Myeloablative autologous haematopoietic stem cell transplantation resets the B cell repertoire to a more naïve state in patients with systemic sclerosis.

OBJECTIVES: Myeloablative autologous haematopoietic stem cell transplant (HSCT) was recently demonstrated to provide significant benefit over cyclophosphamide (CYC) in the treatment of diffuse cutaneous systemic sclerosis (dcSSc) in the Scleroderma: Cyclophosphamide or Transplantation (SCOT) trial. As dysregulation of the B cell compartment has previously been described in dcSSc, we sought to gain insight into the effects of myeloablative autologous HSCT as compared with CYC. METHODS: We sequenced the peripheral blood immunoglobulin heavy chain (IGH) repertoires in patients with dcSSc enrolled in the SCOT trial. RESULTS: Myeloablative autologous HSCT was associated with a sustained increase in IgM isotype antibodies bearing a low mutation rate. Clonal expression was reduced in IGH repertoires following myeloablative autologous HSCT. Additionally, we identified a underusage of immunoglobulin heavy chain V gene 5-51 in patients with dcSSc, and usage normalised following myeloablative autologous HSCT but not CYC treatment. CONCLUSIONS: Together, these findings suggest that myeloablative autologous HSCT resets the IGH repertoire to a more naïve state characterised by IgM-expressing B cells, providing a possible mechanism for the elimination of pathogenic B cells that may contribute to the benefit of HSCT over CYC in the treatment of dcSSc.

Incisional Release and Fat Grafting for the Surgical Management of Perioral Fibrosis in Scleroderma: A Technical Report and Institutional Review.

INTRODUCTION: Scleroderma (systemic sclerosis [SSc]) is a rare autoimmune, connective tissue disorder. Perioral fibrosis is a local cutaneous complication, negatively impacting functional capabilities and aesthetic satisfaction. Fat grafting has been postulated to aid in the management of SSc fibrosis thanks to stem cell enrichment. This technique's success has been demonstrated using different graft origin sites and different injection targets. We aim to demonstrate our SSc patients' success using abdominal fat and perioral target. METHODS: We queried our records for patients with preexisting SSc who underwent incisional release and fat grafting for perioral fibrosis from 2018 to 2021. For perioral release, a semisharp cannula was tunneled under the vermilion border into the vermilion and along the skin. For grafting, cannulas were used to infiltrate the fat with a retrograde filling technique in a radial-fanning manner. Their autoimmune diagnosis, anesthetic risk assessment, systemic disease complications, and degree of presenting symptoms were reviewed along with their postoperative outcomes. RESULTS: From 2018 to 2021, 16 patients diagnosed with SSc were treated with incisional release and fat grafting for the management of perioral fibrosis. Of the SSc patients, 8 presented with limited SSc, and 8 presented with diffuse SSc. The mean patient age was 54.31 years. All SSc patients presented with functional symptoms with the most common concern (n = 9) being "decreased mouth opening." Other common complaints were "difficulty eating" (n = 3) or "difficulty drinking" (n = 2). Some patients (n = 11) also presented with cosmetic concerns with "perioral rhytids" being the most common (n = 6). The mean number of systemic complications, at the time of presentation, was 3.06. The mean anesthetic risk assessment was 2.44. The average amount of fat grafted intraoperatively was 14.89 mL. Two patients with SSc required regrafting. For one patient, this was part of the original treatment plan and for the other due to fat resorption. Patients who followed up reported improved functionality and were pleased aesthetically. CONCLUSIONS: Patients with perioral fibrosis due to SSc can benefit from autologous fat grafting. Incisional release in combination with fat grafting can enhance procedure outcomes. This technique provides beneficial functional and aesthetic outcomes. Patients with both diffuse and limited disease are appropriate candidates for this procedure.

Benefits and risks of haematopoietic stem cell transplantation for systemic sclerosis: A systematic review and meta-analysis.

