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A 60-year-old female patient was admitted to the emergency room for a 7-day history of abdominal bloating, nausea, vomiting, constipation, and lack of flatus. She had been diagnosed with systemic sclerosis (SSc) 10 years ago and had been using methotrexate, sildenafil, and prednisone. She did not present any signs of instability, but physical examination showed malnourishment status and abdominal tenderness and distention. Plain abdominal radiography was suggestive of sigmoid volvulus, confirmed and successfully resolved after endoscopic decompression therapy. Eight months later, the patient developed a new episode of abdominal obstruction. Computed Tomography (CT) scan identified a distended sigmoid colon due to its torsion with gas areas within the bowel wall. This time, endoscopic decompression had failed to treat, so exploratory laparotomy was performed. Colonic distention and sigmoid volvulus were identified during the procedure, after which sigmoidectomy followed by primary anastomosis was performed. Neither perforation nor masses were found. Furthermore, the anatomopathological study was inconsistent with vascular, inflammatory, or neoplastic diseases.

Objectives: In a longitudinal case-control observational study, we evaluated the benefits of a self-management programme for hands developed for patients with SSc., Methods: Patients with SSc included in the intervention group (IG) received a concise self-management programme with emphasis on hand exercises and were evaluated during 24 weeks regarding hand pain, hand function, range of motion, grip and tip and key pinch strength. Results were compared with a control group (CG) with no intervention using an analysis of variance for repeated measures with variables transformed into ranks (P ≤ 0.05). Effect sizes were calculated using Cohen's test., Results: Of 90 patients who were evaluated, seven were excluded at enrolment and 26 were excluded during the follow-up. Data from 57 subjects (IG 40, CG 17) were used for analysis. Groups were similar at baseline, except for the Scleroderma HAQ and tip and key pinch strength. Outcome improvements were noted only in the IG (P ≤ 0.05, large effect size). In the CG, variables did not change or had even worsened (hand grip strength and finger motion)., Conclusions: This self-management programme based on hand exercises for SSc resulted in pain reduction and hand function, strength and range of motion improvement. It can be a simple and useful intervention, especially when a regular rehabilitation programme is not available., (© The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Study Design: This study used a quasi-experimental design where patients were evaluated before and after participation in the self-management program., Introduction: Hands are commonly affected in systemic sclerosis (SSc). Strategies to maintain or improve hand function are indicated upon diagnosis and throughout the course of the disease., Purpose of the Study: The purpose of this study was to develop and evaluate a home-based program for hands in patients with SSc., Methods: A home-based self-management program that consisted of concise instructions about SSc and hand exercises was developed and evaluated in a group of patients with SSc during 8 weeks. Primary outcome measures were hand pain (Visual Analogue Scale) and hand function (Cochin Hand Function Scale). Secondary outcome measures were disability (Scleroderma Health Assessment Questionnaire), finger motion (delta finger-to-palm), grip strength, tip and key pinch strength, Raynaud phenomenon and digital ulcers impact, quality of life (Short Form Health Survey). For comparisons between different times analysis of variance for repeated measures was used. To calculate the effect size (ES), the Cohen's test was performed. To evaluate skin moisturizing and warming habits before and after intervention, the McNemar test was used. Statistical significance was set at P ≤ .05., Results: Twenty-two SSc patients (19 women: 3 men; 16 limited scleroderma: 6 diffuse scleroderma) completed the program. Significant improvements were noted for hand pain (3.97 vs 2.21, ES: 0.69), Cochin Hand Function Scale (19.24 vs 12.48, ES: 0.48), Scleroderma Health Assessment Questionnaire (0.95 vs 0.48, ES: 1.01), delta finger-to-palm (92.86 vs 106.33, ES: 0.40), grip strength (14.43 vs 19, ES: 0.58), tip pinch strength (2.49 vs 4.18, ES: 1.15), key pinch strength (4.01 vs 5.22, ES: 0.76), Raynaud phenomenon impact (0.94 vs 0.47, ES: 0.75), Short Form Health Survey-role physical (47.38 vs 60.14, ES: 0.61), physical functioning (34.62 vs 61.9, ES: 0.18), social functioning (60.71 vs 75.6, ES: 0.64), bodily pain (50.55 vs 63.38, ES: 0.58), vitality (45.95 vs 62, ES: 2.22), mental health (56.62 vs 72.38, ES: 0.84) moisturizing, and cold avoidance habits. Patients considered the program easy to follow with no adverse effects related to exercises., Discussion: We developed a home based hand care program to be offered to SSc patients. Improvements in hand function, strength, disability, motion, and overall quality of life were independent of age, income, education level, disease duration, and skin score. Our findings support those of other studies that reported the benefits of hand exercises in SSc. Some study limitations include the lack of a control group, the small number of subjects and the short-time follow up., Conclusions: This home-based program for patients with SSc improved hand pain, function, mobility, and strength at the end of 8 weeks. Patient adherence and sustained efficacy is still to be determined., (Copyright © 2017 Hanley & Belfus. Published by Elsevier Inc. All rights reserved.)

