AB0658 ROLE OF NAILFOLD CAPILLAROSCOPY IN MONITORING LUNG INVOLVEMENT OF SYSTEMIC SCLEROSIS

Objective.Systemic sclerosis (SSc) is an autoimmune chronic fibrotic disorder. Urotensin II (U-II) is predominantly a vasoactive peptide with fibrotic and prothrombotic features. Like endothelin-1 (ET-1), U-II could play an important role in SSc pathogenesis. We evaluated the possible role of the U-II gene polymorphisms (Thr21Met and Ser89Asn) in the genetic susceptibility to SSc in a Turkish population.Methods.A total of 189 patients with SSc and 205 healthy controls were enrolled in our study. We analyzed the genotype and allele frequencies of the U-II (UTS2) gene polymorphisms Thr21Met and Ser89Asn in patients with SSc and in controls.Results.We found that the Thr21Met polymorphism of the UTS2 gene was markedly associated with the risk of developing SSc (p < 0.0001), but there was no relationship between the Ser89Asn polymorphism and SSc (p > 0.05). Two haplotypes (MS and TS) were markedly associated with SSc (p < 0.05). There were significant associations between the genotype and allele frequencies of UTS2 gene Thr21Met polymorphism and cases with diffuse or limited SSc, systemic or lung involvement, finger flexion deformity, pitting scars at the fingertips, positive anticentromere, or positive antitopoisomerase 1 antibody groups.Conclusion.Our study shows the association between Thr21Met, but not Ser89Asn, in the UTS2 gene and SSc. The results strongly suggest that this single-nucleotide polymorphism may be an important risk factor in the development of SSc, and a powerful indicator of severe skin and lung involvement in patients with SSc.

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The role of nailfold capillaroscopy in the assessment of internal organ involvement in systemic sclerosis: A critical review

Autoimmunity Reviews ◽

10.1016/j.autrev.2017.05.019 ◽

2017 ◽

Vol 16 (8) ◽

pp. 787-795 ◽

Cited By ~ 15

Author(s):

Stergios Soulaidopoulos ◽

Eva Triantafyllidou ◽

Alexandros Garyfallos ◽

George D. Kitas ◽

Theodoros Dimitroulas

Keyword(s):

Systemic Sclerosis ◽

Critical Review ◽

Nailfold Capillaroscopy ◽

Organ Involvement

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The Role of Chest CT in Deciphering Interstitial Lung Involvement: Systemic Sclerosis Versus COVID-19

SSRN Electronic Journal ◽

10.2139/ssrn.3790464 ◽

2021 ◽

Author(s):

Martina Orlandi ◽

Nicholas Landini ◽

Gianluca Sambataro ◽

Cosimo Nardi ◽

Lorenzo Tofani ◽

...

Keyword(s):

Systemic Sclerosis ◽

Chest Ct ◽

Lung Involvement

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Association of Serum Homocysteine Level and Interstitial Lung Disease in Systemic Sclerosis: A Case-control Study

Current Rheumatology Reviews ◽

10.2174/1573397114666180628162907 ◽

2018 ◽

Vol 15 (1) ◽

pp. 74-78

Author(s):

Mohammadali Nazarinia ◽

Asghar Zare ◽

Mohammad javad Fallahi ◽

Mesbah Shams

Keyword(s):

