A RELATIONSHIP BETWEEN THE HONEYCOMB APPEARANCE ON CT SCAN AND LIFE EXPECTANCY IN PATIENTS WITH SCLERODERMA? A SYSTEMATIC REVIEW
Keywords:
Systemic sclerosis, scleroderma, interstitial lung disease, honeycombing, CT scan, HRCT, survival, mortality, prognosisAbstract
Introduction: Systemic sclerosis (SSc) is a complex autoimmune disease frequently complicated by interstitial lung disease (ILD), a leading cause of mortality. The honeycomb appearance on high-resolution computed tomography (HRCT) represents advanced pulmonary fibrosis, but its prognostic significance in SSc-ILD remains debated. This systematic review aims to evaluate the relationship between honeycomb appearance on CT scan and life expectancy in patients with scleroderma.
Methods: A systematic review was conducted following PRISMA guidelines. We screened studies based on predefined criteria: confirmed SSc diagnosis, evaluation of honeycomb pattern on CT, reported survival outcomes, appropriate observational study design, adequate follow-up, and sufficient sample size (>10 patients). Data extraction encompassed study characteristics, CT methodology, honeycomb definition, survival outcomes, and statistical associations. Quality assessment and multivariate analyses were evaluated.
Results: Among 198 included studies, sample sizes ranged from 10 to 62,930 patients, with follow-up periods from 12 months to over 20 years. Honeycombing prevalence in SSc-ILD cohorts ranged from 37.2% to 41.9%, with higher frequency in limited cutaneous SSc. Multiple studies demonstrated significant associations between honeycombing and mortality, with hazard ratios ranging from 1.72 (95% CI 1.38-2.14) to 4.64 (95% CI 1.68-12.81). The association persisted after adjusting for age, gender, pulmonary function tests, and scleroderma subtype. Automated quantitative CT methods (CALIPER) showed improved reproducibility compared to visual scoring.
Discussion: This review provides robust evidence that honeycomb appearance on CT scan is an independent predictor of reduced life expectancy in SSc-ILD patients. Honeycombing represents irreversible fibrotic damage and consistently outperforms inflammatory features (ground-glass opacities) in prognostic value. Heterogeneity in honeycomb definitions and quantification methods remains a limitation.
Conclusion: Honeycomb appearance on HRCT is a critical prognostic marker in SSc-ILD, associated with 2-3 fold increased mortality risk. Standardized CT reporting and incorporation of honeycombing into clinical risk stratification models are recommended. Future research should focus on automated quantitative assessment and validation of honeycombing-specific therapeutic algorithms.
References
A. Adegunsoye, J. Oldham, S. Bellam, S. Montner, Matthew M. Churpek, I. Noth, R. Vij, M. Strek, and Jonathan H. Chung. “Computed Tomography Honeycombing Identifies a Progressive Fibrotic Phenotype with Increased Mortality Across Diverse Interstitial Lung Diseases.” Annals of the American Thoracic Society, 2019.
A. Ariani, E. Bravi, M. Santis, V. Hax, S. Parisi, F. Lumetti, F. Girelli, et al. “OP0063 QUANTITATIVE COMPUTED TOMOGRAPHY PREDICTS 10-YEAR MORTALITY IN INTERSTITIAL LUNG DISEASE RELATED TO SYSTEMIC SCLEROSIS.” Annals of the Rheumatic Diseases, 2020.
A. Ariani, M. Aiello, Mario Silva, V. Alfieri, E. Bonati, F. Lumetti, G. Delsante, N. Sverzellati, and A. Chetta. “Quantitative CT Indexes Are Significantly Associated with Exercise Oxygen Desaturation in Interstitial Lung Disease Related to Systemic Sclerosis.” Clinical Respiratory Journal, 2017.
A. Ariani, M. Silva, S. Parisi, M. Saracco, E. Bravi, F. De Gennaro, C. Benini, et al. “FRI0443 Can Quantitative Chest CT Predict Interstitial Lung Disease Worsening in Systemic Sclerosis? Results from a Multi-Centre Prospective Cohort Study,” 2015.
A. Ariani, Mario Silva, E. Bravi, M. Saracco, S. Parisi, F. De Gennaro, F. Lumetti, et al. “Operator-Independent Quantitative Chest Computed Tomography Versus Standard Assessment of Interstitial Lung Disease Related to Systemic Sclerosis: A Multi-Centric Study.” Modern Rheumatology, 2015.
A. Ariani, Mario Silva, V. Seletti, E. Bravi, M. Saracco, S. Parisi, F. De Gennaro, et al. “Quantitative Chest Computed Tomography Is Associated with Two Prediction Models of Mortality in Interstitial Lung Disease Related to Systemic Sclerosis.” Rheumatology, 2017.
A. Ariani, N. Sverzellati, Andrea Becciolni, G. Milanese, and Mario Silva. “Using Quantitative Computed Tomography to Predict Mortality in Patients with Interstitial Lung Disease Related to Systemic Sclerosis: Implications for Personalized Medicine,” 2020.
A. Ballerie, C. Cavalin, M. Lederlin, Amélie Nicolas, R. Garlantézec, S. Jouneau, V. Lecureur, et al. “Association of Silica Exposure with Chest HRCT and Clinical Characteristics in Systemic Sclerosis.” Seminars in Arthritis & Rheumatism, 2020.
A. Carnevale, Mario Silva, E. Maietti, G. Milanese, M. Saracco, S. Parisi, E. Bravi, et al. “Longitudinal Change During Follow-up of Systemic Sclerosis: Correlation Between High-Resolution Computed Tomography and Pulmonary Function Tests.” Clinical Rheumatology, 2020.
A. Duarte, A. Cordeiro, T. Santiago, M. J. Salvador, and M. Santos. “AB0726 Interstitial Lung Disease in Scleroderma Patients – a Portuguese Portrait.” Scleroderma, Myositis and Related Syndromes, 2018.
A. Fischer, J. Swigris, S. Groshong, C. Cool, H. Sahin, D. Lynch, D. Curran-Everett, J. Gillis, R. Meehan, and K. Brown. “Clinically Significant Interstitial Lung Disease in Limited Scleroderma: Histopathology, Clinical Features, and Survival.” Chest, 2008.
A. Forestier, N. Le Gouellec, A. Duhamel, G. Kramer, T. Pérez, V. Sobanski, S. morell dubois, et al. “THU0430 Description and Prognosis Factors of Systemic Sclerosis-Associated Interstitial Lung Disease Outcome on Serial Hrct.” THURSDAY, 14 JUNE 2018:, 2018.
A. Forestier, N. Le Gouellec, H. Behal, G. Kramer, T. Pérez, V. Sobanski, S. Dubois, et al. “Evolution of High-Resolution CT-Scan in Systemic Sclerosis-Associated Interstitial Lung Disease: Description and Prognosis Factors.” Seminars in Arthritis & Rheumatism, 2020.
A. Fouda, A. Eldin, N. H. Ibrahim, and M. Mostafa. “Pulmonary Involvement in Patients with Systemic Sclerosis,” 2020.
A. Guillen-Del Castillo, C. Pilar Simeón-Aznar, V. Fonollosa-Plá, Serafín Alonso-Vila, M. Reverté-Vinaixa, X. Muñoz, E. Pallisa, Albert Selva-O'allaghan, A. Fernández-Codina, and M. Vilardell‐Tarrés. “Good Outcome of Interstitial Lung Disease in Patients with Scleroderma Associated to Anti-PM/Scl Antibody.” Seminars in Arthritis & Rheumatism, 2014.
A. Haque, K. Divwedi, R. Lewis, A. Charalampopoulos, C. Elliot, A. Hameed, N. Hamilton, et al. “The Effect of Parenchymal Lung Disease on Patient Characteristics and Outcome in SSc-PH: Insights from the ASPIRE Registry.” 13.01 - Pulmonary Hypertension, 2022.
A. Hinze, Y. Radwan, M. Elnagar, R. Kurmann, S. Amin, R. Vassallo, C. Crowson, and B. Bartholmai. “POS0325 RADIOMIC BIOMARKER OF PULMONARY VASCULAR RELATED STRUCTURES PREDICTS MORTALITY IN SYSTEMIC SCLEROSIS,” 2021.
