Involvement In Connective Tissue Disease Research Articles

E012 Burden and risk factors for cardiovascular involvement in connective tissue disease

Abstract Background/Aims Connective tissue diseases (CTD) are associated with several cardiovascular manifestations. Cardiovascular diseases (CVD) are significant cause of morbidity and premature death. The pathogenesis of CVD in CTD patients is incompletely understood and likely multifactorial. Traditional risk factors for atherosclerosis are common among patients with CTD as well as metabolic syndrome. Furthermore, diabetes, dyslipidaemia, hypertension, and obesity may all be exacerbated by connective tissue disease itself or treatment with glucocorticoids. The aim of this study was to assess the burden of CVD and the risk factors for the development of cardiovascular disease in patients with CTD. Methods This was a retrospective study from a single centre. We reviewed clinical records of CTD patients as determined by treating rheumatologist. This CTD cohort included patients with systemic lupus erythematosus (SLE), myositis, systemic sclerosis, Sjogren’s syndrome, mixed and undifferentiated connective tissue diseases. Data on demographic, clinical, laboratory parameters, cardiac investigations, treatment and outcome were collected from electronic patient records. CVD and traditional risk factors were defined using standard criteria. Results A total of 298 patients with CTD were included in the analysis. Mean age of the cohort was 61±17.7 years and majority were females (n = 240, 80.5%). Of the 298 patients, 64 had CVD (21.5%). Most common CVD was ischaemic heart disease (17 patients) followed by hypertension (12), arrhythmia (9), pulmonary arterial hypertension (7), valvular diseases (5), pericardial disease (3), cardiomyopathy (3), myocarditis (2), thromboembolism (3), aortic dissection (2) and coronary spasm (1). Patient with CTD who had CVD were older (72.5±12.2 years) compared to CTD patients who did not develop CVD (non-CVD group, 57.7±17.6 years). The duration of CTD was shorter in patients with CVD (88.9±41.7 months), compared to 106±84.3 months in non-CVD group. The risk factors such as smoking, diabetes and dyslipidaemia was higher in the CVD group. Comparing CVD vs non-CVD patients respectively, systolic BP (147.1±23.0 vs 133.6±18.3mmHg), diastolic BP (83.2 ± 16.3 vs 78.2 ± 11.2mmHg) and serum uric acid (321.8±97.4 vs 292±83.9mg/dL) values were higher and statistically significant (p < 0.05) in CVD group. Patients with CVD had longer exposure to steroids (50%) compared to non-CVD patients (31.6%). They required high dose steroids (9.4% vs 5.6% in non-CVD patients). Current and previous DMARD use was also higher in patients who had CVD (51.6% vs 44.9%). Conclusion This study has demonstrated high burden of cardiovascular disease in CTD patients. Traditional risk factors were more prevalent in the CVD group. DMARD’s and steroid use was also higher in the CVD group. Screening for cardiovascular diseases and disease control in CTD patients is paramount for better outcomes. Further analysis will be carried out for risk factors and relevant outcomes. Disclosure K.K. Garg: None. S. Sampath: None. A. Erinjogunola: None. B. Ting: None. H. Noori: None. G.M. Koduri: None.

Nail involvement in connective tissue diseases: an epidemiological, clinical, and dermoscopic study.