Background/purpose: To evaluate olfactory function in systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and healthy controls over a 2-year period, and to determine the association of olfactory dysfunction with age, disease activity, disease damage, treatment, anxiety and depression symptoms and limbic structures volumes., Methods: Consecutive SLE and SSc patients were enrolled in this study. Clinical, laboratory disease activity and damage were assessed according to diseases specific guidelines. Olfactory functions were evaluated using the Sniffin' Sticks test (TDI). Volumetric magnetic resonance imaging (MRI) was obtained in a 3T Phillips scanner. Amygdalae and hippocampi volumes were analyzed using FreeSurfer® software., Results: We included 143 SLE, 57 SSc and 166 healthy volunteers. Olfactory dysfunction was observed in 78 (54.5%) SLE, 35 (59.3%) SSc patients and in 24 (14.45%) controls (p<0.001) at study entry. SLE and SSc patients had significantly lower mean in all three phases (TDI) of the olfactory assessment when compared with healthy volunteers. In SLE, the presence of olfactory dysfunction was associated with older age, disease activity, higher anxiety and depression symptoms score, smaller left hippocampus volume, smaller left and right amygdalae volume and the presence of anti-ribosomal P (anti-P) antibodies. In SSc the presence of olfactory impairment was associated with older age, disease activity, smaller left and right hippocampi volumes and smaller right amygdala volume. Olfactory function was repeated after a 2-year period in 90 SLE, 35 SSc and 62 controls and was stable in all three groups., Conclusion: Both SLE and SSc patients with longstanding disease had significant reduction in all stages of TDI that maintained stable over a 2-year period. Olfactory dysfunction was associated with age, inflammation and hippocampi and amygdalae volumes. In SLE, additional association with anti-P, anxiety and depression symptoms was observed., (Copyright © 2018. Published by Elsevier B.V.)

The objective of this study is to describe the characteristics of patients with Erasmus syndrome (ES) in a large SSc Brazilian cohort. Nine hundred and forty-seven SSc patients attended at the Scleroderma Outpatient Clinic at two academic medical centers in Brazil and classified as SSc according to the ACR/EULAR criteria were retrospectively studied. Information on demographics, clinical, and laboratory features was obtained by chart review. ES patients had their HLA class II characterized by PCR-SSO method as available. Among the 947 SSc patients studied, nine (0.9 %) had ES. These ES patients were predominantly male (78 %) and smokers (68 %) and presented diffuse SSc (67 %). Mean time of occupational exposure to silica was 13.7 years, with mean age at onset of 47 years. Previous history of tuberculosis was referred by 33 % of the ES patients. All the ES patients presented Raynaud's phenomenon, esophageal involvement, and interstitial lung disease (ILD). Antinuclear antibodies were present in all the ES patients, while anti-topoisomerase I was positive in 44 % and no patient had anticentromere antibody. Three different HLA-DQB alleles (0506, 0305, and 0303) were observed. Compared to non-ES cases, patients with ES were associated with male gender (p < 0.001), diffuse SSc (p < 0.05), ILD (p < 0.05), positive anti-topoisomerase I antibodies (p < 0.05), and death (p < 0.05). Multivariate analysis did not confirm that silicosis is an independent risk factor for SSc. To conclude, ES was rare in this large SSc cohort, although associated with a bad prognosis.

Objective: Systemic sclerosis sine scleroderma (ssSSc) is an infrequent SSc variant characterized by visceral and immunological manifestations of SSc in the absence of clinically detectable skin involvement. We sought to delineate the characteristics of ssSSc in a cohort of Brazilian patients and contrast them with those in the literature., Methods: SSc patients seen at two academic medical centres in Brazil were retrospectively analysed. Patients were classified as ssSSc if they presented with RP, positive ANAs and at least one visceral involvement typical of SSc in the absence of skin thickening. Demographics, clinical and laboratory data were obtained by chart review. Literature review was performed by searching available original studies up until June 2012., Results: Among the 947 consecutive patients with SSc, 79 (8.3%) were classified as ssSSc. Oesophagus was the most frequently affected organ (83.1%), followed by pulmonary involvement (63.2%). Compared with the limited cutaneous form of SSc, telangiectasia was the only variable significantly different after multivariate logistic regression analyses (odds ratio 0.46; 95% CI 0.27, 0.81). Compared with the diffuse cutaneous form of SSc, multivariate analyses revealed that ssSSc patients were less likely to be male (odds ratio 0.15; 95% CI 0.04, 0.57), have digital ulcers (odds ratio 0.26; 95% CI 0.13, 0.51) or anti-Scl70 antibodies (odds ratio 0.19; 95% CI 0.07, 0.55) and less frequently treated with CYC (odds ratio 0.23; 95% CI 0.12, 0.43). These features were comparable to those in the published literature., Conclusion: In this series, patients with ssSSc had a relatively mild disease with good prognosis.

