ARTERIAL INSUFFICIENCIES: Hyperbaric Oxygen Therapy for Selected Problem Wounds.

The use of hyperbaric oxygen (HBO2) for the treatment of selected problem wounds has focused almost entirely on the diabetic foot ulcer (DFU) in recent years. The prevalence of DFUs in today's patient population and the reimbursement available for the treatment of DFUs have given it priority status in discussions about problem wounds, but there are sound fundamental reasons why additional oxygen may have benefits in the treatment of non-DFU wounds.

Hyaluronic acid and platelet-rich plasma, a new therapeutic alternative for scleroderma patients: a prospective open-label study.

BACKGROUND: Systemic sclerosis is a systemic connective tissue disease characterized by endothelium damage, fibrosis, and subsequent atrophy of the skin. Perioral fibrosis produces a characteristic microstomia together with microcheilia, both of which cause severe difficulties and affects patients' daily life, such as eating and oral hygiene. Since there are no effective and specific therapies, we have aimed at evaluating the response to filler injections of hyaluronic acid together with platelet-rich plasma. METHODS: Ten female patients aged between 18 and 70 were included in this study. Each patient was treated with three filler injections of hyaluronic acid and platelet-rich plasma at an interval of 15 to 20 days. Follow-up check-ups were recorded 1, 3, and 24 months after the end of the treatment. During the therapy and the subsequent follow-up, we evaluated the mouth's opening, freedom of movement of the lips, and skin elasticity. RESULTS: After the treatment, patients had achieved good results already after the first injection and the improvement was maintained in the following months, up to 2 years. In particular, 8 (80%) patients showed a greater mouth's opening and increased upper lip's thickness during 1-month follow-up and maintained these results after 2 years (maximum mouth's opening T0 47.61; T3 49.23; T4 48.60 p <  0.0001. Upper lip's thickness T0 4.20; T3 4.75; T4 4.45 p <  0.0001). Moreover, distance between upper and lower incisors (T0 27.05; T3 29.03; T4 28.14 p < 0.0001), inter-commissural distance (T0 49.12; T3 51.44; T4 50.31: p < 0.0001), and lower lip's thickness (T0 3.80; T3 4.85, 5.10; T4 4.25; p < 0.0001) were increased in all of patients in 1-month follow-up, keeping these benefits after 24 months and having a significant increase of skin elasticity 1 month after the end of therapy. CONCLUSIONS: Our study demonstrates that filler injections of hyaluronic acid and platelet-rich plasma represent an efficient local therapeutic alternative for patients affected by scleroderma. The treatment has significantly improved patients' quality of living.

Challenges in the diagnosis and treatment of disabling pansclerotic morphea of childhood: case-based review.

Disabling pansclerotic morphea of childhood (DPMC) is a rare subtype of juvenile localized scleroderma (JLS) characterized by pansclerosis mainly affecting children under the age of 14. This aggressive disease has a poor prognosis due to the rapid progression of deep musculoskeletal atrophy resulting in cutaneous ulceration and severe joint contractures. We describe the challenges in treating a previously well 5-year-old male who has refractory symptoms of DPMC. Over the 29 months, since his initial presentation, we trialed over ten therapies. There was subjective improvement with prednisolone and mycophenolate mofetil (MMF). However, other therapies including biologics and tyrosine kinase inhibitors (TKI) were ineffective. The patient has been referred for hematopoietic stem cell transplant given ongoing disease progression. We conducted a literature search focusing on English articles with keywords including DPMC. Publications with limited information or describing cases aged 20 and above were excluded. Thirty-seven case reports were identified and the reported treatments were evaluated. Methotrexate and corticosteroids have been the most commonly utilized. MMF has been anecdotally effective. Biologics, TKI, and Janus kinase inhibitors lack evidence in DPMC, but have had demonstrated efficacy in similar pathologies including systemic sclerosis, and, thus, have been used for DPMC. Phototherapy has been documented to be reducing skin thickness and stiffness of plaques. Eventually, most children require multi-modal and high-dose immunosuppressive therapies to reduce the inflammation inflicted by the disease. Long-term antibiotics and nutritional support are important in the ongoing care of these patients.

