What is the effect of physical/exercise therapy on physical function in patients with systemic sclerosis?
Systemic sclerosis (SSc) is a relatively rare disease of the connective tissues in the body resulting in debilitating consequences. It is characterized by diffuse fibrosis and abnormalities within the connective tissue of the skin, joints, and eventually internal organs. Specific etiology is not known. Associated signs and symptoms include pain, edema, decreased range of motion, skin thickening, and joint contractures which are all associated with decreased functional ability and reduced quality of life. Unfortunately, research regarding nonpharmaceutical interventions for systemic sclerosis is extremely limited. Research and treatment that is available usually focus on skin lesions and complications to internal organs as the disease progresses. However, physical therapy and/or skilled exercise therapy may be able to address functional concerns and improve the quality of life in both the early and late stages of the disease.
Murphy, et al., utilized an eight-week, in-person, program consisting of tissue mobilizations, thermal modalities, upper extremity mobility, and a home exercise program in patients with early SSc (< 5 years) to determine possible benefits for upper extremity function. The study utilized a single group with pre- and post-test measures to determine improvement in the upper extremity QuickDASH, range of motion, coordination, skin thickening, and Patient-Reported Outcomes Measurement Information System (PROMIS). Patients, on average, had a moderate skin disease and the majority were also being treated with immunosuppressive therapy or were participating in clinical trials. Researchers found that there was not a meaningful improvement at four weeks into the program but did find significant improvement from baseline at the end of the eight weeks. On average, participants improved QuickDASH measures by 14 points, PROMIS by 3.1 points, 41.5 degrees of active motion, and were faster in completing the 9-hole peg test with the left hand. All are significant findings from baseline. However, no significant improvement was shown with right-hand coordination or active motion. Long-term effects were not evaluated, and it is unclear how many sessions are optimal for best results. Additional limitations of this study include the single test group, lack of tracking regarding home exercise program compliance, small sample size, the treating therapist was also the accessor, and that the study focused only on participants in the early stages of the disease (Murphy et al., 2018).
Waszczykowski et al., evaluated the effectiveness of supervised exercise therapy on hand and overall function in SSc with a one-year follow-up. This study evaluated a four-week program of supervised rehabilitation compared to a prescribed home exercise program alone. Assessments were performed at one-, three-, six-, and twelve-month follow-ups. Outcomes used included: QuickDASH, visual analog scale (VAS), Cochin Hand Function Scale (CHFS), Health Assessment Questionnaire Disability Index (HAQ-DI), Scleroderma-HAQ, hand grip, pinch grip, and range of motion. Massage in the whirlpool coupled with upper extremity active exercises, soft tissue massage, and hand joint manipulation was used with the goal of reducing pain and improving hand function. Results indicated significant positive effects for the intervention group at one-, three-, and six-month follow-ups, but only at the one-month follow-up for the control group. At the twelve-month follow-up, no significant improvement was noted indicating limited long-term effects. As a result, researchers recommend regular repetition of the program every six months at a minimum. Limitations of this study include lack of randomization, small sample size, and difficulty blinding participants and researchers (Waszczykowski et al., 2021).
Liem, et al., conducted a systematic review of the effectiveness and safety of exercise therapy in this patient population to help address the limitation of available research. The review consisted of nine articles, four randomized controlled trials and five observational studies. The review compared the interventions of hand exercises, aerobic exercise, and aerobic exercises with resistance training to no exercise. A variety of outcome measures were used to determine the effect on hand function, maximal mouth opening, quality of life, and oxygen uptake. Sample size of the different studies remained small, ranging from four to forty-eight participants with most of them females. It was determined that strengthening and mobility exercises when supported by telemedicine, were more beneficial in hand pain and functioning compared to a similar intervention using only common objects. In addition, two different studies evaluated the effect of exercise on maximal oral aperture. Interventions groups had significant improvement compared to the control groups, however, one group found that the results were short-lived and were not significant at the six-month follow-up. The last set of articles evaluated aerobic and/or resistance exercises. Both utilized a twice a week for twelve-week supervised high-intensity interval training (HIIT) program. After verifying the benefit of the base program, the researchers decided to evaluate the benefit of resistance training in combination with the established HIIT program. Resistance training consisted of chest press, bicep curl, triceps extension, lateral arm raise, and handgrip dynamometer. Results concluded the combined aerobic and resistance training improved muscle strength and function, resting heart rate, and workload and time of exercise at the ventilatory threshold as well as peak exercise were increased. A third study evaluated the effectiveness of an eight-week moderate intensity treadmill program on aerobic capacity and found similar beneficial results (Liem et al., 2019).
