Positive Scl-70 Research Articles

Peripheral and ocular microvascular alterations in systemic sclerosis: observations from capillaroscopic assessments, perfusion peripheral analysis, and optical coherence tomography angiography.

To investigate the correlations between finger microvascular morphology and function in patients with systemic sclerosis (SSc) and the status of ocular microcirculation, as detected by nailfold videocapillaroscopy (NVC), laser speckle contrast analysis (LASCA), and optical coherence tomography angiography (OCTA). The enrollment included 32 SSc patients, classified according to the 2013 ACR/EULAR criteria, and 27 sex- and age-matched healthy controls. The participants underwent comprehensive rheumatological and ophthalmological examinations, as well as NVC, LASCA, and OCTA analysis on the same day at a single center from March to October 2022. SSc patients receiving intravenous prostanoids cycles were assessed at least 1month after infusion. Statistical analysis was conducted using Stata® 15.1. Significant direct correlations were observed between the mean capillary number (at NVC) and the mean perfusion of fingers (at LASCA) with the retinal and choroidal perfusion (at OCTA) (all p < 0.05). In addition, a significantly reduced retinal and choroidal perfusion was detected in SSc patients vs controls (all p < 0.05). Interestingly, diffuse cutaneous SSc (dcSSc) patients exhibited a lower choroidal perfusion (p = 0.03) but an increased choroidal thickness (CT) than limited cutaneous SSc patients (p < 0.001). CT was increased also in patients with positive Scl70 antibodies and with a history of digital ulcers directly correlating with disease duration (r = 0.67, p = 0.001). Finally, the combination of LASCA and OCTA parameters showed a significant discrimination capacity between SSc patients and controls, with an area under the curve of 0.80 [95% CI (0.74, 0.87)]. Peripheral microvascular damage is correlated with impaired ocular microcirculation in SSc. The increased choroidal thickness observed in dcSSc may be related to local sub-endothelial extracellular matrix deposition. The combined analysis of choroidal and fingertip perfusion offers preliminary insights that may complement traditional diagnostic methods for SSc.

VASCULITIS AND ITS VARIOUS PRESENTATIONS: A CASE SERIES

Vasculitis involves a wide spectrum of clinicopathological process with reactive damage to the involved blood vessels. There is loss of vessel integrity instigating haemorrhage &amp; luminal compromise leading to ischemia and necrosis of the tissue supplied by the involved vessels. It may affect varied size and type of blood vessels at different locations. This case series include three cases of vasculitis affecting different organs with varied presentations. Biopsies of three patients with unusual presentations were studied. Their complete history, physical examinations, laboratory investigations including serology were analysed and clinically correlated. The patients presented with different duration of symptoms varying from as short as 1-week days to 6 weeks. Skin lesions were present in two cases. Serology and autoimmune disease markers were negative in all cases except CD56 seen in 10% of plasma in rst case, ANA + was seen in second case and Weakly positive Mi-2 and borderline positive Scl-70 antibodies was seen in third case. Elevated CRP, CRP were seen in all cases. However, pathological features were in concordance with the clinical diagnosis of vasculitis. They were further classied as vasculitis mimicking multiple myeloma, Vasculitis with gastrointestinal manifestation and Henochschonlein purpura. Vasculitis though a rare disease may manifest as an acute or chronic condition. It needs timely diagnosis by clinicopathological examination to aid in further management. It is important to assess the clinical severity in primary and secondary vasculitis, as it determines morbidity and mortality.

Mechanic hands: clinical and capillaroscopy manifestations of patients with connective tissue diseases presented with and without mechanic hands.

The condition known as 'Mechanic's Hands' is a thickened, hyperkeratotic eruption, which is bilaterally symmetric along the fingers, and often occurs in patients with some connective tissue diseases. Nail fold capillaroscopy is a non-invasive technique for evaluation of connective tissue diseases. We evaluated the prevalence of mechanic hands in patients with connective tissue diseases and compared the clinical manifestations and capillaroscopic changes in the patients with and without mechanic hands. The clinical manifestations and capillaroscopy of 576 patients with scleroderma, dermatomyositis, systemic lupus erythematosus, Sjogren's syndrome, undifferentiated and mixed connective tissue diseases were evaluated and compared in patients with and without mechanic hands. A total of 576 patients were enrolled. Mechanic hands were observed in 17.2% of patients: 50% of mixed connective tissue disease, 35% of dermatomyositis, 15.4% of scleroderma, 14.9% of undifferentiated connective tissue disease, 14.3% of Sjogren's syndrome, and no patient with SLE. Among them, 80.8% had abnormal capillaroscopic findings. In dermatomyositis patients, Raynaud's phenomenon, anti-Jo-1 positivity, and some capillaroscopy findings were detected more frequently in patients with mechanic hand. In scleroderma, positive Scl70 and capillary loss were observed more frequently in patients without mechanic hands. Mechanic hands can be a presenting sign of some systemic connective tissue diseases. Probably, finding this sign on examination, especially together with Raynaud's phenomenon or abnormal capillaroscopy, can be helpful in the early diagnosis of the connective tissue diseases and can be used as a predictive and prognostic tool in future studies.