A. Hoffmann-Vold, H. Fretheim, A. Halse, M. Seip, H. Bitter, M. Wallenius, Anne Salberg, et al. “FRI0300 IMPACT OF INTERSTITIAL LUNG DISEASE IN SYSTEMIC SCLEROSIS IN A COMPLETE, NATIONWIDE COHORT.” Scleroderma, Myositis and Related Syndromes, 2019.
A. Hoffmann-Vold, H. Fretheim, A. Halse, M. Seip, H. Bitter, M. Wallenius, T. Garen, et al. “Tracking Impact of Interstitial Lung Disease in Systemic Sclerosis in a Complete Nationwide Cohort.” American Journal of Respiratory and Critical Care Medicine, 2019.
A. Janto, K. Triantafyllias, and A. Schwarting. “Einfluss Der Zeitdauer Bis Zur Diagnose Einer Systemischen Sklerose Auf Lungenfunktion Und Komorbiditäten: Eine Präklinische Und Klinische Analyse.” Zeitschrift Für Rheumatologie (Print), 2019.
A. Jhajj, Harinder Pal S. Gill, C. Hague, Darra T. Murphy, B. Elicker, Jeanette Soon, N. Sulaiman, et al. “Pulmonary Physiology Is Poorly Associated with Radiological Extent of Disease in Systemic Sclerosis-Associated Interstitial Lung Disease.” European Respiratory Journal, 2019.
A. Komócsi, A. Vorobcsuk, R. Faludi, T. Pintér, Z. Lenkey, Gyöngyvér Költő, and L. Czirják. “The Impact of Cardiopulmonary Manifestations on the Mortality of SSc: A Systematic Review and Meta-Analysis of Observational Studies.” Rheumatology, 2012.
A. Le Gall, A. Régent, L. Mouthon, M. Revel, G. Chassagnon, T.N. Hoang-Thi, A.T. Dinh Xuan, et al. “Intérêt Pronostique d’une Évaluation Informatisée Et Automatisée de l’atteinte Pulmonaire Interstitielle Au Scanner Au Cours de La Sclérodermie Systémique.” La Revue de Médecine Interne, 2021.
A. Lopes. “Is Computed Tomography the Ideal Method for the Identification and Management of Lung Disease in Systemic Sclerosis?” Radiologia Brasileira, 2016.
A. Lopes, Domenico Capone, R. Mogami, S. Menezes, F. Guimarães, and R. Levy. “Systemic Sclerosis-Associated Interstitial Pneumonia: Evaluation of Pulmonary Function over a Five-Year Period.” 2011.
A. Morales, Camila Pérez, N. Molano-González, A. Rojas-Villarraga, J. A. Carrillo, J. Anaya, and Especialista en Epidemiología. “Hallazgos Radiológicos de La Enfermedad Pulmonar En Pacientes Colombianos Con Esclerodermia,” 2015.
A. Morales-Cárdenas, Camila Pérez-Madrid, L. Arias, P. Ojeda, María P. Mahecha, A. Rojas-Villarraga, J. A. Carrillo-Bayona, and J. Anaya. “Pulmonary Involvement in Systemic Sclerosis.” Autoimmunity Reviews, 2016.
A. Onat, O. Zengin, M. Balcı, G. Kimyon, B. Kısacık, and O. Pamuk. “AB0670 The Survival and Prognostic Factors of Patients with Systemic Sclerosis: Experience of Two Centers,” 2015.
A. Taouch, W. Ammouri, F. Ibourk el Idrissi, W. Chawad, N. Mouatassim, H. Khibri, M. Maamar, H. Harmouche, Z. Tazi Mezalek, and M. Adnaoui. “AB0927 SYSTEMIC SCLERODERMA: ABOUT 60 CASES.” Annals of the Rheumatic Diseases, 2023.
Aëlle Le Gall, Trieu-Nghi Hoang-Thi, R. Porcher, B. Dunogué, A. Bérézné, L. Guillevin, V. le Guern, et al. “Prognostic Value of Automated Assessment of Interstitial Lung Disease on CT in Systemic Sclerosis.” Rheumatology, 2023.
Alec Peltekian, Karolina J. Senkow, Gorkem Durak, Kevin M Grudzinski, Bradford Bemiss, J.E. Dematte, Carrie Richardson, et al. “Imaging-Based Mortality Prediction in Patients with Systemic Sclerosis.” PRIME@MICCAI, 2025.
Ana Milena Arbeláez Solera, A. Bastidas, Gerardo Quintana, Rafael Valle Oñate, Elsa Reyes, Juan Manuel Bello, D. Jaimes, and Mabel Ávila. “Caracterización de Pacientes Con Compromiso Pulmonar Intersticial Asociado a Esclerosis Sistémica Atendidos En El Hospital Militar Central Desde Enero de 1998 a Mayo de 2008,” 2008.
“Autologous Hematopoietic Stem Cell Transplant in Systemic Sclerosis: Quantitative High Resolution Computed Tomography of the Chest Scoring.” Journal of Rheumatology, 2009.
B. Chang, F. Wigley, B. White, and R. Wise. “Scleroderma Patients with Combined Pulmonary Hypertension and Interstitial Lung Disease.” Journal of Rheumatology, 2003.
B. Demir, M. Can, S. Amikishiyev, N. Aliyeva, B. Artım-Esen, A. Gul, M. Inanç, and Y. Yalçınkaya. “POS1308 DISEASE RELATED ORGAN DAMAGE IN PATIENTS WITH SYSTEMIC SCLEROSIS: PROGRESS OF DAMAGE ACROSS CUTANEOUS SUBTYPES.” Annals of the Rheumatic Diseases, 2023.
B. Gillette. “Scleroderma Patients with Pulmonary Problems Face Higher Mortality Risk,” 2014.
Beatrice Moccaldi, M. Binda, Salvatore Prete, Andrea Martini, A. Cuberli, Maria Favaro, A. Doria, and Elisabetta Zanatta. “Predictors of Persistent Disease Activity and Damage in Systemic Sclerosis: Associations With Mortality in a Single-Center Cohort Study.” Journal of Clinical Rheumatology, 2025.
C. Bruni, M. Occhipinti, M. Pienn, G. Camiciottoli, M. Bartolucci, S. Bosello, Christian Payer, et al. “Lung Vascular Changes as Biomarkers of Severity in Systemic Sclerosis-Associated Interstitial Lung Disease.” Rheumatology, 2022.
C. Derk, G. Huaman, Jayne Littlejohn, F. Otieno, and S. Jimenez. “Predictors of Early Mortality in Systemic Sclerosis: A Case–Control Study Comparing Early Versus Late Mortality in Systemic Sclerosis.” Rheumatology International, 2012.
C. Iannone, M. R. Pellico, L. Morlacchi, V. Rossetti, M. Vicenzi, G. Trignani, P. Airò, et al. “OP0288 OUTCOMES IN PATIENTS WITH SYSTEMIC SCLEROSIS FOLLOWING LUNG TRANSPLANTATION: AN ITALIAN MULTICENTRE EXPERIENCE.” Scientific Abstracts, 2024.
C. Meier, M. Maciukiewicz, M. Brunner, J. Schniering, H. Gabryś, A. Kühnis, O. Distler, T. Frauenfelder, S. Tanadini-Lang, and B. Maurer. “POS0866 TWO-DIMENSIONAL HRCT-BASED RADIOMIC FEATURES IN SSC-ILD DISTINGUISH DRUG RESPONDERS FROM NON-RESPONDERS,” 2021.
C. Nita, L. Groșeanu, D. Opriș-Belinski, M. Popescu, A. Eddan, E. Langball, H. Fretheim, et al. “POS0838 SELECTIVE HRCT SCREENING IN AT-RISK SYSTEMIC SCLEROSIS PATIENTS REVEALS SUBSTANTIAL UNDERDIAGNOSIS OF INTERSTITIAL LUNG DISEASE.” Scientific Abstracts, 2024.
C. Santos, R. Rego Salgueiro, C. M. Morales, C. Castro, and E. Álvarez. “P147 Predictors of Interstitial Lung Involvement in Systemic Sclerosis.” Rheumatology, 2023.
C. Stock, Yang Nan, Yingying Fang, M. Kokosi, V. Kouranos, Peter M. George, Felix Chua, et al. “Deep-Learning CT Imaging Algorithm to Detect Usual Interstitial Pneumonia Pattern in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease: Association with Disease Progression and Survival.” Rheumatology, 2024.