Objectives: The aim of this study was to evaluate human leukocyte antigen (HLA) involvement in the disease expression and poor prognostic clinical features (pulmonary fibrosis and pulmonary arterial hypertension) in patients diagnosed with systemic sclerosis (SSc) in a multiethnic population., Methods: SSc patients followed up between 2008 and 2011 were included, and clinical data were obtained through records review. Molecular HLA typing was performed (polymerase chain reaction amplification technique using specific primer sequences). The statistical analysis involved Fisher's exact test and Pearson's corrected chi-square test. P (values) ≤ 0.05 were considered significant. The delta method was used to estimate the variance of the prevalence ratio (PR)., Results: A total of 141 patients (120 women and 21 men) with SSc were studied, including 33.3% with diffuse cutaneous SSc (dcSSc), 62.4% with limited cutaneous SSc (lcSSc), and 4.3% with sine scleroderma. Pulmonary fibrosis was present in 61 patients (43.3%), and the HLA-A∗30 and DQB1∗04 alleles were related to susceptibility. In contrast, the HLA-DRB1∗01 and DQB1∗05 alleles were protective. Pulmonary arterial hypertension was diagnosed in 19 patients (13.5%) and was associated with HLA-B∗35 and C∗04; in contrast, C∗03 seemed to be protective., Conclusions: Our current study documents the association of some classes I and II HLA alleles with the most severe clinical manifestations in a multiethnic case series. Our findings differed slightly from the previous data in other populations.

Objective: To analyze survival, prognostic factors, and causes of death in a large cohort of patients with systemic sclerosis (SSc)., Methods: From 1991 to 2010, 947 patients with SSc were treated at 2 referral university centers in Brazil. Causes of death were considered SSc-related and non-SSc-related. Multiple logistic regression analysis was used to identify prognostic factors. Survival at 5 and 10 years was estimated using the Kaplan-Meier method., Results: One hundred sixty-eight patients died during the followup. Among the 110 deaths considered related to SSc, there was predominance of lung (48.1%) and heart (24.5%) involvement. Most of the 58 deaths not related to SSc were caused by infection, cardiovascular or cerebrovascular disease, and cancer. Male sex, modified Rodnan skin score (mRSS) > 20, osteoarticular involvement, lung involvement, and renal crisis were the main prognostic factors associated to death. Overall survival rate was 90% for 5 years and 84% for 10 years. Patients presented worse prognosis if they had diffuse SSc (85% vs 92% at 5 yrs, respectively, and 77% vs 87% at 10 yrs, compared to limited SSc), male sex (77% vs 90% at 5 yrs and 64% vs 86% at 10 yrs, compared to female sex), and mRSS > 20 (83% vs 90% at 5 yrs and 66% vs 86% at 10 yrs, compared to mRSS < 20)., Conclusion: Survival was worse in male patients with diffuse SSc, and lung and heart involvement represented the main causes of death in this South American series of patients with SSc.

A 43-year-old woman reported pain in the right hypochondrium, which had started 3 years before and had been worsening for the past few days. Claudication in the superior and inferior limbs, diffuse myalgia, dyspnea, precordialgia followed by dizziness and visual turbidity were added to the clinical picture. In the physical examination bilateral carotid bruit was observed, abdominal aorta murmur and the decrease of the right radial and left pedis pulses and arterial hypertension with difference in the diastolic pressure between limbs >10 mmHg was also observed. On cardiac catheterisation with aortography, right coronary with proximal parietal irregularities, slight pressure increase in right chambers and pulmonary artery, preserved left ventricle contractility, competent valves, carotid and subclavian partial obstruction, severe narrowing of the abdominal aorta below the diaphragm (80%) and right renal artery significant stenosis were observed. Takayasu's arteritis (TA) diagnosis was established according to the ACR criteria based on the clinical symptomatology, on physical and image test findings. Two years later she presented malar rash, photosensitivity, nephropathy, leukopenia, lymphopenia and hemolytic anemia confirming the systemic lupus erythematosus (SLE) diagnosis. TA coexisting with SLE has rarely been reported.