Publications

Building our Knowledge Base

Recent Publications

Scleroderma

Computed Tomography of the Chest to Screen for Interstitial Lung Disease in Patients With Systemic Sclerosis

Bernstein, Elana J., Shervin Assassi, Flavia V. Castelino, Lorinda Chung, Chase Correia, Luke B. Evnin, Tracy M. Frech, et al. “Computed Tomography of the Chest to Screen for Interstitial Lung Disease in Patients with Systemic Sclerosis at Expert Scleroderma Centers in the United States.” ACR Open Rheumatology, 2022. https://doi.org/10.1002/acr2.11434.

 Baseline characteristics of systemic sclerosis patients with restrictive lung disease in a multi-center US-based longitudinal registry

Castelino, Flavia V., John M. VanBuren, Emily Startup, Shervin Assassi, Elana J. Bernstein, Lorinda Chung, Chase Correia, et al. “Baseline Characteristics of Systemic Sclerosis Patients with Restrictive Lung Disease in a Multi‐Center Us‐Based Longitudinal Registry.” International Journal of Rheumatic Diseases 25, no. 2 (2021): 163–74. https://doi.org/10.1111/1756-185x.14253.

Randomized feasibility trial of the Scleroderma Patient-centered Intervention Network Self-Management (SPIN-SELF) Program

Kwakkenbos, Linda, Nora Østbø, Marie-Eve Carrier, Warren R. Nielson, Claire Fedoruk, Brooke Levis, Richard S. Henry, et al. “Randomized Feasibility Trial of the Scleroderma Patient-Centered Intervention Network Self-Management (Spin-Self) Program.” Pilot and Feasibility Studies 8, no. 1 (2022). https://doi.org/10.1186/s40814-022-00994-5.

Effect of Coping Strategies on Patient and Physician Perceptions of Disease Severity and Disability in Systemic Sclerosis

DiRenzo, Dana D., Theresa R. Smith, Tracy M. Frech, Ami A. Shah, and John D. Pauling. “Effect of Coping Strategies on Patient and Physician Perceptions of Disease Severity and Disability in Systemic Sclerosis.” The Journal of Rheumatology 48, no. 10 (2021): 1569–73. https://doi.org/10.3899/jrheum.201612.

The frequency of Raynaud’s phenomenon, very early diagnosis of systemic sclerosis, and systemic sclerosis in a large Veteran Health Administration database

Frech, Tracy M., Maureen A. Murtaugh, Megan Amuan, and Mary Jo Pugh. “The Frequency of Raynaud’s Phenomenon, Very Early Diagnosis of Systemic Sclerosis, and Systemic Sclerosis in a Large Veteran Health Administration Database.” BMC Rheumatology 5, no. 1 (2021). https://doi.org/10.1186/s41927-021-00209-z.

A comprehensive framework for navigating patient care in systemic sclerosis

Saketkoo, Lesley Ann, Tracy Frech, Cecília Varjú, Robyn Domsic, Jessica Farrell, Jessica K. Gordon, Carina Mihai, et al. “A Comprehensive Framework for Navigating Patient Care in Systemic Sclerosis: A Global Response to the Need for Improving the Practice of Diagnostic and Preventive Strategies in SSC.” Best Practice & Research Clinical Rheumatology 35, no. 3 (2021): 101707. https://doi.org/10.1016/j.berh.2021.101707.

Dysphagia, reflux and related sequelae due to altered physiology in scleroderma

Kadakuntla, Anusri, Ankit Juneja, Samantha Sattler, Anusha Agarwal, Drishti Panse, Nardin Zakhary, Anusha Pasumarthi, Lee Shapiro, and Micheal Tadros. “Dysphagia, Reflux and Related Sequelae Due to Altered Physiology in Scleroderma.” World Journal of Gastroenterology 27, no. 31 (2021): 5201–18. https://doi.org/10.3748/wjg.v27.i31.5201.

