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Scleroderma is a rare complication in taxanes therapy. Although individual cases of taxanes-induced scleroderma have been reported, the clinical manifestation and treatment outcomes were reviewed and summarized rarely. This study reported a patient who developed diffuse scleroderma and possible scleroderma renal crisis after paclitaxel therapy for ureter cancer.A PubMed literature review on published cases of taxanes-induced scleroderma up until April 2022 was included for analysis.The search identified 27 patients with adequate information for analysis. Of the 28 patients, including the one presented here, 22 were female. Peripheral edema was the most common symptom in all but one patient, and often accompanied by erythema in 11. Symptoms usually occurred in half of the patients within the 4Taxanes-induced scleroderma is different from idiopathic scleroderma. Physicians should be aware of this condition in order to provide early diagnosis and apply appropriate management in order to avoid serious complications from severe skin sclerosis. Key Points • Scleroderma is a rare but unique and serious complication of taxanes therapy • Skin manifestations and distribution are similar to idiopathic scleroderma, but vascular phenomenon, internal organ involvement and scleroderma-associated auto-antibodies are presented rarely. Skin improvement usually occurs shortly after discontinuing taxanes • The role of immunosuppressive therapy in treating taxanes-induced scleroderma is not clear.

© International League of Associations for Rheumatology (ILAR) 2021, Evidence for the role of sex in the clinical manifestations of systemic sclerosis (SSc) patients is emerging. Some multicenter cohorts have shown that male SSc patients have more severe disease and worse survival. To assess the differences in clinical manifestations and survival in Portuguese SSc patients according to gender. Data from male and female adult SSc patients included in the Rheumatic Diseases Portuguese Register (Reuma.pt) were analysed and compared. Survival was calculated for patients included in Reuma.pt. within the first two years of diagnosis (inception cohort). In total, 1054 adult patients with SSc were included, 12.5% males. No differences in demographic features and comorbidities were found between the sexes, except for a higher rate of cigarette smokers among men. Diffuse cutaneous SSc and anti-topoisomerase antibodies were more prevalent in males than females. Additionally, male patients presented significantly more myositis, interstitial lung disease and gastric involvement. There were no differences in the patterns of drug use between the sexes. During follow-up, more deaths were reported in men than women (12.1% vs 7.3%, p = 0.04). The overall 1-, 3-, and 5-year survivals from diagnosis of the inception cohort (N = 469) for men vs women were 96.4% vs 98.2%, 93% vs 95.9%, and 75.8% vs 93.2%, respectively, with statistically significant differences (p < 0.01). This study confirms the existence of gender differences in clinical and immunological SSc features. Although SSc is less common in men than women, men have a more severe expression of skin and internal organ involvement and worse survival. Key Points • There are differences in SSc disease manifestations between sexes. • Males more commonly have diffuse cutaneous SSc, anti-topoisomerase antibodies, pulmonary and musculoskeletal involvement. • In the inception cohort, men had worse survival rates than women.

To determine limited joint mobility (LJM) of the hand in patients with systemic sclerosis (SSc).LJM was evaluated with "prayer sign" and "tabletop sign" tests. LJM staging was done by Rosenbloom classification method. LJM (+) and LJM (-) patients were compared in terms of demographic findings (gender, age and duration of disease), laboratory results (ESR, CRP, anti-nuclear antibody (ANA), anti-topoisomerase I and anti-centromere), and modified Rodnan skin score (mRss) results.In our study, a total of 217 patients, including 113 patients with a diagnosis of SSc, and 104 as a healthy control group with similar age and gender distribution to these patients, were included. A total of 113 (F=98, M=15) patients (limited cutaneous SSc (lcSSc=71), diffuse cutaneous SSc (dcSSc=42)) were included in this study and LJM positivity was found in 66.4% (lcSSc=38, dcSSc=37). A statistically significant difference was observed in between lcSSc and dcSSc patients according to the presence of LJM (p0.001). There was a moderate positivity relationship between LJM and mRss (lcSSc r=0.449, p0.001; dcSSc r=0.565, p0.001).In our study, it was found that LJM staging correlated with mRss and dcSSc patients had more severe LJM findings than lcSSc. We conclude that "prayer sign" and "tabletop sign" tests used in hand evaluation in SSc patients have similar clinical results with mRss and can be simple bedside tests in daily practice. Key Points • This is the first study examining limited joint mobility (LJM) with "prayer sign" and "tabletop sign" tests in systemic sclerosis (SSc) patients. • "Prayer sign" and "tabletop sign" tests can be easily performed in daily practice. • We found Rosenbloom LJM staging correlated with modified Rodnan skin score. LJM of the hand can be a good prognostic indicator for early stage SSc patients.