Overall, it is difficult to make evidence-based clinical decisions due to the lack of quality evidence. The rarity of the disease makes obtaining a suitable sample size to account for the required effect size, and difficulty blinding participants and researchers. Due to these factors, it is difficult to determine the true validity of the results. However, many of the participants had to travel more than thirty minutes for their sessions, and the adherence rate stayed high. This speaks to the feasibility of using the mentioned interventions in this population in the clinic. Unfortunately, the proposed intervention programs all varied in frequency, intensity, duration, and interventions with a highly individualized approach. In-clinic and home exercise programs were often individualized based on patients’ values and goals for treatment. Most of them also focused mainly on hand/upper extremity function without evaluating lower extremity and community/home-based mobility at all. Most of which also focused mainly on hand/upper extremity function without evaluating lower extremity and community/home-based mobility at all. Furthermore, it is challenging to determine the true effects across multiple studies because of the wide variety of outcome measures used. For example, the Functional Index for Hand OsteoArthritis was used in one study but not in others. Use of this type of outcome measure also poses its own questions with validity considering it has been validated for a different patient population than the one being examined. Even well-known and validated outcome measures, such as the QuickDASH, have a debate regarding the minimal clinically important difference score ranging from eight to fourteen. Although the evidence is weak for exercise therapy in patients with SSc, by culminating all the evidence it can be determined that exercise therapy is safe for these patients with no to minimal adverse effects. Results seem to indicate that using a combination of modalities, supervised exercise, and home exercise programs provides the most benefit and that these programs should be continued intermittently long-term because of the short-lived effects.
Liem, S. I., Vliet Vlieland, T. P., Schoones, J. W., & de Vries-Bouwstra, J. K. (2019). The effect and safety of exercise therapy in patients with systemic sclerosis: A systematic review. Rheumatology Advances in Practice, 3(2). https://doi.org/10.1093/rap/rkz044
Murphy, S., Barber, M., Homer, K., Dodge, C., Cutter, G., & Khanna, D. (2018). Occupational therapy treatment to improve upper extremity function in individuals with early systemic sclerosis: A pilot study. Arthritis Care & Research , 70(11), 1653–1660. https://doi.org/10.1002/acr.23522
Waszczykowski, M., Dziankowska-Bartkowiak, B., Podgórski, M., Fabiś, J., & Waszczykowska, A. (2021). Role and effectiveness of complex and supervised rehabilitation on overall and hand function in systemic sclerosis patients—one-year follow-up study. Scientific Reports, 11(15174). https://doi.org/10.1038/s41598-021-94549-y
What is the effect of physical/exercise therapy on physical function in patients with systemic sclerosis?
Systemic sclerosis (SSc) is a relatively rare disease of the connective tissues in the body resulting in debilitating consequences. It is characterized by diffuse fibrosis and abnormalities within the connective tissue of the skin, joints, and eventually internal organs. Specific etiology is not known. Associated signs and symptoms include pain, edema, decreased range of motion, skin thickening, and joint contractures which are all associated with decreased functional ability and reduced quality of life. Unfortunately, research regarding nonpharmaceutical interventions for systemic sclerosis are extremely limited. Research and treatment that is available, usually focuses on skin lesions and complications to internal organs as the disease progresses. However, physical therapy and/or skilled exercise therapy may be able to address functional concerns and improve quality of life in both the early and late stages of the disease.