Exercise as a multi-modal disease-modifying medicine in systemic sclerosis

Pettersson, Henrik, Helene Alexanderson, Janet L. Poole, Janos Varga, Malin Regardt, Anne-Marie Russell, Yasser Salam, et al. “Exercise as a Multi-Modal Disease-Modifying Medicine in Systemic Sclerosis: An Introduction by the Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis (G-Forss).” Best Practice & Research Clinical Rheumatology 35, no. 3 (2021): 101695. https://doi.org/10.1016/j.berh.2021.101695.

A randomised, double-blind, placebo-controlled phase 3 study of lenabasum in diffuse cutaneous systemic sclerosis

Spiera, Robert, Dinesh Khanna, Masataka Kuwana, Daniel E. Furst, Tracy M. Frech, Laura Hummers, Wendy Stevens, et al. “A Randomised, Double-Blind, Placebo-Controlled Phase 3 Study of Lenabasum in Diffuse Cutaneous Systemic Sclerosis: Resolve-1 Design and Rationale.” Clinical and Experimental Rheumatology 39, no. 4 (2021): 124–33. https://doi.org/10.55563/clinexprheumatol/i80zh7.

Clinical characteristics, visceral involvement, and mortality in at-risk or early diffuse systemic sclerosis

Jaafar, Sara, Alain Lescoat, Suiyuan Huang, Jessica Gordon, Monique Hinchcliff, Ami A. Shah, Shervin Assassi, et al. “Clinical Characteristics, Visceral Involvement, and Mortality in at-Risk or Early Diffuse Systemic Sclerosis: A Longitudinal Analysis of an Observational Prospective Multicenter US Cohort.” Arthritis Research & Therapy 23, no. 1 (2021). https://doi.org/10.1186/s13075-021-02548-1.

Predictive Significance of Serum Interferon-Inducible Protein Score for Response to Treatment in Systemic Sclerosis

Assassi, Shervin, Ning Li, Elizabeth R. Volkmann, Maureen D. Mayes, Dennis Rünger, Jun Ying, Michael D. Roth, et al. “Predictive Significance of Serum Interferon‐Inducible Protein Score for Response to Treatment in Systemic Sclerosis–Related Interstitial Lung Disease.” Arthritis & Rheumatology 73, no. 6 (2021): 1005–13. https://doi.org/10.1002/art.41627.

Does hand involvement in systemic sclerosis limit completion of patient-reported outcome measures?  

Frech, T.M., VanBuren, J.M., Startup, E. et al. Does hand involvement in systemic sclerosis limit completion of patient-reported outcome measures?. Clin Rheumatol 40, 965–971 (2021). https://doi.org/10.1007/s10067-020-05467-9

Open Access Clinical Bedside Tools to Assess Systemic Sclerosis Vasculopathy

Frech, Tracy M., Zhining Ou, and Angela P. Presson. “Clinical Bedside Tools to Assess Systemic Sclerosis Vasculopathy: Can Digital Thermal Monitoring and Sublingual Microscopy Identify Patients with Digital Ulcers?” The Journal of Rheumatology 48, no. 10 (2021): 1566–68. https://doi.org/10.3899/jrheum.201234.

Does sublingual microscopy correlate with nailfold videocapillaroscopy in systemic sclerosis?

Radic, M., Thomas, J., McMillan, S. et al. Does sublingual microscopy correlate with nailfold videocapillaroscopy in systemic sclerosis?. Clin Rheumatol 40, 2263–2266 (2021). https://doi.org/10.1007/s10067-020-05495-5

Raynaud Phenomenon in Systemic Sclerosis

Thomas, Julie K., Mislav Radic, Jordan R. Tucker, Rebecca Overbury, and Tracy M. Frech. “Raynaud Phenomenon in Systemic Sclerosis: Does Digital Thermal Monitoring Correlate to Specific Nailfold Videocapillaroscopy Abnormalities?” The Journal of Rheumatology 48, no. 2 (2020): 247–50. https://doi.org/10.3899/jrheum.191371.