OBJECTIVES: We aimed to evaluate the efficacy and safety of haematopoietic stem cell transplantation (HSCT) in patients with systemic sclerosis. METHODS: A systematic literature review and meta-analysis were carried out. We compared survival outcomes using the Kaplan-Meier method with patient-level data between HSCT and intravenous pulse cyclophosphamide. Additionally, the incidence rate of treatment-related deaths with HSCT was pooled using a random-effect model. RESULTS: Of the 2091 articles screened, 22 were included: 3 randomized controlled trials and 19 observational studies. HSCT studies showed significant improvement in the skin thickness score and lung function. Despite treatment-related deaths being higher in HSCT than in intravenous pulse cyclophosphamide, the Kaplan-Meier analysis showed a high survival rate of 2 years post-transplant (log-rank, P = 0.004). The pooled frequency of transplant-related death from 700 systemic sclerosis patients was 6.30% (95% confidence interval 4.21-8.38). However, the estimated frequency of treatment-related deaths has been reducing over the last decade. CONCLUSIONS: HSCT is an effective treatment for systemic sclerosis, but the optimal indications must be carefully determined by balancing the risks.

Assessing the effect of PRP addition to facial micro-lipofilling for patients suffering from Scleroderma: A prospective routine care analysis.

BACKGROUND: Combining fat graft with platelet derived products is now common practice in regenerative surgery. We proposed to assess the safety and efficacy of Platelet-Rich Plasma (PRP) addition to a micro-lipofilling protocol for facial treatment of patients suffering from Systemic Sclerosis (SSc). OBJECTIVE: Main objective was to evaluate the improvement of the Mouth Handicap In Systemic Sclerosis (MHISS) scale score at 6 months post-therapy. METHOD: Included SSc patients had a MHISS score equal or up to 20. Surgery was performed under general anesthesia. Micro-fat and PRP (CCA-NA from DEPA Classification) were mixed in a 70/30 ratio, before injection in peri-oral sites according to a specific protocol. Efficacy criteria were recorded at baseline, 3 and 6 months. Moreover, we compared this cohort (current study) to a former (2015) non-enriched micro-lipofilling cohort in the same indication, using the same protocol. RESULTS: Thirteen women patients with mean age of 53.2 years (±14.3) have been included. At baseline, mean MHISS score was 29.5 (±8.7) and significantly decreased to 22.5 (±7.8) at 6 months (P=0.016), corresponding to a 22.0% of improvement from baseline, with a mean decrease of 6.5 points (±7.5) at 6 months. Patients received a mean volume of 30.8ml PRP-micro-fat (±8.1ml). CONCLUSION: PRP addition appeared beneficial, however, controlled studies are required to determine its superiority to facial micro-lipofilling.

Mixed connective tissue disease after thymectomy in refractory myasthenia gravis.

Connective tissue diseases, including systemic lupus erythematosus (SLE) and systemic sclerosis are classic models of autoimmunity; diseases with large-scale loss of tolerance and subsequent development of pathogenic autoreactive lymphocytes and tissue targeting autoantibodies. Here we report a case of mixed connective tissue disease, with features of systemic lupus erythematosus and systemic sclerosis developing in a patient 10 years post thymectomy for myasthenia gravis. The patient developed acute cutaneous lupus, Raynaud's with digital ulcers, arthritis and lymphopenia. Her myasthenia continued to be resistant to treatment and her rheumatic disease progressed despite aggressive therapy. We performed a database search of MEDLINE, EMBASE, Scopus, and Web of Science for articles of similar cases post thymectomy from inception to August 2021, using the terms "systemic lupus erythematosus" (or systemic sclerosis, or connective tissue disease) and "myasthenia gravis" and "thymectomy". We identified 41 cases, 28 of SLE post thymectomy, 8 related to systemic sclerosis, 5 with mixed connective tissue disease and highlighted their different presentation and serology. We explore the role of the thymus, tolerance and myasthenia gravis in the development of connective tissue disease. This highlights the complexity of concurrent autoimmune diseases and their autoantibodies.

Conceptual Paper: Abdominoplasty and Liposuction in Systemic Sclerosis.