Murphy, et al.,utilized an eight week, in-person, program consisting of tissue mobilizations, thermal modalities, upper extremity mobility, and a home exercise program in patients with early SSc (< 5 years) to determine possible benefit for upper extremity function. The study utilized a single group with pre- and post-test measures to determine improvement on the upper extremity QuickDASH, range of motion, coordination, skin thickening, and Patient-Reported Outcomes Measurement Information System (PROMIS). Patients, on average, had moderate skin disease and the majority were also being treated with immunosuppressive therapy or were participating in clinical trials. Researchers found that that there was not a meaningful improvement at four weeks into the program but did find significant improvement from baseline at the end of the eight-week period. On average, participants improved QuickDASH measures by 14 points, PROMIS by 3.1 points, 41.5 degrees of active motion, and were faster in completing the 9-hole peg test with the left hand. All are significant findings from baseline. However, no significant improvement was shown with right hand coordination or active motion. Long-term effects were not evaluated, and it is unclear how many sessions are optimal for best results. Additional limitations of this study include the single test group, lack of tracking regarding home exercise program compliance, small sample size, the treating therapist was also the accessor, and that the study focused only on participants in the early stages of the disease (Murphy et al., 2018).
Waszczykowski et al., evaluated the effectiveness of supervised exercise therapy on hand and overall function in SSc with a one-year follow-up. This study evaluated a four-week program of supervised rehabilitation compared to a prescribed home exercise program alone. Assessments were performed at one-, three-, six-, and twelve-month follow-ups. Outcomes used included: QuickDASH, visual analog scale (VAS), Cochin Hand Function Scale (CHFS), Health Assessment Questionnaire Disability Index (HAQ-DI), Scleroderma-HAQ, hand grip, pinch grip, and range of motion. Massage in the whirlpool coupled with upper extremity active exercises, soft tissue massage, hand joint manipulation was used with the goal of reducing pain and improving hand function. Results indicated significant positive effects for the intervention group at one-, three-, and six-month follow-ups, but only at the one-month follow-up for the control group. At the twelve-month follow-up, no significant improvement was noted indicating limited long-term effects. As a result, researchers recommend regular repetition of the program at every six-months at minimum. Limitations of this study include lack of randomization, small sample size, and difficulty blinding participates and researchers (Waszczykowski et al., 2021).
Liem, et al., conducted a systematic review of the effectiveness and safety of exercise therapy in this patient population to help address the limitation of available research. The review consisted of nine articles, four randomized controlled trials and five observational studies. The review compared the interventions of hand exercises, aerobic exercise, and aerobic exercises with resistance training to no exercise. A variety of outcome measures were used to determine the effect on hand function, maximal mouth opening, quality of life, and oxygen uptake. Sample size of the different studies remained small, ranging from four to forty-eight participants with most of them females. It was determined that strengthening and mobility exercises, when supported by telemedicine, was more beneficial in hand pain and functioning compared to a similar intervention using only common objects. In addition, two different studies evaluated the effect of exercise on maximal oral aperture. Interventions groups had significant improvement compared to the control groups, however, one group found that the results were short lived and were not significant at the six-month follow-up. The last set of articles evaluated aerobic and/or resistance exercises. Both utilized a twice a week for twelve-weeks supervised high-intensity interval training (HIIT) program. After verifying a benefit from the base program, the researchers decided to evaluate the benefit of resistance training in combination with the established HIIT program. Resistance training consisted of chest press, bicep curl, triceps extension, lateral arm raise, and handgrip dynamometer. Results concluded the combined aerobic and resistance training improved muscle strength and function, resting heart rate, and workload and time of exercise at ventilatory threshold as well as peak exercise were increased. A third study evaluated the effectiveness of an eight-week moderate intensity treadmill program on aerobic capacity and found similar beneficial results (Liem et al., 2019).