Chiara Giraudo, G. Fichera, M. Binda, Beatrice Moccaldi, Elisabetta Cocconcelli, A. Cuberli, Anna Michielin, et al. “Does Body Composition Matter in Patients with Systemic Sclerosis?” Rheumatology, 2025.
Claudia Iannone, M. R. Pellico, L. Morlacchi, V. Rossetti, M. Vicenzi, Lorenzo Beretta, A. Severino, et al. “Pulmonary and Extra-Pulmonary Effects of Lung Transplantation in an Italian Cohort of Patients with Systemic Sclerosis.” Rheumatology, 2024.
D. Artene, and C. Ancuta. “AB0740 INTERSTITIAL LUNG DISEASE SYSTEMIC SCLEROSIS: AN EAST-EUROPEAN EUSTAR CENTER EXPERIENCE.” Annals of the Rheumatic Diseases, 2022.
D. Khanna, C. Tseng, Niloofar Farmani, V. Steen, D. Furst, P. Clements, M. Roth, et al. “Clinical Course of Lung Physiology in Patients with Scleroderma and Interstitial Lung Disease: Analysis of the Scleroderma Lung Study Placebo Group.” Arthritis & Rheumatism, 2011.
D. Khanna, V. Nagaraja, C. Tseng, F. Abtin, R. Suh, G. Kim, A. Wells, et al. “Predictors of Lung Function Decline in Scleroderma-Related Interstitial Lung Disease Based on High-Resolution Computed Tomography: Implications for Cohort Enrichment in Systemic Sclerosis–Associated Interstitial Lung Disease Trials.” Arthritis Research & Therapy, 2015.
D. Khorolskii, Alesya A. Klimenko, E. S. Pershina, E. P. Mikheeva, Natalya M. Babadayeva, A. Kondrashov, Nadezhda A. Shostak, and E. Zhilyaev. “PREDICTION OF INTERSTITIAL LUNG DISEASE PROGRESSION IN SYSTEMIC SCLEROSIS PATIENTS: A COHORT STUDY ANALYSIS.” The Bulletin of Contemporary Clinical Medicine, 2024.
D. Launay, M. Rémy-Jardin, U. Michon-Pasturel, I. Mastora, E. Hachulla, M. Lambert, V. Delannoy, et al. “High Resolution Computed Tomography in Fibrosing Alveolitis Associated with Systemic Sclerosis.” Journal of Rheumatology, 2006.
D. T. Wada, Fabrício Arantes de Almeida, Daniela A de Moraes, J. Dias, J. Baddini-Martinez, M. Oliveira, and M. Koenigkam-Santos. “Automatic Quantitative Computed Tomography Evaluation of the Lungs in Patients With Systemic Sclerosis Treated With Autologous Stem Cell Transplantation.” Journal of Clinical Rheumatology, 2019.
D. Tárnoki, Á. Tárnoki, V. Skakun, K. Karlinger, E. Bárczi, A. Bohács, and V. Müller. “Relationship Between HRCT Changes and Clinical Parameters in Patients with Scleroderma.” Imaging, 2018.
D. Tashkin, E. Volkmann, C. Tseng, Hyun J. Kim, J. Goldin, P. Clements, D. Furst, et al. “Relationship Between Quantitative Radiographic Assessments of Interstitial Lung Disease and Physiological and Clinical Features of Systemic Sclerosis.” Annals of the Rheumatic Diseases, 2014.
Daniel Sánchez-Cano, N. Ortego-Centeno, J. Callejas, V. Pla, R. Ríos-Fernandez, C. Tolosa-Vilella, Gerard Espinosa-Garriga, et al. “Interstitial Lung Disease in Systemic Sclerosis: Data from the Spanish Scleroderma Study Group.” Rheumatology International, 2018.
Daniela Castillo Saldana, C. Hague, Darra T. Murphy, H. Coxson, J. Tschirren, S. Peterson, J. Sieren, M. Kirby, and C. Ryerson. “Association of Computed Tomography Densitometry with Disease Severity, Functional Decline, and Survival in SSc-ILD.” Annals of the American Thoracic Society, 2020.
E. A. Vallejos, F. Felder, S. Leiva, J. Enghelmayer, R. Gomez, P. Rossi, F. Zenón, Gabriela Legarreta, S. Acuña, and Mariano M Volpacchio. “Predictors of Pulmonary-Functional Decline in Systemic Sclerosis: A University Hospital Experience.” ILD/DPLD of Known Origin, 2019.
E. Cozzani, A. Muracchioli, G. Murdaca, Mirko Beccalli, Simone Caprioli, P. Zentilin, P. Ameri, et al. “Correlation Between Skin and Affected Organs in 52 Sclerodermic Patients Followed in a Diseases Management Team: Development of a Risk Prediction Model of Organ-Specific Complications.” Frontiers in Immunology, 2021.
E. Edigin, V. Prado, M. Salazar, and H. Shaka. “SYSTEMIC SCLEROSIS WITH LUNG INVOLVEMENT INCREASES INPATIENT MORTALITY: ANALYSIS OF THE NATIONAL INPATIENT SAMPLE,” 2020.
E. Hachulla, P. Carpentier, V. Gressin, E. Diot, Y. Allanore, J. Sibilia, D. Launay, et al. “Risk Factors for Death and the 3-Year Survival of Patients with Systemic Sclerosis: The French ItinérAIR-Sclérodermie Study.” Rheumatology, 2008.
E. Volkmann, D. Tashkin, M. Sim, Ning Li, E. Goldmuntz, L. Keyes‐Elstein, A. Pinckney, et al. “Short-Term Progression of Interstitial Lung Disease in Systemic Sclerosis Predicts Long-Term Survival in Two Independent Clinical Trial Cohorts.” Annals of the Rheumatic Diseases, 2018.
E. Volkmann, D. Tashkin, Michael D. Roth, J. Goldin, and G. Kim. “Early Radiographic Progression of Scleroderma.” Chest, 2021.
E. Volkmann, D. Tashkin, Michael S. Roth, J. Goldin, and G. Kim. “OP0267 SHORT-TERM CHANGES IN THE RADIOGRAPHIC EXTENT OF INTERSTITIAL LUNG DISEASE PREDICT LONG-TERM MORTALITY IN SYSTEMIC SCLEROSIS,” 2021.
Ebru Özden Yılmaz, Döndü Üsküdar Cansu, and Cengiz Korkmaz. “Sklerodermalı Hastalarımızın Klinik Ve Laboratuvar Sonuçları Ve Prognozu: Tek Merkez Deneyimi.” OSMANGAZİ JOURNAL OF MEDICINE, 2018.
F. Bonella, and P. Caramaschi. “The Ambitious Goal of Validating Prognostic Biomarkers for Systemic Sclerosis-Related Interstitial Lung Disease.” Journal of Rheumatology, 2013.
F. Cacciapaglia, E. D. Lorenzis, A. Corrado, S. Bosello, M. Fornaro, F. Montini, L. Urso, et al. “FRI0230 THE 2009-2019 SURVIVAL AND MORTALITY PREDICTORS IN A LARGE MULTICENTRE SYSTEMIC SCLEROSIS COHORT.” Annals of the Rheumatic Diseases, 2020.
F. Cacciapaglia, M. Fornaro, F. Montini, L. Urso, S. Colella, L. Coladonato, M. G. Anelli, E. Praino, G. Lopalco, and F. Iannone. “AB0640 SURVIVAL AND MORTALITY PREDICTORS IN SYSTEMIC SCLEROSIS: RESULTS FROM A MONOCENTRIC ITALIAN COHORT.” Abstracts Accepted for Publication, 2019.
F. Godinho De Amorim, C. Verrastro, E. Santos, and C. Kayser. “POS1252 CALIPER PARAMETERS AS PREDICTORS OF MORTALITY IN SYSTEMIC SCLEROSIS: A LONGITUDINAL STUDY.” Annals of the Rheumatic Diseases, 2023.
Fathi Elbraky, Khaled D. Alsaeiti, Fathiyah M Aboulqasim, and Saleh M. Alawgali. “Demographic Characteristics and Clinical Manifestations of Interstitial Lung Disease with Systemic Sclerosis in Eastern Part of Libya.” Libyan International Medical University Journal, 2021.
