Rapidly Progressive Systemic Sclerosis–associated Interstitial Lung Disease After Intravesical Bacillus Calmette-Guérin Therapy for Early-stage Bladder Cancer

2021 ◽  
pp. jrheum.201625
Author(s):  
Gonçalo Boleto ◽  
Jérôme Avouac ◽  
Yannick Allanore
Keyword(s):  
Bladder Cancer ◽  
Systemic Sclerosis ◽  
Respiratory Failure ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Early Stage ◽  
Wide Spectrum ◽  
Progressive Systemic Sclerosis ◽  
Bacillus Calmette Guerin ◽  
Common Cause

Despite recent advances in the management of systemic sclerosis–associated interstitial lung disease (SSc-ILD), it remains the most common cause of death and a significant contributor to morbidity.1,2 SSc-ILD is characterized by a wide spectrum of disease courses, with some patients having limited nonprogressive fibrosis, whereas others develop rapid and extensive fibrosis leading to respiratory failure.3

scholarly journals P33 Rituximab as rescue therapy in advanced progressive systemic sclerosis associated interstitial lung disease

Thorax ◽  
2015 ◽  
Vol 70 (Suppl 3) ◽  
pp. A92.2-A92
Author(s):  
M Kokosi ◽  
P Saunders ◽  
K Karagiannis ◽  
F Chua ◽  
TM Maher ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Rescue Therapy ◽  
Progressive Systemic Sclerosis

Elevated Serum Krebs von den Lungen-6 in Early Disease Predicts Subsequent Deterioration of Pulmonary Function in Patients with Systemic Sclerosis and Interstitial Lung Disease

2016 ◽  
Vol 43 (10) ◽  
pp. 1825-1831 ◽  
Author(s):  
Masataka Kuwana ◽  
Yuichiro Shirai ◽  
Tsutomu Takeuchi
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Early Stage ◽  
Characteristic Curve ◽  
Elevated Serum ◽  
Initial Assessment ◽  
Baseline Serum ◽  
Disease Modifying Drugs ◽  
Highly Correlated

Objective.To identify predictors of poor prognosis in patients with systemic sclerosis (SSc) associated with interstitial lung disease (ILD).Methods.Fifty patients with early-stage SSc-ILD who had never received disease-modifying drugs and were either observed for ≥ 10 years or died from ILD-related causes were enrolled. The baseline variables of patients who developed endstage lung disease (ESLD) were compared with those of patients who remained ESLD-free, and the Cox proportional hazard model was used to identify initial factors that correlated with ESLD development.Results.Sixteen patients (32%) developed ESLD during 173.5 ± 64.7 months of followup. Elevated serum Krebs von den Lungen-6 (KL-6) at initial assessment was highly correlated with ESLD development (p = 0.0002). Receiver-operating characteristic curve analysis revealed that a KL-6 value of 1273 U/ml effectively discriminated patients who developed ESLD from those who did not. Patients with KL-6 > 1273 U/ml were less likely to remain ESLD-free compared with those with lower KL-6 levels (p < 0.0001). Multivariate analysis showed that KL-6 > 1273 U/ml was the most reliable predictor of ESLD development (OR 51.2, 95% CI 7.6–343, p < 0.0001). Finally, the initial KL-6 level correlated with the forced vital capacity (FVC) decline rate (r = 0.58, p < 0.0001).Conclusion.The natural course of SSc-ILD is highly variable. Baseline serum KL-6 is a biomarker potentially useful for predicting FVC decline.

Interstitial lung disease in progressive systemic sclerosis: high-resolution CT versus radiography.

Radiology ◽  
1990 ◽  
Vol 176 (3) ◽  
pp. 755-759 ◽  
Author(s):  
H Schurawitzki ◽  
R Stiglbauer ◽  
W Graninger ◽  
C Herold ◽  
D Pölzleitner ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
High Resolution ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Progressive Systemic Sclerosis ◽  
High Resolution Ct

scholarly journals Clinical and Pathological Features of Breast Cancer in Systemic Sclerosis: Results from the Sclero-Breast Study