The assessment of nail changes in connective tissue diseases (CTD) has been rarely explored in previous studies. The use of dermoscopy to study vascular changes in nailfolds is an interesting diagnostic technique. The aim of the study was to describe the epidemiological, clinical, and dermoscopic features of nail lesions in CTD. A prospective study was performed at the Dermatology Department of Habib Thameur Hospital (Tunis, Tunisia) in collaboration with the Internal Medicine Department over a period of 15 months, from July 2020 to September 2021, including patients diagnosed with systemic sclerosis (SS), systemic lupus erythematosus (SLE) and dermatomyositis (DM). Our study included 48 patients. Nail involvement was found in 44 cases. Dermoscopic nailfold abnormalities were identified in 37 cases. The most common clinical features were ragged cuticle, nailfold erythema, and onycholysis. Additionally, splinter hemorrhage, longitudinal ridging, lunula abnormalities, melanonychia, trachyonychia, leukonychia, increase in transverse curvature, parrot beak nail, half and half nails, and onychorrhexis were described. Nailfold dermoscopy showed a normal pattern in 10 cases, a nonspecific pattern in nine cases (SLE), and a scleroderma pattern in 29 cases (SS and DM). The scleroderma pattern was further categorized into an early pattern (6), an active pattern (14), and a late pattern (9). Normal pattern was observed solely in patients in remission. The late scleroderma pattern was associated with disease duration and systemic involvement. In SLE, disease activity correlated with onycholysis, nailfold erythema, and pathologic pattern in dermoscopy. However, patients with DM displayed a positive correlation between pulmonary involvement and scleroderma pattern. Nail involvement in CTD includes a diverse range of abnormalities. Despite being nonspecific, it can provide crucial clues for establishing a diagnosis. Nailfold dermoscopy serves as a mirror for microangiopathy, enabling the detection of changes at an initial stage, and thus, it becomes a diagnostic and prognostic tool.

RENAL INVOLVEMENT IN PATIENTS WITH RHEUMATOID ARTHRITIS- A HOSPITAL-BASED OBSERVATIONAL STUDY.

Background: Rheumatoid arthritis is a chronic multisystem, immuno-inammatory disease characterized by articular and extra-articular manifestations. The disease's hallmark is synovial inammation and potential to cause cartilage damage, bone erosion, and subsequent damage to joint integrity. Rheumatoid arthritis is a systemic disease with a variety of extra-articular manifestations. The predominant well-recognized systemic manifestations include Sjogren's syndrome, pulmonary, cardiac, neurological, renal, gastrointestinal, dermal involvement, vasculitis, and Felty's syndrome. Regarding renal involvement in connective tissue disease, the focus is on systemic lupus erythematosus (SLE). The affection of kidneys in RA is more or less overlooked and mainly attributed to drugs (NSAIDs, DMARDs like gold and d-penicillamine, which are less commonly used in today's scenario. The other primary etiology of kidney involvement in RA is secondary amyloidosis, commonly associated with the long-term disease process. Vasculitis with RA is a relatively less common etiology of renal involvement in RA. Renal involvement in Rheumatoid arthritis has been well documented with a prevalence of 1-5%. Although impairment of renal function is often mild to moderate, renalrelated mortality signicantly contributes to the increased mortality of RA. The kidney is involved in RA with both glomerular and tubular damage. Renal damage in RA, however, is usually asymptomatic and is detected on laboratory investigation. It is often difcult to differentiate between damage due to disease activity and drugs used to treat RA. It is a hospita Methods: l based observational study conducted at Gauhati Medical college, Assam, for one year. All Adult (> 18 years) patients fullled the 2010 American College of Rheumatology Criteria for Rheumatoid arthritis were included. Patients with diabetes mellitus congestive heart failure, urinary tract infection, long standing and uncontrolled hypertension, preexisting renal disease , overlap syndrome and pregnant patients were excluded. Detailed clinical history was taken from all included patients regarding onset and duration of disease. Disease activity was measured using disease activity score -28 -(DAS-28). Routine investigation, ESR, CRP, renal function test, ultrasonography and urine for microalbuminuria were measured in all included patients. The maximum age was Results: 68 years, the minimum was 20 years, and the mean age was 40.22 ± 11.58. The study group included 38 females and 12 males accounting for 76% and 24%, respectively. The mean disease duration was 18.6 months with a range of 3-60 months. All patients presented with polyarthritis(100%). History of morning stiffness was present in 54% of patients, while 46% had generalized weakness and constitutional symptoms. In our study, microalbuminuria was found in 15 patients (30%). Mmicroalbuminuria was seen more commonly in the age group between 41-50 years. Microalbuminuria was signicantly associated with higher ESR values. Out of 15 patients positive for microalbuminuria, 13 patients had ESR >50, and 7 had ESR >100 with a p-value <0.001. Mean CRP was 42.45 mg/l in microalbuminuria-positive patients as compared to 16.19mg/l in microalbuminuria-negative patient. Out of 15 patients with microalbuminuria positive had evidence of erosion on x-ray, with a p-value of 0.017. Conclusion: From the present study, it can be concluded that the peak age of the disease is 31-50 years, with female preponderance. Polyarthritis is the most common presenting symptom, and most patients have an insidious onset. Constitutional symptoms like fever, malaise, and anorexia are more common among the extra-articular features. Asymptomatic renal involvement is common, and microalbuminuria is frequently seen in patients with Rheumatoid Arthritis. The presence of microalbuminuria is a sensitive indicator of increased renal vascular permeability in rheumatoid arthritis patients. Immunological methods for detecting microalbuminuria should be routinely used in all Rheumatoid Arthritis patients to detect renal involvement in its initial phase to devise the most appropriate treatment.