Systemic Sclerosis ◽

Healthy Subjects ◽

Case Control Study ◽

Homocysteine Level ◽

Case Control ◽

Control Group ◽

Lung Involvement ◽

Serum Homocysteine ◽

The Mean ◽

Control Study

Background:Systemic sclerosis is a disorder of connective tissue with unknown cause, affecting the skin and internal organs, characterized by fibrotic changes.Objective:To determine the correlation between serum homocysteine level and interstitial lung involvement in systemic sclerosis. </P><P> Materials and Methods: In this case – control study, 59 patients who fulfilled the ACR/EULAR classification criteria for systemic sclerosis and were referred to Hafez Hospital of Shiraz, Iran, were included as the case group. Fifty nine healthy subjects were involved as the control group. Patients were divided into two groups based on interstitial lung involvement and two subtypes, diffuse and limited type. Serum homocysteine, vitamin B12, and folate levels compared between the controls, and cases groups.Results:Of 59 case and control group, 53 (%89.8) were female and the mean age did not differ in both groups (P=0.929). Thirty five (%59.3) patients had interstitial lung involvement and 38(%64.4) had diffuse cutaneous systemic sclerosis. The mean serum homocysteine level was 13.9±6.3 µmol/L in the case and 13.7±9.2 µmol/L in the control group (P=0.86). The mean serum homocysteine level did not differ between the patients with and without interstitial lung involvement (P=0.52). The patients with lung involvement was older than those without lung involvement (P=0.004). Lung disease was more common in diffuse type (P=0.014).Conclusion:In our study, serum homocysteine level did not differ between the patients and healthy subjects. Also, there was no correlation between serum homocysteine level and lung involvement, but lung involvement was more common in older patients and also diffuse subtype.

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Systematic Review of the Role of Microparticles in Systemic Sclerosis

Current Rheumatology Reviews ◽

10.2174/1573397109666140103001139 ◽

2014 ◽

Vol 9 (4) ◽

pp. 279-300 ◽

Cited By ~ 1

Author(s):

James Dunne ◽

Julius Bankole ◽

Kevin Keen

Keyword(s):

Systematic Review ◽

Systemic Sclerosis

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Impaired Angiogenesis in Systemic Sclerosis: The Emerging Role of the Antiangiogenic VEGF165b Splice Variant

Trends in Cardiovascular Medicine ◽

10.1016/j.tcm.2012.05.011 ◽

2011 ◽

Vol 21 (7) ◽

pp. 204-210 ◽

Cited By ~ 34

Author(s):

Mirko Manetti ◽

Serena Guiducci ◽

Lidia Ibba-Manneschi ◽

Marco Matucci-Cerinic

Keyword(s):

Systemic Sclerosis ◽

Splice Variant ◽

Impaired Angiogenesis

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AB0440 CLINICAL AND IMMUNOLOGICAL FEATURES OF THE SYSTEMIC SCLEROSIS-OVERLAP SYNDROMES

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.2550 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 1248.2-1248

Author(s):

O. Desinova ◽

M. Starovoytova ◽

L. P. Ananyeva ◽

O. Koneva ◽

L. Garzanova ◽

...

Keyword(s):

