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Rheumatology (Oxford, England) Sep 2023SSc is an autoimmune disease characterized by excessive fibrosis in multiple organs, including the gastrointestinal (GI) tract. GI symptoms of SSc such as intestinal...
OBJECTIVES
SSc is an autoimmune disease characterized by excessive fibrosis in multiple organs, including the gastrointestinal (GI) tract. GI symptoms of SSc such as intestinal pseudo-obstruction (IPO) are often refractory to conventional intervention and can result in longer in-hospital stay or even increased mortality. We aimed to summarize the insights to date regarding the efficacy of IVIG against GI symptoms of SSc to unveil what we should focus on in future studies.
METHODS
Herein we report the response of GI symptoms in three cases with SSc-myositis overlap who received IVIG administration. We also conducted a systematic literature review to summarize previous reports regarding the efficacy of IVIG upon the GI manifestations of SSc, according to the PRISMA 2020 guideline.
RESULTS
The case series demonstrated remarkable and rapid improvement of GI symptoms, including IPO, after IVIG administration. The literature review revealed that previous reports also support the efficacy and safety of IVIG against GI manifestations of SSc. However, they were all retrospective studies and lacking description of the short-term outcome after IVIG administration with objective and quantitative metrics.
CONCLUSION
IVIG seems to be a promising therapeutic option for the management of GI symptoms in SSc, including IPO. Investigators should focus more on short-term outcomes to properly assess the therapeutic benefit of IVIG, ideally using reliable quantitative measures in a multicentre randomized placebo-controlled setting.
Topics: Humans; Immunoglobulins, Intravenous; Retrospective Studies; Scleroderma, Systemic; Gastrointestinal Diseases; Intestinal Pseudo-Obstruction
PubMed: 36825818
DOI: 10.1093/rheumatology/kead093 -
BMJ Open Feb 2023Cardiac involvement in patients with systemic sclerosis (SSc) is associated with poor prognosis. Early detection of myocardial impairment is essential for treatment. The... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
Cardiac involvement in patients with systemic sclerosis (SSc) is associated with poor prognosis. Early detection of myocardial impairment is essential for treatment. The present study aimed to systematically review the value of detecting subclinical myocardial impairment in SSc patients using myocardial strain obtained from speckle tracking echocardiography (STE).
DESIGN
A systematic review and meta-analysis.
DATA SOURCES
The PubMed, Embase and Cochrane library databases were searched in the period from the earliest available indexing date to 30 September 2022.
ELIGIBILITY CRITERIA FOR SELECTING STUDIES
Studies evaluating myocardial function in SSc patients comparison with healthy controls based on myocardial strain data obtained from STE were included.
DATA EXTRACTION AND SYNTHESIS
Ventricle and atrium data on myocardial strain were extracted to assessing the mean difference (MD).
RESULTS
A total of 31 studies were included in the analysis. Left ventricular global longitudinal strain (MD: -2.31, 95% CI -2.85 to -1.76), left ventricular global circumferential strain (MD: -2.93, 95% CI -4.02 to -1.84) and left ventricular global radial strain (MD: -3.80, 95% CI -5.83 to -1.77) was significantly lower in SSc patients than in healthy controls. Right ventricular global wall strain (MD: -2.75, 95% CI -3.25 to -2.25) was also decreased in SSc patients. STE revealed significant differences in several atrial parameters including left atrial reservoir strain (MD: -6.72, 95% CI -10.09 to -3.34) and left atrial conduit strain (MD: -3.26, 95% CI -6.50 to -0.03), as well as right atrial reservoir strain (MD: -7.37, 95% CI -11.20 to -3.53) and right atrial conduit strain (MD: -5.44, 95% CI -9.15 to -1.73). There were no differences in left atrial contractile strain (MD: -1.51, 95% CI -5.34 to 2.33).
CONCLUSION
SSc patients have a lower strain than healthy controls for the majority of STE parameters, indicating the presence of an impaired myocardium involving both the ventricle and atrium.