Identifying barriers and facilitators to physical activity for people with scleroderma

Harb, Sami, Julie Cumin, Danielle B. Rice, Sandra Peláez, Marie Hudson, Susan J. Bartlett, Alexandra Roren, et al. “Identifying Barriers and Facilitators to Physical Activity for People with Scleroderma: A Nominal Group Technique Study.” Disability and Rehabilitation 43, no. 23 (2020): 3339–46. https://doi.org/10.1080/09638288.2020.1742391.

HLA and autoantibodies define scleroderma subtypes and risk in African and European Americans

Gourh, Pravitt, Sarah A. Safran, Theresa Alexander, Steven E. Boyden, Nadia D. Morgan, Ami A. Shah, Maureen D. Mayes, et al. “HLA and Autoantibodies Define Scleroderma Subtypes and Risk in African and European Americans and Suggest a Role for Molecular Mimicry.” Proceedings of the National Academy of Sciences 117, no. 1 (2019): 552–62. https://doi.org/10.1073/pnas.1906593116.

Outcomes of patients with systemic sclerosis treated with rituximab  

Elhai, Muriel, Marouane Boubaya, Oliver Distler, Vanessa Smith, Marco Matucci-Cerinic, Juan José Alegre Sancho, Marie-Elise Truchetet, et al. “Outcomes of Patients with Systemic Sclerosis Treated with Rituximab in Contemporary Practice: A Prospective Cohort Study.” Annals of the Rheumatic Diseases 78, no. 7 (2019): 979–87. https://doi.org/10.1136/annrheumdis-2018-214816.

Linear scleroderma “en coup de sabre” with extensive brain involvement

Magro, C.M., Halteh, P., Olson, L.C. et al. Linear scleroderma “en coup de sabre” with extensive brain involvement—Clinicopathologic correlations and response to anti-Interleukin-6 therapy. Orphanet J Rare Dis 14, 110 (2019). https://doi.org/10.1186/s13023-019-1015-7

Protean Neurologic Manifestations of Two Rare Dermatologic Disorders 

Wallach, A.I., Magro, C.M., Franks, A.G. et al. Protean Neurologic Manifestations of Two Rare Dermatologic Disorders: Sweet Disease and Localized Craniofacial Scleroderma. Curr Neurol Neurosci Rep 19, 11 (2019). https://doi.org/10.1007/s11910-019-0929-8

Risk Factors for Mortality and Cardiopulmonary Hospitalization in Systemic Sclerosis  

Hsu, Vivien M., Lorinda Chung, Laura K. Hummers, Ami Shah, Robert Simms, Marcy Bolster, Faye N. Hant, et al. “Risk Factors for Mortality and Cardiopulmonary Hospitalization in Systemic Sclerosis Patients at Risk for Pulmonary Hypertension, in the Pharos Registry.” The Journal of Rheumatology 46, no. 2 (2018): 176–83. https://doi.org/10.3899/jrheum.180018.

The patient experience of Raynaud’s phenomenon in systemic sclerosis

Pauling, John D, Lesley Ann Saketkoo, Marco Matucci-Cerinic, Francesca Ingegnoli, and Dinesh Khanna. “The Patient Experience of Raynaud’s Phenomenon in Systemic Sclerosis.” Rheumatology 58, no. 1 (2018): 18–26. https://doi.org/10.1093/rheumatology/key026.

Utility of B-type natriuretic peptides in the assessment of patients with systemic sclerosis

Chung L, Fairchild RM, Furst DE, et al. Utility of B-type natriuretic peptides in the assessment of patients with systemic sclerosis-associated pulmonary hypertension in the PHAROS registry. Clin Exp Rheumatol. 2017;35 Suppl 106(4):106-113.

Current and future direction in the management of scleroderma 

Brady, S.M., Shapiro, L. & Mousa, S.A. Current and future direction in the management of scleroderma. Arch Dermatol Res 308, 461–471 (2016). https://doi.org/10.1007/s00403-016-1647-6

Survival and Predictors of Mortality in Systemic Sclerosis

Chung, Lorinda, Robyn T. Domsic, Bharathi Lingala, Firas Alkassab, Marcy Bolster, M. E. Csuka, Chris 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 66, no. 3 (2014): 489–95. https://doi.org/10.1002/acr.22121.