The challenge of pet therapy in systemic sclerosis: evidence for an impact on pain, anxiety, neuroticism and social interaction.

OBJECTIVES: The aim of our study was to evaluate the effect of animal-assisted intervention (AAI), a complementary support to traditional therapies focused on the interaction between animals and human beings, in improving psychological trait, anxiety and pain in a cohort of systemic sclerosis (SSc) patients. METHODS: 42 SSc patients, undergoing iloprost intravenous infusion, were divided in three groups: 1) 14 patients submitted to 20 AAI sessions; 2) 14 patients engaged in alternative social activity (control group 1 - C1); and 3) 14 patients without any alternative activity (control group 2 - C2). All patients underwent Visual Analog Scale (VAS), the State-anxiety (STAI-S) and emotional faces at the beginning (s0) and at the end (s1) of each single session, while General Anxiety State-Trait Anxiety Inventory (STAI-T), Beck Depression Inventory (BDI), Social Interaction Anxiety Scale (SIAS), Eysenck Personality Questionnaire-Revised (EPQ-R), the Social Phobia Scale (SPS), the Toronto Alexythymia Scale (TAS-20), the Thought Control Questionnaire (TCQ) were administered at baseline (t0) and at the end of the project (t1). RESULTS: AAI group showed a significant decrease of the anxiety state level in respect to the two control groups (p<0.001). VAS scale resulted lower both in AAI (p < 0.001) and C1 group (p<0.01). Moreover, STAI-T and TAS scores were significantly reduced in AAI group (p<0.001). TCQ scale showed that patients treated with AAI, compared to control group C2, had greater capacity to avoid unpleasant and unwanted thoughts (p<0.05). In AAI group, the EPQ-R test revealed an enhancement of extroversion trait compared to both control groups (p<0.05). CONCLUSIONS: Our data show that AAI significantly reduces pain perception, anxiety, neuroticism and ameliorates patients' social interaction, therefore it may be a useful to allow a better compliance to traditional therapies.

Prognostic profile of systemic sclerosis: analysis of the clinical EUSTAR cohort in China.

BACKGROUND: Systemic sclerosis is a disease that has significant clinical heterogeneity. This study aims to determine the causes and risk factors of death in a single center European League Against Rheumatism Scleroderma Trials and Research Group (EUSTAR) cohort at the Peking Union Medical College Hospital (PUMCH) in China. METHODS: Patients clinically diagnosed with systemic sclerosis (SSc) between Feb 2009 and Dec 2015 were prospectively recruited from the EUSTAR database and Chinese Rheumatism Data Center (CRDC) of the PUMCH. Baseline and follow-up data were collected. Kaplan-Meier analysis was used to estimate survival, and Cox proportional hazards regression analysis was used to identify factors associated with mortality. RESULTS: A total of 448 patients were included in the cohort, of whom 56.7% had limited cutaneous systemic sclerosis (lcSSc). The average age at diagnosis was 42.8 ± 12.1 years. The prevalence of interstitial lung disease (ILD) was 382/447 (85.5%). Among 402 patients, 348 of them took glucocorticoid during the disease course; 374 patients received immunosuppressors. Across 2167 patient-years, 40 patients died. Of these, 27 deaths were attributable to SSc, with pulmonary arterial hypertension (PAH) being the leading cause of death. The median survival time was 53 months. Survival rates from disease diagnosis were 97.0%, 94.6%, 91.1% and 87.8% at 1, 3, 5 and 10 years, respectively. Independent prognostic factors for mortality were PAH (HR 6.248, 95% CI 2.855, 13.674) and arrhythmia (HR 4.729, 95% CI 1.588, 14.082). Tripterygium wilfordii Hook F (TwHF) (log-rank test 7.851, p 0.005) and methotrexate (MTX) (log-rank test 7.925, p = 0.005) were found in survival analysis to be protective treatments against mortality. Patients who used cyclophosphamide (CTX) during the disease course had poorer prognosis (log-rank test 5.177, p = 0.023). CONCLUSIONS: In china, although there is a high prevalence of ILD in patients with SSc (85.5%), most of them have reserved pulmonary function, which means that interstitial lung disease (ILD) is not the most important factor in the death of patients with SSc and also is not a risk factor for poor prognosis. Only ILD with pulmonary dysfunction is associated with poor outcome. The 10-year cumulative rate (87.8%) in patients with SSc in China is slightly lower than the Europe, and pulmonary arterial hypertension (PAH) and arrhythmia at baseline are independent prognostic factors, whereas PAH instead of ILD is the leading cause of death in patients with SSc. Interestingly, the Chinese traditional medicine TwHF, as a protective factor for survival deserves further study.

