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Pharmacotherapy Options in the Management of Raynaud's Phenomenon.

Hinze AM, Wigley FM.

Curr Treatm Opt Rheumatol. 2018 Sep;4(3):235-254. doi: 10.1007/s40674-018-0102-6. Epub 2018 Jul 4.


Longitudinal assessment of interstitial lung disease in single lung transplant recipients with scleroderma.

Hinze AM, Lin CT, Hussien AF, Perin J, Venado A, Golden JA, Boin F, Brown RH, Wise RA, Wigley FM.

Rheumatology (Oxford). 2019 Aug 27. pii: kez341. doi: 10.1093/rheumatology/kez341. [Epub ahead of print]


Epigenetic activation and memory at a TGFB2 enhancer in systemic sclerosis.

Shin JY, Beckett JD, Bagirzadeh R, Creamer TJ, Shah AA, McMahan Z, Paik JJ, Sampedro MM, MacFarlane EG, Beer MA, Warren D, Wigley FM, Dietz HC.

Sci Transl Med. 2019 Jun 19;11(497). pii: eaaw0790. doi: 10.1126/scitranslmed.aaw0790.


Clinical and Molecular Phenotyping in Scleromyxedema Pre- and Post-Treatment with Intravenous Immunoglobulin.

Mecoli CA, Talbot CC Jr, Fava A, Cheadle C, Boin F, Wigley FM, Hummers LK.

Arthritis Care Res (Hoboken). 2019 Apr 22. doi: 10.1002/acr.23908. [Epub ahead of print]


ANCA-associated vasculitis in scleroderma: A renal perspective

Kant S, Shah AA, Hummers LK, Wigley FM, Geetha D.

Clin Nephrol. 2018 Dec;90(6):413-418. doi: 10.5414/CN109445.


Vascular complications in systemic sclerosis: a prospective cohort study.

Mecoli CA, Shah AA, Boin F, Wigley FM, Hummers LK.

Clin Rheumatol. 2018 Sep;37(9):2429-2437. doi: 10.1007/s10067-018-4148-5. Epub 2018 May 26.


Brief Report: Whole-Exome Sequencing to Identify Rare Variants and Gene Networks That Increase Susceptibility to Scleroderma in African Americans.

Gourh P, Remmers EF, Boyden SE, Alexander T, Morgan ND, Shah AA, Mayes MD, Doumatey A, Bentley AR, Shriner D, Domsic RT, Medsger TA Jr, Steen VD, Ramos PS, Silver RM, Korman B, Varga J, Schiopu E, Khanna D, Hsu V, Gordon JK, Saketkoo LA, Gladue H, Kron B, Criswell LA, Derk CT, Bridges SL Jr, Shanmugam VK, Kolstad KD, Chung L, Jan R, Bernstein EJ, Goldberg A, Trojanowski M, Kafaja S, Maksimowicz-McKinnon KM, Mullikin JC, Adeyemo A, Rotimi C, Boin F, Kastner DL, Wigley FM.

Arthritis Rheumatol. 2018 Oct;70(10):1654-1660. doi: 10.1002/art.40541. Epub 2018 Aug 29.


Autoantibodies and scleroderma phenotype define subgroups at high-risk and low-risk for cancer.

Igusa T, Hummers LK, Visvanathan K, Richardson C, Wigley FM, Casciola-Rosen L, Rosen A, Shah AA.

Ann Rheum Dis. 2018 Aug;77(8):1179-1186. doi: 10.1136/annrheumdis-2018-212999. Epub 2018 Apr 20.


Pyridostigmine for the treatment of gastrointestinal symptoms in systemic sclerosis.

Ahuja NK, Mische L, Clarke JO, Wigley FM, McMahan ZH.

Semin Arthritis Rheum. 2018 Aug;48(1):111-116. doi: 10.1016/j.semarthrit.2017.12.007. Epub 2018 Jan 5.


Clinical and serological features of systemic sclerosis in a multicenter African American cohort: Analysis of the genome research in African American scleroderma patients clinical database.

Morgan ND, Shah AA, Mayes MD, Domsic RT, Medsger TA Jr, Steen VD, Varga J, Carns M, Ramos PS, Silver RM, Schiopu E, Khanna D, Hsu V, Gordon JK, Gladue H, Saketkoo LA, Criswell LA, Derk CT, Trojanowski MA, Shanmugam VK, Chung L, Valenzuela A, Jan R, Goldberg A, Remmers EF, Kastner DL, Wigley FM, Gourh P, Boin F.

Medicine (Baltimore). 2017 Dec;96(51):e8980. doi: 10.1097/MD.0000000000008980.


Right Ventricular Myofilament Functional Differences in Humans With Systemic Sclerosis-Associated Versus Idiopathic Pulmonary Arterial Hypertension.

Hsu S, Kokkonen-Simon KM, Kirk JA, Kolb TM, Damico RL, Mathai SC, Mukherjee M, Shah AA, Wigley FM, Margulies KB, Hassoun PM, Halushka MK, Tedford RJ, Kass DA.

Circulation. 2018 May 29;137(22):2360-2370. doi: 10.1161/CIRCULATIONAHA.117.033147. Epub 2018 Jan 19.


Impact of Radiation Therapy on Scleroderma and Cancer Outcomes in Scleroderma Patients With Breast Cancer.

Shah DJ, Hirpara R, Poelman CL, Woods A, Hummers LK, Wigley FM, Wright JL, Parekh A, Steen VD, Domsic RT, Shah AA.

