Resolution of skin fibrosis and joint contractures in aggressive diffuse systemic sclerosis using autologous stem cell transplantation

The use of haematopoietic stem cell transplantation (HSCT) has now expanded beyond the domain of haematological diseases. Increasingly, the benefits of intense immunosuppression in the management of severe autoimmune diseases are being recognized. In diffuse systemic sclerosis (SSc), there has been increasing evidence of the efficacy of HSCT in improving morbidity and mortality. We present the first Australian patient to undergo autologous HSCT for SSc and review the current literature in the use of HSCT in SSc. Remarkably, the patient had complete resolution of skin disease (modified Rodnan skin score 27/51–0/51), tenosynovitis, synovitis and myositis.     

Magnetic resonance imaging in pachydermodactyly

The magnetic resonance imaging (MRI) findings of a teenage boy with pachydermodactyly are described. The findings include fusiform soft tissue swelling around the proximal interphalangeal joints of the hands, with sparing of the tendons and ligaments. There was no capsular involvement and no evidence of periosteal reaction.     

Predictors of clinical response to intraarticular Hylan injections -- a prospective study using synovial fluid measures, clinical outcomes, and magnetic resonance imaging

OBJECTIVE: To evaluate synovial fluid (SF) and clinical and imaging predictors of clinical response in patients receiving intraarticular Hylan GF-20 injections. METHODS: Thirty-two patients with mild to moderate osteoarthritis (OA) of the knee [OsteoArthritis Research Society International (OARSI) grades I-II] were followed over 6 months. SF and clinical and radiographic measures were assessed. Patella and tibial cartilage volume and cartilage defect scores were measured at baseline and 6 months using magnetic resonance imaging (MRI). The primary outcome measure was the relationship between SF measures and clinical response as defined by the OARSI-Outcome Measures in Rheumatology Clinical Trials responder criteria for OA ("High improvement" >or= 50% improvement in pain or function; absolute change >or= 20 NU on Western Ontario and McMaster University Osteoarthritis Index questionnaire). Secondary outcomes included MRI outcomes (change in cartilage volume and cartilage defect scores). RESULTS: Fifteen patients achieved "High improvement." High baseline SF hyaluronic acid (HA) concentration was a statistically significant predictor of clinical response with odds ratio (OR) 6.04 (p < 0.02). HA concentration was divided into tertiles and fitted to a univariate regression model against clinical response. A baseline HA concentration value of > 2 mg/ml provided the greatest tradeoff between sensitivity and specificity with values of 60% and 77%, respectively, a likelihood ratio of 2.55, and OR of 4.88. Baseline clinical and radiological measures did not predict clinical response in this cohort with mild to moderate OA. Nineteen subjects had MRI at both timepoints. No change was noted in cartilage volumes or cartilage defect scores over 6 months. There was no association between baseline HA concentration and baseline cartilage volume. CONCLUSION: Baseline SF HA concentration predicts clinical response in patients receiving intraarticular Hylan. This has implications for the selection of patients who are likely to respond to this therapy.     

Reconstructive hand surgery for scleroderma joint contractures

Systemic scleroderma can cause significant hand deformity and functional impairment. Surgery is often avoided due to the perceived risks of wound healing. The most common surgical procedures have been digital sympathectomy, arthrodesis or arthroplasty of the proximal interphalangeal (PIP) or both, and metacarpophalangeal (MCP) joints. We describe herein successful soft tissue hand surgery in 2 patients for treatment of scleroderma claw deformities without the use of arthrodesis or arthroplasty. At the MCP joint, the tight capsules were excised, and the collateral ligaments and volar plates were released. At the PIP joints, the volar plates were released and the tight palmar skin was released, resulting in marked improvement of joint position. Intensive hand therapy was used to maximize function. In these 2 patients with claw deformity, we found that tight volar skin was the main contributor to flexion contracture at the PIP level. In contrast, joint capsule contracture was the main contributor to hyperextension deformity at the MCP level.