Fatigue in systemic sclerosis: a systematic review.

OBJECTIVES: To systematically review fatigue in systemic sclerosis (SSc) in terms of prevalence, features, correlates, predictors and management. METHODS: We performed a literature search in PubMed (Medline), EBSCO and COCHRANE databases up to June 2017 selecting articles regarding fatigue in SSc. The articles finally selected fulfilled the following eligibility criteria: written in English, referred to fatigue in SSc, reporting original data, including validated questionnaires measuring fatigue. RESULTS: A total of 43 records were included. Fatigue in SSc has a prevalence similar to that of other rheumatic diseases and is one of the most prevalent and debilitating symptom experienced by SSc patients. Fatigue leads to a significant impairment of quality of life, parenting, household and work ability. Fatigue is associated with psychosocial factors (depression, pain and sleep disorders), sociodemographic factors and clinical manifestations of the disease (pulmonary and gastrointestinal involvement). Indeed, the relationship with scores of disease activity is uncertain. Pharmacological therapeutic approaches were broadly ineffective in reducing fatigue. More encouraging results concern physical activity, complementary and alternative medicine. CONCLUSIONS: Adequate management of fatigue could lead to a marked improvement of the patient's quality of life, also contributing to reduction in SSc indirect costs.

Systemic sclerosis: Association between physical function, handgrip strength and pulmonary function.

BACKGROUND: Systemic sclerosis (SSc) is a multisystem disease affecting the skin, respiratory system and skeletal muscles. In SSc patients, hand function disability is the major factor limiting daily activities. AIM: To evaluate the association of physical function with handgrip strength and pulmonary function in SSc patients. A further aim was to assess the relationship between handgrip strength and pulmonary function in patients with SSc. METHOD: A cross-sectional study in which 28 patients with SSc underwent isometric handgrip strength (IHGS) measurement and pulmonary function tests and completed the Health Assessment Questionnaire Disability Index (HAQ-DI) to measure physical function. RESULTS: The HAQ-DI scores were associated with the IHGS (rs = -0.599, P = 0.001) and pulmonary function parameters, particularly the diffusion capacity for carbon monoxide (DLco; rs = -0.642, P = 0.0004). CONCLUSION: In patients with SSc, the degree of physical disability is associated with both hand grip strength and pulmonary function. However, there is no relationship between handgrip strength and pulmonary function in these patients.

Efficacy of intensive hand physical therapy in patients with systemic sclerosis.

