Presence Of Systemic Sclerosis Research Articles

Organ changes after SARS-CoV-2 infection in a patient with systemic sclerosis according to autopsy

The article presents a description of a clinical case with a fatal outcome due to SARS-CoV-2 coronavirus-induced bilateral viral pneumonia with areas of pneumofibrosis, complicated by acute respiratory failure. The presence of systemic sclerosis in this patient aggravated the course of the disease and became one of the causes of death. Autopsy revealed signs of bilateral pneumonia with lesions in both lungs and areas of pneumofibrosis.Histological examination revealed alveoli with rupture of interalveolar septa, areas of atelectasis, serous-purulent exudate with desquamated alveolocytes, places with organization of exudate, zones of pneumofibrosis. Scleroderma cardiosclerosis, linear necrosis of cardiomyocytes were present in the heart. There were signs of multiple organ failure – pulmonary edema, cerebral edema.

Detection of systemic sclerosis in patients with pulmonary hypertension

Introduction. The problem of early detection of pulmonary arterial hypertension (PAH) is closely related to the diagnosis of systemic sclerosis (SSc). It is especially important in patients with slight manifestations of the disease, making it diffcult to verify the diagnosis before the clinic of right ventricular heart failure, and low knowledge of the general practitioners of the available diagnostic opportunities.The aim of the work was to identify the earliest clinical signs of PAH associated with SSc for its early diagnosis.Material and methods. The study included 51 patients with limited SSc. The investigators evaluated the extent of skin lesion, levels of the level of N­terminal brain natriureticpropeptide (NT­proBNP) and uric acid, the fluorescent pattern and titer of antinuclear factor. All patients underwent an echocardiography, nailfold videocapillaroscopy, a comprehensive assessment of external respiratory functions. When there were symptoms of PAH, right heart catheterization was performed to confrm it.Results. PAH was detected in 19 of the 51 patients included in the study. Patients with PAH were signifcantly older than those without PAH: (60±8) and (54±7) years, respectively (p=0.02); there were also differences between these groups in the signs of right cardiac remodeling (p<0.05), in the indices estimated in the six­minute walk test (p<0.05), in the assessment of lung diffusing capacity (p<0.001), in levels of NT­proBNP and uric acid (p<0.001), in the density of the nailfold capillaries (p=0.009); in a semiquantitative assessment of nailfold capillary alterations (p=0.022). Among patients with PAH associated with SSc, seven patients were diagnosed with SSc for the frst time after referring to cardiologists and verifying the genesis of PAH, while other 12 patients were previously observed by rheumatologists. Differences in the duration of the period from the onset of symptoms to the diagnosis of SSc (p=0.043) were revealed between these groups.Conclusion. The study shows the probability of the presence of SSc in patients with suspected idiopathic PAH, that determines the necessity of additional immunological and instrumental examinations in such patients.

Nailfold Capillaroscopy Characteristics of Antisynthetase Syndrome and Possible Clinical Associations: Results of a Multicenter International Study.

To describe nailfold videocapillaroscopy (NVC) features of patients with antisynthetase syndrome (AS) and to investigate possible correlations with clinical and serological features of the disease. We retrospectively analyzed NVC images of 190 patients with AS [females/males 3.63, mean age 49.7 ± 12.8 yrs, median disease duration 53.7 mos (interquartile range 82), 133 anti-Jo1 and 57 non-anti-Jo1-positive patients]. For each patient, we examined number of capillaries, giant capillaries, microhemorrhages, avascular areas, ramified capillaries, and the presence of systemic sclerosis (SSc)-like pattern. Finally, we correlated NVC features with clinical and serological findings of patients with AS. Concomitantly, a historical cohort of 75 patients with antinuclear antibody-negative primary Raynaud phenomenon (RP) and longterm followup was used as a control group (female/male ratio 4.13/1, mean age 53.9 ± 17.6 yrs) for NVC measures. NVC abnormalities were observed in 62.1% of AS patients compared with 29.3% of primary RP group (p < 0.001). An SSc-like pattern was detected in 67 patients (35.3%) and it was associated with anti-Jo1 antibodies (p = 0.002) and also with a longer disease duration (p = 0.004). Interestingly, there was no significant correlation between the presence of SSc-like pattern and RP, and only 47% of patients with SSc-like pattern had RP. NVC abnormalities are commonly observed in AS, independently from the occurrence of RP. The presence of an SSc-like pattern could allow identification of a more defined AS subtype, and prospective studies could confirm the association with clinical and serological features of AS.