BACKGROUND: Surgical interventions in patients with systemic sclerosis (SSc), in particular plastic procedures, might cause undesired consequences. Notably, liposuction seems to possess greater risk as adipose tissue has been shown to play an important role in treating wounds and ulcers in patients with SSc. While anticentromere antibodies were found to be correlated with vasculopathy in SSc, patients with SSc and anticentromere antibodies might be more vulnerable to surgical wound complications following liposuction. A 46-year-old female patient, who had been diagnosed with SSc at the age of 31 years, had antinuclear as well as anticentromere antibodies. She underwent abdominoplasty with liposuction and developed severe skin necrosis of the abdomen following the procedure and at the site of liposuction. The correlation with anticentromere and the role of liposuction in skin necrosis in SSc are presented.

Autologous Fat Grafting for the Oral and Digital Complications of Systemic Sclerosis: Results of a Prospective Study.

BACKGROUND: Systemic sclerosis is a connective tissue disease. Skin involvement of the mouth and hand may compromise function and quality of life. Autologous fat grafting has been described as a specific treatment of these clinical features. We report the results of our prospective study designed to treat and prevent skin complications in systemic sclerosis. MATERIALS AND METHODS: We treated 25 patients with mouth and/or hand involvement (microstomia, xerostomia, skin sclerosis, Raynaud's phenomenon and long-lasting digital ulcers) with autologous fat grafting, according to the Coleman's technique, around the mouth and/or at the base of each finger. The surgical procedures were repeated in each patient every 6 months for a total of two or three times. Clinical data were collected before the first surgery and again 6 months after each surgical procedure. Pain, skin thickness, saliva production and disability were assessed with validated tests. RESULTS: Overall we performed 63 autologous fat grafting sessions (either on the mouth, on the hands or on both anatomical areas). Results at 6 moths after the last session included improvement of xerostomia evaluated with a sialogram, reduction of the skin tension around the mouth and, in the hands, reduction of the Raynaud phenomenon as well as skin thickness. Pain was reduced while the perception of disability improved. Digital ulcers healed completely in 8/9 patients. CONCLUSIONS: Our results confirm the efficacy and safety of autologous fat grafting for the treatment of skin complications and digital ulcers due to systemic sclerosis. In addition, the patients' subjective well-being improved. Level of evidence V This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Upper Extremity Angiographic Patterns in Systemic Sclerosis: Implications for Surgical Treatment.

PURPOSE: Conventional angiography is often used in the preoperative work-up of hand surgery patients with systemic sclerosis. The goal of this study was to propose a classification system based on the pattern of arterial involvement in a series of upper extremity angiograms. The authors hypothesized that this classification system would demonstrate high inter- and intrarater reliability. METHODS: A retrospective review of 110 upper extremity angiograms in patients with systemic sclerosis (obtained between 1996 and 2017) was performed. Images were classified into 4 types based on the patency of the radial and ulnar arteries at the wrist, and into 3 subtypes based on the patency of the superficial and deep palmar arches. Classification reliability was compared with Fleiss' Kappa (for inter-rater) and Cohen's (for intrarater) coefficient between 4 fellowship-trained hand surgeons and a hand fellow. RESULTS: The inter-rater reliability between all 5 observers using types alone was 0.83 (0.80-0.85), whereas the inter-rater reliability using subtypes was 0.64 (confidence interval [CI] 95%, 0.62-0.65). The intrarater reliability using types alone ranged from 0.80 to 0.95, whereas intrarater reliabilities using subtypes were 0.81 (CI 95%, 0.72-0.90), 0.78 (CI 95%, 0.69-0.87), 0.87 (CI 95%, 0.80-0.95), 0.64 (CI 95%, 0.53-0.75), and 0.92 (CI 95%, 0.86-0.98) for the 4 attendings and a hand fellow, respectively. Fifty-seven percent of angiograms were interpreted as having loss of ulnar artery patency at the wrist (type 2) with 77% having additional loss of superficial palmar arch patency (type 2A). CONCLUSIONS: This large series of angiograms in patients with systemic sclerosis demonstrates a classification system for conventional angiography that shows high inter-rater and intrarater reliability using type alone. When subtypes were used, the inter-rater and intrarater reliabilities decreased to moderate and moderate-to-high, respectively. CLINICAL RELEVANCE: This study represents the first step in establishing a classification system that, by grouping patients with similar angiogram findings, may allow for targeted research into risk stratification, monitoring, and treatment in systemic sclerosis.