Ferit zuhur, S. Zuhur, Cigdem Zuhur, B. Musellim, and G. Ongen. “Survival in Progressive Systemic Sclerosis with Pulmonary Involvement: A Single-Center Experience in Istanbul, Turkey.” Rheumatology International, 2012.
Fernanda Godinho de Amorim, Ernandez Rodrigues Dos Santos, Carlos Gustavo Yuji Verrastro, and Cristiane Kayser. “Quantitative Chest Computed Tomography Predicts Mortality in Systemic Sclerosis: A Longitudinal Study.” PLoS ONE, 2024.
G. Camiciottoli, I. Orlandi, M. Bartolucci, E. Meoni, F. Nacci, S. Diciotti, Chiara Barcaroli, et al. “Lung CT Densitometry in Systemic Sclerosis: Correlation with Lung Function, Exercise Testing, and Quality of Life.” Chest, 2007.
G. Ooi, M. Mok, K. Tsang, Y. Wong, P. Khong, P. Fung, Sophia S. C. Chan, et al. “Interstitial Lung Disease in Systemic Sclerosis: An HRCT-Clinical Correlative Study.” Acta Radiologica, 2003.
G. Pugnet, A. Petermann, S. Collot, P. Otal, P. Lansiaux, A. Maria, N. Ait Abdallah, et al. “POS0069 LONGITUDINAL CHANGES ON QUANTITATIVE CHEST HIGH-RESOLUTION COMPUTED TOMOGRAPHY IN EARLY DIFFUSE SYSTEMIC SCLEROSIS-RELATED INTERSTITIAL LUNG DISEASE AFTER AUTOLOGOUS HEMATOPOIETIC STEM CELL TRANSPLANTATION.” Annals of the Rheumatic Diseases, 2022.
G. Pugnet, A. Petermann, S. Collot, P. Otal, P. Lansiaux, N. A. Abdallah, G. Lorillon, et al. “Changes on Chest HRCT in Systemic Sclerosis-Related Interstitial Lung Disease After Autologous Haematopoietic Stem Cell Transplantation.” Rheumatology, 2022.
Gulfidan Cakmak, Tuba Selçuk Can, Ş. Gundogdu, C. Akman, Hande Ikitimur, B. Musellim, and G. Ongen. “Relationship Between Abnormalities on High-Resolution Computerized Tomography, Pulmonary Function, and Bronchoalveolar Lavage in Progressive Systemic Sclerosis.” Sarcoidosis Vasculities and Diffuse Lung Diseases, 2016.
H. Abida, Z. Meddeb, C. Abdelkéfi, A. El Ouni, S. Toujani, S. Hamzeoui, T. Larbi, and K. Bouslama. “AB0893 CORRELATIONS BETWEEN CT SCAN, CLINICAL AND RESPIRATORY FUNCTIONAL DATA IN SYSTEMIC SCLEROSIS ASSOCIATED INTERSTITIAL LUNG DISEASE.” Annals of the Rheumatic Diseases, 2023.
H. Kwon, E. Kang, J. Park, D. J. Go, E. Lee, Y. Song, Hyun‐Ju Lee, and Eun-Bong Lee. “A Decision Model for the Watch-and-Wait Strategy in Systemic Sclerosis-Associated Interstitial Lung Disease.” Rheumatology, 2015.
H. Kwon, E. Kang, J.K. Park, D. Go, E.Y. Lee, Y. Song, H.-J. Lee, and E.B. Lee. “AB0622 Clinical Characteristics of Systemic Sclerosis Patients with Interstitial Lung Disease Who Do Not Require Immunosuppressive Treatment,” 2014.
H. Poormoghim, M. M. Lakeh, M. Mohammadipour, Shima Talehy-Moineddin, and F. Sodagari. “Pulmonary Survival Study in 91 Patients with Systemic Sclerosis.” Rheumatology International, 2011.
H. Yabuuchi, Y. Matsuo, H. Tsukamoto, S. Sunami, T. Kamitani, S. Sakai, M. Hatakenaka, et al. “Correlation Between Pretreatment or Follow-up CT Findings and Therapeutic Effect of Autologous Peripheral Blood Stem Cell Transplantation for Interstitial Pneumonia Associated with Systemic Sclerosis.” European Journal of Radiology, 2011.
H. Yamakawa, T. Ogura, Shintaro Sato, T. Nishizawa, R. Kawabe, T. Oba, A. Kato, et al. “The Potential Utility of Anterior Upper Lobe Honeycomb-Like Lesion in Interstitial Lung Disease Associated with Connective Tissue Disease.” Respiratory Medicine, 2020.
Hyun J. Kim, D. Tashkin, D. Gjertson, Matthew S. Brown, E. Kleerup, S. Chong, J. Belperio, et al. “Transitions to Different Patterns of Interstitial Lung Disease in Scleroderma with and Without Treatment.” Annals of the Rheumatic Diseases, 2016.
I. Marie, St�phane Dominique, H. Lévesque, Philippe Ducrott�, P. Denis, M. Hellot, and H. Courtois. “Esophageal Involvement and Pulmonary Manifestations in Systemic Sclerosis.” Arthritis & Rheumatism, 2001.
J. Goldin, D. Lynch, D. Strollo, R. Suh, D. Schraufnagel, P. Clements, R. Elashoff, et al. “High-Resolution CT Scan Findings in Patients with Symptomatic Scleroderma-Related Interstitial Lung Disease.” Chest, 2008.
J. Goldin, R. Elashoff, Hyun J. Kim, X. Yan, D. Lynch, D. Strollo, M. Roth, et al. “Treatment of Scleroderma-Interstitial Lung Disease with Cyclophosphamide Is Associated with Less Progressive Fibrosis on Serial Thoracic High-Resolution CT Scan Than Placebo: Findings from the Scleroderma Lung Study.” Chest, 2009.
J. Jacob, B. Bartholmai, S. Rajagopalan, A. Brun, R. Egashira, R. Karwoski, M. Kokosi, A. Wells, and D. Hansell. “Evaluation of Computer-Based Computer Tomography Stratification Against Outcome Models in Connective Tissue Disease-Related Interstitial Lung Disease: A Patient Outcome Study.” BMC Medicine, 2016.
J. Morisset, B. Elicker, Xiaowen Hu, S. Le, J. Ryu, K. Jones, B. Ley, et al. “Predictors of Mortality and Risk Prediction Among Patients with Scleroderma Related Interstitial Lung Disease,” 2015.
J. Morisset, E. Vittinghoff, B. Elicker, Xiaowen Hu, S. Le, J. Ryu, K. Jones, et al. “Mortality Risk Prediction in Scleroderma‐Related Interstitial Lung Disease: The SADL Model.” Chest, 2017.
J. Raja, S. Muthusamy, and C. Ng. “AB0458 SURVIVAL OF SCLERODERMA PATIENTS WITH INTERSTITIAL LUNG DISEASE: OBSERVATION DATA FROM A MALAYSIAN TERTIARY CENTRE.” Annals of the Rheumatic Diseases, 2021.
J. Schniering, M. Maciukiewicz, H. Gabryś, M. Brunner, Christian Blüthgen, C. Meier, S. Braga-Lagache, et al. “Computed Tomography-Based Radiomics Decodes Prognostic and Molecular Differences in Interstitial Lung Disease Related to Systemic Sclerosis.” European Respiratory Journal, 2021.
J. Schniering, M. Maciukiewicz, H. Gabryś, M. Brunner, Christian Blüthgen, C. Meier, S. Braga-Lagache, et al. “Resolving Phenotypic and Prognostic Differences in Interstitial Lung Disease Related to Systemic Sclerosis by Computed Tomography-Based Radiomics.” medRxiv, 2020.
Jatin P. Singh, G. Kokenberger, Lucas Pu, Ernest G. Chan, Alaa Ali, Kaveh Moghbeli, Tong Yu, Chadi A. Hage, Pablo G. Sanchez, and J. Pu. “Predicting Post-Lung Transplant Survival in Systemic Sclerosis Using CT-Derived Features from Preoperative Chest CT Scans.” European Radiology, 2024.
Jonathan H. Chung, S. Montner, A. Adegunsoye, Cathryn T. Lee, J. Oldham, A. Husain, H. MacMahon, I. Noth, R. Vij, and M. Strek. “CT Findings, Radiologic-Pathologic Correlation, and Imaging Predictors of Survival for Patients With Interstitial Pneumonia With Autoimmune Features.” AJR. American Journal of Roentgenology, 2017.