In systemic sclerosis, baseline extent of radiological involvement is an important outcome predictor and baseline absence of radiological involvement suggests a more favourable prognosis. As current predictive models are based on cohorts with variable disease duration, we aim to assess disease dynamics in early disease.Patients were included from the prospective longitudinal Belgian Systemic Sclerosis Cohort. We included patients with a disease duration = 36 months at baseline with available baseline thoracic high-resolution computed tomography (HRCT) images and longitudinal pulmonary function test (PFT) results until 42 months of follow-up.Fifty-two patients were included; 50% were male and 44% suffered from diffuse cutaneous systemic sclerosis. A total of 46% carried anti-topoisomerase 1 antibodies. The mean disease duration at baseline visit was 11 months. At baseline visit, 40.4% (21/52) patients had HRCT abnormalities. Patients with abnormal HRCT findings more frequently suffered from diffuse cutaneous systemic sclerosis (p 0.05) and less frequently carried anti-centromere antibodies (p 0.05). Patients without CT abnormalities at baseline had a shorter disease duration (9 ± 7 months versus 14 ± 12 months). After 42 months, 8/52 patients, including 3 patients with normal HRCT findings at baseline, died due to SSc-related manifestations. Progression of lung fibrosis occurred in 16 patients at month 42, including 7 patients with normal CT at baseline. No clear predictors of progression could be identified.In early SSc patients, the disease dynamics differ from the large published cohorts. Progressive lung fibrosis and mortality can also occur in patients without radiological abnormalities at baseline. Key Points • Disease dynamics in early SSc differ from more established SSc. • In early SSc, progressive pulmonary fibrosis can occur in patients without CT abnormalities at baseline. • In early SSc, more stringent pulmonary follow-up is warranted both in lcSSc and dcSSc.

To determine the prevalence of clinical manifestations and incidence rate of cardiopulmonary complications in a comparison between men and women with early SSc. An inception cohort of early-SSc patients at the Rheumatology Clinic, Maharaj Nakorn Chiang Mai Hospital, between January 2010 and June 2016, was used. All patients were assessed for clinical manifestations and underwent ECG, echocardiography, and HRCT at the study entry and then annually. One hundred and fifteen patients (46 male, 90 dcSSc) with a mean (SD) disease duration of 11.6 months (8.8) at cohort entry were enrolled during a mean (SD) observational period of 3.8 years (1.6). At enrollment, the male group had a higher prevalence of dcSSc subtype (91.3% vs. 69.5%, p = 0.006), hypo-hyperpigmentation (84.8% vs. 65.2%, p = 0.021), myositis (26.1% vs. 10.1%, p = 0.024), and right ventricular dysfunction [RVD] (8.7% vs. 0%, p = 0.024) compared with women. At the last visit, the male group had a higher cumulative prevalence of digital ulcers (47.8% vs. 27.5%, p = 0.026), telangiectasia (93.5% vs. 69.6%, p = 0.002), joint contracture (69.6% vs. 43.5%, p = 0.006), tendon friction rub (39.1% vs. 20.3%, p = 0.027), LVEF