Quantitative assessment of left ventricular myocardial involvement in patients with connective tissue disease: a 3.0T contrast-enhanced cardiovascular magnetic resonance study

The aim of this study was to evaluate left ventricular (LV) myocardial involvement in connective tissue disease (CTD) patients using multiparemetric imaging derived from cardiovascular magnetic resonance (CMR). CMR was performed on 146 CTD patients (comprising of 74 with idiopathic inflammatory myopathy (IIM) and 72 with non-IIM) and 72 healthy controls and included measures of LV global strains [including peak strain (PS), peak systolic (PSSR) and diastolic strain rate (PDSR)], myocardial perfusion [including upslope, max signal intensity (MaxSI), and time to maximum signal intensity (TTM)], and late gadolinium enhancement (LGE) parameters. Univariable and multivariable linear regression analyses were performed to determine the association between LV deformation and microvascular perfusion, as well as LGE. Our results indicated that CTD patients had decreased global longitudinal PS (GLPS), PSSR, PDSR, and myocardial perfusion (all p < 0.017) compared with normal controls. Non-IIM patients exhibited lower LV global strain and longer TTM than IIM patients. The presence of LGE was independently associated with global radial PS (GRPS: β = − 0.165, p = 0.011) and global circumferential PS (GCPS: β = − 0.122, p = 0.022). TTM was independently correlated with GLPS (β = − 0.156, p = 0.027). GLPS was the best indicator for differentiating CTD patients from normal controls (area under curve of 0.78). This study indicated that CTD patients showed impaired LV global myocardial deformation and microvascular perfusion, and presence of LGE. Cardiac involvement might be more severe in non-IIM patients than in IIM patients. Impaired microvascular perfusion and the presence of LGE were independently associated with LV global deformation.

Pulmonary involvement in connective tissue disease due to Coronavirus 19: a case report

Coronavirus disease (COVID-19) is a disease caused by the new coronavirus called severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) emerging in Wuhan, China's Hubei province. The epidemic spread exponentially across the world and became a pandemic [1-3]. Fever, dry cough, headache, and shortness of breath all typical symptoms, and can lead to death with acute respiratory distress syndrome. Diabetes mellitus, hypertension, coronary heart disease, chronic obstructive pulmonary disease and chronic kidney disease tend to be major risk factors for mortality [4]. Real-time fluorescence reverse transcription- polymerase chain reaction (RT-PCR) is the preferred initial and reference diagnostic test for COVID-19. Chest computed tomography (CT) has gained importance due to the long result time and false negativity of the RT-PCR test [5, 6]. Typical imaging findings for COVID-19 pneumonia are peripheral, bilateral, ground glass opacities with or without consolidation, and the differential diagnosis comprises influenza pneumonia, organizing pneumonia, drug toxicity, and connective tissue disease [7].&#x0D; Due to immune dysfunction, steroid usage and immunotherapy, connective tissue diseases are vulnerable to viral infections. Few studies have identified the presence of concurrent COVID-19 in patients with connective tissue diseases [8, 9]. In this article, we aimed to present the chest CT findings of a patient with connective tissue diseases who received immunosuppressed therapy