Topics: Humans; Atrial Fibrillation; Echocardiography; Heart Atria; Heart Ventricles; Scleroderma, Systemic; Reproducibility of Results; Ventricular Function, Left
PubMed: 36797018
DOI: 10.1136/bmjopen-2022-063364 -
Rheumatology International Jul 2023A literature review on new-onset autoimmune connective tissue diseases (ACTDs) following COVID-19 is lacking. We evaluated potential associations between COVID-19 and...
A literature review on new-onset autoimmune connective tissue diseases (ACTDs) following COVID-19 is lacking. We evaluated potential associations between COVID-19 and the development of new-onset ACTDs. The "population" was adults with disease terms for ACTDs, including systemic lupus erythematosus (SLE), Sjogren's syndrome, systemic sclerosis (SSc), idiopathic inflammatory myositis (IIM), anti-synthetase syndrome, mixed CTD and undifferentiated CTD, and "intervention" as COVID-19 and related terms. Databases were searched for English-language articles published until September 2022. We identified 2236 articles with 28 ultimately included. Of the 28 included patients, 64.3% were female, with a mean age was 51.1 years. The USA reported the most cases (9/28). ACTD diagnoses comprised: 11 (39.3%) IIM (including four dermatomyositis); 7 (25%) SLE; four (14.3%) anti-synthetase syndrome; four (14.3%) SSc; two (7.1%) other ACTD (one lupus/MCTD overlap). Of eight, four (14.3%) patients (including that with lupus/MCTD) had lupus nephritis. The average time from COVID-19 to ACTD diagnosis was 23.7 days. A third of patients were admitted to critical care, one for treatment of haemophagocytic lymphohistiocytosis in SLE (14 sessions of plasmapheresis, rituximab and intravenous corticosteroids) and nine due to COVID-19. 80% of patients went into remission of ACTD following treatment, while three (10%) patients died-one due to macrophage activation syndrome with anti-synthetase syndrome and two from unreported causes. Our results suggest a potential association between COVID-19 and new-onset ACTDs, notably in young females, reflecting more comprehensive CTD epidemiology. The most common diagnosis in our cohort was IIM. The aetiology and mechanisms by which ACTDs emerge following COVID-19 remain unknown and require further research.
Topics: Adult; Humans; Female; Middle Aged; Male; Mixed Connective Tissue Disease; Incidence; COVID-19; Connective Tissue Diseases; Autoimmune Diseases; Lupus Erythematosus, Systemic; Scleroderma, Systemic; Prognosis; Lupus Nephritis; Myositis
PubMed: 36786873
DOI: 10.1007/s00296-023-05283-9 -
International Journal of Nephrology and... 2023Nephrogenic systemic fibrosis (NSF) is a rare disorder that occurs in association majorly with chronic kidney disease (CKD). The lack of collective quantitative data on... (Review)
Review
AIM
Nephrogenic systemic fibrosis (NSF) is a rare disorder that occurs in association majorly with chronic kidney disease (CKD). The lack of collective quantitative data on its clinical manifestations and the different treatment options' efficacy, call the need for our investigation.
METHODS
A systematic review was conducted covering a timeline from inception up to July 2022 without any restrictions. Article screening and data extraction were performed independently on PubMed, Google Scholar, ScienceDirect, and Cochrane Library. The keywords that we used were CKD, NSF, Gadolinium enduced fibrosis, etc; shortlisted articles were assessed for risk of bias. Data were presented as frequencies and percentages, with a confidence interval of 95%. A chi-square test was also done to find significant relationships, with a -value <0.05 considered significant.
RESULTS
We had 83 patients in this review consisting of 44 (55.7%) females with a mean age of 51.4±14.6 years. Sixty-nine (83.1%) patients had chronic kidney disease predisposition to NSF. Previous exposure to gadolinium-based contrast dyes was seen in 66 (79.5%) patients). The most common symptom in patients was cutaneous lesions in 69 (83.1%) patients. The most used treatments were ultraviolet therapy, renal transplant, and extracorporeal photopheresis; in 13.3% of the patients each. Condition in most patients either improved (67.1%) or remained stable (11.8%). Chi-square testing found that the treatments offered were also seen to be significantly related to outcome (p=0.015).