Overall, it is difficult to make evidence-based clinical decisions due to the lack of quality evidence. The rarity of the disease makes obtaining a suitable sample size to account for the required effect size, and difficulty blinding participants and researchers. Due to these factors, it is difficult to determine the true validity of the results. However, many of the participants had to travel more than thirty minutes for their sessions, and the adherence rate stayed high. This speaks to the feasibility of using the mentioned interventions in this population in the clinic. Unfortunately, the proposed intervention programs all varied in frequency, intensity, duration, and interventions with a highly individualized approach. In-clinic and home exercise programs were often individualized based on patients’ values and goals for treatment. Most of which also focused mainly on hand/upper extremity function without evaluating lower extremity and community/home-based mobility at all. Furthermore, it is challenging to determine the true effects across multiple studies because of the wide variety of outcome measures used. For example, the Functional Index for Hand OsteoArthritis was used in one study but in no others. Use of this type of outcome measure also poses its own questions with validity considering it has been validated for a different patient population than the one being examined. Even well-known and validated outcome measures, such as the QuickDASH, have debate regarding the minimal clinically important difference score ranging from eight to fourteen. Although the evidence is weak for exercise therapy in patients with SSc, by culminating all the evidence it can be determined that exercise therapy is safe for these patients with none to minimal adverse effects. Results seem to indicate that using a combination of modalities, supervised exercise, and home exercise program provides the most benefit, and that these programs should be continued intermittently long-term because of the short-lived effects.
Liem, S. I., Vliet Vlieland, T. P., Schoones, J. W., & de Vries-Bouwstra, J. K. (2019). The effect and safety of exercise therapy in patients with systemic sclerosis: A systematic review. Rheumatology Advances in Practice, 3(2). https://doi.org/10.1093/rap/rkz044
Murphy, S., Barber, M., Homer, K., Dodge, C., Cutter, G., & Khanna, D. (2018). Occupational therapy treatment to improve upper extremity function in individuals with early systemic sclerosis: A pilot study. Arthritis Care & Research , 70(11), 1653–1660. https://doi.org/10.1002/acr.23522
Waszczykowski, M., Dziankowska-Bartkowiak, B., Podgórski, M., Fabiś, J., & Waszczykowska, A. (2021). Role and effectiveness of complex and supervised rehabilitation on overall and hand function in systemic sclerosis patients—one-year follow-up study. Scientific Reports, 11(15174). https://doi.org/10.1038/s41598-021-94549-y
What is the effect of physical/exercise therapy on physical function in patients with systemic sclerosis?
Systemic sclerosis (SSc) is a relatively rare disease of the connective tissues in the body resulting in debilitating consequences. It is characterized by diffuse fibrosis and abnormalities within the connective tissue of the skin, joints, and eventually internal organs. Specific etiology is not known. Associated signs and symptoms include pain, edema, decreased range of motion, skin thickening, and joint contractures which are all associated with decreased functional ability and reduced quality of life. Unfortunately, research regarding nonpharmaceutical interventions for systemic sclerosis is extremely limited. Research and treatment that is available usually focus on skin lesions and complications to internal organs as the disease progresses. However, physical therapy and/or skilled exercise therapy may be able to address functional concerns and improve the quality of life in both the early and late stages of the disease.
Murphy, et al., utilized an eight-week, in-person, program consisting of tissue mobilizations, thermal modalities, upper extremity mobility, and a home exercise program in patients with early SSc (< 5 years) to determine possible benefits for upper extremity function. The study utilized a single group with pre- and post-test measures to determine improvement in the upper extremity QuickDASH, range of motion, coordination, skin thickening, and Patient-Reported Outcomes Measurement Information System (PROMIS). Patients, on average, had a moderate skin disease and the majority were also being treated with immunosuppressive therapy or were participating in clinical trials. Researchers found that there was not a meaningful improvement at four weeks into the program but did find significant improvement from baseline at the end of the eight weeks. On average, participants improved QuickDASH measures by 14 points, PROMIS by 3.1 points, 41.5 degrees of active motion, and were faster in completing the 9-hole peg test with the left hand. All are significant findings from baseline. However, no significant improvement was shown with right-hand coordination or active motion. Long-term effects were not evaluated, and it is unclear how many sessions are optimal for best results. Additional limitations of this study include the single test group, lack of tracking regarding home exercise program compliance, small sample size, the treating therapist was also the accessor, and that the study focused only on participants in the early stages of the disease (Murphy et al., 2018).