K. Antoniou, D. Hansell, N. Goh, K. Samara, Elizabeth Renzoni, M. Toby, N. Siafakas, and A. Wells. “Scleroderma Lung Disease: A Scleroderma Physiologic Index (SPI) Derived from Lung Physiology and Oxygen Saturation,” 2013.
K. Mark. “Quantitative Thorax-CT-Analyse Bei Patienten Mit Systemischer Sklerose Vor Und Nach Autologer Stammzelltransplantation : Vergleich Der Ergebnisse Mit Lungenfunktionstests Und Mit Klinischen Parametern,” 2018.
K. Meridor, I. Sagy, and Y. Molad. “Anti-Ro/SS-A Antibody Is Associated with Worse Pulmonary Outcome and Reduced Overall Survival in Systemic Sclerosis.” Modern Rheumatology, 2021.
K. Morrisroe, D. Hansen, W. Stevens, L. Ross, J. Sahhar, G. Ngian, Catherine L. Hill, et al. “Predictors and Prognosis of Pulmonary Hypertension Complicating Interstitial Lung Disease in Systemic Sclerosis.” Rheumatology, 2024.
K. Morrisroe, W. Stevens, J. Sahhar, G. Ngian, N. Ferdowsi, D. Hansen, Shreeya Patel, et al. “The Clinical and Economic Burden of Systemic Sclerosis Related Interstitial Lung Disease.” Rheumatology, 2019.
K. Ninagawa, M. Kato, Yasuka Kikuchi, H. Sugimori, M. Kono, Y. Fujieda, I. Tsujino, and T. Atsumi. “Predicting the Response to Pulmonary Vasodilator Therapy in Systemic Sclerosis with Pulmonary Hypertension by Using Quantitative Chest CT.” Modern Rheumatology, 2022.
Khune Akash. “AB0665 OUTCOME OF INTERSTITIAL LUNG DISEASE IN PATIENTS WITH SCLERODERMA, A 5 YEAR PROSPECTIVE COHORT STUDY.” Abstracts Accepted for Publication, 2019.
L. Ananyeva, O. Ovsyannikova, V. Lesnyak, O. Koneva, O. Desinova, and M. Starovoytova. “AB0627 Slow Progressive Interstitial Lung Disease Associated with Systemic Sclerosis: Distinct Disease Phenotype?” 2014.
L. Cano-García, Aimara García-Studer, S. Manrique-Arija, F. Ortiz-Márquez, R. Redondo-Rodriguez, P. Borregón-Garrido, N. Mena-Vázquez, and Antonio Fernández-Nebro. “Accrual of Organ Damage and One-Year Mortality in Systemic Sclerosis: A Prospective Observational Study.” Seminars in Arthritis & Rheumatism, 2024.
L. Chung, R. Domsic, B. Lingala, Firas Alkassab, M. Bolster, M. Csuka, C. Derk, et al. “Survival and Predictors of Mortality in Systemic Sclerosis‐Associated Pulmonary Arterial Hypertension: Outcomes From the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry.” Arthritis Care & Research, 2014.
L. Gargani, C. Bruni, C. Romei, P. Frumento, A. Moreo, G. Ágoston, S. Guiducci, et al. “Prognostic Value of Lung Ultrasound B-Lines in Systemic Sclerosis.” Chest, 2020.
L. M. Díaz, R. G. Velez, and I. García. “AB0591 ANALYSIS OF A COHORT OF PATIENTS WITH SYSTEMIC SCLEROSIS AND INTERSTITIAL LUNG DISEASE,” 2020.
L. Méndez Díaz, R. G. Gil Velez, and I. Madroñal García. “AB0591 ANALYSIS OF A COHORT OF PATIENTS WITH SYSTEMIC SCLEROSIS AND INTERSTITIAL LUNG DISEASE.” Annals of the Rheumatic Diseases, 2020.
L. Schachna, T. Medsger, J. Dauber, F. Wigley, N. Braunstein, B. White, V. Steen, et al. “Lung Transplantation in Scleroderma Compared with Idiopathic Pulmonary Fibrosis and Idiopathic Pulmonary Arterial Hypertension.” Arthritis & Rheumatism, 2006.
L. V. de Oliveira Martins, Sandra Maximiano de Oliveira, Juliana Silvatti, Fernanda Godinho de Amorim, C. V. Agapito Tito, and C. Kayser. “Mortality in Systemic Sclerosis–Associated Interstitial Lung Disease in Brazil.” Journal of Clinical Rheumatology, 2021.
L. V. Teplova, Л. П. Ананьева, V. N. Lesnyak, Майя Николаевна Старовойтова, О. В. Денисова, Т. А. Невская, and Елена Николаевна Александрова. “СИСТЕМНАЯ СКЛЕРОДЕРМИЯ С ИНТЕРСТИЦИАЛЬНЫМПОРАЖЕНИЕМ ЛЕГКИХ: СРАВНИТЕЛЬНАЯ КЛИНИЧЕСКАЯХАРАКТЕРИСТИКА С БОЛЬНЫМИ БЕЗ ПОРАЖЕНИЯ ЛЕГКИХ.” RSP, 2010.
Lize Vanaken, Nicholas Landini, J. Lenaerts, E. Claeys, J. Lenaerts, W. Wuyts, J. Verschakelen, and E. De Langhe. “Progressive Lung Fibrosis and Mortality Can Occur in Early Systemic Sclerosis Patients Without Pulmonary Abnormalities at Baseline Assessment.” Clinical Rheumatology, 2020.
M. Aringer. “Verlauf Der Interstitiellen Lungenerkrankung Bei Systemischer Sklerose.” Kompass Autoimmun, 2020.
———. “Verlauf Der Interstitiellen Lungenerkrankung Bei Systemischer Sklerose.” Kompass Pneumologie, 2021.
M. Breda, M. Lazzaroni, F. Franceschini, and P. Airó. “POS0324 SMOKING HISTORY AND SCLERODERMA CLINICAL TRIALS CONSORTIUM DAMAGE INDEX (SCTC-DI) AS INDEPENDENT PREDICTORS OF MORTALITY IN SYSTEMIC SCLEROSIS IN A SINGLE-CENTRE ITALIAN COHORT,” 2021.
M. Colaci, M. Sebastiani, A. Manfredi, D. Giuggioli, G. Cassone, C. U. Manzini, Cecilia Ghizzoni, S. Cerri, and C. Ferri. “Lung Involvement in Systemic Sclerosis: Role of High Resolution Computed Tomography and Its Relationship with Other Pulmonary and Clinico-Serological Features.” Journal of Biological Regulators and Homeostatic Agents, 2014.
M. Freire, M. Alonso, E. Alvarez, L. Gonzalez, A. Rivera, and B. Sopeña. “AB0823 Scleroderma and Silica: Erasmus Syndrome in West North Spain,” 2013.
M. Furukawa, E.G. Chan, J. Ryan, Chadi A. Hage, and Pablo G. Sanchez. “Outcomes of Lung Transplantation for Scleroderma Versus Other Indications: Insigts from a Single Center.” JHLT Open, 2025.
M. I. Acosta Colmán, G. Avila Pedretti, M. Acosta, C. P. Simeón Aznar, Vicent Fonollosa Plá, and Miquel Villardel Torrés. “Can We Predict the Severity of Pulmonary Hypertension in Patients with Scleroderma?” Reumatología Clínica, 2012.
M. Jacob, A. Mathew, R. Goel, and John Mathew. “Clinical Profile and Outcome of Pulmonary Hypertension With and Without Interstitial Lung Disease in Scleroderma.” Journal of Clinical Rheumatology and Immunology, 2024.
M. Lazzaroni, C. Campochiaro, E. Marasco, J. D. de Vries-Bouwstra, F. Franceschini, F. del Galdo, C. Denton, et al. “SAT0328 OUTCOME OF INTERSTITIAL LUNG DISEASE (ILD) IN ANTI-PM/SCL PATIENTS WITH SYSTEMIC SCLEROSIS: RESULTS FROM AN EUSTAR CASE-CONTROL STUDY.” 2020.
M. Lazzaroni, L. Moschetti, Marta Breda, F. Franceschini, and P. Airò. “Long-Term Organ Damage Accrual and Late Mortality in Systemic Sclerosis.” Clinical and Experimental Rheumatology, 2023.