The Role of Optical Coherence Tomography in Differential Diagnosis of Multiple Sclerosis and Autoimmune Connective Tissue Diseases with CNS Involvement.

The purpose of this study was to examine whether application of optical coherence tomography (OCT) measurements can provide a useful biomarker for distinguishing central nervous system (CNS) involvement in autoimmune connective tissue diseases (CTD) from multiple sclerosis (MS). An observational study included non-optic neuritis eyes of 121 individuals: 59 patients with MS, 30 patients with CNS involvement in CTD, and 32 healthy controls. OCT examination was performed in all subjects to measure retinal nerve fiber layer (RNFL) thickness, ganglion cell complex (GCC) thickness, ganglion cell layer-inner plexiform layer (GCIPL) thickness, and volume of the macula. There was a significant group effect with regard to superior optic disc RNFL, macular RNFL, GCC, and GCIPL thickness, and macular volume. Post-hoc analysis revealed that MS patients have significantly smaller macular volume and thinner superior optic disc RNFL, macular RNFL, GCC, and GCIPL compared to healthy controls. CTD patients have significantly smaller superior optic disc RNFL, GCIPL, and GCC thickness compared to healthy controls. However, no significant group differences were observed between the patient groups (MS vs. CTD) on any outcome. Although a prominent retinal thinning may be a useful biomarker in MS patients, in a general population of individuals with a confirmed CNS involvement the use of OCT is not specific enough to discriminate between MS and autoimmune CTD.

Diagnosing renal involvement in connective tissue disease: interpretation of anti-nuclear autoantibody tests.

Diagnosing renal involvement in connective tissue disease: interpretation of anti-nuclear autoantibody tests.

Histopathology of the muscle in rheumatic diseases

The presence of muscular symptoms is common in rheumatological clinical practice, but often the differential diagnosis between muscular involvement in connective tissue diseases, vasculitis and drug-induced myopathy may be difficult. In addition to clinical assessment, laboratory analysis and instrumental examinations, muscle biopsy may help to clarify the diagnosis in patients with muscular involvement. The purpose of this review is to provide a critical analysis of the current medical literature on muscular histopathology, to help clinicians to identify when to perform muscular biopsy and to provide a practical guide to a better understanding of the pathology report. Moreover, we provide an overview of the muscular involvement and the most common histopathological findings in rheumatic diseases.

Renal Involvement in Connective Tissue Diseases

Renal involvement is common and heterogenous in connective tissue diseases and has a main influence on prognosis and mortality. In systemic lupus erythematosus proliferative glomerulonephritis is the most common manifestation, while in primary Sjogren's syndrome interstitial nephritis with tubular dysfunction is the predominant pathological feature. In systemic sclerosis the most serious renal manifestation is scleroderma renal crisis characterized by abrupt onset of hypertension and acute kidney injury associated with an increase in plasma renin activity. Risk factors for scleroderma renal crisis are diffuse cutaneous involvement, treatment with corticosteroids > 15 mg prednisolone/day and treatment with calcineurin inhibitors.Regular measurement of urine sediment, proteinuria-to-urine creatinine ratio, tubular proteinuria, measurement of plasma creatinine, and office as well as home blood pressure monitoring are strongly recommended. Diagnostic kidney biopsy is essential in differentiating the different types of lupus nephritis and renal involvement in sjogren's syndrome.The optimal treatment of lupus nephritis varies with the classification of the morphological findings present on renal biopsy. The treatment of interstitial nephritis in sjogren's syndrome consists of immunosuppression e. g. corticosteroids. Renal tubular acidosis should be corrected by sodium alkali and potassium alkali. Angiotensin-converting enzyme inhibitors play a major role in the treatment of scleroderma renal crisis, they should be continued also in patients progressing to end-stage renal disease.

