable and associated with serious consequences, these concerns are realistic and in themselves do not represent anxiety disorders, for which irrational fear is typically a central component. Nonetheless, fear of progression can affect HRQL substantially. In a cross-sectional study of 215 patients with SSc from the Netherlands, fear of progression was highly associated with symptoms of depression.

Fatigue

Fatigue from a chronic medical disease is characterized by persistent exhaustion that is disproportionate to exertion and not relieved by rest. Fatigue is the most commonly experienced symptom of SSc and has a substantial impact on HRQL, as well as the ability to perform daily activities, including work. In one Canadian study, 89% of 464 patients with SSc reported fatigue at least some of the time, and 81% of these patients indicated that fatigue had at least a moderate impact on their daily function. Levels of fatigue in SSc are similar to those experienced by patients with other rheumatic diseases and by patients with cancer undergoing active treatment. Cross sectionally, greater fatigue in SSc is associated with increased medical comorbidities, current smoking, pain, breathing problems, and gastrointestinal symptoms. Longitudinally, fatigue severity has been associated with pain; severity of gastrointestinal involvement; and psychological variables, specifically ineffective coping skills.

Sleep

Significant sleep disruption is common in SSc and has broad implications for patients. A polysomnography study of 27 patients with SSc found that, compared with age-adjusted norms, patients with SSc had reduced sleep efficiency and rapid eye movement sleep, as well as increased arousal and slow wave sleep. Sleep disruption was associated with esophageal dyskinesia and dyspnea, which are common complications of SSc, as well as restless legs syndrome. Observational studies have linked dyspnea, pain, fatigue, pruritus, gastrointestinal symptoms, and depressive symptoms with self-reported poor sleep quality and sleep disruption in SSc.

Pain

Between 60% and 83% of patients with SSc report experiencing pain at any given time, and pain levels in SSc are similar to levels reported in chronic pain and rheumatic conditions. Pain in SSc is associated with reduced HRQL, functional disability, work disability, sleep problems, and symptoms of depression. Patients with SSc describe their pain as both localized and generalized in quality, and sources can include pain from Raynaud phenomenon, gastrointestinal pain, joint and musculoskeletal pain, skin pain, and pain caused by calcinosis and ulcers. Pain ratings are higher among patients with diffuse SSc compared with patients with limited disease, although this difference is generally small. Based on patient reports, overall pain levels are associated with sleep problems, fatigue, and symptoms of depression, as well as physical function, reduced ability to perform daily activities, work disability, and poorer HRQL. How patients with SSc describe their pain and the degree to which they think they can manage it often reflects psychosocial factors, which should be considered in assessment and intervention, especially as related to pharmacologic treatment.

Pruritus

Pruritus, or itch, is common in SSc and is associated with HRQL, even after controlling for sociodemographic and other SSc symptom variables. Overall, 43% of 959 patients with SSc from a Canadian registry reported pruritus on most days in the last month. This rate was slightly higher, but not statistically significant, among patients with early SSc (<5 years since onset of non-Raynaud symptoms, 46%) versus those with longer disease duration (≥5 years, 41%). The presence of pruritus is more common among patients with greater skin and gastrointestinal involvement.

Body Image

Acquired disfigurement from an injury or medical illness is often linked to problems with body image, including social avoidance. Appearance changes in highly visible areas of the body, particularly the face and hands, are common in patients with SSc and contribute to body image distress, which in turn can be associated with symptoms of anxiety and depression. Several cross-sectional studies have reported that appearance changes of the face, including changes to the mouth, as well as hand involvement, including skin thickening, have consistently been related to body image distress, including dissatisfaction with appearance, decreased appearance self-esteem, and symptoms of anxiety and depression. Other appearance changes, including telangiectasias, may also be associated with body image distress. Social discomfort caused by changes in appearance is also related to age. Younger patients, for whom the importance of meeting new people and developing intimate relationships is more pronounced, may experience a greater negative impact of appearance changes on social relationships.