Systemic Sclerosis ◽

Pathological Process ◽

Point Of View ◽

Joint Involvement ◽

Laboratory Findings ◽

Overlap Syndromes ◽

Digital Ischemia ◽

Immune Alterations ◽

Clinical Variants

Background:Systemic Sclerosis (SSc) overlap syndromes (SSc with polymyositis / dermatomyositis (PM/DM), rheumatoid arthritis (RA), etc.) still remain a group of very heterogenous and not very well studied clinical variants of SSc that are characterized by certain clinical and immunological features.Objectives:Identify clinical and immunological features of the SSc-overlap syndromesMethods:80 pts with SSc-PM/DM and 35 pts with SSc-RA undergoing standard clinical examination and laboratory immunological evaluation.Results:ANA Hep2 was positive in 98% of SSc-PM/DM pts; a-Scl-70 was in 34%, a - PM-Scl and RF were in 20%. ACA (6%), a-RNP (9%), and a - Jo-1 (5%) were significantly less common. Correlation analysis showed significant prevalence of conduction abnormalities in pts with a-Scl-70- (p<0.03); PM-Scl was rarely associated with cardiac arrhythmia (p<0.02) and pericarditis (p<0.03), but there was an association between ACA and presence of digital ischemia (p<0.04). Three pts with limited skin had Scl-70 and PM-Scl antibodies, two of them manifested clinical features of DM. A-Jo-1 was found in 3 pts with a longstanding disease (14,10 and 7 years), and one of these pts was also positive for a-Scl-70. All pts had limited skin and two had interstitial lung disease with FVC values of 79% and 74.8%.ANA Hep2 was positive in 96% of SSc-RA pts; a-Scl-70 – in 28%, and a-RNP - in 30%. RF-positivity was in 72% of pts, and Anti-CCP - in 27%. Simultaneous Anti-CCP and a-Scl-70 was found in one case, and Anti-CCP - anti-RNP – in another, both were associated with low RF titers. All pts had early joint involvement which became prevailing in subsequent years, and onset of the disease between 30 and 36 years. There was a correlation between laboratory signs of inflammatory activity and immunological disorders: ESR and a-Scl-70 (p<0.03). Anti-CCP and a-Scl-70 co-positivity was a significantly less frequent phenomenon (p<0.04). There was a remarkable 28% proportion of a-Scl-70 cases in SSc-RA with limited cutaneous which is usually characterized by ACA-positivity.Conclusion:SSc-PM/DM and SSc-RA appear to be an active disease from the immunological point of view, confirming therefore an important role of immune alterations in disease progression. Laboratory findings display specific pathogenetic features of SSc-overlap syndromes; laboratory abnormalities can be used to measure the activity and specify characteristics of the pathological process.Disclosure of Interests:None declared

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Lung involvement in systemic sclerosis is associated with adverse hospital outcomes: insights from the National Inpatient Sample

Journal of Investigative Medicine ◽

10.1136/jim-2020-001743 ◽

2021 ◽

pp. jim-2020-001743

Author(s):

Jesse Osemudiamen Odion ◽

Armaan Guraya ◽

Chukwudi Charles Modijeje ◽

Osahon Nekpen Idolor ◽

Eseosa Jennifer Sanwo ◽

...

Keyword(s):

Systemic Sclerosis ◽

Hospital Charge ◽

Hospital Length Of Stay ◽

Lung Involvement ◽

National Inpatient Sample ◽

Inpatient Mortality ◽

Total Hospital ◽

Total Hospital Charge ◽

Icd 10 ◽

Secondary Outcomes

This study aimed to compare outcomes of systemic sclerosis (SSc) hospitalizations with and without lung involvement. The primary outcome was inpatient mortality while secondary outcomes were hospital length of stay (LOS) and total hospital charge. Data were abstracted from the National Inpatient Sample (NIS) 2016 and 2017 database. This database is the largest collection of inpatient hospitalization data in the USA. The NIS was searched for SSc hospitalizations with and without lung involvement as principal or secondary diagnosis using International Classification of Diseases 10th Revision (ICD-10) codes. SSc hospitalizations for patients aged ≥18 years from the above groups were identified. Multivariate logistic and linear regression analysis was used to adjust for possible confounders for the primary and secondary outcomes, respectively. There were over 71 million discharges included in the combined 2016 and 2017 NIS database. 62,930 hospitalizations were for adult patients who had either a principal or secondary ICD-10 code for SSc. 5095 (8.10%) of these hospitalizations had lung involvement. Lung involvement group had greater inpatient mortality (9.04% vs 4.36%, adjusted OR 2.09, 95% CI 1.61 to 2.73, p<0.0001), increase in mean adjusted LOS of 1.81 days (95% CI 0.98 to 2.64, p<0.0001), and increase in mean adjusted total hospital charge of $31,807 (95% CI 14,779 to 48,834, p<0.0001), compared with those without lung involvement. Hospitalizations for SSc with lung involvement have increased inpatient mortality, LOS and total hospital charge compared with those without lung involvement. Collaboration between the pulmonologist and the rheumatologist is important in optimizing outcomes of SSc hospitalizations with lung involvement.

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