S100A8 and S100A9 play important roles in immune and inflammatory disorders. The role of the two proteins in systemic sclerosis (SSc) remains unknown. Fifty-seven diffuse cutaneous SSc (dcSSc) patients, 31 limited cutaneous SSc (lcSSc) patients were recruited in the present study. The expression of S100A8 and S100A9 in plasma was measured using an enzyme-linked immunosorbent assay and the mRNA levels in peripheral blood were assessed using reverse transcriptase quantitative PCR. The expression and distribution of S100A8, S100A9, and receptor for advanced glycation end products (RAGE), in skin tissues was analyzed by immunohistochemistry. The plasma concentrations of S100A8 and S100A9 were significantly higher in dcSSc patients than in normal controls and lcSSc patients. Both S100A8 and S100A9 levels were significantly increased in dcSSc patients with lung or kidney involvement. Increased plasma levels of S100A8 and S100A9 in dcSSc patients were associated with several autoantibodies. Transcription levels of S100A8 and S100A9 in peripheral blood were found elevated in both dcSSc and lcSSc patients than normal controls. Immunohistochemistry demonstrated higher S100A8 and S100A9 expression in sclerotic skin than in normal skin. The number of S100A8, S100A9, or RAGE positive fibroblasts was also significantly increased. Highly elevated expression of both S100A8 and S100A9 was found in dcSSc patients. There was close correlation with disease severity and serological abnormalities, suggesting that the two proteins may play important roles in the development of systemic sclerosis.

The purpose of this study was to assess the impact of enteric-coated mycophenolate sodium (EC-MPS) on skin and pulmonary manifestations of patients with progressive systemic sclerosis (Ssc). A prospective, open-label single-centre trial with EC-MPS 2 × 720 mg/day over 12 months and a long-term follow-up of 50 months were conducted. Modified Rodnan skin score (mRSS) was used to assess the skin and pulmonary function tests to assess the pulmonary involvement. In order to quantify the extent of alveolitis/fibrosis via densitometry, the high attenuation value, median lung density and percentiles of lung tissue densities were obtained by high-resolution computed tomography. Eleven patients were included. Three patients had to stop medication before month 6 (2× side effects, 1× progression). For the remaining eight patients, the median mRSS was non-significantly reduced from 13.5 at baseline to 11 at month 12. According to the CT histography, median lung density and high attenuation values remained stable. However, the course of percentiles -200 to -300 and particularly -300 to -400 Hounsfield units slightly increased in seven of eight patients after 12 months, suggesting worsening of pulmonary involvement. Accordingly, median diffusing capacity for carbon monoxide showed a tendency to decline (75.1 % vs. 70.2) while forced vital capacity non-significantly improved (78.0 vs. 85.5 %) during the study. Four patients are still on EC-MPS without clinical signs of progression after 50 months follow-up. EC-MPS showed non-significant improvement of the skin. Pulmonary fibrosis remained stable with only a slight tendency towards progression which might be ascribed to the medication as well as the natural course of the disease. CT histography appears to be a sensitive method for the detection of progression of pulmonary fibrosis and therefore should be considered for further studies in Ssc.

Skin tightness is a major clinical manifestation of systemic sclerosis (SSc). Importantly for both clinicians and patients, spontaneous regression of the fibrosis process has been documented. The purpose of this study is to identify the incidence and related clinical characteristics of spontaneous regression among Thai SSc patients. A historical cohort with 4 years of follow-up was performed among SSc patients over 15 years of age diagnosed with SSc between January 1, 2005 and December 31, 2006 in Khon Kaen, Thailand. The start date was the date of the first symptom and the end date was the date of the skin score ≤2. To estimate the respective probability of regression and to assess the associated factors, the Kaplan–Meier method and Cox regression analysis was used. One hundred seventeen cases of SSc were included with a female to male ratio of 1.5:1. Thirteen patients (11.1%) experienced regression. The incidence rate of spontaneous skin regression was 0.31 per 100 person-months and the average duration of SSc at the time of regression was 35.9 ± 15.6 months (range, 15.7–60 months). The factors that negatively correlated with regression were (a) diffuse cutaneous type, (b) Raynaud’s phenomenon, (c) esophageal dysmotility, and (d) colchicine treatment at onset with a respective hazard ratio (HR) of 0.19, 0.19, 0.26, and 0.20. By contrast, the factor that positively correlated with regression was active alveolitis with cyclophosphamide therapy at onset with an HR of 4.23 (95% CI, 1.23–14.10). After regression analysis, only Raynaud’s phenomenon at onset and diffuse cutaneous type had a significantly negative correlation to regression. A spontaneous regression of the skin fibrosis process was not uncommon among Thai SSc patients. The factors suggesting a poor predictor for cutaneous manifestation were Raynaud’s phenomenon, diffuse cutaneous type while early cyclophosphamide therapy might be related to a better skin outcome.