Lung transplantation in scleroderma: recent advances and lessons.

PURPOSE OF REVIEW: The purpose of this review is to highlight recent data regarding feasibility and outcomes following lung transplantation for patients with systemic sclerosis related pulmonary disease as well as to emphasize areas of uncertainly in need of further study. We include a description of our centre's approach to lung transplant evaluation and posttransplant management in this complex patient population. RECENT FINDINGS: Historical data have demonstrated that patients with scleroderma have an increased risk of complications following lung transplantation owing to the multisystem nature of disease, particularly concurrent gastrointestinal, cardiac and renal involvement. Emerging data support the safety of lung transplant in appropriately selected patients with scleroderma-related interstitial lung disease and pulmonary arterial hypertension. SUMMARY: Accumulating evidence validates that a diagnosis of scleroderma is not a priori a contraindication to lung transplant. In the carefully selected patient, both short-term and long-term outcomes following lung transplantation are comparable to counterparts with fibrotic lung disease or pulmonary arterial hypertension. However, further prospective study to detail how these patients should be evaluated and managed posttransplant is definitely needed. Cardiac disease is an emerging cause of morbidity and mortality in the scleroderma population and deserves particular attention during the pre and posttransplant period.

Molecular recognition patterns of anti-topoisomerase I-antibodies in patients with systemic sclerosis before and after autologous stem cell transplantation.

OBJECTIVES: To evaluate the effect of autologous stem cell transplantation (aSCT) on antibody (ab) reactivity towards linear epitopes of topoisomerase-I (topo-I/Scl70) in patients with systemic sclerosis (SSc) and to correlate antibody reactivities with clinical outcome after aSCT. METHODS: Fourteen anti-topo-I/Scl70-positive SSc-patients were analysed before and after non-myeloablative aSCT. Five patients showed ongoing good response (group 1), 9 had primarily responded but later relapsed or did not respond (group 2). Patients' sera were tested by ELISA against full length (fl) topo-I and 45 overlapping 25-mer peptides. Furthermore, for comparison sera from patients with anti-topo-I-negative SSc (n=12), other collagen disorders (n=6), and from 21 healthy controls (HC) were analysed. RESULTS: Anti-topo-I-positive SSc-sera showed significantly higher IgG-reactivity as compared to HC towards 34 of the 45 peptides. Especially peptide 39 (aa647-671) emerged as a immunodominant epitope being recognised predominantly by anti-topo-I-positive SSc-sera. Reactivity towards 17 of the 45 peptides decreased after aSCT in group 1- and 2-patients. Before aSCT, group 1-patients had lower antibody reactivity towards peptide 39 than group 2-patients. There was no change in peptide-specificity after aSCT. CONCLUSIONS: Reactivity towards topo-I-epitopes is heterogeneous in SSc, but peptide 39 (aa647-671) may be another immunodominant epitope besides the published epitope aa489-573. Antibody reactivity to this peptide 39 was higher in group 2- than in group 1-patients. Peptide recognition pattern did not change after aSCT.

Surgical management of gastroesophageal reflux disease in patients with systemic sclerosis.