Computed Tomography of the Chest to Screen for Interstitial Lung Disease in Patients With Systemic Sclerosis

Bernstein, Elana J., Shervin Assassi, Flavia V. Castelino, Lorinda Chung, Chase Correia, Luke B. Evnin, Tracy M. Frech, et al. “Computed Tomography of the Chest to Screen for Interstitial Lung Disease in Patients with Systemic Sclerosis at Expert Scleroderma Centers in the United States.” ACR Open Rheumatology, 2022. https://doi.org/10.1002/acr2.11434.

 Baseline characteristics of systemic sclerosis patients with restrictive lung disease in a multi-center US-based longitudinal registry

Castelino, Flavia V., John M. VanBuren, Emily Startup, Shervin Assassi, Elana J. Bernstein, Lorinda Chung, Chase Correia, et al. “Baseline Characteristics of Systemic Sclerosis Patients with Restrictive Lung Disease in a Multi‐Center Us‐Based Longitudinal Registry.” International Journal of Rheumatic Diseases 25, no. 2 (2021): 163–74. https://doi.org/10.1111/1756-185x.14253.

Randomized feasibility trial of the Scleroderma Patient-centered Intervention Network Self-Management (SPIN-SELF) Program

Kwakkenbos, Linda, Nora Østbø, Marie-Eve Carrier, Warren R. Nielson, Claire Fedoruk, Brooke Levis, Richard S. Henry, et al. “Randomized Feasibility Trial of the Scleroderma Patient-Centered Intervention Network Self-Management (Spin-Self) Program.” Pilot and Feasibility Studies 8, no. 1 (2022). https://doi.org/10.1186/s40814-022-00994-5.

Effect of Coping Strategies on Patient and Physician Perceptions of Disease Severity and Disability in Systemic Sclerosis

DiRenzo, Dana D., Theresa R. Smith, Tracy M. Frech, Ami A. Shah, and John D. Pauling. “Effect of Coping Strategies on Patient and Physician Perceptions of Disease Severity and Disability in Systemic Sclerosis.” The Journal of Rheumatology 48, no. 10 (2021): 1569–73. https://doi.org/10.3899/jrheum.201612.

The frequency of Raynaud’s phenomenon, very early diagnosis of systemic sclerosis, and systemic sclerosis in a large Veteran Health Administration database

Frech, Tracy M., Maureen A. Murtaugh, Megan Amuan, and Mary Jo Pugh. “The Frequency of Raynaud’s Phenomenon, Very Early Diagnosis of Systemic Sclerosis, and Systemic Sclerosis in a Large Veteran Health Administration Database.” BMC Rheumatology 5, no. 1 (2021). https://doi.org/10.1186/s41927-021-00209-z.

A comprehensive framework for navigating patient care in systemic sclerosis

Saketkoo, Lesley Ann, Tracy Frech, Cecília Varjú, Robyn Domsic, Jessica Farrell, Jessica K. Gordon, Carina Mihai, et al. “A Comprehensive Framework for Navigating Patient Care in Systemic Sclerosis: A Global Response to the Need for Improving the Practice of Diagnostic and Preventive Strategies in SSC.” Best Practice & Research Clinical Rheumatology 35, no. 3 (2021): 101707. https://doi.org/10.1016/j.berh.2021.101707.

Dysphagia, reflux and related sequelae due to altered physiology in scleroderma

Kadakuntla, Anusri, Ankit Juneja, Samantha Sattler, Anusha Agarwal, Drishti Panse, Nardin Zakhary, Anusha Pasumarthi, Lee Shapiro, and Micheal Tadros. “Dysphagia, Reflux and Related Sequelae Due to Altered Physiology in Scleroderma.” World Journal of Gastroenterology 27, no. 31 (2021): 5201–18. https://doi.org/10.3748/wjg.v27.i31.5201.