Sexual Function

Sexual dysfunction is a common problem among women with SSc. Compared with women in the general population, women with SSc are significantly less likely to be sexually active, and sexually active women with SSc are significantly more likely to be sexually impaired. Factors that are independently associated with being sexually active include younger age, fewer gastrointestinal symptoms, and less severe Raynaud phenomenon symptoms. Among women who are sexually active, sexual impairment is associated with older age, as well as with more severe skin involvement and breathing problems. Vaginal pain is 8 times as common among women with impairment compared with those without.

Among men with SSc, erectile dysfunction (ED) is common with onset typically occurring several years after the manifestation of the first non-Raynaud symptoms. In the general population, ED is typically associated with atherosclerosis, but in SSc penile blood flow is impaired because of both myointimal proliferation of small arteries and corporal fibrosis. Men with SSc who have ED are significantly more likely to be older than those without ED and tend to report non-SSc risk factors (eg, alcohol consumption) at higher rates. SSc factors associated with ED include severe cutaneous, muscular, or renal involvement; diffuse disease; increased pulmonary pressures; restrictive lung disease;, endothelial dysfunction; and microvascular damage. Most men with SSc who have ED do not receive treatment. Among those who do, sildenafil seems to be commonly used, but its efficacy has not been established in SSc.

Depression

Depression involves symptoms that may include sadness, loss of interest or pleasure, feelings of guilt or low self-esteem, poor concentration, and disturbed sleep or appetite. A study of 345 patients with SSc enrolled in a Canadian registry reported that the prevalence of major depressive disorder (MDD) was 4% for the past 30 days, 11% for the past 12 months, and 23% for lifetime. A French study of 50 hospitalized patients with SSc and 50 patients with SSc who attended a patient organization meeting found that 19% had current MDD and 56% had lifetime MDD, and rates were higher in hospitalized (28% current) versus nonhospitalized (10% current) patients. Depression is substantially more common in patients with SSc than in the general population and may be more prevalent than in other rheumatic diseases. However, many patients with SSc and other chronic diseases who meet criteria for MDD at a given time point do not meet criteria consistently. In the Canadian sample, only 3 of 12 patients with SSc who had a current major depressive episode at baseline met diagnostic criteria 1 month later. Some episodes may be time limited and may resolve without targeted intervention or treatment. Others may reflect ongoing moderate symptoms that only variably meet criteria for formal diagnosis. Many patients with SSc describe ongoing emotional distress from the burden of living with the disease, but differentiate this qualitatively from what they consider clinical depression. Cross sectionally, factors associated with symptoms of depression in SSc include greater overall disease burden, which may involve degree of gastrointestinal involvement; breathing problems; skin involvement; and tender joints.

Anxiety and Fear

Anxiety can be a normal reaction to stress; however, it may also lead to mental health problems when experienced in excess. To date, only 1 study documented the prevalence of anxiety disorders among patients with SSc. In that study, 49% of 50 hospitalized patients and 50 patients who attended a patient meeting had at least 1 current anxiety disorder, and 64% met criteria for at least 1 anxiety disorder in their lifetimes. Social anxiety and generalized anxiety disorder were the most common. There was no difference in prevalence between hospitalized and nonhospitalized patients.

The course of SSc is highly unpredictable and patients may perceive the future as uncertain. For patients, worry about the future, including fear of disease progression, fear of becoming physically disabled, and fear of being dependent on others, is an important source of stress. Because SSc is unpredictable and associated with serious consequences, these concerns are realistic and in themselves do not represent anxiety disorders, for which irrational fear is typically a central component. Nonetheless, fear of progression can affect HRQL substantially. In a cross-sectional study of 215 patients with SSc from the Netherlands, fear of progression was highly associated with symptoms of depression.

Fatigue

Fatigue from a chronic medical disease is characterized by persistent exhaustion that is disproportionate to exertion and not relieved by rest. Fatigue is the most commonly experienced symptom of SSc and has a substantial impact on HRQL, as well as the ability to perform daily activities, including work. In one Canadian study, 89% of 464 patients with SSc reported fatigue at least some of the time, and 81% of these patients indicated that fatigue had at least a moderate impact on their daily function. Levels of fatigue in SSc are similar to those experienced by patients with other rheumatic diseases and by patients with cancer undergoing active treatment. Cross sectionally, greater fatigue in SSc is associated with increased medical comorbidities, current smoking, pain, breathing problems, and gastrointestinal symptoms. Longitudinally, fatigue severity has been associated with pain; severity of gastrointestinal involvement; and psychological variables, specifically ineffective coping skills.