BACKGROUND: Systemic sclerosis (scleroderma) is frequently associated with both gastroesophageal reflux disease (GERD) and simultaneous esophageal dysmotility. Anti-reflux procedures in this patient population must account for the existing physiology of each patient and likely disease progression. We aim to compare perioperative and intermediate outcomes of fundoplication versus gastric bypass for the treatment of GERD. METHODS: After IRB approval, patients with systemic sclerosis undergoing fundoplication or gastric bypass for the treatment of GERD from 2004 to 2016 were identified. Demographics, perioperative data, immediate complications, and symptom improvement were retrieved and analyzed. RESULTS: Fourteen patients with systemic sclerosis underwent surgical treatment of GERD during the defined study period. Average body mass index was 26 kg/m2. Seven fundoplications (2 Nissens, 4 Toupets, and 1 Dor) and 7 Roux-en-Y gastric bypasses (RYGB) were performed. No 30-day mortality was observed in either group. Median follow-up was 97 months for the fundoplication group (range 28-204 months), and 19 months for the RYGB group (range 1-164 months). Preoperatively, dysphagia, heartburn, and regurgitation were present in 71% (n = 10), 86% (n = 12), and 64% (n = 9) of patients, respectively. Eleven patients had pH study prior to surgical intervention, and 91% of them had abnormal acid exposure. Esophagitis was evident in 85% (n = 11) of patients during preoperative upper endoscopy, and two patients had Barrett's esophagus. Impaired esophageal motility was present in all RYGB patients and 71% of fundoplication patients. Of the patients who had assessment of their GERD symptoms at follow-up, all five patients in the RYGB group and only 3 (50%) patients in the fundoplication group reported symptom improvement or resolution. CONCLUSIONS: Laparoscopic RYGB as an anti-reflux procedure is safe and may provide an alternative to fundoplication in the treatment of GERD for systemic sclerosis patients with esophageal dysmotility.

Lung Transplantation in Patients With Systemic Sclerosis.

PURPOSE OF REVIEW: The purpose of this study was to highlight recent findings on evaluation for lung transplantation and outcomes after transplantation in patients with systemic sclerosis (scleroderma). RECENT FINDINGS: The recognition that extra-pulmonary disease manifestations can seriously compromise post-transplant outcomes has changed the way patients are screened for lung transplant candidacy. Reluctance to transplant subjects affected by scleroderma has been driven by the complexity and multisystem nature of this disease. Multiple recent reports provide convincing findings that scleroderma patients undergoing lung transplantation have similar outcomes as those with other non-rheumatologic pulmonary conditions, even when significant esophageal dysmotility is present. New evidence supports the notion that scleroderma should not be a contraindication for referral to lung transplant. Future studies are needed to improve risk stratification, to define protocols for screening and management of extra-pulmonary complications, and to optimize immunosuppression before and after transplant.

Kidney transplantation in patients with systemic sclerosis: a nationwide multicentre study.

Kidney transplantation is one of the therapeutic options for end-stage renal disease (ESRD) in systemic sclerosis (SS). Current evidence demonstrates poorer patient and graft survival after transplantation in SS than in other primary kidney diseases. All the patients presenting ESRD associated with SS who had received a kidney allograft between 1987 and 2013 were systematically included from 20 French kidney transplantation centres. Thirty-four patients received 36 kidney transplants during the study period. Initial kidney disease was scleroderma renal crisis in 76.4%. Extrarenal involvement of SS was generally stable, except cardiac and gastrointestinal involvements, which worsened after kidney transplantation in 45% and 26% of cases, respectively. Patient survival was 100%, 90.3% and 82.5% at 1, 3 and 5 years post-transplant, respectively. Pulmonary involvement of SS was an independent risk factor of death after transplantation. Death-censored graft survival was 97.2% after 1 and 3 years, and 92.8% after 5 years. Recurrence of scleroderma renal crisis was diagnosed in three cases. In our study, patient and graft survivals after kidney transplantation can be considered as excellent. On this basis, we propose that in the absence of extrarenal contraindication, SS patients presenting with ESRD should be considered for kidney transplantation.

Determinants of unemployment amongst Australian systemic sclerosis patients: results from a multicentre cohort study.