17. How to Look at Renal Involvement in Connective Tissue Disease and Systemic Vasculitis

17. How to Look at Renal Involvement in Connective Tissue Disease and Systemic Vasculitis

17. How to Look at Renal Involvement in Connective Tissue Disease and Systemic Vasculitis

17. How to Look at Renal Involvement in Connective Tissue Disease and Systemic Vasculitis

Clinical Study of Cardiovascular Involvement in Patients with Connective Tissue Diseases

Objective: The aim of this study was to evaluate cardiovascular involvement in patients with connective tissue diseases. Methods: A total of 99 patients (mean age 36.6 ± 10.7 years) with connective tissue diseases were included in the study. Patients were assessed based on their sex, age, disease duration, cardiovascular symptoms, erythrocyte sedimentation rate, antinuclear antibody, and anti-double stranded DNA antibodies. Moreover, cardiac investigations including electrocardiography (ECG), chest X-ray and echocardiography were performed. Results: The study included 90 (90.9%) females and 9 (9.09%) males, aged between 18 and 64 years. Chest pain, palpitation, dyspnoea on exertion, syncope, fatigue, and cardiac murmur were the most cardiac symptoms and signs of patients. ECG abnormalities associated with sinus bradycardia, sinus tachycardia, ST interval and T waves, were found in 27% patients. Chest X-ray with cardiomegaly was found in 10 (10.1%) patients. Echocardiography abnormalities were observed in 32% of patients with pericardial involvement, mitral regurgitation, and diastolic dysfunction. Moreover, the incidence of pericardial effusion was found to be significantly (p higher in patients with connective tissue diseases. Conclusion: The study indicates the correlation between disease activity, disease duration and abnormal echocardiography of cardiovascular involvement in connective tissue diseases.

Assessment of cardiovascular involvement in Connective Tissue Disease: Let’s open Pandora’s box

Connective Tissue Disease (CTD) is characterized by autoimmune activation and systemic inflammatory response, affecting several internal organs. Cardiac involvement represents one of the most severe complications associated with higher rates of death and reduced life expectancy in this population. Cardiovascular abnormalities occurring in CTD encompass different aspects of cardiac disease, such as pericarditis, myocarditis, coronary artery disease, heart failure due to systolic and diastolic dysfunction and valvular disease. Considering the increased risk of cardiovascular disease (CVD) in CTD, early assessment and diagnosis of cardiovascular involvement is of utmost importance. However, CVD in CTD has often an atypical clinical presentation, and traditional diagnostic approaches such as transthoracic echocardiography have low sensitivity in the early stages. Cardiac magnetic resonance imaging can identify subtle morphological changes of the myocardium and contribute to earlier diagnosis of CVD in patients with CTD.

Ventricular Tachycardia and Sudden Cardiac Death in Connective Tissue Diseases: Can Cardiovascular Magnetic Resonance Play a Role?