2021 ◽  
Vol 11 (6) ◽  
pp. 580
Author(s):  
Angela Toss ◽  
Amelia Spinella ◽  
Chrystel Isca ◽  
Caterina Vacchi ◽  
Guido Ficarra ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Early Stage ◽  
Fold Increase ◽  
Breast Conserving Surgery ◽  
Pathological Features ◽  
Luminal A ◽  
Clinical And Pathological Features

Systemic Sclerosis (SSc) is a chronic disease associated with a 1.5-fold increase in cancer risk, including lung cancer, hematological malignancies, and breast cancer (BC). This is a retrospective study aiming to explore the clinical and pathological features of BC developed by SSc patients. A total of 54.5% of patients developed BC before SSc (median interval: 5 years), whereas 45.5% of patients developed BC after SSc (median delay: 8 years). A total of 93.1% of patients were diagnosed with an early stage tumor. Among invasive carcinomas, 70.8% presented with a low Mib1, 8.3% with a tubular histotype, and 42.8% with a Luminal A-like phenotype. A total of 66.6% of patients underwent breast-conserving surgery and 55.5% RT. A total of 40% of patients developed interstitial lung disease after RT and 20% diffuse cutaneous SSc. The cause of death of the six deceased patients was PAH. A significant association was observed between the use of immunosuppressive therapy and diffuse skin extension, negative ACA, positive Anti-Scl-70, and interstitial lung disease, but not BC status. SSc patients developed BC at a good prognosis, suggesting a de-escalation strategy of cancer therapies. In particular, ionizing radiation and chemotherapeuticals should be limited to higher-risk cases. Finally, proper screening is mandatory in order to allow for early cancer detection in SSc patients.

The role of vasodilators in patients with progressive systemic sclerosis. Interstitial lung disease and pulmonary hypertension

CHEST Journal ◽  
1985 ◽  
Vol 88 (4) ◽  
pp. 263S-265 ◽  
Author(s):  
J. Ohar ◽  
C. Polatty ◽  
A. Robichaud ◽  
A. Fowler ◽  
G. Vetrovec ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Progressive Systemic Sclerosis

Procollagen Type I and III Aminoterminal Propeptide Levels and Severity of Interstitial Lung Disease in Mexican Women With Progressive Systemic Sclerosis

2015 ◽  
Vol 51 (9) ◽  
pp. 440-448 ◽  
Author(s):  
Laura Gonzalez-Lopez ◽  
Alberto D. Rocha-Muñoz ◽  
Eva M. Olivas-Flores ◽  
Araceli Garcia-Gonzalez ◽  
Ana R. Peguero-Gómez ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Progressive Systemic Sclerosis ◽  
Type I ◽  
Mexican Women ◽  
Procollagen Type ◽  
Procollagen Type I

scholarly journals Circulating biomarkers of systemic sclerosis – interstitial lung disease

2020 ◽  
Vol 5 (2_suppl) ◽  
pp. 41-47 ◽  
Author(s):  
Anna-Maria Hoffmann-Vold ◽  
Håvard Fretheim ◽  
Chantal Meier ◽  
Britta Maurer
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Application ◽  
Topoisomerase I ◽  
Early Stage ◽  
Organ Damage ◽  
Disease Diagnosis ◽  
Surfactant Protein D ◽  
Circulating Biomarkers

Interstitial lung disease is a frequent organ manifestation in systemic sclerosis and is associated with high mortality. It is crucial to diagnose interstitial lung disease in systemic sclerosis and to assess severity and identify patients prone to progression at an early stage to ultimately decrease organ damage and improve outcome. Circulating anti-topoisomerase-I autoantibodies have long been associated with the presence and development of systemic sclerosis – interstitial lung disease, evidence on their potential to further predict the clinical course of systemic sclerosis is however conflicting. C-reactive protein is a marker of infection and systemic inflammation with widespread clinical application and is elevated in systemic sclerosis with a tendency towards higher abundancy in patients with early disease. The role of other circulating biomarkers is promising but hampered by the lack of standardized criteria and guidelines for sample/data collection, analyses, reporting and validation and has not reached prime time for clinical application. However, epithelial markers including Krebs von den Lungen-6 and surfactant protein D and several cytokines and chemokines including CCL2 and CCL18 for severity assessment of systemic sclerosis – interstitial lung disease patients at the time of interstitial lung disease diagnosis and to predict interstitial lung disease progression have been reported and seem to be promising candidate biomarkers in the future.

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