OBJECTIVES: We sought to assess employment status, risk factors for unemployment and the associations of unemployment with patients' health related quality of life (HRQoL). METHODS: All patients enrolled in a systemic sclerosis (SSc) longitudinal cohort study, completed an employment questionnaire on enrolment. Clinical manifestations were defined based on presence at the time of enrolment. Summary statistics, chi-square tests, univariate and multivariable logistic regression were used to determine the associations of various risk factors with employment. RESULTS: Among 1587 SSc patients, 160 (20%) were unemployed at the time of cohort enrolment excluding retired patients. Of these, 63% had limited disease subtype. Mean (±SD) age at enrollment was 51.9 (±10.4) years; 13 years below the average retirement age in Australia. Mean (±SD) disease duration at recruitment was 11.1 (±10.9) years. Multivariable regression analysis revealed the presence of digital amputation (OR 3.9, 95%CI 1.7-9.1, p=0.002), diffuse disease subtype (OR 2.2, 95%CI 1.3-3.5, p-value=0.002), sicca symptoms (OR 2.7, 95%CI 1.6-4.4, p<0.001), a physical job (OR 1.8, 95%CI 1.1-3.1, p=0.03) and pulmonary arterial hypertension (OR 2.2, 95%CI 1.1-4.5, p=0.02) to be associated with unemployment. Unemployed patients had consistently poorer HRQoL scores in all domains (physical, emotional and mental health) of the SF-36 form than those who were employed. CONCLUSIONS: SSc is associated with substantial work disability and unemployment, which is in turn associated with poor quality of life. Raising awareness, identifying modifiable risk factors and implementing employment strategies and work place modifications are possible ways of reducing this burden.

Dental implants in patients with oral mucosal diseases - a systematic review.

To reveal dental implants survival rates in patients with oral mucosal diseases: oral lichen planus (OLP), Sjögren's syndrome (SjS), epidermolysis bullosa (EB) and systemic sclerosis (SSc). A systematic literature search using PubMed/Medline and Embase databases, utilising MeSH and search term combinations identified publications on clinical use implant-prosthetic rehabilitation in patients with OLP, SjS, EB, SSc reporting on study design, number, gender and age of patients, follow-up period exceeding 12 months, implant survival rate, published in English between 1980 and May 2015. After a mean observation period (mOP) of 53·9 months (standard deviation [SD] ±18·3), 191 implants in 57 patients with OLP showed a survival rate (SR) of 95·3% (SD ±21·2). For 17 patients with SjS (121 implants, mOP 48·6 ± 28·7 months), 28 patients with EB (165 implants, mOP 38·3 ± 16·9 months) and five patients with SSc (38 implants, mOP 38·3 ± 16·9 months), the respective SR was 91·7 ± 5·97% (SjS), 98·5 ± 2·7% (EB) and 97·4 ± 4·8% (SSc). Heterogeneity of data structure and quality of reporting outcomes did not allow for further comparative data analysis. For implant-prosthetic rehabilitation of patients suffering from OLP, SjS, EB and SSc, no evidence-based treatment guidelines are presently available. However, no strict contraindication for the placement of implants seems to be justified in patients with OLP, SjS, EB nor SSc. Implant survival rates are comparable to those of patients without oral mucosal diseases. Treatment guidelines as for dental implantation in patients with healthy oral mucosa should be followed.

[LEFT ATRIAL APPENDAGE CLOSURE IN A PATIENT WITH SYSTEMIC SCLEROSIS]. / LE CAS CLINIQUE DU MOIS. Fermeture percutanée de L'auricule gauche chez une patiente souffrant de sclérodermie.

We report the clinical history of a 69 year-old female who suffered from systemic sclerosis and in whom we performed a percutaneous left atrial appendage closure due to recurrent gastrointestinal bleedings under anticoagulant therapy for chronic atrial fibrillation. We review the impact of scleroderma on the cardiac and digestive systems and discuss the issue of anticoagulation and its alternatives in uncommon clinical situations. We also describe the indications, technical aspects and potential complications of percutaneous left atrial appendage closure.

[A case of combination therapy by vacuum assisted closure along with skin graft for scleroderma with common wart].

Scleroderma is a kind of connective tissue disease characterized by skin and other systems fibrosis. The underlying mechanisms for this disease are poorly understood due to its complexity. It is very difficult for scleroderma patient to heal the wound due to the special pathological characteristic of scleroderma.

Procedural pain management in the treatment of scleroderma digital ulcers.