Increasing evidence supports the concept of macrophage migration inhibitory factor (MIF) as a central proinflammatory cytokine in autoimmune diseases. To further evaluate its role in systemic sclerosis (SSc), serum levels of MIF were determined by enzyme-linked immunoassay, and correlations to clinical manifestations were analyzed in 43 patients. MIF levels were significantly increased in patients (median, 18.8; range

Objective: To determine the prevalence of anti-cyclic citrullinated peptide (CCP) antibodies in systemic sclerosis (SSc) and to assess any association between the presence of anti-CCP, radiographic features, and clinical manifestations. Materials and methods: Anti-CCP antibodies and rheumatoid factor (RF) were tested in serum samples from 75 patients with SSc (64 women and 11 men), with a mean age of 59.4 years (range 24–85) with either diffuse (dcSSc) and limited (lcSSc) cutaneous involvement. As a control group, 22 age- and sex-matched healthy controls (HCs) were examined. Standard radiographs of the hands and wrists were examined in each patient. Results: The presence of anti-CCP was found in sera of 10.6% (8/75) patients with SSc (lcSSc 3 of 44, 6.8%; dcSSc 5 of 31, 16.1%). None of the HCs was positive for anti-CCP. The positivity of RF was observed in 19 of 75 (25.3%) SSc patients (lcSSc 10 of 44, 22.7%; dcSSc 9 of 31, 29%). Statistically significant association was found between anti-CCP positivity and the presence of arthritis (p

This study aims to analyze differences among established disease damage indicators in patients with limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dcSSc). Fifty patients with lcSSc and 55 patients with dcSSc were included in this study. Difference in mean disease duration between the two subgroups of patients was not statistically significant (z=-0.88, p=0.38). Patients with lcSSc and dcSSc were compared, and differences in vascular, esophageal, lung, heart, renal, and musculoskeletal involvement were statistically assessed using chi (2), Mann-Whitney, and Kruskal-Wallis tests. Using the technique of nailfold capillaroscopy, we found normal capillaries or nonspecific capillary change in 10.0% of the patients with lcSSc and only in 3.6% of the patients with dcSSc. Dilated capillaries without loss of capillaries were found in 42% of the patients with lcSSc and in 10.9% of the patients with dcSSc (p=0.05). However, severe capillary damage (loss of capillaries) was noticed more frequently in patients with dcSSc (dcSSc/lcSSc: 85.5%/48.0%, p=0.002). Pitting scars or digital ulcers were found in 46.0% of the patients with lcSSc and in 67.3% of the patients with dcSSc (p=0.04). We did not notice a significant difference in frequency of fingertip osteolysis and telangiectasia. Esophageal hypomotility was found in 64% of the patients with lcSSc and in 85.5% of the patients with dcSSc (p

Fibrinogen-like protein 2 (FGL2), a member of the fibrinogen-related superfamily of proteins, is expressed on the surface of macrophages, T cells, and endothelial cells and directly cleaves prothrombin to thrombin. The aim of this study is to determine the serum FGL2 level and its association with clinical parameters in patients with systemic sclerosis (SSc). Serum FGL2 level was examined by enzyme-linked immunosorbent assay in 61 patients with SSc, 24 patients with systemic lupus erythematosus, and 24 healthy individuals. In a retrospective longitudinal study, sera from 13 patients with SSc were analyzed. The serum FGL2 level was increased in patients with SSc compared with healthy individuals (P