Performance of Forced Vital Capacity and Lung Diffusion Cutpoints for Associated Radiographic Interstitial Lung Disease in Systemic Sclerosis

Forced vital capacity (FVC) and DLCO are used for screening of systemic sclerosis-associated interstitial lung disease (SSc-ILD). The study purpose was to determine the sensitivity, specificity, and negative predictive value (NPV) (proportion of true negative screening tests) of FVC and DLCO thresholds for SSc-ILD on chest high-resolution computed tomography (HRCT) scans. Patients fulfilling American College of Rheumatology 2013 SSc criteria with a chest HRCT scan and pulmonary function tests (PFT) were studied. A thoracic radiologist quantified radiographic ILD. Optimal FVC and DLCO % predicted thresholds for ILD were identified using receiver-operating characteristic curves. The FVC and DLCO combinations with greatest sensitivity and specificity were also determined. Subanalysis was performed in patients with positive Scl-70 autoantibodies. The study included 265 patients. Of 188 (71%) with radiographic ILD, 59 (31%) had "normal" FVC (≥ 80% predicted), and 65 out of 151 (43%) had "normal" DLCO (≥ 60% predicted). FVC < 80% (sensitivity 0.69, specificity 0.73), and DLCO < 62% (sensitivity 0.60, specificity 0.70) were optimal thresholds for radiographic SSc-ILD. All FVC and DLCO threshold combinations evaluated had NPV < 0.70. The NPV for radiographic ILD for FVC < 80% was lower in patients with positive Scl-70 autoantibody (NPV = 0.05) compared to negative Scl-70 autoantibody (NPV = 0.57). Radiographic ILD is prevalent in SSc despite "normal" PFT. No % predicted FVC or DLCO threshold combinations yielded high NPV for SSc-ILD screening. "Normal" FVC and DLCO in patients with SSc, especially those with positive Scl-70 autoantibodies, should not obviate consideration of HRCT for ILD evaluation.

Acute Interstitial Pneumonia Presenting as Diffuse Alveolar Damage With Positive Scl-70 Antibodies: Cart Before the Horse?

Acute Interstitial Pneumonia Presenting as Diffuse Alveolar Damage With Positive Scl-70 Antibodies: Cart Before the Horse?

SAT0198 Rituximab for the Treatment of Systemic Sclerosis Associated Interstitial Lung Disease: a Case Series

BackgroundRituximab (RTX) is a chimeric mAb against human CD20 that depletes peripheral B cells and introduced for systemic rheumatic diseases. Basic research and clinical studies have provided preliminary data pointing...

AB0748 Study of antinucleolar pattern sera in an autoimmunity laboratory: antigenic specificities and clinical association

BackgroundIndirect ImmunoFluorescence (IIF) nucleolar pattern has a low prevalence, being usually associated with Systemic Sclerosis (SSc). Despite the increasing number of tools that help us to identify antigenic specificities many...

Esophageal dysmotility associated with systemic sclerosis: a high-resolution manometry study

Esophageal involvement occurs in about 80% of patients with systemic sclerosis, with a marked diminution of peristaltic pressures in the distal two-thirds of the esophagus. Our aims were to more fully characterize esophageal motility disorders in systemic sclerosis using high-resolution manometry (HRM) and to determine predictive factors of esophageal involvement. Fifty-one patients (46 females) with systemic sclerosis were included in this retrospective study. Esophageal motility was characterized with HRM. The demographic data, esophageal symptoms, presence of other organ involvement, and autoantibody profile (anti-Scl70 antibodies [Scl70], anticentromere antibodies [ACA]) were recorded for all patients. Esophageal body dysmotility was present in 33 patients (67.3%) and was associated with hypotensive esophagogastric junction in 27 patients (55.1%). The velocity of proximal contractions was higher in patients with esophageal body dysmotility compared to patients with normal peristalsis (median 10.8 cm/s vs. 5.5, P = 0.04). The amplitude of middle esophageal contraction but not of distal esophageal contraction was reduced in patients with hypoperistalsis. Diffuse esophageal skin involvement, presence of Scl70 and absence of ACA were associated with esophageal involvement. Esophageal symptoms encountered in 87.5% of patients were not predictive of esophageal dysmotility. This HRM series confirms the high prevalence of esophageal body dysmotility in systemic sclerosis. Diffuse skin involvement, positive Scl70 and negative ACA, but not esophageal symptoms, may predict esophageal body dysmotility.