Exercise as a multi-modal disease-modifying medicine in systemic sclerosis

Pettersson, Henrik, Helene Alexanderson, Janet L. Poole, Janos Varga, Malin Regardt, Anne-Marie Russell, Yasser Salam, et al. “Exercise as a Multi-Modal Disease-Modifying Medicine in Systemic Sclerosis: An Introduction by the Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis (G-Forss).” Best Practice & Research Clinical Rheumatology 35, no. 3 (2021): 101695. https://doi.org/10.1016/j.berh.2021.101695.

A randomised, double-blind, placebo-controlled phase 3 study of lenabasum in diffuse cutaneous systemic sclerosis

Spiera, Robert, Dinesh Khanna, Masataka Kuwana, Daniel E. Furst, Tracy M. Frech, Laura Hummers, Wendy Stevens, et al. “A Randomised, Double-Blind, Placebo-Controlled Phase 3 Study of Lenabasum in Diffuse Cutaneous Systemic Sclerosis: Resolve-1 Design and Rationale.” Clinical and Experimental Rheumatology 39, no. 4 (2021): 124–33. https://doi.org/10.55563/clinexprheumatol/i80zh7.

Clinical characteristics, visceral involvement, and mortality in at-risk or early diffuse systemic sclerosis

Jaafar, Sara, Alain Lescoat, Suiyuan Huang, Jessica Gordon, Monique Hinchcliff, Ami A. Shah, Shervin Assassi, et al. “Clinical Characteristics, Visceral Involvement, and Mortality in at-Risk or Early Diffuse Systemic Sclerosis: A Longitudinal Analysis of an Observational Prospective Multicenter US Cohort.” Arthritis Research & Therapy 23, no. 1 (2021). https://doi.org/10.1186/s13075-021-02548-1.

Predictive Significance of Serum Interferon-Inducible Protein Score for Response to Treatment in Systemic Sclerosis

Assassi, Shervin, Ning Li, Elizabeth R. Volkmann, Maureen D. Mayes, Dennis Rünger, Jun Ying, Michael D. Roth, et al. “Predictive Significance of Serum Interferon‐Inducible Protein Score for Response to Treatment in Systemic Sclerosis–Related Interstitial Lung Disease.” Arthritis & Rheumatology 73, no. 6 (2021): 1005–13. https://doi.org/10.1002/art.41627.

Does hand involvement in systemic sclerosis limit completion of patient-reported outcome measures?  

Frech, T.M., VanBuren, J.M., Startup, E. et al. Does hand involvement in systemic sclerosis limit completion of patient-reported outcome measures?. Clin Rheumatol 40, 965–971 (2021). https://doi.org/10.1007/s10067-020-05467-9

Open Access Clinical Bedside Tools to Assess Systemic Sclerosis Vasculopathy

Frech, Tracy M., Zhining Ou, and Angela P. Presson. “Clinical Bedside Tools to Assess Systemic Sclerosis Vasculopathy: Can Digital Thermal Monitoring and Sublingual Microscopy Identify Patients with Digital Ulcers?” The Journal of Rheumatology 48, no. 10 (2021): 1566–68. https://doi.org/10.3899/jrheum.201234.

Does sublingual microscopy correlate with nailfold videocapillaroscopy in systemic sclerosis?

Radic, M., Thomas, J., McMillan, S. et al. Does sublingual microscopy correlate with nailfold videocapillaroscopy in systemic sclerosis?. Clin Rheumatol 40, 2263–2266 (2021). https://doi.org/10.1007/s10067-020-05495-5

Raynaud Phenomenon in Systemic Sclerosis

Thomas, Julie K., Mislav Radic, Jordan R. Tucker, Rebecca Overbury, and Tracy M. Frech. “Raynaud Phenomenon in Systemic Sclerosis: Does Digital Thermal Monitoring Correlate to Specific Nailfold Videocapillaroscopy Abnormalities?” The Journal of Rheumatology 48, no. 2 (2020): 247–50. https://doi.org/10.3899/jrheum.191371.