OBJECTIVES: To evaluate the efficacy of a three-week period of complex and intensive hand physical therapy on hand function in patients with systemic sclerosis (SSc). METHODS: Thirty-one patients with SSc were treated. Hand stretching exercises, ergotherapy supplemented with thermal and mud baths, whirlpool therapy and soft tissue massage were daily used during a three-week period. The control SSc group (n=22) received similar management for their large joints leaving out their hands. The primary outcomes of this study were the Health Assessment Questionnaire (HAQ) and the Disabilities of the Arm, Shoulder and Hand (DASH). Hand Anatomic Index (HAI), Cochin Hand Function (CHFT) and clinical characteristics were also assessed before starting the therapy and six months afterwards. RESULTS: Six months after the investigation period, only the group receiving hand physical therapy showed improvement in HAQ and DASH scores compared to the baseline values (p<0.05). The improvement in median HAQ value (25%-75% quartiles) reached the clinical meaningful rate (baseline 1.125/0.625-1.625/ versus 0.75/0.25-1.5/ at six months). Visual analogue scales of global pain (p<0.01) and Raynaud's phenomenon (p<0.05) also had better results than at baseline. HAI, gripping strength and CHFT also showed some improvement, but did not reach the significance level of change by the end of the six-month observation period. CONCLUSIONS: The complex physical therapy caused favourable changes in both the HAQ and the DASH indicating that this particular program had some long-term beneficial effect on hand function in patients with SSc.

Thermography Improves Clinical Assessment in Patients with Systemic Sclerosis Treated with Ozone Therapy.

Objective. Treatment of scleroderma is challenging and limited. The aim of our study was to evaluate the usefulness of thermography in assessment of the clinical condition (joints movability and skin thickness) in clinically advanced patients with systemic sclerosis before and after ozone therapy. Method. The study included 42 patients aged 32 to 73 years with advanced systemic sclerosis hospitalized in the university clinic between 2003 and 2006. Thermography and clinical examinations were conducted at baseline and after two series of bath in water with ozone. Results. The comparison of results showed significant increase in skin temperature by 2.5°C, significant increase in interphalangeal joints movability by 18 degrees, and significant decrease in skin score by 14.7 points. The skin temperature was correlated with skin score (r = -0.59) and joints movability (r = +0.8). Conclusions. Ozone therapy shows positive effect on clinical parameters and skin temperature as measured with thermography. The study indicated possibility of introducing ozonotherapy as an independent therapy in cases with low level of progression or during remission periods and as additional treatment in patients with advanced disease requiring immunosuppressive treatment. Thermography is useful in assessment of skin condition showing strong correlation between skin temperature and clinical parameters.

Effects of intense pulsed light in microstomia in patients with systemic sclerosis: A pilot study.

INTRODUCTION: The effects of intense pulsed light (IPL) on collagen structures are well known in the treatment of photodamaged skin. OBJECTIVE: The objective of this study was to investigate the effect of IPL on sclerotic skin by treating patients with microstomia due to systemic sclerosis. METHODS AND MATERIALS: 13 patients all with microstomia and systemic sclerosis were treated with IPL, PR (530-750 nm filter) and/or VL (555-950 nm filter) applicator. They were treated in the perioral area 8 times with 3-4 weeks of interval and follow-up for 6 months. The outcomes were the inter-incisal distance and the inter-ridge distance. RESULTS: A significant increase in mouth opening of 4.1 mm (95% confidence interval, 1726-6638, p < 0.005) was found in the inter-ridge distance when comparing the distance before treatment with the distance at six-month follow-up. No significant difference was found in the inter-incisal distance. The patients experienced improved mobility and better control of lip movements after the treatments. CONCLUSION: IPL can improve the inter-ridge distance between the lips in patients with microstomia due to systemic sclerosis but does not affect the inter-incisal distance, which is also dependent on the mobility of the mandibular joints. This treatment can be considered an adjunctive therapy in patients with microstomia due to systemic sclerosis.

Stiff hands and feet, facial deformities.

The appearance of the skin on this woman's face, hands, and feet helped us to recognize an advanced case of an autoimmune disease.

Increased inspiratory esophagogastric junction pressure in systemic sclerosis: an add-on to antireflux barrier.