CONCLUSION
The findings in this study provide a quantitative measurement of NSF's presentations and treatment efficacies. This serves to make way for researchers to form comprehensive guidelines on the presentation-based treatment of NSF.
PubMed: 36660606
DOI: 10.2147/IJNRD.S392231 -
Medicine Jan 2023Chronic graft versus host disease (cGVHD) is a systemic immune-mediated complication that occurs in approximately half of patients undergoing allogeneic hematopoietic...
RATIONALE
Chronic graft versus host disease (cGVHD) is a systemic immune-mediated complication that occurs in approximately half of patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HCT), and remains the leading cause of late morbidity and mortality. cGVHD involves a heterogeneous group of organic manifestations, many of which mimic autoimmune diseases such as scleroderma, primary biliary cholangitis, Sjögren syndrome and polymyositis.
PATIENT CONCERNS
A 60-years-old female with a history of allo-HCT developed de novo cGVHD 11 months after allo-HCT with isolated liver involvement. The patient presented with jaundice, cytolysis, cholestasis and concomitant acute digital ischemia. Liver biopsy and autoimmunity tests were performed and were found to be compatible with immune-mediated liver damage. Nailfold capillaroscopy revealed microangiopathy, characterized by avascular areas and some enlarged capillaries resembled an early systemic sclerosis pattern.
DIAGNOSIS
Biliary cholangitis-like and digital ischemia related to cGVHD.
INTERVENTIONS
The patient was treated with high-dose prednisone and ursodeoxycholic acid, and extracorporeal photopheresis. The patient required hospital admission for administration of intravenous prostacyclin due to refractory Raynaud syndrome.
OUTCOMES
After 6 to 8 weeks, the patient achieved a good response, with evident clinical improvement and progressive normalization of liver function.
LESSONS
cGVHD is a multiorgan pathological condition, and this case emphasizes that a multidisciplinary team, including rheumatologists, should be involved in the follow-up of allo-transplant patients to ensure that the clinical complications are adequately addressed. Early intervention is critical for improving patient' prognosis.In addition, we performed a systemic literature review based on published case articles on hepatic cGVHD and digital ischemia published up to August 2022. To the best of our knowledge, this is the first reported case of such an association.
Topics: Humans; Female; Middle Aged; Transplantation, Homologous; Hematopoietic Stem Cell Transplantation; Graft vs Host Disease; Cholangitis; Bronchiolitis Obliterans Syndrome; Scleroderma, Systemic; Ischemia; Chronic Disease
PubMed: 36637943
DOI: 10.1097/MD.0000000000032495 -
Pharmaceuticals (Basel, Switzerland) Dec 2022The role of Bronchoalveolar Lavage (BAL) in the evaluation of systemic sclerosis (SSc) interstitial lung disease (ILD) is still controversial. The aim of this systematic... (Review)
Review
The role of Bronchoalveolar Lavage (BAL) in the evaluation of systemic sclerosis (SSc) interstitial lung disease (ILD) is still controversial. The aim of this systematic literature review was to investigate the use of BAL in SSc-ILD, and to focus on the pros and cons of its real-life application. Methods: PubMed, Cochrane, and Embase were questioned from inception until 31 December 2021. Results: Eighteen papers were finally analyzed. A positive correlation was observed between lung function and BAL cytology; in particular, BAL neutrophilia/granulocytosis was related to lower diffusing capacity for carbon monoxide (DLCO) values and lower forced vital capacity (FVC). Moreover, a positive correlation between BAL cellularity and high-resolution computed tomography (HRCT) findings has been reported by several authors. Cytokines, chemokines, growth factors, coagulation factors, and eicosanoids have all been shown to be present, more often and in higher quantities in SSc-ILD patients than in the health control and, in some cases, they were related to more severe pulmonary disease. There was no consensus regarding the role of BAL cellularity as a predictor of mortality.