M. Maciukiewicz, J. Schniering, H. Gabryś, M. Brunner, Christian Blüthgen, C. Meier, M. Guckenberger, et al. “OP0150 MACHINE LEARNING APPROACHES FOR RISK MODELLING IN INTERSTITIAL LUNG DISEASE ASSOCIATED WITH SYSTEMIC SCLEROSIS USING HIGH DIMENSIONAL IMAGE ANALYSIS,” 2021.
M. Marius, T. Xenitidis, I. Koetter, N. Konstantin, J. Henes, and C. Kloth. “AB0619 Autologous Non-Myeloablative Hematopoietic Stem Cell Transplantation for Systemic Sclerosis: The Benefit of Quantitative Chest-CT Analysis for Response of Lung Involvement and Comparison with Results of Pulmonary Function Tests and Clinical Tests,” 2016.
M. Massad, C. Powell, J. Kpodonu, Cimenga Tshibaka, Ziad Hanhan, N. Snow, and A. Geha. “Outcomes of Lung Transplantation in Patients with Scleroderma.” World Journal of Surgery, 2005.
M. Mayes. “Race, Scleroderma, and Survival: Why Is There a Difference?” Journal of Rheumatology, 2005.
M. Nazarinia, E. Esmaeilzadeh, Yasaman Emami, and A. Salehi. “One Decade Distinct Features, Morbidity and Mortality of Scleroderma: A Cross-Sectional Study.” Clinical and Experimental Rheumatology, 2016.
M. Nikpour, and M. Baron. “Mortality in Systemic Sclerosis: Lessons Learned from Population-Based and Observational Cohort Studies.” Current Opinion in Rheumatology, 2014.
M. Ninaber, J. Stolk, J. Smit, Ernest J Le Roy, L. Kroft, M. E. Bakker, Jeska K de Vries Bouwstra, A. Schouffoer, M. Staring, and B. Stoel. “Lung Structure and Function Relation in Systemic Sclerosis: Application of Lung Densitometry.” European Journal of Radiology, 2015.
M. Okamoto, K. Fujimoto, Junko Sadohara, Kiyomi Furuya, S. Kaieda, T. Miyamura, E. Suematsu, et al. “A Retrospective Cohort Study of Outcome in Systemic Sclerosis-Associated Interstitial Lung Disease.” Respiratory Investigation, 2016.
M. Ozmen, C. Gumus, E. Otman, Kazım Ayberk Sinci, İ. Kurut Aysin, D. Solmaz, and S. Akar. “Tomographic Fibrosis Score in the Patients with Systemic Sclerosis-Associated Interstitial Lung Disease.” European Journal of Rheumatology, 2023.
M. Park. “Recent Advances in Predicting Mortality and Progression of Systemic Sclerosis–Associated Interstitial Lung Disease.” Tuberculosis and Respiratory Diseases, 2020.
M. Rojas-Giménez, R. Castro, C. López-Medina, J. C. Gutiérrez, M. Aguirre-Zamorano, and A. E. Contreras. “AB0432 SURVIVAL AND PROGNOSIS IN SYSTEMIC SCLEROSIS: RESULTS FROM A SINGLE-CENTER COHORT,” 2021.
M. Sieren, H. Grasshoff, G. Riemekasten, Lennart Berkel, F. Nensa, R. Hosch, Jörg Barkhausen, Roman Kloeckner, and Franz Wegner. “Computed Tomography-Derived Quantitative Imaging Biomarkers Enable the Prediction of Disease Manifestations and Survival in Patients with Systemic Sclerosis.” RMD Open, 2025.
M. Vlachou, H. Fayed, Adele Dawson, S. Reddecliffe, Alexander Stevenson, R. Thomson, B. Schreiber, and J. Coghlan. “Intravenous Prostanoids in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: A Single Centre Experience.” Rheumatology, 2021.
M. Vonk, W. V. D. Hombergh, H. Knaapen, F. V. D. Hoogen, and Jaap Fransen. “AB0652 Early Organ Involvement, Survival and Causes of Death in an Unselected Cohort of Dutch Patients with Systemic Sclerosis,” 2017.
M. Vonk, W. V. D. Hombergh, H. Knaapen, S. Simons, J. Fransen, and F. V. D. Hoogen. “AB0652 Early Organ Involvement, Survival and Causes of Death in an Unselected Cohort of Dutch Patients with Systemic Sclerosis,” 2017.
M. Zompatori, M. Leone, M. Giannotta, N. Galiè, M. Palazzini, M. Reggiani, L. Bono, and Gian Stefano Pollini. “Pulmonary Hypertension and Systemic Sclerosis: The Role of High-Resolution Computed Tomography.” La Radiologia Medica, 2013.
Maddalena Angela Di Lellis. “Systemische Sklerose - HRCT Erlaubt Aussage Über Behandlungserfolg Bei ILD.” Aktuelle Rheumatologie, 2016.
———. “Systemische Sklerose - HRCT Erlaubt Aussage Über Behandlungserfolg Bei ILD,” 2016.
Maria M. Crespo, Christian A. Bermudez, M. Dew, B. Johnson, M. P. George, J. Bhama, M. Morrell, et al. “Lung Transplant in Patients with Scleroderma Compared with Pulmonary Fibrosis. Short- and Long-Term Outcomes.” Annals of the American Thoracic Society, 2016.
Michelle J. Connolly, S. Abdullah, Deborah A. Ridout, Benjamin E Schreiber, J. Haddock, and J. G. Coghlan. “Prognostic Significance of Computed Tomography Criteria for Pulmonary Veno-Occlusive Disease in Systemic Sclerosis-Pulmonary Arterial Hypertension.” Rheumatology, 2017.
N. Azizah. “PROFIL KLINIS, TATA LAKSANA, DAN PROGNOSIS PASIEN SKLERODERMA DI INSTALASI RAWAT JALAN REUMATOLOGI RSUD DR. SOETOMO PERIODE 01 JANUARI 2016- 31 DESEMBER 2018,” 2019.
N. Gerolymatou, N. Koletsos, M. Karakosta, T. Memi, A. Drosos, and P. Voulgari. “AB0838 PULMONARY AND CARDIOVASCULAR MANIFESTATIONS IN SYSTEMIC SCLEROSIS: RESULTS FROM A SSC PATIENTS COHORT AT A TERTIARY CENTRE OF NW GREECE.” Annals of the Rheumatic Diseases, 2023.
N. Goh, R. Hoyles, C. Denton, D. Hansell, E. Renzoni, T. Maher, A. Nicholson, and A. Wells. “Short‐Term Pulmonary Function Trends Are Predictive of Mortality in Interstitial Lung Disease Associated With Systemic Sclerosis.” Arthritis & Rheumatology, 2017.
N. Landini, M. Orlandi, C. Bruni, E. Carlesi, C. Nardi, L. Calistri, G. Morana, S. Tomassetti, S. Colagrande, and M. Matucci-Cerinic. “Computed Tomography Predictors of Mortality or Disease Progression in Systemic Sclerosis–Interstitial Lung Disease: A Systematic Review.” Frontiers in Medicine, 2022.
N. Le Gouellec, A. Duhamel, J. Faivre, L. Guillaume, V. Sobanski, T. Pérez, E. Hachulla, P. Hatron, M. Rémy-Jardin, and D. Launay. “AB0614 Prognostic Factors of Functional Outcome in Systemic Sclerosis-Associated Interstitial Lung Disease,” 2014.
N. Le Gouellec, J. Faivre, A. Hachulla, E. Hachulla, T. Pérez, S. Morell-Dubois, P. Hatron, M. Rémy-Jardin, and D. Launay. “FRI0254 Prognosis Factors for Survival and Progression-Free Survival in Systemic Sclerosis–Associated Interstitial Lung Disease,” 2013.
O. Koneva, L. Garzanova, O. Ovsyannikova, N. Kovaleva, L. Ananyeva, O. Desinova, M. Starovoytova, and R. Shayakhmetova. “AB0726 Study of the Association of Various X-Ray Symptoms of Interstitial Lung Disease (ILD) in Patients with Systemic Scleroderma(SSc) with Indices of Lung Functional Tests.” Annals of the Rheumatic Diseases, 2022.
