Building our Knowledge Base

Recent Publications


Magro CM, Halteh P, Olson LC, Kister I, Shapiro L.  Linear scleroderma “en coup de sabre” with extensive brain involvement-Clinicopathologic correlations and response to anti-interleukin-6 therapy. Orphanet J Rare Disease 2019  May 16; 14 (1). PMID: 31096996.

Wallach AI, Magro CM, Franks AG Jr, Shapiro L , Kister I.  Protean Neurologic Manifestations of Two Rare Dermatologic Disorders:  Sweet Disease and Localized Craniofacial Scleroderma. Current Neurol Neurosci Rep  2019  Feb 12; 19(3). PMID: 30747288.

Oliver B, Boehm M, Rosing DR, Shapiro LS, Dempsey DT, Merkel PA, Lee CR, Cowen EW. Diffuse atrophic papules and plaques, intermittent abdominal pain, paresthesias, and cardiac abnormalities in a 55-year-old woman. J Am Acad Dermatol. 2016 Dec;75(6):1274-1277. doi: 10.1016/j.jaad.2016.09.015. Epub 2016 Oct 4. PubMed PMID: 27717619; PubMed Central PMCID: PMC5112129. 

Chung L, Fairchild RM, Furst DE, Li S, Alkassab F, Bolster MB, Csuka ME, Derk CT, Domsic RT, Fischer A, Frech T, Gomberg-Maitland M, Gordon JK, Hinchcliff M, Hsu V, Hummers LK, Khanna D, Medsger TA, Molitor JA, Preston IR, Schiopu E, Shapiro L, Hant F, Silver R, Simms R, Varga J, Steen VD, Zamanian RT. Utility of B-type natriuretic peptides in the assessment of patients with systemic sclerosis-associated pulmonary hypertension in the PHAROS registry. Clin Exp Rheumatol. 2016 Nov 10. [Epub ahead of print] PubMed PMID: 27908301. 

Brady SM, Shapiro L, Mousa SA. Current and future direction in the management of scleroderma. Arch Dermatol Res. 2016 Sep;308(7):461-71. doi: 10.1007/s00403-016-1647-6. Epub 2016 Apr 30. Review. PubMed PMID: 27139430.

Hinchcliff M, Khanna S, Hsu VM, Lee J, Almagor O, Chang RW, Steen V, Chung L; PHAROS Investigators. Survival in systemic sclerosis-pulmonary arterial hypertension by serum autoantibody status in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry. Semin Arthritis Rheum. 2015 Dec;45(3):309-14. doi: 10.1016/j.semarthrit.2015.06.011. Epub 2015 Jun 19. PubMed PMID: 26210782; PubMed Central PMCID: PMC4656087.

Hsu VM, Chung L, Hummers LK, Wigley F, Simms R, Bolster M, Silver R, Fischer A, Hinchcliff ME, Varga J, Goldberg AZ, Derk CT, Schiopu E, Khanna D, Shapiro LS, Domsic RT, Medsger T, Mayes MD, Furst D, Csuka ME, Molitor JA, Alkassab F, Steen VD. Development of pulmonary hypertension in a high-risk population with systemic sclerosis in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) cohort study. Semin Arthritis Rheum. 2014 Aug;44(1):55-62. doi: 10.1016/j.semarthrit.2014.03.002. Epub 2014 Mar 5. PubMed PMID: 24709277.

Judson MA, Shapiro L, Freitas S, Polychronopoulos VS, Highland KB. Concomitant sarcoidosis and a connective tissue disease: review of the clinical findings and postulations concerning their association. Respir Med. 2013 Sep;107(9):1453-9. doi: 10.1016/j.rmed.2013.01.004. Epub 2013 Feb 27. Review. PubMed PMID: 23452728. 