Anti-CCP (cyclic citrullinated peptide) is considered the most useful laboratory tool in the diagnosis of rheumatoid arthritis (RA). Some authors have also found this autoantibody in patients with scleroderma (SSc). The study aimed to investigate the prevalence of anti-CCP antibodies in SSc patients from Southern Brazil and their association with clinical and serological profile of the disease. We studied 76 patients with SSc and 100 healthy volunteers for presence of anti-CCP. SSc patients charts were reviewed for clinical and laboratory data. In the SSc group, the diffuse form was present in 20.5%; 62.8% had the limited form; 14.1% had overlap with systemic lupus or polymyositis and 2.5% had SSc sine scleroderma. Anti-CCP was found in nine of 78 (11.5%) SSc patients and in one of 100 healthy volunteers (p = 0.0054). No relationship was found with arthritis, skin Rodnan m score, esophageal dysmotility, myocarditis, pulmonary hypertension and lung fibrosis. Positive association was observed with arthralgias (p = 0.02). Also, no relationship was noted with the presence of anti-centromere antibodies, anti-Scl-70, anti-RNP or rheumatoid factor. Anti-CCP are more common in SSc patients than in controls. Arthralgias but not arthritis or rheumatoid factor are more frequent in anti-CCP positive patients.

Systemic sclerosis (SSc) is complicated by pulmonary hypertension and right ventricle (RV) failure in approximately 10% of the patients. Factors influencing the reactivity of pulmonary circulation to vasodilators are not established, while the examination of vasoreactivity is important in determining the treatment, because systemic administration of oral vasodilators can induce severe adverse events in nonresponders. The mechanism of RV failure in SSc is unclear and may result either from increased RV afterload or intrinsic myocardial disease. The aim of the study was to assess the reactivity of pulmonary circulation to inhaled nitric oxide (iNO) and to evaluate its influence on RV function in SSc patients with elevated right ventricle systolic pressure (RVSP). In 60 SSc patients aged 24–73 (58 females, two males; 33 patients with limited SSc and 27 with diffuse SSc), echocardiographic examination with tissue Doppler echocardiography (TDE) was performed. RV function was measured by systolic (S) and early diastolic (E) velocity of tricuspid annulus by TDE. In patients with RVSP >45 mmHg, the reactivity of pulmonary circulation was assessed by iNO test. High-resolution computerized tomography (HRCT) was performed to assess the extent of pulmonary fibrosis. Of 14 SSc subjects with elevated RVSP (13 females, one male; RVSP 47–62 mmHg), positive reaction to iNO was observed in five (RVSP decreased from 51.6 ± 3.7 to 32.24 ± 2.3 mmHg); nine patients were not reactive (RVSP 53.5 ± 5.7 mmHg before iNO vs. 49.6 ± 6.7 mmHg). RV systolic function was decreased in patients with elevated RVSP as compared to the patients with normal pulmonary pressure (S velocity 13.2 ± 1.3 vs. 14.4 ± 1.6 cm/s, respectively, p

Autoimmune hepatitis (AIH) is a chronic hepatitis of unknown etiology characterized by continuing hepatocellular necrosis and inflammation that afflicts 100,000 to 200,000 persons in the United States. It is a rare manifestation of systemic sclerosis. Only about nine reports of this association have been previously reported in the literature. Importantly, all cases had the limited clinical form of systemic. The authors describe herein the first report of a patient with diffuse systemic sclerosis who was diagnosed with AIH with positive antimitochondrial antibody and had an excellent response to immunosuppressive drugs. We also briefly review the literature regarding this issue.

Pulmonary arterial hypertension is a recognized clinical component of systemic autoimmune diseases, especially systemic sclerosis. Mutations in the bone morphogenetic protein receptor 2 gene reported in sporadic and familial primary pulmonary arterial hypertension have failed to be detected in patients with either scleroderma spectrum disease or underlying connective tissue diseases. Activin receptor-like kinase 1 (ALK-1) gene has recently been linked to the pathogenesis of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia, which has some resemblance with the CREST syndrome. The presence of mutations in the ALK-1 gene in ten patients with underlying connective tissue diseases was investigated.

Macrovascular involvement in scleroderma has received relatively little attention. We hereby describe a 5.6-cm ascending aortic aneurysm in a 56-year-old man presenting with increased dyspnea, diagnosed with antibody-negative, rapidly progressive diffuse cutaneous scleroderma. The patient had sclerosis and induration of all extremities, face, thorax and abdomen. Other features included Raynaud's phenomenon, arthralgia, dysphagia, dyspnea attributed to pulmonary fibrosis, pericardial effusion, and cardiomyopathy. To the best of our knowledge, there is only one other report to date in the English language of a thoracic aortic aneurysm associated with scleroderma.