Increased Epicardial Fat Volume Is Independently Associated with the Presence and Severity of Systemic Sclerosis

The study aimed to determine if intrathoracic fat volumes are associated with the presence and severity of systemic sclerosis (SSc), defined by the presence of pulmonary arterial hypertension (PAH). A total of 265 patients were included in the study, 202 of whom had SSc (134 had SSc with no PAH and 68 had SSc-associated PAH) and who underwent high-resolution computed tomography, and 63 controls who underwent coronary computed tomography angiography with calcium scoring. Intrathoracic and epicardial (EFV) fat volumes were quantified by manual tracing of the mediastinum and the pericardium, the difference of which represents the extrapericardial fat volume. Associations between these three fat volumes and the presence and severity of SSc, adjusted for cardiovascular risk factors and interstitial lung disease, were evaluated by logistic regression analysis. Of the 202 patients with SSc, the mean age was 55 years (ranged from 20 to 86), and 79% (159 of 202) were women. Adjusted EFV (odds ratio [OR]: 1.065; 95% confidence interval [CI]: 1.046-1.084, P = < 0.0001), extrapericardial fat volume (OR: 1.028, 95% CI: 1.017-1.038, P = < 0.0001), and intrathoracic fat volume (OR: 1.033, 95% CI: 1.023-1.043, P = 0.001) were associated with the presence of SSc. Only EFV was associated with SSc severity (adjusted OR: 1.010, 95% CI: 1.003-1.018, P = 0.007). Increased epicardial fat volume is associated with the presence and severity of SSc, independent of cardiovascular risk factors and interstitial lung disease.

AB0812 Usefulness of mouth handicap in systemic sclerosis scale in systemic sclerosis spanish patients

BackgroundThe Mouth Handicap in Systemic Sclerosis Scale (MHISS) is a 12-item questionnaire proposed and validated by Mouthon L et al (Ann Rheum Dis 2007; 66: 1654-1655) which assesses mouth disability...

Toll-like receptor-mediated, enhanced production of profibrotic TIMP-1 in monocytes from patients with systemic sclerosis: role of serum factors

ObjectivesTo investigate whether monocytes contribute to matrix deposition in systemic sclerosis (SSc) by production of tissue-inhibitor of metalloproteinase-1 (TIMP-1).MethodsMatrix metalloproteinase-1 (MMP-1) and TIMP-1 expression and secretion were measured by qRT-PCR...

Primary Biliary Cirrhosis Associated with Systemic Sclerosis: Diagnostic and Clinical Challenges

Patients with primary biliary cirrhosis (PBC) often have concurrent limited systemic sclerosis (SSc). Conversely, up to one-fourth of SSc patients are positive for PBC-specific antimitochondrial antibodies (AMA). The mechanisms responsible for the co-occurrence of these diseases are largely unknown. Genetic, epigenetic, environmental, and infectious factors appear to be important for the pathogenesis of the disease, but the hierarchy of events are not well defined. Patients with SSc and PBC have an increased morbidity and mortality compared with the general population, but whether the presence of both diseases in an affected individual worsens the prognosis and/or outcome of either disease is not clear. Some case reports suggested that the presence of SSc in PBC patents is associated with a more favorable prognosis of the liver disease, whereas others report an increased mortality in patients with PBC and SSc compared to patients with PBC alone. This paper discusses the features of patients with PBC-associated SSc. Our aims are to clarify some of the pathogenetic, diagnostic, and clinical challenges that are currently faced in the routine management of these patients. We also intend to provide some practical hints for practitioners that will assist in the early identification of patients with PBC-associated SSc.

Systemic sclerosis and bone loss: the role of the disease and body composition

Objectives: Studies on body composition are not available in systemic sclerosis (SSc). As this variable may play an important role in bone loss we have analysed bone mineral density (BMD) and body composition in SSc patients and healthy controls.Methods: Forty‐three postmenopausal SSc patients and 47 healthy postmenopausal women were studied. Patients with intestinal malabsorption, renal failure, current or past history of smoking or using osteopenic drugs were excluded. BMD and body composition was evaluated by dual X‐ray absorptiometry (DXA).Results: A higher frequency of osteoporosis in the lumbar spine (32.5%) and femoral neck (51.1%) was observed in SSc patients when compared to controls (14.8% vs. 19.1%; p<0.01). Multiple linear regression analysis revealed an association between the presence of SSc and low BMD. Body composition showed a reduced lean mass (33.15 vs. 39.99 g; p<0.01) and fat mass (21.05 vs. 26.82 g; p<0.01) in SSc when compared to controls. Lean mass was an important factor related to BMD in the lumbar spine and femoral neck.Conclusion: SSc may be an independent factor for low BMD. The low lean mass in these patients emphasizes the need for appropriate additional therapeutic measures to reduce bone loss in SSc patients.

Systemic sclerosis could mask the presentation of psoriasis in a patient with symptomatic and bilateral sacroiliitis

Psoriasis is rarely associated with systemic sclerosis (SSc), and sacroiliitis associated to Connective Tissue Diseases is also rather rare. In this report we describe a case of a young woman with SSc who developed symptomatic and bilateral sacroiliitis. The clinical pattern of sacroiliac involvement of this patient resembles that of psoriatic sacroiliitis. HLA typing was compatible with both SSc and psoriasis. Psoriatic sacroiliitis could not be diagnosed, but, on the basis of these observations, the patient described could represent a case of PsA without psoriasis, and psoriatic cutaneous involvement masked by the presence of SSc.