Ventricular tachycardia (VT) and sudden cardiac death (SCD) are deleterious manifestations of cardiac involvement in connective tissue diseases (CTD). In rheumatoid arthritis (RA), the commonest cause of SCD is coronary artery disease that may lead to acute coronary syndrome and VT. In systemic lupus erythematosus (SLE), VT is due either to coronary artery disease or to acute myocarditis. VT/SCD can be also assessed in polymyostis (PM) and dermatomyositis (DM). Finally, VT was described in 7-13%, while SCD in 5-21% of unselected SSc patients. Cardiovascular magnetic resonance (CMR) has been already used as screening tool in CTDs, because of its capability to evaluate noninvasively and without radiation, function, inflammation, perfusion defects and fibrosis. Apart of the impaired ventricular function, which can be by itself a predisposing factor for VT/SCD, the commonest causes that can potentially lead to VT/SCD in CTDs are myocarditis, coronary artery disease with ischemic type fibrosis, microvascular disease, nonischemic type fibrosis and heart failure. The detection of perfusion defects by CMR can be served as a tool to select those patients, who should undergo x ray coronary angiography and/or angioplasty. The location and extent of myocardial scar can guide further electrophysiologic study and/or intervention. On the other hand, in those with myocarditis, microvascular disease and/or heart failure, a modification in both cardiac and rheumatic medication may be recommended after evaluation of CMR findings. CMR in CTDs with VT/SCD may guide risk stratification, rheumatic and cardiac therapeutic decision making and therefore it should be included as a reliable adjunct in the evaluation of these patients.

Abnormalities in lung volumes and airflow in children with newly diagnosed connective tissue disease.

Connective tissue diseases (CTDs) of childhood are rare inflammatory disorders, involving various organs and tissues including respiratory system. Pulmonary involvement in patients with CTDs is uncommon but may cause functional impairment. Data on prevalence and type of lung function abnormalities in children with CTDs are scarce. Thus, the aim of this study was to asses pulmonary functional status in children with newly diagnosed CTD and follow the results after two years of the disease course. There were 98 children (mean age: 13 ± 3; 76 girls), treated in Department of Pediatric Rheumatology, Institute of Rheumatology, Warsaw and 80 aged-matched, healthy controls (mean age 12.7 ± 2.4; 50 girls) included into the study. Study procedures included medical history, physical examination, chest radiograph and PFT (spirometry and whole body-plethysmography). Then, the assessment of PFT was performed after 24 months. FEV₁, FEV₁/FVC and MEF50 were significantly lower in CTD as compared to control group, there was no difference in FVC and TLC. The proportion of patients with abnormal lung function was significantly higher in the study group, 41 (42%) vs 9 (11%). 24-months observation didn't reveal progression in lung function impairment. Lung function impairment is relatively common in children with CTDs. Although restrictive ventilatory pattern is considered typical feature of lung involvement in CTDs, airflow limitation could also be an initial abnormality.

Cardiovascular Involvement in Connective Tissue Disease: The Role of Interstitial Lung Disease

ObjectiveThe aim of this study was to assess cardiovascular involvement in patients with connective tissue disease (CTD), and determine whether interstitial lung disease (ILD) in these patients is associated with elevated cardiovascular risk.MethodsThis study evaluated a retrospective cohort of 436 CTD patients admitted to a large teaching hospital in Zhejiang province, China, along with an additional 436 participants of an annual community health screening conducted in the physical examination center who served as age- and gender-matched controls. Demographic, clinical, serologic and imaging characteristics, as well as medications used by each participant were recorded. Cardiovascular involvement was defined by uniform criteria. Correlations between clinical/serologic factors and cardiovascular involvement were determined by univariate and multivariate analyses.ResultsCTD patients had a significantly higher cardiovascular involvement rate than controls (64.7% vs 23.4%), with higher rates of diabetes, hypertension, and hyperlipidemia, elevated systolic and diastolic pressures, C-reactive protein, total cholesterol, and low-density lipoprotein cholesterol, and lower albumin and high-density lipoprotein cholesterol (all p < 0.05). Furthermore, CTP patients with cardiovascular involvement were significantly older, had higher systolic and diastolic pressures, C-reactive protein, glucose, and uric acid, higher rates of diabetes, hypertension, and use of moderate- to high-dose glucocorticoids, and longer disease duration compared to patients without involvement (all p < 0.05). Moreover, CTD in patients with cardiovascular involvement was more likely to be complicated by ILD (p < 0.01), which manifested as a higher alveolar inflammation score (p < 0.05). In the multivariate analysis, cardiovascular involvement in CTD patients was associated with age, systolic pressure, body mass index, uric acid, disease duration > 2 years, use of moderate- to high-dose glucocorticoids, and ILD with a high alveolar inflammation score.ConclusionCardiovascular involvement is increased in CTD patients, and is associated with ILD with a higher alveolar inflammation score. Thus, early-stage echocardiography and CT scans should be used to detect potential cardiovascular complications in these patients.

