Resolution of paraneoplastic PM/Scl-positive systemic sclerosis after curative resection of a pancreatic tumour

Bruni, Cosimo; Lages, Ana Clara Ribeiro; Patel, Hitesh; Nihtyanova, Svetlana I.; Green, Bryan; Abu-Hilal, Mohammed; Harvey, Joanne E.; Ong, Voon H; Cerinic, Marco Matucci; Derrett-Smith, Emma; Denton, Christopher P.

doi:10.1093/rheumatology/kew382

Cited by 18 publications

(13 citation statements)

References 5 publications

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“…Similarly, good outcomes were observed in anti-PM/Scl+ subjects, who overall demonstrated a low incidence of PH, SRC, and cardiac SSc, although approximately one-half of these patients did develop clinically significant PF within the first 15 years of disease (35). Mortality rates, although low in the first 10 years of disease, appeared to increase faster than among the other antibody groups in the second decade of disease, possibly being related to the progression of clinically significant PF or to the development of malignancies (36).…”

Section: Discussionmentioning

confidence: 97%

Using Autoantibodies and Cutaneous Subset to Develop Outcome‐Based Disease Classification in Systemic Sclerosis

Nihtyanova

Sarı

Harvey

et al. 2020

Arthritis & Rheumatology

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164

150

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Objective To describe the associations between autoantibodies, clinical presentation, and outcomes among patients with systemic sclerosis (SSc) in order to develop a novel SSc classification scheme that would incorporate both antibodies and the cutaneous disease subset as criteria. Methods Demographic and clinical characteristics, including cutaneous subset, time of disease and organ complication onset, and autoantibody specificities, were determined in a cohort of SSc subjects. Survival analysis was used to assess the effect of the autoantibodies on organ disease and death. Results The study included 1,325 subjects. Among the antibody/skin disease subsets, anticentromere antibody–positive patients with limited cutaneous SSc (lcSSc) (n = 374) had the highest 20‐year survival (65.3%), lowest incidence of clinically significant pulmonary fibrosis (PF) (8.5%) and scleroderma renal crisis (SRC) (0.3%), and lowest incidence of cardiac SSc (4.9%), whereas the frequency of pulmonary hypertension (PH) was similar to the mean value in the SSc cohort overall. The anti–Scl‐70+ groups of patients with lcSSc (n = 138) and patients with diffuse cutaneous SSc (dcSSc) (n = 149) had the highest incidence of clinically significant PF (86.1% and 84%, respectively, at 15 years). Anti‐Scl‐70+ patients with dcSSc had the lowest survival (32.4%) and the second highest incidence of cardiac SSc (12.9%) at 20 years. In contrast, in anti‐Scl‐70+ patients with lcSSc, other complications were rare, and these patients demonstrated the lowest incidence of PH (6.9%) and second highest survival (61.8%) at 20 years. Anti–RNA polymerase antibody–positive SSc patients (n = 147) had the highest incidence of SRC (28.1%) at 20 years. The anti–U3 RNP+ SSc group (n = 56) had the highest incidence of PH (33.8%) and cardiac SSc (13.2%) at 20 years. Among lcSSc patients with other autoantibodies (n = 295), the risk of SRC and cardiac SSc was low at 20 years (2.7% and 2.4%, respectively), while the frequencies of other outcomes were similar to the mean values in the full SSc cohort. Patients with dcSSc who were positive for other autoantibodies (n = 166) had a poor prognosis, demonstrating the second lowest survival (33.6%) and frequent organ complications. Conclusion These findings highlight the importance of autoantibodies, cutaneous subset, and disease duration when assessing morbidity and mortality in patients with SSc. Our novel classification scheme may improve disease monitoring and benefit future clinical trial designs in SSc.

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Section: Discussionmentioning

confidence: 97%

Using Autoantibodies and Cutaneous Subset to Develop Outcome‐Based Disease Classification in Systemic Sclerosis

Nihtyanova

Sarı

Harvey

et al. 2020

Arthritis & Rheumatology

Self Cite

164

150

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“…Bruni et al. 9 observed 14 SRCs among 80 patients with SSc and anti-PM/Scl antibodies (5.7%). In this work, steroid treatment did not appear to be a risk factor; nevertheless, our case strongly suggests a cause-effect relationship.…”

Section: Discussionmentioning

confidence: 99%

Scleroderma Renal Crisis in a Systemic Sclerosis With Anti-PM/Scl Antibodies

Jacquier¹,

Mousson²,

Rebibou³

et al. 2019

Kidney International Reports

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“…A recent case of a patient with anti-PM/Scl positive diffuse scleroderma and myositis with a coincident pancreatic neoplasm supports this possibility (57). This patient had evidence of increased nuclear expression of the 100 kDa component of the PM/Scl complex in her cancerous tissue compared to normal pancreatic tissue.…”

Section: Implications For Cancer Screening and Scleroderma Therapeuticsmentioning

confidence: 95%

“…This has been observed across a wide range of tumor types but has been most notable with breast cancer (1, 26–27–28–29), where in one series, 61% of patients developed breast cancer and scleroderma within 12 months of each other. The description that scleroderma dramatically improves with treatment of the underlying cancer in some cases is intriguing also (57–58–59). These reports suggest that careful study of patients with a clustering of cancer and scleroderma may provide insight into the mechanisms linking the two diseases.…”

Section: Potential Mechanisms Linking Cancer and Sclerodermamentioning

confidence: 99%

Mechanistic and clinical insights at the scleroderma-cancer interface

Shah

Casciola‐Rosen

2017

Journal of Scleroderma and Related Disorders

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Emerging data suggest tantalizing links between cancer and systemic inflammatory rheumatic syndromes. In scleroderma, patients may have an increased risk of cancer secondary to chronic inflammation and damage from the disease, malignant transformation promoted by immunosuppressive therapies, a shared susceptibility to both cancer and autoimmunity, or a common inciting exposure. However, it is increasingly recognized that a subset of patients develop cancer around the time that scleroderma clinically manifests, raising the question of cancer-induced autoimmunity. In this review, we discuss data suggesting a mechanistic link between cancer and the development of scleroderma, and the clinical implications of these findings.

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Resolution of paraneoplastic PM/Scl-positive systemic sclerosis after curative resection of a pancreatic tumour

Cited by 18 publications

References 5 publications

Using Autoantibodies and Cutaneous Subset to Develop Outcome‐Based Disease Classification in Systemic Sclerosis

Using Autoantibodies and Cutaneous Subset to Develop Outcome‐Based Disease Classification in Systemic Sclerosis

Scleroderma Renal Crisis in a Systemic Sclerosis With Anti-PM/Scl Antibodies

Mechanistic and clinical insights at the scleroderma-cancer interface

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