Anti-Ro52/TRIM21 is independently associated with pulmonary arterial hypertension and mortality in a cohort of systemic sclerosis patients.

Objective: We undertook a comprehensive cross-sectional analysis of a multicentred Australian cohort of systemic sclerosis (SSc) patients to evaluate the associations of anti-Ro52/TRIM21 with SSc pulmonary involvement.Method: The study included 596 patients from the Australian Scleroderma Cohort Study database whose anti-Ro52/TRIM21 status was known. Anti-Ro52/TRIM21 was measured via line immunoassay. Data on demographic variables, autoantibody profiles, presence of interstitial lung disease (ILD), presence of pulmonary arterial hypertension (PAH), oxygen saturation, Six-Minute Walk Test distance, Borg dyspnoea score, and lung function tests were extracted. SPSS software was used to examine associations using univariate and multivariate analyses.Results: Anti-Ro52/TRIM21 was present in 34.4% of SSc patients. In the cross-sectional analysis, anti-Ro52/TRIM21 was independently associated with PAH [odds ratio 1.75, 95% confidence interval (CI) 1.05-2.90], but not ILD or other surrogate measures of pulmonary involvement such as average patient oxygen saturation. The antibody, however, was also associated with a higher forced vital capacity/diffusing capacity of the lung for carbon monoxide ratio. Prospectively, anti-Ro52/TRIM21 was also associated with an increased risk of death in patients with SSc (hazard ratio 1.62, 95% CI 1.11-2.35), independent of confounding factors. The primary cause of death appeared to be related to PAH and/or ILD, and anti-Ro52/TRIM21 was associated with PAH-related complications.Conclusion: Anti-Ro52/TRIM21 was independently associated with PAH and mortality in SSc patients. Future longitudinal studies are recommended to investigate the timing and pathogenic mechanisms of this autoantibody in PAH.

Arterial stiffness is increased in systemic sclerosis: a cross-sectional comparison with matched controls.

OBJECTIVES: Increased arterial stiffness is a predictor of cardiovascular and all-cause mortality. Atherosclerosis may be increased in systemic sclerosis (SSc). Our aims were to determine if arterial stiffness is elevated and to evaluate correlates of arterial stiffness in SSc. METHODS: We carried out two studies: 1. a comparison of arterial stiffness in 40 SSc patients free from cardiovascular disease or significant vascular manifestations of SSc and 40 healthy controls (HC), and 2. an analysis of determinants of arterial stiffness in 80 SSc patients free from cardiovascular disease. RESULTS: In Study 1, the groups were well-matched for age (52.2 vs. 50.0 years, p=0.432) and sex (80% female in both). SSc patients had higher augmentation index (AIx) than HC (31.0% [IQR 25.7-38.7] vs. 23.8% [IQR 13.5- 30.1], p<0.001). Pulse wave velocity (PWV) was also higher, however this did not reach statistical significance (6.9 m/s [IQR 6.0-8.3] vs. 6.5 m/s [IQR 6.1-7.4], p=0.275). In Study 2, age (p<0.001) and calcium channel blocker (CCB) therapy (p=0.016) were independently associated with higher AIx; and age (p<0.001), disease duration (p=0.042) and systolic blood pressure (p=0.001) with higher PWV. CONCLUSIONS: SSc patients had higher AIx than HC. The paradoxical association between CCB therapy and higher AIx could reflect generalised vasculopathy rather than atherosclerotic disease. Prospective studies in larger cohorts are warranted to clarify this point and elucidate other determinants of arterial stiffness in SSc.

The impact of gastroesophageal reflux disease and its treatment on interstitial lung disease outcomes.

BACKGROUND: To determine the relationship between gastroesophageal reflux disease (GORD) and its treatment and interstitial lung disease in patients with systemic sclerosis (SSc). METHODS: SSc patients from the Australian Scleroderma Cohort Study (ASCS) were included. GORD was defined as self-reported GORD symptoms, therapy with a proton pump inhibitor (PPI) or histamine 2 receptor antagonist (H2RA) and/or the presence of reflux oesophagitis diagnosed endoscopically. The impact of GORD and its treatment on ILD features (including severity and time to ILD development) and survival was evaluated. RESULTS: GORD was a common manifestation affecting 1539/1632 (94%) of SSc patients. GORD affected 450/469 (96%) of those with SSc-ILD cohort. In SSc-ILD, there was no relationship between the presence of GORD or its treatment and time to ILD development or ILD severity. However, GORD treatment was associated with improved survival in those with ILD (p = 0.002). Combination therapy with both a PPI and a H2RA was associated with a greater survival benefit than single agent therapy with PPI alone (HR 0.3 vs 0.5 p < 0.050 respectively). CONCLUSION: GORD is a common SSc disease manifestation. While the presence or treatment of GORD does not influence the development or severity of ILD, aggressive GORD treatment, in particular with a combination of PPI and H2RA, is associated with improved survival in those with SSc-ILD.

Imaging of systemic lupus erythematosus. Part I: CNS, cardiovascular, and thoracic manifestations.

Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease that has a relapsing and remitting course. It has a wide range of non-specific symptoms with various organ manifestations. In 1982, the American College of Rheumatology (ACR) published the revised criteria for the classification of SLE. The diagnosis of SLE may be made if four or more of the 11 ACR criteria are present, either serially or simultaneously, during any interval of observation. Whilst the diagnosis of SLE is based on clinical and laboratory features, with no universally accepted radiological diagnostic criteria, imaging is nonetheless useful for diagnosing specific organ manifestations, monitoring disease progression, and identifying complications secondary to immunosuppressive therapy. In this review, we describe the spectrum of radiological findings of SLE in various organ systems and compile a list of organ manifestations including the most frequently occurring diseases as well as the rare but not-to-be-missed diseases. This review aims to serve as a concise reference tool in an endeavour to assist clinicians and radiologists in the diagnosis and monitoring of this disease. This pictorial review presents the various radiological findings of CNS, cardiovascular and thoracic manifestation of SLE. The gastrointestinal, renal and musculoskeletal systems will be covered in part II.

Imaging of systemic lupus erythematosus. Part II: gastrointestinal, renal, and musculoskeletal manifestations.

Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease that has a relapsing and remitting course. It has a wide range of presentations with various organ manifestations. In this review, we have compiled the radiological findings of gastrointestinal, renal, and musculoskeletal manifestations of SLE.

Smooth muscle myopathy as an underrecognized manifestation of active systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with protean manifestations. We here present a case of unexplained diarrhoea and abdominal pain in a patient with SLE. Investigations revealed dilatation of stomach, small bowel and colonic wall, biliary and pancreatic ducts, renal collecting systems and ureters as well as thoracic aorta and major pulmonary arteries, as manifestations of a smooth muscle myopathy that was responsive to immunosuppressive therapy with cyclosporin A.