AIM: To investigate crural diaphragm (CD) function in systemic sclerosis (SSc) using high-resolution manometry and standardized inspiratory maneuvers. METHODS: Eight SSc volunteers (average age, 40.1 years; one male) and 13 controls (average age, 32.2 years; six males) participated in the study. A high-resolution manometry/impedance system measured the esophagus and esophagogastric junction (EGJ) pressure profile during swallows and two respiratory maneuvers: sinus arrhythmia maneuver (SAM; the average of six EGJ peak pressures during 5-s deep inhalations) and threshold maneuver (TM; the EGJ peak pressures during forced inhalation under 12 and 24 cmH2O loads). Inspiratory diaphragm lowering (IDL) was taken as the displacement of the EGJ high-pressure zone during the SAM. RESULTS: SSc patients had lower mean lower esophageal sphincter pressure than controls during normal breathing (19.7±2.8 mmHg vs 32.2±2.7 mmHg, P=0.007). Sinus arrhythmia maneuver pressure was higher in SSc patients than in controls (142.6±9.4 mmHg vs 104.6±13.8 mmHg, P=0.019). Sinus arrhythmia maneuver pressure normalized to IDL was also higher in SSc patients than in controls (83.8±13.4 mmHg vs 37.5±6.9 mmHg, P=0.005). Threshold maneuver pressures normalized to IDL were also greater in SSc patients than in controls (TM 12 cmH2O: 85.1±16.4 mmHg vs 43.9±6.3 mmHg, P=0.039; TM 24 cmH2O: 85.2±16.4 mmHg vs 46.2±6.6 mmHg, P=0.065). Inspiratory diaphragm lowering in SSc patients was less than in controls (2.1±0.3 cm vs 3±0.2 cm, P=0.011). CONCLUSION: SSc patients had increased inspiratory EGJ pressure. This is an add-on to EGJ pressure and indicates that the antireflux barrier can be trained.

Acupuncture-based modalities: novel alternative approaches in the treatment of gastrointestinal dysmotility in patients with systemic sclerosis.

BACKGROUND: The gastrointestinal (GI) dysmotility of systemic sclerosis (SSc, scleroderma) patients requires careful evaluation and intervention. The lack of effective prokinetic drugs motivate researchers to search for alternative treatments. OBJECTIVES: We present an overview of the pathophysiology of SSc GI dysmotility and the advances in its management, with particular focus on acupuncture-related modalities and innovative therapies. DATA SOURCES: Original research articles were identified based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guideline methodology. We have searched the MEDLINE database using Medical Subject Heading (MeSH) for all English and non-English articles with an English abstract from 2005 to October 2012. RESULTS: Only four original articles of various study designs were found studying Complementary and Alternative Medicine (CAM) therapies for SSc patients. Despite the small patient study numbers, CAM treatments, acupressure, and transcutaneous electroacupuncture, showed self-reported and physiologic evidence of improvement of GI functioning and/or symptoms in SSc patients. CONCLUSIONS: CAM therapies include experimental modalities with the potential to offer relief of symptoms from GI dysmotility. Larger studies are needed to investigate their optimal use in patient subsets to tailor therapies to patient needs.

Prolonged treatment with transcutaneous electrical nerve stimulation (TENS) modulates neuro-gastric motility and plasma levels of vasoactive intestinal peptide (VIP), motilin and interleukin-6 (IL-6) in systemic sclerosis.

PURPOSE: We assessed the effects of transcutaneous electrical nerve stimulation (TENS) on neurogastric functioning in scleroderma patients. METHODS: Seventeen SSc patients underwent 30 min TENS treatment >10Hz at GI acupuncture points PC6 and ST36, once (acute TENS) and then after two weeks of TENS sessions for 30 min twice daily (prolonged TENS). Data collected at Visits 1 and 2 included gastric myoelectrical activity (GMA) by surface electrogastrography (EGG), heart rate variability (HRV) by surface electrocardiography (EKG), GI specific symptoms and health related SF-36 questionnaires. Plasma VIP, motilin and IL-6 levels were determined. Statistical analyses were performed by Student's t-test, Spearman Rank and p-values <0.05 were considered significant. RESULTS: 1. Only after prolonged TENS, the percentages of normal slow waves and average slow wave coupling (especially channels 1, 2 reflecting gastric pacemaker and corpus regions) were significantly increased; 2. the percentage of normal slow waves was significantly correlated to sympathovagal balance; 3. Mean plasma VIP and motilin levels were significantly decreased after acute TENS, (vs. baseline), generally maintained in the prolonged TENS intervals. Compared to baseline, mean plasma IL-6 levels were significantly increased after acute TENS, but significantly decreased after prolonged TENS. 4. After prolonged TENS, the frequency of awakening due to abdominal pain and abdominal bloating were significantly and modestly decreased, respectively. CONCLUSIONS: In SSc patients, two weeks of daily TENS improved patient GMA scores, lowered plasma VIP, motilin and IL-6 levels and improved association between GMA and sympathovagal balance. This supports the therapeutic potential of prolonged TENS to enhance gastric myoelectrical functioning in SSc.