O. Koneva, Nataliya Kovaleva, L. Garzanova, Olga Ovsynnikova, O. Desinova, L. Ananyeva, M. Starovoytova, and R. Shayakhmetova. “DYNAMICS OF PULMONARY FUNCTIONAL TESTS (PFTS) AND RADIOGRAPHY DURING IMMUNOSUPPRESSIVE THERAPY (IST) OF INTERSTITIAL LUNG DISEASE (ILD) IN SYSTEMIC SCLEROSIS (SSC).” Chest, 2022.
O. Meyer. “Prognostic Markers for Systemic Sclerosis.” Joint, Bone, Spine : Revue Du Rhumatisme, 2006.
O. Moore, N. Goh, T. Corte, H. Rouse, O. Hennessy, V. Thakkar, J. Byron, et al. “FRI0243 Extent of Disease on High-Resolution CT Lung Is a Predictor of Decline and Mortality in Systemic Sclerosis-Related Interstitial Lung Disease,” 2013.
———, et al. “THU0235 Identifying and Quantifying Prognostic Factors in Systemic Sclerosis-Related Interstitial Lung Disease Using a Time-Varying Covariate Survival Model,” 2013.
O. Ovsyannikova, L. Ananyeva, O. Koneva, L. Garzanova, R. Shayakhmetova, O. Desinova, M. Starovoytova, and Anna KHELKOVSKAYA-SERGEEVA. “ASSOCIATION OF EUROPEAN SCLERODERMA STUDY GROUP ACTIVITY INDEX WITH PROGRESSION PULMONARY ALTERATIONS BY HRCT IN PATIENTS WITH SYSTEMIC SCLEROSIS OVER A 5-YEAR PERIOD.” Chest, 2023.
O. Ovsyannikova, and O. Koneva. “AB0633 Association of Decrease Diffusing Lung Capacity with Progression Pulmonary Alterations by HRCT in Patients with Systemic Sclerosis over A Five-Year Period,” 2014.
O. Ovsyannikova, O. Koneva, L. Ananieva, L. Garzanova, and O. Desinova. “RELATIONSHIP BETWEEN LUNG FUNCITON TEST AND DIGITAL ULCERS (DUS) IN PATIENTS WITH SYSTEMIC SCLEROSIS (SSC) OVER A FIVE-YEAR PERIOD,” 2020.
O. Salamo, Rodeo Abrencillo, E. Bernstein, John Varga, Francesco Boin, S. Assassi, and Isabel C MIRA-AVENDANO. “IS THE DEGREE OF SKIN FIBROSIS MEASURED BY THE MODIFIED RODNAN SKIN SCORE DIFFERENT BETWEEN PATIENTS WITH PROGRESSIVE SCLERODERMA-RELATED INTERSTITIAL LUNG DISEASE?” Chest, 2023.
P. Bauer, D. Schiavo, T. Osborn, D. Levin, J. S. St. Sauver, A. Hanson, D. Schroeder, and J. Ryu. “Influence of Interstitial Lung Disease on Outcome in Systemic Sclerosis: A Population-Based Historical Cohort Study.” Chest, 2013.
P. Fisichella, N. Reder, J. Gagermeier, and E. Kovacs. “Usefulness of pH Monitoring in Predicting the Survival Status of Patients with Scleroderma Awaiting Lung Transplantation.” Journal of Surgical Research, 2014.
P. Pradère, I. Tudorache, J. Magnusson, L. Savale, O. Brugière, B. Douvry, M. Reynaud‐Gaubert, et al. “Lung Transplantation for Scleroderma Lung Disease: An International, Multicenter, Observational Cohort Study.” The Journal of Heart and Lung Transplantation, 2018.
Pimchanok Palawisut, Duangkamol Aiewruengsurat, P. Kaenmuang, A. Navasakulpong, and W. Tanomkiat. “Pulmonary Function Change and Survival of Systemic Sclerosis-Associated Interstitial Lung Disease in Different Imaging Patterns: A 20-Year Retrospective Study.” Journal of Clinical Rheumatology and Immunology, 2025.
R. Castro, R. Mariscal-Ocaña, M. Rojas-Giménez, J. C. Gutiérrez, A. E. Contreras, C. López-Medina, N. Shamsutdinova, et al. “AB0451 TO EVALUATE THE PREVALENCE OF INTERSTITIAL LUNG DISEASE (ILD) AND/OR PULMONARY ARTERIAL HYPERTENSION (PAH) IN PATIENTS AFFECTED BY SYSTEMIC SCLEROSIS (SSC) AND TO DETERMINE THE FACTORS ASSOCIATED WITH ILD.” Annals of the Rheumatic Diseases, 2021.
R. Janardana, A. Irodi, P. Chebbi, L. Rv, D. Danda, and G. Mahasampath. “OP0145 Scleroderma Related Interstitial Lung Disease and Mycophenolate : Long Term Outcomes.” THURSDAY, 14 JUNE 2018, 2018.
R. Su, Mihoko V Bennett, S. Jacobs, T. Hunter, C. Bailey, E. Krishnan, G. Rosen, and L. Chung. “An Analysis of Connective Tissue Disease-Associated Interstitial Lung Disease at a US Tertiary Care Center: Better Survival in Patients with Systemic Sclerosis.” Journal of Rheumatology, 2011.
R. Takei, M. Arita, S. Kumagai, Yuhei Ito, F. Tokioka, T. Koyama, Rintaro Saito, K. Nishimura, Hironobu Tokumasu, and T. Ishida. “Radiographic Fibrosis Score Predicts Survival in Systemic Sclerosis‐associated Interstitial Lung Disease.” Respirology (Carlton South. Print), 2018.
Robert W Koschik, N. Fertig, M. Lucas, R. Domsic, and T. Medsger. “Anti-PM-Scl Antibody in Patients with Systemic Sclerosis.” Clinical and Experimental Rheumatology, 2012.
Roozbeh Sharif, Maureen D. Mayes, F. Tan, O. Gorlova, L. Hummers, Ami A. Shah, D. Furst, et al. “IRF5 Polymorphism Predicts Prognosis in Patients with Systemic Sclerosis.” Annals of the Rheumatic Diseases, 2012.
S. Amikishiyev, Y. Yalçınkaya, K. Mammadova, N. Aliyeva, G. Durak, B. Artım-Esen, A. Gül, A. Bilge, G. Okumus, and Murat Inanç. “Mortality and Associated Factors in Patients with Systemic Sclerosis Associated Pulmonary Hypertension with and Without Interstitial Lung Disease: A Long-Term Follow-up Study.” Modern Rheumatology, 2024.
S. Assassi, D. D. del Junco, K. Sutter, T. Mcnearney, J. Reveille, A. Karnavas, P. Gourh, et al. “Clinical and Genetic Factors Predictive of Mortality in Early Systemic Sclerosis.” Arthritis & Rheumatism, 2009.
S. Dewi, Waliyyuddin Robbani, and I. D. Kulsum. “Lung Abnormalities in Systemic Sclerosis Patients Through Spirometry, Chest X-Ray, and High-Resolution Computed Tomography Scan.” Althea Medical Journal, 2022.
S. Günther, S. Maître, A. Bérézné, X. Jaïs, A. Seferian, O. Sitbon, G. Simonneau, L. Mouthon, M. Humbert, and D. Montani. “Frequency and Impact on Prognosis of Signs of Pulmonary Veno-Occlusive Disease on High Resolution Computed Tomography in Patients with Scleroderma Associated Pulmonary Arterial Hypertension,” 2011.
S. Jacobsen, S. Ullman, G. Shen, A. Wiik, and P. Halberg. “Influence of Clinical Features, Serum Antinuclear Antibodies, and Lung Function on Survival of Patients with Systemic Sclerosis.” Journal of Rheumatology, 2001.
S. Kalender, T. Demirci Yildirim, F. Dulger, S. Gulle, and M. Birlik. “POS0816 HRCT ANNUAL SCORE: IT MAY BE USEFUL TO MONITOR HRCT ANNUAL SCORE TO TRACK PULMONARY INVOLVEMENT IN SYSTEMIC SCLEROSIS.” Scientific Abstracts, 2024.
S. Keret, Y. Braun-Moscovici, M. Yigla, V. Shataylo, L. Guralnik, and A. Balbir-Gurman. “POS0871 CHARACTERISTICS OF INTERSTITIAL LUNG DISEASE IN PATIENTS WITH SYSTEMIC SCLEROSIS DURING LONG TERM FOLLOW-UP, SINGLE CENTER EXPERIENCE,” 2021.