Waszczykowski et al., evaluated the effectiveness of supervised exercise therapy on hand and overall function in SSc with a one-year follow-up. This study evaluated a four-week program of supervised rehabilitation compared to a prescribed home exercise program alone. Assessments were performed at one-, three-, six-, and twelve-month follow-ups. Outcomes used included: QuickDASH, visual analog scale (VAS), Cochin Hand Function Scale (CHFS), Health Assessment Questionnaire Disability Index (HAQ-DI), Scleroderma-HAQ, hand grip, pinch grip, and range of motion. Massage in the whirlpool coupled with upper extremity active exercises, soft tissue massage, and hand joint manipulation was used with the goal of reducing pain and improving hand function. Results indicated significant positive effects for the intervention group at one-, three-, and six-month follow-ups, but only at the one-month follow-up for the control group. At the twelve-month follow-up, no significant improvement was noted indicating limited long-term effects. As a result, researchers recommend regular repetition of the program every six months at a minimum. Limitations of this study include lack of randomization, small sample size, and difficulty blinding participants and researchers (Waszczykowski et al., 2021).
Liem, et al., conducted a systematic review of the effectiveness and safety of exercise therapy in this patient population to help address the limitation of available research. The review consisted of nine articles, four randomized controlled trials and five observational studies. The review compared the interventions of hand exercises, aerobic exercise, and aerobic exercises with resistance training to no exercise. A variety of outcome measures were used to determine the effect on hand function, maximal mouth opening, quality of life, and oxygen uptake. Sample size of the different studies remained small, ranging from four to forty-eight participants with most of them females. It was determined that strengthening and mobility exercises when supported by telemedicine, were more beneficial in hand pain and functioning compared to a similar intervention using only common objects. In addition, two different studies evaluated the effect of exercise on maximal oral aperture. Interventions groups had significant improvement compared to the control groups, however, one group found that the results were short-lived and were not significant at the six-month follow-up. The last set of articles evaluated aerobic and/or resistance exercises. Both utilized a twice a week for twelve-week supervised high-intensity interval training (HIIT) program. After verifying the benefit of the base program, the researchers decided to evaluate the benefit of resistance training in combination with the established HIIT program. Resistance training consisted of chest press, bicep curl, triceps extension, lateral arm raise, and handgrip dynamometer. Results concluded the combined aerobic and resistance training improved muscle strength and function, resting heart rate, and workload and time of exercise at the ventilatory threshold as well as peak exercise were increased. A third study evaluated the effectiveness of an eight-week moderate intensity treadmill program on aerobic capacity and found similar beneficial results (Liem et al., 2019).
Overall, it is difficult to make evidence-based clinical decisions due to the lack of quality evidence. The rarity of the disease makes obtaining a suitable sample size to account for the required effect size, and difficulty blinding participants and researchers. Due to these factors, it is difficult to determine the true validity of the results. However, many of the participants had to travel more than thirty minutes for their sessions, and the adherence rate stayed high. This speaks to the feasibility of using the mentioned interventions in this population in the clinic. Unfortunately, the proposed intervention programs all varied in frequency, intensity, duration, and interventions with a highly individualized approach. In-clinic and home exercise programs were often individualized based on patients’ values and goals for treatment. Most of them also focused mainly on hand/upper extremity function without evaluating lower extremity and community/home-based mobility at all. Most of which also focused mainly on hand/upper extremity function without evaluating lower extremity and community/home-based mobility at all. Furthermore, it is challenging to determine the true effects across multiple studies because of the wide variety of outcome measures used. For example, the Functional Index for Hand OsteoArthritis was used in one study but not in others. Use of this type of outcome measure also poses its own questions with validity considering it has been validated for a different patient population than the one being examined. Even well-known and validated outcome measures, such as the QuickDASH, have a debate regarding the minimal clinically important difference score ranging from eight to fourteen. Although the evidence is weak for exercise therapy in patients with SSc, by culminating all the evidence it can be determined that exercise therapy is safe for these patients with no to minimal adverse effects. Results seem to indicate that using a combination of modalities, supervised exercise, and home exercise programs provides the most benefit and that these programs should be continued intermittently long-term because of the short-lived effects.
