UK Scleroderma Study Group (UKSSG) guidelines on the diagnosis and management of scleroderma renal crisis.

The UK Scleroderma Study Group developed guidelines on the diagnosis and management of scleroderma renal crisis (SRC) based on best available evidence and clinical experience. SRC is characterised by the acute onset of severe hypertension and acute kidney injury. Current strategies to reduce the associated morbidity and mortality include identifying at risk patients to aid early diagnosis. ACE inhibitor therapy should be lifelong in all patients, regardless of whether they require renal replacement therapy. Patients with SRC may recover renal function up to 3 years after the crisis, most often within 12 to 18 months.

Characteristics and Survival of Anti-U1 RNP Antibody-Positive Patients With Connective Tissue Disease-Associated Pulmonary Arterial Hypertension.

OBJECTIVE: Pulmonary arterial hypertension (PAH) is a severe complication of connective tissue diseases (CTDs). This study aimed to investigate the clinical and hemodynamic characteristics and survival of anti-U1 RNP-positive patients with CTD-associated PAH, with a focus on systemic sclerosis (SSc)-associated PAH. METHODS: We implemented a prospective database that included patients with CTD-associated PAH for whom there were clinical, autoantibody, and mortality data. We compared clinical and hemodynamic characteristics to anti-U1 RNP antibody status. We then assessed whether anti-U1 RNP antibodies could be a prognostic factor in CTD-associated PAH with a focus on SSc-associated PAH. RESULTS: We studied a total of 342 patients with CTD-associated PAH, of whom 36 (11%) were anti-U1 RNP antibody positive. Anti-U1 RNP-positive patients were younger and less functionally impaired than were anti-U1 RNP-negative patients in CTD- and SSc-associated PAH. Hemodynamic parameters were similar in anti-U1 RNP-positive and anti-U1 RNP-negative patients. In CTD-associated PAH, anti-U1 RNP positivity was associated with decreased mortality in univariable analysis (hazard ratio 0.34 [95% confidence interval 0.18-0.65], P < 0.001). In multivariable analysis, anti-U1 RNP positivity was also associated with decreased mortality (hazard ratio 0.44 [95% confidence interval 0.20-0.97], P = 0.043) independently of age, sex, functional parameters, lung involvement, and hemodynamic parameters. Results were similar in SSc-associated PAH, although the association between anti-U1 RNP positivity and survival did not reach significance in univariable (hazard ratio 0.47 [95% confidence interval 0.22-1.02], P = 0.055) and multivariable (hazard ratio 0.47 [95% confidence interval 0.20-1.11], P = 0.085) analyses. CONCLUSION: Anti-U1 RNP positivity was associated with distinct clinical characteristics and survival in CTD- and SSc-associated PAH. While hemodynamic parameters were similar in anti-U1 RNP-positive and anti-U1 RNP-negative patients, our results suggest that anti-U1 RNP positivity could be a factor protecting against mortality in CTD- and SSc-associated PAH.

Scleroderma renal crisis.

Systemic sclerosis is an autoimmune connective tissue disorder characterized by endothelial cell injury, vascular hyper-reactivity, obliterative microvasculopathy and excess collagen deposition, particularly within the dermis of the skin. Table 1 outlines the various terms that are used to describe the extent and location of sclerosis. Limited cutaneous and diffuse cutaneous are the two main forms that exist.

Fixation of ankle syndesmotic injuries: comparison of tightrope fixation and syndesmotic screw fixation for accuracy of syndesmotic reduction.

BACKGROUND: Ankle syndesmotic injuries are complex and require anatomic reduction and fixation to restore the normal biomechanics of the ankle joint and prevent long-term complications. PURPOSE: The aim of this study is to compare the accuracy and maintenance of syndesmotic reduction using TightRope versus syndesmotic screw fixation. STUDY DESIGN: Cohort study; Level of evidence, 2. METHODS: This cohort study included consecutive patients treated for ankle syndesmotic diastases between July 2007 and June 2009. Single slice axial computed tomography (CT) scans of both the ankles together were performed at the level of syndesmosis, 1 cm above the tibial plafond. A greater than 2-mm widening of syndesmosis compared with the untreated contralateral ankle was considered significant malreduction. Clinical outcomes were measured using the American Orthopaedics Foot and Ankle Society (AOFAS) and Foot and Ankle Disability Index (FADI) scores. RESULTS: Forty-six of 55 eligible patients participated in the study; 23 patients were in the TightRope group and 23 in the syndesmotic screw group. The average age was 42 years in the TightRope and 40 years in the syndesmotic screw group, and the mean follow-up time was 2.5 years (range, 1.5-3.5 years). The average width of normal syndesmosis was 4.03 ± 0.89 mm. In the TightRope group, the mean width of syndesmosis was 4.37 mm (SD, ±1.12 mm) (P = .30, t test) compared with 5.16 mm (SD, ±1.92 mm) in the syndesmotic screw group (P = .01, t test). Five of 23 ankles (21.7%) in the syndesmotic screw group had syndesmotic malreduction, whereas none of the TightRope group showed malreduction on CT scans (P = .04, Fisher exact test). Average time to full weightbearing was 8 weeks in the TightRope group and 9.1 weeks in the syndesmotic screw group. There was no significant difference between the TightRope and syndesmotic screw groups in mean postoperative AOFAS score (89.56 and 86.52, respectively) or FADI score (82.42 and 81.22, respectively). Regression analysis confirmed malreduction of syndesmosis as the only independent variable that affected the clinical outcome (regression coefficient, -12.39; t = -2.43; P = .02). CONCLUSION: The results of this study indicate that fixation with TightRope provides a more accurate method of syndesmotic stabilization compared with screw fixation. Syndesmotic malreduction is the most important independent predictor of clinical outcomes; therefore, care should be taken to reduce the syndesmosis accurately.

Remission in psoriatic arthritis: is it possible and how can it be predicted?

INTRODUCTION: Since remission is now possible in psoriatic arthritis (PsA) we wished to examine remission rates in PsA patients following anti tumour necrosis factor alpha (TNFalpha) therapy and to examine possible predictors of response. METHODS: Analysis of a prospective patient cohort attending a biologic clinic, between November 2004 and March 2008, was performed prior to commencing therapy and at regular intervals. Baseline clinical characteristics including demographics, previous disease-modifying antirheumatic drug (DMARD) response, tender and swollen joint counts, early morning stiffness, pain visual analogue score, patient global assessment, C reactive protein (CRP) and health assessment questionnaire (HAQ) were collected. RESULTS: A total of 473 patients (152 PsA; 321 rheumatoid arthritis (RA)) were analyzed. At 12 months remission, defined according to the disease activity score using 28 joint count and CRP (DAS28-CRP), was achieved in 58% of PsA patients compared to 44% of RA patients, significant improvement in outcome measures were noted in both groups (P<0.05). Analysis of a subgroup of PsA and RA patients matched for DAS28-CRP at baseline also showed higher numbers of PsA patients achieving remission. Linear regression analysis identified the HAQ at baseline as the best predictor of remission in PsA patients (P<0.001). CONCLUSIONS: DAS28 remission is possible in PsA patients at one year following anti-TNF therapy, at higher rates than in RA patients and is predicted by baseline HAQ.