THORACIC INVOLVEMENT IN CONNECTIVE TISSUE DISEASES: RADIOLOGICAL PATTERNS AND FOLLOW-UP.

Connective tissue diseases (CTDs) are a heterogeneous group of idiopathic inflammatory diseases involving various organs. A thoracic involvement is frequent, and chest-CT represents the imaging technique of reference in its assessment. Pulmonary abnormalities related to CTDs are various; although several disease-specific aspects have been described, the two most clinically relevant complications are represented by interstitial lung disease and pulmonary arterial hypertension. The early identification of a thoracic involvement, with the adoption of specific therapies, can significantly change patient's prognosis. The aim of this article is to review the most common typical and atypical CT features of thoracic involvement occurring in CT, especially focusing on interstitial lung disease.

AB0922 Pulmonary Involvement in Connective Tissue Diseases - A Retrospective Analysis of A Multidisciplinary Approach

BackgroundThe lung is a frequent target of connective tissue diseases (CTDs) and all components of the respiratory system are at risk. Interstitial lung disease (ILD) is frequently seen in CTDs,...

Cardiac Tissue Characterization and the Diagnostic Value of Cardiovascular Magnetic Resonance in Systemic Connective Tissue Diseases

Accurate diagnosis of cardiovascular involvement in connective tissue diseases (CTDs) remains challenging. We hypothesized that cardiovascular magnetic resonance (CMR) demonstrates cardiac lesions in symptomatic CTD patients with normal echocardiography. CMR from 246 CTD patients with typical cardiac symptoms (TCS; n = 146, group A) or atypical cardiac symptoms (ATCS; n = 100, group B) was retrospectively evaluated. Group A included 9 patients with inflammatory myopathy (IM), 35 with sarcoidosis, 30 with systemic sclerosis (SSc), 14 with systemic lupus erythematosus (SLE), 10 with rheumatoid arthritis (RA), and 48 with small vessel vasculitis. Group B included 25 patients with RA, 20 with SLE, 20 with sarcoidosis, 15 with SSc, 10 with IM, and 10 with small vessel vasculitis. CMR was performed by 1.5T; left ventricular ejection fraction, T2 ratio (edema imaging), and late gadolinium enhancement (LGE; fibrosis imaging) were evaluated. Acute and chronic lesions were characterized as LGE positive plus T2 ratio >2 and T2 ratio ≤2, respectively. According to LGE, lesions were characterized as diffuse subendocardial, subepicardial, and subendocardial/transmural due to vasculitis, myocarditis, and myocardial infarction, respectively. A stress study by dobutamine echocardiography or stress, nuclear, or adenosine CMR was performed in CTD patients with negative rest CMR. Abnormal CMR was identified in 32% (27% chronic) and 15% (12% chronic) of patients with TCS and ATCS, respectively. Lesions due to vasculitis, myocarditis, and myocardial infarction were evident in 27.4%, 62.6%, and 9.6% of CTD patients, respectively. Stress studies in CTD patients with negative CMR revealed coronary artery disease in 20%. CMR in symptomatic CTD patients with normal echocardiography can assess disease acuity and identify vasculitis, myocarditis, and myocardial infarction.

FRI0099 Pulmonary fibrosis and connective tissue diseases: Follow-up of lung involvement in rheumatoid arthritis and systemic sclerosis

BackgroundLung involvement in connective tissue diseases (CTDs) is a significant cause of morbidity and mortality. The most frequent pulmonary component affected is the interstistium. The most common histopathologic pattern of...