Identifying barriers and facilitators to physical activity for people with scleroderma

Harb, Sami, Julie Cumin, Danielle B. Rice, Sandra Peláez, Marie Hudson, Susan J. Bartlett, Alexandra Roren, et al. “Identifying Barriers and Facilitators to Physical Activity for People with Scleroderma: A Nominal Group Technique Study.” Disability and Rehabilitation 43, no. 23 (2020): 3339–46. https://doi.org/10.1080/09638288.2020.1742391.

HLA and autoantibodies define scleroderma subtypes and risk in African and European Americans

Gourh, Pravitt, Sarah A. Safran, Theresa Alexander, Steven E. Boyden, Nadia D. Morgan, Ami A. Shah, Maureen D. Mayes, et al. “HLA and Autoantibodies Define Scleroderma Subtypes and Risk in African and European Americans and Suggest a Role for Molecular Mimicry.” Proceedings of the National Academy of Sciences 117, no. 1 (2019): 552–62. https://doi.org/10.1073/pnas.1906593116.

Outcomes of patients with systemic sclerosis treated with rituximab  

Elhai, Muriel, Marouane Boubaya, Oliver Distler, Vanessa Smith, Marco Matucci-Cerinic, Juan José Alegre Sancho, Marie-Elise Truchetet, et al. “Outcomes of Patients with Systemic Sclerosis Treated with Rituximab in Contemporary Practice: A Prospective Cohort Study.” Annals of the Rheumatic Diseases 78, no. 7 (2019): 979–87. https://doi.org/10.1136/annrheumdis-2018-214816.

Linear scleroderma “en coup de sabre” with extensive brain involvement

Magro, C.M., Halteh, P., Olson, L.C. et al. Linear scleroderma “en coup de sabre” with extensive brain involvement—Clinicopathologic correlations and response to anti-Interleukin-6 therapy. Orphanet J Rare Dis 14, 110 (2019). https://doi.org/10.1186/s13023-019-1015-7

Protean Neurologic Manifestations of Two Rare Dermatologic Disorders 

Wallach, A.I., Magro, C.M., Franks, A.G. et al. Protean Neurologic Manifestations of Two Rare Dermatologic Disorders: Sweet Disease and Localized Craniofacial Scleroderma. Curr Neurol Neurosci Rep 19, 11 (2019). https://doi.org/10.1007/s11910-019-0929-8

Risk Factors for Mortality and Cardiopulmonary Hospitalization in Systemic Sclerosis  

Hsu, Vivien M., Lorinda Chung, Laura K. Hummers, Ami Shah, Robert Simms, Marcy Bolster, Faye N. Hant, et al. “Risk Factors for Mortality and Cardiopulmonary Hospitalization in Systemic Sclerosis Patients at Risk for Pulmonary Hypertension, in the Pharos Registry.” The Journal of Rheumatology 46, no. 2 (2018): 176–83. https://doi.org/10.3899/jrheum.180018.

The patient experience of Raynaud’s phenomenon in systemic sclerosis

Pauling, John D, Lesley Ann Saketkoo, Marco Matucci-Cerinic, Francesca Ingegnoli, and Dinesh Khanna. “The Patient Experience of Raynaud’s Phenomenon in Systemic Sclerosis.” Rheumatology 58, no. 1 (2018): 18–26. https://doi.org/10.1093/rheumatology/key026.

Utility of B-type natriuretic peptides in the assessment of patients with systemic sclerosis

Chung L, Fairchild RM, Furst DE, et al. Utility of B-type natriuretic peptides in the assessment of patients with systemic sclerosis-associated pulmonary hypertension in the PHAROS registry. Clin Exp Rheumatol. 2017;35 Suppl 106(4):106-113.

Current and future direction in the management of scleroderma 

Brady, S.M., Shapiro, L. & Mousa, S.A. Current and future direction in the management of scleroderma. Arch Dermatol Res 308, 461–471 (2016). https://doi.org/10.1007/s00403-016-1647-6

Survival and Predictors of Mortality in Systemic Sclerosis

Chung, Lorinda, Robyn T. Domsic, Bharathi Lingala, Firas Alkassab, Marcy Bolster, M. E. Csuka, Chris 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 66, no. 3 (2014): 489–95. https://doi.org/10.1002/acr.22121.