Sleep

Significant sleep disruption is common in SSc and has broad implications for patients. A polysomnography study of 27 patients with SSc found that, compared with age-adjusted norms, patients with SSc had reduced sleep efficiency and rapid eye movement sleep, as well as increased arousal and slow wave sleep. Sleep disruption was associated with esophageal dyskinesia and dyspnea, which are common complications of SSc, as well as restless legs syndrome. Observational studies have linked dyspnea, pain, fatigue, pruritus, gastrointestinal symptoms, and depressive symptoms with self-reported poor sleep quality and sleep disruption in SSc.

Pain

Between 60% and 83% of patients with SSc report experiencing pain at any given time, and pain levels in SSc are similar to levels reported in chronic pain and rheumatic conditions. Pain in SSc is associated with reduced HRQL, functional disability, work disability, sleep problems, and symptoms of depression. Patients with SSc describe their pain as both localized and generalized in quality, and sources can include pain from Raynaud phenomenon, gastrointestinal pain, joint and musculoskeletal pain, skin pain, and pain caused by calcinosis and ulcers. Pain ratings are higher among patients with diffuse SSc compared with patients with limited disease, although this difference is generally small. Based on patient reports, overall pain levels are associated with sleep problems, fatigue, and symptoms of depression, as well as physical function, reduced ability to perform daily activities, work disability, and poorer HRQL. How patients with SSc describe their pain and the degree to which they think they can manage it often reflects psychosocial factors, which should be considered in assessment and intervention, especially as related to pharmacologic treatment.

Pruritus

Pruritus, or itch, is common in SSc and is associated with HRQL, even after controlling for sociodemographic and other SSc symptom variables. Overall, 43% of 959 patients with SSc from a Canadian registry reported pruritus on most days in the last month. This rate was slightly higher, but not statistically significant, among patients with early SSc (<5 years since onset of non-Raynaud symptoms, 46%) versus those with longer disease duration (≥5 years, 41%). The presence of pruritus is more common among patients with greater skin and gastrointestinal involvement.

Body Image

Acquired disfigurement from an injury or medical illness is often linked to problems with body image, including social avoidance. Appearance changes in highly visible areas of the body, particularly the face and hands, are common in patients with SSc and contribute to body image distress, which in turn can be associated with symptoms of anxiety and depression. Several cross-sectional studies have reported that appearance changes of the face, including changes to the mouth, as well as hand involvement, including skin thickening, have consistently been related to body image distress, including dissatisfaction with appearance, decreased appearance self-esteem, and symptoms of anxiety and depression. Other appearance changes, including telangiectasias, may also be associated with body image distress. Social discomfort caused by changes in appearance is also related to age. Younger patients, for whom the importance of meeting new people and developing intimate relationships is more pronounced, may experience a greater negative impact of appearance changes on social relationships.

Sexual Function

Sexual dysfunction is a common problem among women with SSc. Compared with women in the general population, women with SSc are significantly less likely to be sexually active, and sexually active women with SSc are significantly more likely to be sexually impaired. Factors that are independently associated with being sexually active include younger age, fewer gastrointestinal symptoms, and less severe Raynaud phenomenon symptoms. Among women who are sexually active, sexual impairment is associated with older age, as well as with more severe skin involvement and breathing problems. Vaginal pain is 8 times as common among women with impairment compared with those without.

Among men with SSc, erectile dysfunction (ED) is common with onset typically occurring several years after the manifestation of the first non-Raynaud symptoms. In the general population, ED is typically associated with atherosclerosis, but in SSc penile blood flow is impaired because of both myointimal proliferation of small arteries and corporal fibrosis. Men with SSc who have ED are significantly more likely to be older than those without ED and tend to report non-SSc risk factors (eg, alcohol consumption) at higher rates. SSc factors associated with ED include severe cutaneous, muscular, or renal involvement; diffuse disease; increased pulmonary pressures; restrictive lung disease;, endothelial dysfunction; and microvascular damage. Most men with SSc who have ED do not receive treatment. Among those who do, sildenafil seems to be commonly used, but its efficacy has not been established in SSc.