S. Mathai, L. Hummers, H. Champion, F. Wigley, A. Zaiman, P. Hassoun, and R. Girgis. “Survival in Pulmonary Hypertension Associated with the Scleroderma Spectrum of Diseases: Impact of Interstitial Lung Disease.” Arthritis & Rheumatism, 2009.
S. Nihtyanova, B. Schreiber, V. Ong, Daniel W. Rosenberg, P. Moinzadeh, J. Coghlan, A. Wells, and C. Denton. “Prediction of Pulmonary Complications and Long‐Term Survival in Systemic Sclerosis.” Arthritis & Rheumatology, 2014.
S. Nihtyanova, and C. Denton. “Scleroderma Lung Involvement, Autoantibodies, and Outcome Prediction: The Confounding Effect of Time.” Journal of Rheumatology, 2017.
S. Panopoulos, V. Bournia, G. Konstantonis, K. Fragiadaki, P. Sfikakis, and M. Tektonidou. “Predictors of Morbidity and Mortality in Early Systemic Sclerosis: Long-Term Follow-up Data from a Single-Centre Inception Cohort.” Autoimmunity Reviews, 2018.
S. Patiwetwitoon, S. Wangkaew, J. Euathrongchit, N. Kasitanon, and W. Louthrenoo. “High-Resolution Computed Tomographic Findings in Systemic Sclerosis–Associated Interstitial Lung Disease: Comparison Between Diffuse and Limited Systemic Sclerosis.” Journal of Clinical Rheumatology, 2012.
S. Plastiras, S. Karadimitrakis, P. Ziakas, P. Vlachoyiannopoulos, H. Moutsopoulos, and G. Tzelepis. “Scleroderma Lung: Initial Forced Vital Capacity as Predictor of Pulmonary Function Decline.” Arthritis & Rheumatism, 2006.
S. Sousa, A. Duarte, I. Cordeiro, L. Teixeira, J. Canas da Silva, A. Cordeiro, and M. Santos. “AB0636 Interstitial Lung Disease in Scleroderma Portuguese Patients,” 2016.
S. V. Kocheril, B. Appleton, E. Somers, E. Kazerooni, K. Flaherty, F. Martinez, B. Gross, and L. Crofford. “Comparison of Disease Progression and Mortality of Connective Tissue Disease-Related Interstitial Lung Disease and Idiopathic Interstitial Pneumonia.” Arthritis & Rheumatism, 2005.
S. Walsh, N. Sverzellati, A. Devaraj, G. Keir, A. Wells, and D. Hansell. “Connective Tissue Disease Related Fibrotic Lung Disease: High Resolution Computed Tomographic and Pulmonary Function Indices as Prognostic Determinants.” Thorax, 2013.
S. Wangkaew, J. Euathrongchit, P. Watanawittawas, N. Kasitanon, S. Puntana, and W. Louthrenoo. “SAT0478 Incidence And Predictors of Interstitial Lung Disease (ILD) in Early Systemic Sclerosis Patients: Inception Cohort Study,” 2015.
S. Wangkaew, J. Euathrongchit, Pittaporn Wattanawittawas, and N. Kasitanon. “Correlation of Delta High-Resolution Computed Tomography (HRCT) Score with Delta Clinical Variables in Early Systemic Sclerosis (SSc) Patients.” Quantitative Imaging in Medicine and Surgery, 2016.
Siahmet Atlı, Gamze Kırkıl, Mutlu Kutluöztürk, Önsel Öner, S. Koca, Ayşe Murat Aydın, and S. Doğru. “Parameters Effective on Survival in Connective Tissue Disease-Related Interstitial Lung Disease.” Journal of Health Sciences and Medicine, 2024.
Società Italiana Di Reumatologia. “PO:32:185 | Deep-Learning Analysis of High-Resolution Computed Tomography Images Predicts Progression and Mortality in Systemic Sclerosis Related Interstitial Lung Disease.” Reumatismo, 2025.
“SSc-ILD: Reflux Und Ösophagusdilatation Keine Prädiktoren Für Krankheitsverlauf.” Aktuelle Rheumatologie, 2024.
Stefan Nowak, D. Jaffuel, Jacques Morel, E. Ahmed, A. Gamez, C. Boissin, J. Charriot, Nicolas Molinari, and Arnaud Bourdin. “Phenotypes and Prognosis of Systemic Sclerosis: A Cluster Analysis.” Respiratory Medical Research, 2025.
T. Odani, S. Yasuda, M. Kono, T. Kurita, Y. Fujieda, K. Otomo, Y. Kon, T. Horita, and T. Atsumi. “THU0256 Effectiveness of Autologous Hematopoietic Stem Cell Transplantation for Interstitial Lung Diseases in Patients with Systemic Sclerosis,” 2013.
Tiffany A Winstone, D. Assayag, P. Wilcox, J. Dunne, C. Hague, J. Leipsic, H. Collard, and C. Ryerson. “Predictors of Mortality and Progression in Scleroderma-Associated Interstitial Lung Disease: A Systematic Review.” Chest, 2014.
V. León, W. I. Girón Matute, F. Serrano, B. S. Benavente, C. C. Riera, and L. P. Maestú. “SADL Scale as a Predictor of Mortality in Patients with ILD Associated with Scleroderma.” ILD / DPLD of Known Origin, 2021.
V. Steen, and T. Medsger. “Changes in Causes of Death in Systemic Sclerosis, 1972–2002.” Annals of the Rheumatic Diseases, 2007.
Vincent Sobanski, J. D. de Vries-Bouwstra, A. Hoffmann-Vold, D. Huscher, Margarida Alves, M. Matucci-Cerinic, G. Riemekasten, et al. “Lung Function and Skin Fibrosis Changes as Predictors of Survival in SSc-Associated Interstitial Lung Disease: A EUSTAR Study.” Rheumatology, 2025.
W. Chebbi, S. Ayed, N. Boudawara, L. Boussoffara, S. Mahjoub, M. Sfar, and J. Knani. “Frequency and Impact of Interstitial Lung Disease on Clinical State and Mortality in Systemic Scleroderma,” 2012.
W. Wu, S. Jordan, N. Graf, J. Pena, J. Curram, Y. Allanore, M. Matucci-Cerinic, et al. “SAT0513 Progressive Skin Fibrosis Is Associated with a Decline in Lung Function and Poorer Survival in Patients with Diffuse Cutaneous Systemic Sclerosis: A European Scleroderma Trials and Research (EUSTAR) Analysis.” Saturday, 16 JUNE 2018, 2018.
Wanlong Wu, S. Jordan, N. Graf, J. de Oliveira Pena, J. Curram, Y. Allanore, M. Matucci-Cerinic, et al. “Progressive Skin Fibrosis Is Associated with a Decline in Lung Function and Worse Survival in Patients with Diffuse Cutaneous Systemic Sclerosis in the European Scleroderma Trials and Research (EUSTAR) Cohort.” Annals of the Rheumatic Diseases, 2019.
Y. Ahmad, Christine Zhou, K. Olmstead, Sarah Khan, J. Windholz, N. Gupta, J. Gomez-Arroyo, and J.M. Elwing. “SYSTEMIC SCLEROSIS-ASSOCIATED PRECAPILLARY PULMONARY HYPERTENSION WITH AND WITHOUT INTERSTITIAL LUNG DISEASE: OBSERVATIONS FROM A SINGLE REFERRAL CENTER COHORT,” 2020.
Y. Gc, R. Samant, A. Mahashur, P. Sagdeo, and G. Kakade. “AB0627 Predictors of Ild and Its Progression in A Cohort of Scleroderma Patients from Western India: A Prospective Observational Cohort Study,” 2016.
Л. П. Ананьева. “Интерстициальное Поражение Легких, Ассоциированное с Системной Склеродермией (Прогрессирующим Системным Склерозом),” 2017.
О. А. Конева, О. В. Десинова, Л. П. Ананьева, and Наталья Васильевна Ковалева. “Значение Компьютерной Томографии Высокого Разрешения в Оценке Эффективности Ритуксимаба у Пациентов с Интерстициальным Поражением Легких При Системной Склеродермии,” 2018.
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