Abstracts

Scleroderma

Farrell JF, Shapiro LS, Miller M. Clinical Pharmacist As Part of the Interprofessional Team Improves Quality of Care in Patients with Rheumatic Disease Abstract 215. 2017 ACR/ARHP Annual Meeting; 2017 November 05; San Diego , CA, USA. c2017. 

Overbury R, Frech TM, Murtaugh M, Steen VD, PHAROS investigators. A Normal Pulmonary Diffusion Capacity Is Rare in Pulmonary Artery Hypertension in Systemic Sclerosis. 2016 ACR/ARHP Annual Meeting. 2016 ACR/ARHP Annual Meeting; 2016 November 13; Washington, DC, United States of America. c2016. 

Sievert M, Machin DR, Donato AJ, Murtaugh M, Pauling JD, et al. Laser Speckled Imaging and Videomicroscopy Assessment of Sublingual Perfusion in Systemic Sclerosis and Healthy Controls. • Abstract Number: 2979 • 2015 ACR/ARHP Annual Meeting; 2015 November; c2015. 

Frech TM, Machin DR, Gates PE, Domsic RT, Shapiro LS, et al. Assessment of Sublingual Frenulum Perfusion in Systemic Sclerosis. Abstract Number: 2980 • 2015 ACR/ARHP Annual Meeting; 2015 November; San Francisco, CA, USA. c2015. 

Shapiro LS, Saketkoo L, Farrell JF, Fligelstone K. Development of a “Renal Crisis Prevention Card” As an Educational Tool Aimed at Improving Outcomes in High-Risk Patients with Systemic Sclerosis. • Abstract Number: 2716 • 2014 ACR/ARHP Annual Meeting; 2014 November; Boston, MA, USA. c2014. 

Frech T, Pauling J, Murtaugh M, Shapiro L, Choi B, et al. Assessment of Sublingual Abnormalities in Systemic Sclerosis. Abstract Number: 1694 • 2014 ACR/ARHP Annual Meeting; 2014 November; Boston, MA, USA. c2014. 

Degos Disease

Gastrointestinal Kohlmeier–Degos disease

Sattler, S.S., Magro, C.M., Shapiro, L. et al. Gastrointestinal Kohlmeier–Degos disease: a narrative review. Orphanet J Rare Dis 17, 172 (2022). https://doi.org/10.1186/s13023-022-02322-9

Exploring the pathophysiologic basis of constrictive pericarditis of Kohlmeier Degos disease

Magro, Cynthia, and Lee Shapiro. “Exploring the Pathophysiologic Basis of Constrictive Pericarditis of Kohlmeier Degos Disease: A Case Series and Review of the Literature.” Annals of Diagnostic Pathology 59 (2022): 151943. https://doi.org/10.1016/j.anndiagpath.2022.151943.

Neurological Involvement in Malignant Atrophic Papulosis  

Yousefi S, Borhani-Haghighi A, Safari A, Shapiro L. Neurological Involvement in Malignant Atrophic Papulosis: A Comprehensive Review of Literature. Neurol India. 2022;70(1):5-10. doi:10.4103/0028-3886.338719

Atrophic papulosis: the wedge-shaped skin necrosis overlays an active lesion

Zouboulis, C.C., L.S. Shapiro, and C.M. Magro. “Atrophic Papulosis: The Wedge‐Shaped Skin Necrosis Overlays an Active Lesion.” Journal of the European Academy of Dermatology and Venereology 35, no. 10 (2021). https://doi.org/10.1111/jdv.17377

Clinical and laboratory prognosticators of atrophic papulosis (Degos disease) 

Lu, J.D., Sachdeva, M., Silverberg, O.M. et al. Clinical and laboratory prognosticators of atrophic papulosis (Degos disease): a systematic review. Orphanet J Rare Dis 16, 203 (2021). https://doi.org/10.1186/s13023-021-01819-z

Enhanced cutaneous Rock2 expression

Magro, Cynthia, Ziv Schwartz, Jad Saab, and Amin Hedayat. “Enhanced Cutaneous ROCK2 Expression as a Marker of Rho Kinase Pathway Activation in Autoimmune Disease and Kohlemeier-Degos Disease.” Annals of Diagnostic Pathology 44 (2020): 151414. https://doi.org/10.1016/j.anndiagpath.2019.151414.

