Effects of non-pharmacological therapies on hand function and the ability to perform daily activities in people with systemic sclerosis: A systematic review and meta-analysis of randomized control trials

Background

Systemic sclerosis (SSc) is an autoimmune rheumatic disease. Individuals with a diagnosis of SSc describe repercussions on their activities of daily living and instrumental activities of daily living that affect their everyday functional capacity. The objective of this systematic review was to explore the effectiveness of non-pharmacological interventions to improve hand function and the ability to perform activities of daily living.

Methods

A systematic review was conducted on the Cochrane Library, Medline/PubMed, OTseeker, PEDro, Scopus, Web of Science up to September 10, 2022. Inclusion criteria were defined following PICOS recommendations (Populations, Intervention, Comparison and Outcome measures). Methodological quality was assessed with the Downs and Black Scale and risk of bias was assessed using version 2 of the Cochrane risk-of-bias tool for randomized trials (RoB 2). A meta-analysis of each outcome was performed.

Results

A total of 8 studies met the inclusion criteria, providing data on 487 individuals with SSc. The non-pharmacological intervention applied the most was exercise. The effects of non-pharmacological interventions were better than those of the waiting list or no treatment control conditions in both outcomes – hand function (mean difference [MD] = −6.98; 95% CI [−11.45, − 2.50], P = 0.002, I² = 0%) and performance of daily activities (MD = −0.19; 95% CI [−0.33, − 0.04], P = 0.01, I² = 0%). Moderate risk of bias was found in the majority of the studies included.

Conclusion

There is emerging evidence that non-pharmacological interventions can improve hand function and performance of daily activities in individuals with a diagnosis of SSc. Given the moderate risk of bias found in the studies included, the results should be considered with caution.     

Tumor necrosis factor-α inhibitor-induced morphea and psoriasiform dermatitis in a pediatric patient with Crohn's disease

Tumor necrosis factor-alpha inhibitor therapy for inflammatory bowel disease may be associated with paradoxical cutaneous adverse events, most commonly psoriasiform eruptions. We present the case of a pediatric female patient with Crohn's disease who developed multiple concurrent cutaneous eruptions while on infliximab treatment, including morphea, psoriasiform dermatitis, and genital lichen sclerosus. Although refractory to skin-directed treatments, all three conditions resolved upon discontinuation of infliximab, supporting their development as a paradoxical reaction to infliximab therapy.     

Is External Cervical Resorption an Established Manifestation of Systemic Sclerosis? A Case Report

Scleroderma, or systemic sclerosis, is a multisystem autoimmune disorder characterized by hardening and fibrosis of the skin. To date, only a small number of case reports have established a relationship between scleroderma and external cervical resorption (ECR). The aim of this case report is to document the case of a patient with multiple external cervical resorption lesions, who was referred to our unit. A 54-year-old female patient, with a 10-year history of systemic sclerosis diagnosed by her rheumatologist, was referred to our unit regarding extensive ECR. A total of 14 maxillary and mandibular teeth with ECR were detected by clinical examination and cone-beam computed tomography. The characteristic vascularity of resorptive defects with profuse bleeding upon probing was not evident. The patient declined any active treatment owing to the desire to avoid lengthy and unpredictable treatment, which may hasten the loss of her teeth. General practitioners should be aware of the relationship between connective tissue disorders and ECR. Although not well established in the literature, the vascular changes implicated in scleroderma may stimulate the odontoclastic processes involved in ECR.     

The co-occurrence of segmental vitiligo and linear morphea in a pediatric patient and a review of the literature

Linear morphea and segmental vitiligo are both autoimmune diseases that are observed in the pediatric population, with rare reports of their co-existence. We describe a case of linear morphea and segmental vitiligo with an overlapping distribution in a pediatric patient and review the literature. Including our own case, we summarize 10 cases of co-occurring segmental vitiligo and morphea in pediatric patients; most of these lesions follow a blaschkolinear distribution, and none of the patients had autoimmune thyroid disease. Although uncommon, the coexistence of segmental vitiligo and linear morphea within lines of Blaschko can occur, and this case suggests that linear morphea and segmental vitiligo may be disorders related to genetic mosaicism.     

Presenting characteristics and progression of pediatric-onset morphea: Interim analysis of a prospective registry

Morphea is a rare fibrosing disorder with a highly variable disease course, which can complicate management. Here, we present a prospective cohort study describing the current treatments used in the management of pediatric-onset morphea and assessing responses to systemic and topical therapies. Most patients demonstrated inactive disease by 1 year, regardless of treatment, though recurrences were common in our cohort overall (39%). Our results support the need for continuous monitoring of all children with morphea following the completion of treatment, including topical treatment, due to high rates of disease relapse.