Transient repetitive exposure to low level light therapy enhances collateral blood vessel growth in the ischemic hindlimb of the tight skin mouse.

The tight skin mouse (Tsk(-/+)) is a model of scleroderma characterized by impaired vasoreactivity, increased oxidative stress, attenuated angiogenic response to VEGF and production of the angiogenesis inhibitor angiostatin. Low-level light therapy (LLLT) stimulates angiogenesis in myocardial infarction and chemotherapy-induced mucositis. We hypothesize that repetitive LLLT restores vessel growth in the ischemic hindlimb of Tsk(-/+) mice by attenuating angiostatin and enhancing angiomotin effects in vivo. C57Bl/6J and Tsk(-/+) mice underwent ligation of the femoral artery. Relative blood flow to the foot was measured using a laser Doppler imager. Tsk(-/+) mice received LLLT (670 nm, 50 mW cm(-2), 30 J cm(-2)) for 10 min per day for 14 days. Vascular density was determined using lycopersicom lectin staining. Immunofluorescent labeling, Western blot analysis and immunoprecipitation were used to determine angiostatin and angiomotin expression. Recovery of blood flow to the ischemic limb was reduced in Tsk(-/+) compared with C57Bl/6 mice 2 weeks after surgery. LLLT treatment of Tsk(-/+) mice restored blood flow to levels observed in C57Bl/6 mice. Vascular density was decreased, angiostatin expression was enhanced and angiomotin depressed in the ischemic hindlimb of Tsk(-/+) mice. LLLT treatment reversed these abnormalities. LLLT stimulates angiogenesis by increasing angiomotin and decreasing angiostatin expression in the ischemic hindlimb of Tsk(-/+) mice.

Long-term N-acetylcysteine therapy in systemic sclerosis interstitial lung disease: a retrospective study.

Systemic sclerosis (SSc) is associated with interstitial lung diseases. The primary endpoints of this study were changes between baseline and month 24 in single-breath carbon monoxide diffusing capacity (DLco). The secondary endpoints were: vital capacity (VC), forced expired volume in 1 sec (FEV1), total lung capacity (TLC), scores of high resolution computed tomography (HRCT) of the chest, number of adverse effects. In this study, we retrospectively investigated data from SSc patients who had undergone therapy with high-dose intravenous N-acetylcysteine (NAC) at a dosage of 15 mg/Kg/h for 5 consecutive hours every 14 days. After NAC therapy median values of DLco (69.5 vs 77.7%), VC (99 vs 101.3%) and TLC (93 vs 98.3%) significantly increased. We did not observe any significant changes from baseline in FEV1 value and HRTC score. The improvement in lung function was more evident in SSc patients without radiological signs of pulmonary fibrosis than in patients with pulmonary fibrosis. In SSc patients with mild-moderate pulmonary fibrosis intravenous NAC administration slows the rate of deterioration of DLco, VC and TLC. In conclusion, this retrospective study demonstrates that long-term therapy with intravenous NAC ameliorates pulmonary function tests in SSc patients.

Spinal cord stimulation utilization to treat the microcirculatory vascular insufficiency and ulcers associated with scleroderma: a case report and review of the literature.