OBJECTIVES: Digital ulcers (DU) may develop in half of systemic sclerosis (SSc) patients; they are often resistant to treatments. Deep wound debridement is crucial for DU healing, but very difficult to carry out without adequate procedural pain management. Here, we report the results of our experience on procedural pain management in scleroderma DU. METHODS: The study included 51 DU observed in 32 consecutive SSc patients; procedural pain was treated following a definite schedule: local lidocaine and prilocaine (25 mg of either agent per gram of cream, EMLA 5%) were initially used in all cases, followed by local and oral morphine, according to the severity of pain scored on a 10 cm visual analogue scale (VAS). RESULTS: At baseline, higher pain VAS was recorded in more severe (p=0.0001) and/or infected DU (p=0.0001). Good compliance to DU debridement was observed in patients with mild pain (VAS ≤4) treated with only EMLA, and in 5 cases with moderate-severe pain (VAS >4) at baseline. While, the majority of DU with moderate-severe pain (34/39) needed a combined therapy with EMLA and local morphine (8/34) or with EMLA, local and oral morphine (26/34). On the whole, pain management during DU debridement required only EMLA application in 33% of cases, EMLA plus local morphine in 16%, while combined EMLA, local and oral morphine were necessary in 51%, generally with more severe and/or infected lesions. CONCLUSIONS: The present study showed valuable control of procedural pain during DU debridement with sequential, combined analgesic treatment.

Scleroderma and the temporomandibular joint: reconstruction in 2 variants.

PURPOSE: This article reviews the pathophysiology of scleroderma (systemic sclerosis [SSc]) and its destructive effects on the mandible in general and the temporomandibular joint (TMJ) in particular. It discusses the considerations of operating on patients with devastating chronic disease and presents 2 cases of TMJ reconstruction in patients with the diagnosis. PATIENTS AND METHODS: Two patients with different degrees of SSc involvement underwent TMJ reconstruction with costochondral grafts. The patients represent the surgical considerations pertinent to this disease and different outcomes as determined by the variance in severity of their afflictions. RESULTS: The 2 patients tolerated the surgeries well and exhibited improvement in function in the long-term. One patient thrives and continues to do well despite her SSc approximately 10 years postoperatively; the second patient died of her disease approximately 9 years after her initial surgical care. CONCLUSIONS: The experience with these 2 cases showed that patients with SSc can safely undergo TMJ reconstruction with anticipated good results, but that the overall severity of the disease remains paramount in determining the feasibility of corrective surgery under this diagnosis.

Digital Sympathectomy in Patients With Scleroderma: An Overview of the Practice and Referral Patterns and Perceptions of Rheumatologists.

PURPOSE: Periarterial sympathectomy is a treatment option for patients with systemic sclerosis (SSc) suffering from digital vasculopathy. Despite potential benefits of ulcer healing, pain improvement, and amputation prevention, this operation appears to be infrequently performed. The aims of our study are as follows: (1) to assess national digital sympathectomy rates in patients with SSc and (2) to improve our understanding of referring physicians' perceptions of operative management and access to hand surgeons. Our hypothesis is that rheumatologists' practices largely influence their referral patterns for digital sympathectomy. METHODS: To determine the rates and demographics of hospitalized patients with SSc who had undergone digital sympathectomy, we queried the Nationwide Inpatient Sample from 2006 to 2010. Additionally, we mailed a self-administered survey to a national sample of 500 board-certified rheumatologists to elicit their practice patterns and perceptions of digital sympathectomy. Using logistic regression, we analyzed potential predictor variables associated with rheumatologists performing the following: (1) routinely counseling patients about digital sympathectomy and (2) expressing the desire to refer these patients for operative evaluation. RESULTS: Of 348,539 hospitalizations associated with a diagnosis of SSc, only 0.2% were for digital sympathectomy. Our questionnaire revealed that only 50% of rheumatologists routinely counseled, whereas 67% expressed the desire to refer. Factors associated with increased rheumatologists' interest in surgical management for patients with SSc included positive perception of the operation's efficacy, comfort with postoperative management, and interdisciplinary relationship with a hand surgeon. DISCUSSION: Critical components to increasing appropriate utilization of digital sympathectomy include enhancing rheumatologists' understanding of the operation, comfort with postoperative management, and promoting strong, interdisciplinary relationships with hand surgeons. Increasing education and awareness, as well as establishing a solid referral network of hand surgeons may thereby improve patient access to digital sympathectomy.