PubMed: 36559035
DOI: 10.3390/ph15121584 -
International Journal of Molecular... Oct 2022Systemic sclerosis (SSc) is an autoimmune disease characterized by skin and internal organ fibrosis and microvascular impairment, which can affect major organs,... (Review)
Review
Systemic sclerosis (SSc) is an autoimmune disease characterized by skin and internal organ fibrosis and microvascular impairment, which can affect major organs, including the heart. Arrhythmias are responsible for approximately 6% of deaths in patients with SSc, and mainly occur due to myocardial fibrosis, which causes electrical inhomogeneity. The aim of this study was to determine the frequency of arrhythmias and conduction disturbances in SSc cohorts, and to identify the characteristics and risk factors associated with the occurrence of dysrhythmias in patients with SSc. A systematic literature review using PubMed, Embase, Web of Science and Scopus databases was performed. Full-text articles in English with arrhythmias as the main topic published until 21 April 2022 were included. Most prevalent arrhythmias were premature supraventricular and ventricular contractions, while the most frequent conduction disturbance was represented by right bundle branch block (RBBB). Elevated concentrations of N-terminal prohormones of brain natriuretic peptides (NT-pro BNP) were associated with numerous types of atrial and ventricular arrhythmias, and with the occurrence of RBBB. A lower value of the turbulence slope (TS) emerged as an independent predictor for ventricular arrhythmias. In conclusion, dysrhythmias are frequent in SSc cohorts. Paraclinical and laboratory parameters are useful instruments that could lead to early diagnosis in the course of the disease.
Topics: Humans; Electrocardiography; Arrhythmias, Cardiac; Heart; Scleroderma, Systemic; Autoimmune Diseases
PubMed: 36361752
DOI: 10.3390/ijms232112963 -
Blood Pressure Monitoring Feb 2023Nailfold video-capillaroscopy (NVC) is an inexpensive method of assessing microcirculation. We reviewed the literature to assess whether changes to the nailfold...
OBJECTIVES
Nailfold video-capillaroscopy (NVC) is an inexpensive method of assessing microcirculation. We reviewed the literature to assess whether changes to the nailfold capillaries exist in patients with cardiovascular disease (CVD).
METHODS
We searched PubMed, Scopus and Cochrane Library databases for original research articles relating to the use of noninvasive microvascular assessment in patients with CVD. Methodological quality was assessed with the 'Quality Assessment Tool for Observational Cohort and Cross-sectional Studies.' The results obtained from NVC were analysed qualitatively and compared with other forms of microvascular assessment.
RESULTS
In total 2759 articles were screened, of which 22 studies involving 562 patients (~40% women) with CVD were included. Mean age ranged between 3.7-68.4 years (cases) and 4.0-58.0 years (controls). Reduced capillary density and increased capillary dimensions were seen in patients with pulmonary arterial hypertension (PAH). Among patients with systemic sclerosis, advanced scleroderma patterns can be used to identify patients with or at risk of developing PAH. Functional nailfold changes precede structural changes in patients with hypertension. However, the studies were heterogeneous in the diagnosis of disease and the measurement of nailfold parameters. Most studies did not exclude conditions with altered nailfold features, and only one study performed a power calculation. Furthermore, abnormal nailfold findings are present in patients without systemic disease.
CONCLUSIONS
Structural and functional changes to the nailfold are a feature of established CVD and precede the development of PAH. However, heterogeneity in measurement and abnormal findings in healthy participants limit their use in the wider population.
Topics: Humans; Female; Child, Preschool; Child; Adolescent; Young Adult; Adult; Middle Aged; Aged; Male; Cardiovascular Diseases; Microscopic Angioscopy; Cross-Sectional Studies; Blood Pressure; Scleroderma, Systemic
PubMed: 36281701
DOI: 10.1097/MBP.0000000000000624 -
Thorax Aug 2023Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is rare, poorly understood, with heterogeneous characteristics resulting in difficult diagnosis. We... (Meta-Analysis)
Meta-Analysis
UNLABELLED
Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is rare, poorly understood, with heterogeneous characteristics resulting in difficult diagnosis. We aimed to systematically review evidence of soluble markers in peripheral blood or bronchoalveolar lavage fluid (BALF) as biomarkers in SSc-ILD.