OBJECTIVE: To report a case of scleroderma with associated Raynaud's phenomenon and its successful treatment with spinal cord stimulation. To demonstrate the use of transcutaneous oxygen pressure monitoring to guide the progression from trial to implantation and to assess post-implantation microcirculatory recovery. DESIGN: Case report and literature review. PATIENT: A 51-year-old female with scleroderma, associated Raynaud's phenomenon, and a non-healing 3.7-cm lower extremity ischemic ulcer. Ankle-brachial indexes demonstrated normal macrocirculation, but transcutaneous oxygen pressures demonstrated significant microcirculatory insufficiency. INTERVENTION: Treatment was a spinal cord stimulator implantation after a successful trial. Transcutaneous oxygen pressures were interpreted during the trial and post-implantation stages. Results. Based on a 5-day trial that documented improvements in transcutaneous oxygen pressures and pain relief, the patient underwent implantation. At 4 months, the ischemic ulcer had healed. The patient had significant improvement in pain control and reduced Raynaud's phenomenon signs and symptoms. At 18 months, the patient continued to have improvement with no associated complications. A literature review demonstrated only four published reports, including a total of 18 patients, on spinal cord stimulator treatment for scleroderma and associated Raynaud's phenomenon. CONCLUSIONS: We report the healing of a greater than 3-cm ischemic ulcer in an individual with normal macrocirculation but severe microcirculatory insufficiency from scleroderma. Improvements in microcirculation correlated with wound healing. Spinal cord stimulation may be considered for select individuals with microcirculatory reserves that can be modulated with treatment.

Fibroblast abnormalities in the pathogenesis of systemic sclerosis.

Systemic sclerosis (SSc) is a chronic systemic disease characterized by autoimmunity, vascular lesions and progressive fibrosis. The fibrotic component is dominant in SSc compared with other vascular or autoimmune diseases and determines its prognosis and therapeutic refractoriness. Fibroblasts are responsible for abnormal extracellular matrix accumulation. Studies in cultured SSc skin fibroblasts have facilitated the identification of potential pathways involved in their profibrotic phenotype. Profibrotic fibroblasts characterized by abnormal growth and extracellular matrix synthesis may differentiate or expand from normal resident fibroblasts. Recruitment of bone marrow-derived progenitors and transdifferentiation of different cell lineages might also be involved. Multiple factors and signaling pathways appear to be involved in the development or persistence of the SSc fibroblast phenotype. Although their relative relevance and interplay are unclear, aberrant TGF-ß signaling seems pivotal and constitutes the best characterized therapeutic target.

Effect of a multi-faceted intervention on gingival health among adults with systemic sclerosis.

OBJECTIVES: To evaluate the effect of adaptive oral hygiene devices and orofacial exercise to improve gingival health among adults with systemic sclerosis (SSc). METHODS: Forty-eight patients with SSc were assigned randomly to the multifaceted oral health intervention or usual dental care control group. Participants in the intervention group received a rechargeable, powered Oral-B® oscillating-rotating-pulsating toothbrush and a Reach® Access™ Flosser that has a toothbrush-like handle. For those with an oral aperture of less than 40 mm, orofacial exercises were taught. Participants in the control group were each given a manual toothbrush and dental floss. Participants in both groups received instructions and demonstration on the use of the devices, and were requested to perform the respective intervention twice a day for 6 months. Evaluations were at baseline, 3-, and 6-months. The main outcome was gingival index (GI), an indicator of gingival inflammation. RESULTS: Both groups showed significant reduction in GI scores at 6 months (ps<0.005). Reduction in GI scores of the intervention group at 6 months was 20.8% which is considered to be clinically significant. Compared to the control group, the intervention group showed a significant and larger reduction in GI score by 8% at 6 months (p=0.0007). CONCLUSIONS: Results support the use of adaptive devices and orofacial exercise to improve gingival health in adults with SSc when compared to use of manual toothbrushing and finger-held flossing. Recommending and educating patients with SSc to use adaptive devices to clean the tooth surfaces looks promising for long-term oral health improvement.