Walker KM, Pope J; participating members of the Scleroderma Clinical Trials Consortium (SCTC).; Canadian Scleroderma Research Group (CSRG). Treatment of systemic sclerosis complications: what to use when first-line treatment fails--a consensus of systemic sclerosis experts. Semin Arthritis Rheum. 2012 Aug;42(1):42-55. doi: 10.1016/j.semarthrit.2012.01.003. Epub 2012 Mar 29. PubMed PMID: 22464314. 

Frech TM, Gordon J, Chung L, Bolster M, Segal B, et al. Vasodilatory Therapeutics and Digital Ulcerations in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Cohort. Rheumatology. 2012 February 15. 

Walker KM, Pope J; Scleroderma Clinical Trials Consortium.; Canadian Scleroderma Research Group. Expert agreement on EULAR/EUSTAR recommendations for the management of systemic sclerosis. J Rheumatol. 2011 Jul;38(7):1326-8. doi: 10.3899/jrheum.101262. Epub 2011 Apr 1. PubMed PMID: 21459952. 

Hinchcliff M, Fischer A, Schiopu E, Steen VD; PHAROS Investigators. Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS): baseline characteristics and description of study population. J Rheumatol. 2011 Oct;38(10):2172-9. doi: 10.3899/jrheum.101243. Epub 2011 Aug 15. PubMed PMID: 21844142; PubMed Central PMCID: PMC3230328. 

Shapiro LS. Case 26-2001: scleroderma renal crisis and polymyositis. N Engl J Med. 2002 Jun 13;346(24):1916-8; author reply 1916-8. PubMed PMID: 12066814. 

Kremer JM, Shapiro LS, Morell M, Kelly S. Topical Tretonoin Improves Skin Scores on the Face and hands in Patients with PSS. Arthritis and rheumatism.1998; 41:5102. 

Shapiro LS. Effective Intervention in Scleroderma Renal Crisis. The Journal of musculoskeletal medicine. 1997; 14:25-36. 

Sacks DG, Okano Y, Steen VD, Curtiss E, Shapiro LS, Medsger TA Jr. Isolated pulmonary hypertension in systemic sclerosis with diffuse cutaneous involvement: association with serum anti-U3RNP antibody. J Rheumatol. 1996 Apr;23(4):639-42. PubMed PMID: 8730118. 

Shapiro LS. Large vessel arterial thrombosis in systemic sclerosis associated with antiphospholipid antibodies. J Rheumatol. 1990 May;17(5):685-8. Review. PubMed PMID: 2193157. 

Shapiro LS, Prince RK, Buckingham RB, Rodnan GP. D-penicillamine treatment of progressive systemic sclerosis (scleroderma): a comparison of clinical and in vitro effects. J Rheumatol. 1983 Apr;10(2):316-8. PubMed PMID: 6864687.

Degos Disease

Wang, SC, Patel H, Shapiro LS, Mookherjee S. Kohlmeier-Degos disease with constrictive pericarditis and atrial fibrillation.  J. Echocardiography.2018 July. PMID:  29974345.

Toledo AE, Shapiro LS, Farrell JF, Magro CM, Polito J. Laparoscopy shows superiority over endoscopy for early detection of malignant atrophic papulosis gastrointestinal complications: a case report and review of literature. BMC Gastroenterol. 2015 Nov 2;15:156. doi: 10.1186/s12876-015-0387-y. Review. PubMed PMID: 26527039; PubMed Central PMCID: PMC4630896. 

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 Sep 17;21(9). pii: 13030/qt7dk8q7n1. PubMed PMID: 26437280. 

Toledo-Garcia AE, Farrell JF, Shapiro LS. Malignant Atrophic Papulosis Is Challenging to Diagnose, Treat. The Rheumatologist. 2014 July 01; 8(7):25-28. Chung L, Domsic RT, Lingala B, Alkassab F, Bolster M, Csuka ME, Derk C, Fischer A, Frech T, Furst DE, Gomberg-Maitland M, Hinchcliff M, Hsu V, Hummers LK, Khanna D, Medsger TA Jr, Molitor JA, Preston IR, Schiopu E, Shapiro L, Silver R, Simms R, Varga J, Gordon JK, Steen VD. 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 Res (Hoboken). 2014 Mar;66(3):489-95. doi: 10.1002/acr.22121. PubMed PMID: 23983198. 