The aim of the study is to assess the natural course of systemic sclerosis (SSc) in patients with interstitial lung involvement and to evaluate the effects of treatment.Sixty SSc patients with interstitial lung involvement were included in the study and history and retrospective data records of the patients were reviewed.It was observed that 47 patients (78.3%) had Raynaud's phenomenon, 7 patients (11.7%) had skin involvement, 5 patients (8.3%) had joint involvement, and 1 patient (1.7%) had gastrointestinal system involvement as the first manifestation of the disease. Lung involvement had developed on an average of 113 +/- 106 months after the first manifestation of the disease and was apparent within the first year in 10 patients (16.7%), between 1 and 2 years in 3 patients (5%), between 2 and 3 years in 2 patients (3.3%), between 3 and 4 years in 5 patients (8.3%), between 4 and 10 years in 21 patients (35%), and after 10 years in 19 patients (31.7%). When the symptoms of lung involvement appeared, 37 patients (61.7%) were not receiving treatment while 23 (38.3%) were using an immunosuppressive agent. The time interval of lung involvement for the treated patients was 131 +/- 95 months while it was 101 +/- 112 months in untreated patients (p0.05).In SSc patients with interstitial pulmonary involvement, the disease frequently starts with Raynaud's phenomenon and pulmonary symptoms tend to appear at a mean of 7 years after the onset of disease. The first sign of the disease, the probability of interstitial pulmonary involvement, is highest during the first 15 years after. Although this probability decreases after 15 years, in up to 10% of the patients, interstitial pulmonary involvement can still occur even up to 40 years. Immunosuppressive treatment is not effective in preventing the development of pulmonary involvement. However, it delays the manifestation of pulmonary symptoms for nearly 4 years.

This study aims to characterize the clinical features of a cohort of patients diagnosed with systemic sclerosis (SSc) after the age of 75 and compare them to a group diagnosed at a younger age. We record the review of 769 patients diagnosed with SSc over the past 16 years. Utilizing a nested case-control model, we compare demographics, disease severity, morbidity, and mortality data of all patients diagnosed after the age of 75 to sex- and disease-type-matched, randomly selected group of patients diagnosed with SSc before the age of 60. Twelve patients were diagnosed with SSc after the age of 75, seven with the diffuse, and five with the limited form. It took longer to diagnose SSc in the older patients, and comparison of disease severity revealed a worse pulmonary picture and a more frequent development of malignancy in the older patients as compared with the younger ones. During a mean follow-up of 36.2 months, our cohort of patients did not have worsening in their disease severity, though 6 months after the last follow-up, six patients died. We conclude that a diagnosis of SSc at an older age appears to be a poor prognostic indicator related to both disease severity and comorbidities. A higher clinical suspicion will lead to an earlier diagnosis and a potential decrease in morbidity and mortality.

We describe a patient with diffuse systemic sclerosis and presyncopal episodes where the electrocardiogram revealed complete atrioventricular heart block associated with left posterior and right heart bundle block. The patient underwent implantation of a permanent pacemaker.

Texto completo:acesso restrito. p. 50-51 Submitted by JURANDI DE SOUZA SILVA (jssufba@hotmail.com) on 2013-01-25T12:06:55Z No. of bitstreams: 1 art%3A10.1007%2Fs10067-003-0823-1.pdf: 164389 bytes, checksum: 10ea308e7d9b19b37e3c1a5bdb5575bd (MD5) Made available in DSpace on 2013-01-25T12:06:55Z (GMT). No. of bitstreams: 1 art%3A10.1007%2Fs10067-003-0823-1.pdf: 164389 bytes, checksum: 10ea308e7d9b19b37e3c1a5bdb5575bd (MD5) Previous issue date: 2004 Progressive systemic sclerosis (PSS) can involve the skin diffusely or in a limited way. Rarely cutaneous involvement can assume a hypertrophic form, described in the literature as keloidal scleroderma. The authors describe a PSS patient who presented cutaneous manifestations compatible with the diagnosis of this clinical situation. The clinical features, pathogenic mechanism and therapeutic approach are discussed and a broad review of the literature is given.