Systemic sclerosis in Sarawak: a profile of patients treated in the Sarawak General Hospital

We performed a cross-sectional study of the demography, clinical and laboratory features of patients with systemic sclerosis patients followed up in our centre from 1984 to 2007. There were 23 cases with the majority of them (96%) being female. They have a mean age of 50.3 years and a mean disease duration of 6.02 (SD 5.82) years. Our patients comprised of multi-ethnic groups with predominantly Chinese (52%), Sarawak natives (35%) and Malays (13%). They have a mean lag time to diagnosis of 24.8 (SD 34.8) months. All the patients have sclerodermatous skin changes with 16(70%) having diffuse scleroderma and 7(30%) having limited scleroderma. The common clinical manifestations found in our patients were Raynaud's phenomenon (91%), sclerodactyly (65%), digital ulcers (52%) and pulmonary fibrosis (52%). There was low incidence of pulmonary hypertension (13%) and renal involvement (4%). The majority of our patients (67%) have positive ANA with 33% positive Scl-70. The majority received calcium channel blockers (87%), aspirin (48%) and low-dose prednisolone (48%). One patient developed adenocarcinoma of the lung on follow-up. This study demonstrated the rarity of systemic sclerosis in our centre with considerable lag time to diagnosis in our patients. Diffuse cutaneous systemic scleroderma is more common in our centre with rare pulmonary hypertension and renal involvement.

Scleroderma Renal Crisis: A Rare Complication of Systemic Sclerosis with Poor prognosis-experience in a Medical Center in Central Taiwan

Objective: Scleroderma renal crisis (SRC) is the most life-threatening complication of systemic sclerosis (SSc), which is characterized by malignant hypertension and rapidly progressive renal failure. In this study, we investigate the incidence, clinical features, treatment and outcome of our SRC patients. Patients and Methods: We reviewed the records of 161 patients with SSc, seen from January 1991 to December 2005 in the Department of Rheumatology, Taichung Veterans General Hospital, Taiwan. Tests for anti-nuclear antibodies (ANA) in sera were screened against Hep-2 cells by indirect immunofluorescence assay. Quantification of autoantibodies to extractable nuclear antigen, including anti-Scl-70 antibody (Scl-70) and anti-centromere antibody, were tested with the AtheNa Multi-Lyte ANA test system. Data collected from the records included gender, age at onset, age at diagnosis, clinical manifestations, laboratory data, treatment and outcome. Fisher's exact test was used to compare differences in categorical variables. Results: Seventy-eight (48.4%) patients were limited type SSc and 83 (51.6%) patients were diffuse type SSc. Four (2.5%) patients of a total of 161 patients had SRC. All of our SRC patients were diffuse type with positive Scl-70. SRC occurred at 37.7±55.72 months (range from 1-120 months) after the diagnosis of SSc. The duration between Raynaud's phenomenon to disease onset was 3.75 ± 2.06 months. There were significant difference at myocardial involvement (p＜0.05) and pericardial effusion (p＜0.05) between the SRC and Non-SRC diffuse SSc patients. Two patients died within four months after the SRC, one patient required permanent dialysis and one patient partly regained renal function. Conclusion: SRC is a rare complication in Taiwanese SSc patients and has a poor prognosis. Whether early administration of angiotensin converting enzyme inhibitor might prevent or ameliorate the onset of SRC needs further investigation.

Scleroderma overlap syndromes.

Scleroderma is a connective tissue disease that causes fibrosis and vascular abnormalities, but that also has an autoimmune component. Many patients with scleroderma have a positive antinuclear antibody, and there can be family histories of other connective tissue diseases such as systemic lupus erythematosus (SLE) and rheumatoid arthritis. Some patients have features of scleroderma and other autoimmune conditions. This article will review recent literature to help in the understanding of scleroderma with overlap features. Recent reports of scleroderma overlap with rheumatoid arthritis suggest distinct features of diffuse scleroderma with positive Scl-70, pulmonary fibrosis, and later seropositive erosive rheumatoid arthritis. SLE rarely occurs with scleroderma. Sjögren syndrome symptoms are common in scleroderma. In primary Sjögren syndrome, anticentromere antibody positive patients have more Raynaud phenomenon. Antibodies that occur in scleroderma that are thought to be specific are present in other connective tissue diseases. For instance, Scl-70 antibody is reported in as many as 35% of patients with scleroderma but can be present in 25% of patients with SLE. Myositis or myopathy can be features of scleroderma. Scleroderma overlap with polymyositis is frequently anti-PM Scl antibody positive, whereas anti-Jo-1 does not normally occur in the overlap of scleroderma and polymyositis but is usually exclusively positive in polymyositis with arthritis and alveolitis. A better prognosis is found with PM Scl antibody in myositis. Vasculitis is not a typical feature of scleroderma, but has been reported. Eosinophilic fasciitis is rare, and the onset could be associated with simvastatin.