Magro CM, Wang X, Garrett-Bakelman F, Laurence J, Shapiro LS, DeSancho MT. The effects of Eculizumab on the pathology of malignant atrophic papulosis.Orphanet J Rare Dis. 2013 Nov 26;8:185. doi: 10.1186/1750-1172-8-185. PubMed PMID: 24279613; PubMed Central PMCID: PMC3879088. 

Shapiro LS, Toledo-Garcia AE, Farrell JF. Effective treatment of malignant atrophic papulosis (Köhlmeier-Degos disease) with treprostinil--early experience. Orphanet J Rare Dis. 2013 Apr 4;8:52. doi: 10.1186/1750-1172-8-52. PubMed PMID: 23557362; PubMed Central PMCID: PMC3636001. 

Magro CM, Poe JC, Kim C, Shapiro L, Nuovo G, Crow MK, Crow YJ. Degos disease: a C5b-9/interferon-α-mediated endotheliopathy syndrome. Am J Clin Pathol. 2011 Apr;135(4):599-610. doi: 10.1309/AJCP66QIMFARLZKI. PubMed PMID: 21411783. 



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

Magro, C and Shapiro, L.  in Vasculitis, Oxford University Press, 2020 (in review).  Kohlmeier-Degos Disease.  

Shapiro LS, Farrell JF, Lukasiewicz RL, Merkel PA, Rosing D, et al. Systemic Degos Disease: Long Term Survival on Combined Therapy with Eculizumab and Treprostinil Abstract1 166. 2017 ACR/ARHP Annual Meeting; 2017 November 06; San DIego, CA, USA. c2017. 

Toledo-Garcia A, Shapiro LS, Farrell JF. Malignant Atrophic Papulosis (MAP) Complicating Connective Tissue Diseases (CTDs). • Abstract Number: 2197 • 2014 ACR/ARHP Annual Meeting; 2014 November; Boston, MA, USA. c2014. 

Shapiro LS, Farrell JF, Toledo-Garcia A. Treprostinil Use in Malignant Atrophic Papulosis (Köhlmeier-Degos Disease): Review of Worldwide Experience to Date. • Abstract Number: 2196 • 2014 ACR/ARHP Annual Meeting; 2014 November; Boston, MA, USA. c2014. 

Toledo-Garcia A, Shapiro LS, Farrell JF. Eculizumab Treatment of Malignant Atrophic Papulosis (Köhlmeier-Degos Disease): World Experience to Date. • Abstract Number: 2195 • 2014 ACR/ARHP Annual Meeting; 2014 November; Boston, MA, USA. c2014. 

Farrell JF, Shapiro LS, Kremer JM, Toledo-Garcia A. Pharmacist-Developed Letters May Enhance Success in Obtaining Insurer Approval for OffLabel Use of Biologics. Abstract Number: 2444 • 2014 ACR/ARHP Annual Meeting; 2014 November 14; Boston, MA, USA. c2014. 

Shapiro LS, Toledo-Garcia AE, Farrell JF. Effective Treatment of Degos Disease with Treprostinil. 2nd Systemic Sclerosis World Congress, 2012. Abstracts from the 2rd Systemic Sclerosis World Congress, 2012; 2012 February; Madrid, Spain. 

Polito J, Toledo-Garcia AE, Shapiro LS. Early Detection of Systemic Degos Disease (DD) or Malignant Atrophic Papulosis May Increase Survival. American College of Gastroenterology. ACG Annual Scientific Meeting; 2010 October; San Antonio, TX, United States of America. 

Book Chapters


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.