METHOD
Five databases were screened for observational or interventional, peer-reviewed studies in adults published between January 2000 and September 2021 that assessed levels of biomarkers in peripheral blood or BALF of SSc-ILD patients compared with healthy controls. Qualitative assessment was performed using Critical Appraisal Skills Programme (CASP) checklists. Standardised mean difference (SMD) in biomarkers were combined in random-effects meta-analyses where multiple independent studies reported quantitative data.
RESULTS
768 published studies were identified; 38 articles were included in the qualitative synthesis. Thirteen studies were included in the meta-analyses representing three biomarkers: KL6, SP-D and IL-8. Greater IL-8 levels were associated with SSc-ILD in both peripheral blood and BALF, overall SMD 0.88 (95% CI 0.61 to 1.15; I=1%). Greater levels of SP-D and KL-6 were both estimated in SSc-ILD peripheral blood compared with healthy controls, at an SMD of 1.78 (95% CI 1.50 to 2.17; I=8%) and 1.66 (95% CI 1.17 to 2.14; I=76%), respectively.
CONCLUSION
We provide robust evidence that KL-6, SP-D and IL-8 have the potential to serve as reliable biomarkers in blood/BALF for supporting the diagnosis of SSc-ILD. However, while several other biomarkers have been proposed, the evidence of their independent value in diagnosis and prognosis is currently lacking and needs further investigation.
PROSPERO REGISTRATION NUMBER
CRD42021282452.
Topics: Adult; Humans; Lung Diseases, Interstitial; Interleukin-8; Pulmonary Surfactant-Associated Protein D; Scleroderma, Systemic; Biomarkers; Lung
PubMed: 36261273
DOI: 10.1136/thorax-2022-219226 -
Journal of Scleroderma and Related... Oct 2022Severe digital ischemia, including digital ulcers and gangrene, is considered rare in patients with antisynthetase antibodies. This study aimed to elucidate the clinical...
OBJECTIVE
Severe digital ischemia, including digital ulcers and gangrene, is considered rare in patients with antisynthetase antibodies. This study aimed to elucidate the clinical features of antisynthetase-positive patients complicated with digital ulcers and/or gangrene using a systematic literature review and case series in a single-center cohort.
METHODS
A systematic literature review was conducted to identify reports describing antisynthetase-positive cases with digital ulcers and/or gangrene. Our cohort of consecutive patients with antisynthetase antibodies was stratified by the history of severe digital ischemia. Demographic and clinical features and outcomes in patients with severe digital ischemia identified in the systematic literature review and our cohort were compared with those in patients without severe digital ischemia in our cohort.
RESULTS
The systematic literature review revealed 12 antisynthetase-positive patients with severe digital ischemia from one case series and eight case reports. Seven (7%) of 100 patients with antisynthetase antibodies in our cohort had a record of severe digital ischemia. Severe digital ischemia was often found at presentation and was associated with the classification of systemic sclerosis with or without myositis overlap. Clinical features associated with severe digital ischemia in antisynthetase-positive patients included Raynaud's phenomenon ( < 0.001), digital pitting scars ( = 0.001), and nailfold capillary abnormality ( = 0.02). Outcomes of severe digital ischemia were generally favorable with vasodilators.
CONCLUSION
Severe digital ischemia is an overlooked complication in antisynthetase-positive patients. Antisynthetase antibodies should be measured in patients presenting with digital ulcers or gangrene, especially in those with systemic sclerosis phenotype and features associated with antisynthetase antibodies in the absence of systemic sclerosis-specific autoantibodies.
PubMed: 36211206
DOI: 10.1177/23971983221090857