Kohlmeier–Degos disease with constrictive pericarditis and atrial fibrillation

Wang, S.C., Patel, H., Shapiro, L.S. et al. Kohlmeier–Degos disease with constrictive pericarditis and atrial fibrillation. J Echocardiogr 16, 192–193 (2018). https://doi.org/10.1007/s12574-018-0386-4

Benign atrophic papulosis (Köhlmeier–Degos disease)

Zouboulis, C.C., A. Theodoridis, M. Brunner, and C.M. Magro. “Benign Atrophic Papulosis (Köhlmeier-Degos Disease): The Wedge-Shaped Dermal Necrosis Can Resolve with Time.” Journal of the European Academy of Dermatology and Venereology 31, no. 10 (2017): 1753–56. https://doi.org/10.1111/jdv.14355.

Diffuse atrophic papules and plaques, intermittent abdominal pain, paresthesias, and cardiac abnormalities in a 55-year-old woman

Oliver, Brittany, Manfred Boehm, Douglas R. Rosing, Lee S. Shapiro, Daniel T. Dempsey, Peter A. Merkel, Chyi-Chia Richard Lee, and Edward W. Cowen. “Diffuse Atrophic Papules and Plaques, Intermittent Abdominal Pain, Paresthesias, and Cardiac Abnormalities in a 55-Year-Old Woman.” Journal of the American Academy of Dermatology 75, no. 6 (2016): 1274–77. https://doi.org/10.1016/j.jaad.2016.09.015.

Laparoscopy shows superiority over endoscopy for early detection of malignant atrophic papulosis gastrointestinal complications

Toledo, A.E., Shapiro, L.S., Farrell, J.F. et al. Laparoscopy shows superiority over endoscopy for early detection of malignant atrophic papulosis gastrointestinal complications: a case report and review of literature. BMC Gastroenterol 15, 156 (2015). https://doi.org/10.1186/s12876-015-0387-y

Opioid associated intravenous and cutaneous microvascular drug abuse

Magro CM, Toledo-Garcia A, Pala O, Momtahen S, Shapiro L. Opioid associated intravenous and cutaneous microvascular drug abuse (skin-popping) masquerading as Degos disease (malignant atrophic papulosis) with multiorgan involvement. Dermatol Online J. 2015;21(9):13030/qt7dk8q7n1.

Effective treatment of malignant atrophic papulosis (Köhlmeier-Degos disease) with treprostinil  

Shapiro, L.S., Toledo-Garcia, A.E. & Farrell, J.F. Effective treatment of malignant atrophic papulosis (Köhlmeier-Degos disease) with treprostinil – early experience. Orphanet J Rare Dis 8, 52 (2013). https://doi.org/10.1186/1750-1172-8-52

Degos Disease: A C5b-9/Interferon-α–Mediated Endotheliopathy Syndrome

Magro, Cynthia M., Jonathan C. Poe, Connie Kim, Lee Shapiro, Gerard Nuovo, Mary K. Crow, and Yanick J. Crow. “Degos Disease.” American Journal of Clinical Pathology 135, no. 4 (2011): 599–610. https://doi.org/10.1309/ajcp66qimfarlzki.

Book Chapters

Scleroderma

Shapiro, L, Saketkoo, L.A., Steen, V.D.  in Pigmented Ethnic Skin and Imported Dermatoses, Springer International Publishing, 2018.   Scleroderma in Pigmented Populations, 471-480.