Arthritis Care Res (Hoboken). 2018 Oct;70(10):1517-1524. doi: 10.1002/acr.23505.


Determining the Risk Factors and Clinical Features Associated With Severe Gastrointestinal Dysmotility in Systemic Sclerosis.

McMahan ZH, Paik JJ, Wigley FM, Hummers LK.

Arthritis Care Res (Hoboken). 2018 Sep;70(9):1385-1392. doi: 10.1002/acr.23479. Epub 2018 Aug 6.


Treating Raynaud phenomenon: Beyond staying warm.

Shapiro SC, Wigley FM.

Cleve Clin J Med. 2017 Oct;84(10):797-804. doi: 10.3949/ccjm.84a.17025. Review.


Editorial: Clinical Trials in Raynaud's Phenomenon: A Spoonful of Sugar (Pill) Makes the Medicine Go Down (in Flames).

Seibold JR, Wigley FM.

Arthritis Rheumatol. 2017 Dec;69(12):2256-2258. doi: 10.1002/art.40307. Epub 2017 Nov 10. No abstract available.


Evaluation of cancer-associated myositis and scleroderma autoantibodies in breast cancer patients without rheumatic disease.

Shah AA, Rosen A, Hummers LK, May BJ, Kaushiva A, Roden RBS, Armstrong DK, Wigley FM, Casciola-Rosen L, Visvanathan K.

Clin Exp Rheumatol. 2017 Sep-Oct;35 Suppl 106(4):71-74. Epub 2017 Jun 19.


Association of Fibrosing Myopathy in Systemic Sclerosis and Higher Mortality.

Paik JJ, Wigley FM, Shah AA, Corse AM, Casciola-Rosen L, Hummers LK, Mammen AL.

Arthritis Care Res (Hoboken). 2017 Nov;69(11):1764-1770. doi: 10.1002/acr.23291.



Shah AA, Rosen A, Hummers LK, Wigley FM, Xu G, Elledge SJ, Casciola-Rosen L.

Arthritis Rheumatol. 2017 Sep;69(9):1915-1916. doi: 10.1002/art.40132. Epub 2017 Jul 11. No abstract available.


The Therapeutic Efficacy of Botulinum Toxin in Treating Scleroderma-Associated Raynaud's Phenomenon: A Randomized, Double-Blind, Placebo-Controlled Clinical Trial.

Bello RJ, Cooney CM, Melamed E, Follmar K, Yenokyan G, Leatherman G, Shah AA, Wigley FM, Hummers LK, Lifchez SD.

Arthritis Rheumatol. 2017 Aug;69(8):1661-1669. doi: 10.1002/art.40123. Epub 2017 Jun 26.


Brief Report: Anti-RNPC-3 Antibodies As a Marker of Cancer-Associated Scleroderma.

Shah AA, Xu G, Rosen A, Hummers LK, Wigley FM, Elledge SJ, Casciola-Rosen L.

Arthritis Rheumatol. 2017 Jun;69(6):1306-1312. doi: 10.1002/art.40065.


Exhaled nitric oxide in pulmonary arterial hypertension associated with systemic sclerosis.

Cao Z, Mathai SC, Hummers LK, Shah AA, Wigley FM, Lechtzin N, Hassoun PM, Girgis RE.

Pulm Circ. 2016 Dec;6(4):545-550. doi: 10.1086/688768.


Independent Association of Severity of Muscle Weakness With Disability as Measured by the Health Assessment Questionnaire Disability Index in Scleroderma.

Paik JJ, Wigley FM, Mejia AF, Hummers LK.

Arthritis Care Res (Hoboken). 2016 Nov;68(11):1695-1703. doi: 10.1002/acr.22870. Epub 2016 Oct 9.


Raynaud's Phenomenon.

Wigley FM, Flavahan NA.

N Engl J Med. 2016 Aug 11;375(6):556-65. doi: 10.1056/NEJMra1507638. Review. No abstract available.


Risk of Digital Vascular Events in Scleroderma Patients Who Have Both Anticentromere and Anti-Interferon-Inducible Protein 16 Antibodies.

McMahan ZH, Wigley FM, Casciola-Rosen L.

Arthritis Care Res (Hoboken). 2017 Jun;69(6):922-926. doi: 10.1002/acr.22978.


Unique Abnormalities in Right Ventricular Longitudinal Strain in Systemic Sclerosis Patients.

Mukherjee M, Chung SE, Ton VK, Tedford RJ, Hummers LK, Wigley FM, Abraham TP, Shah AA.

Circ Cardiovasc Imaging. 2016 Jun;9(6). pii: e003792. doi: 10.1161/CIRCIMAGING.115.003792. Epub 2016 Jun 7.


Right Ventricular Functional Reserve in Pulmonary Arterial Hypertension.

Hsu S, Houston BA, Tampakakis E, Bacher AC, Rhodes PS, Mathai SC, Damico RL, Kolb TM, Hummers LK, Shah AA, McMahan Z, Corona-Villalobos CP, Zimmerman SL, Wigley FM, Hassoun PM, Kass DA, Tedford RJ.

Circulation. 2016 Jun 14;133(24):2413-22. doi: 10.1161/CIRCULATIONAHA.116.022082. Epub 2016 May 11.


Enrichment of Scleroderma Vascular Disease-Associated Autoantigens in Endothelial Lineage Cells.

McMahan ZH, Cottrell TR, Wigley FM, Antiochos B, Zambidis ET, Park TS, Halushka MK, Gutierrez-Alamillo L, Cimbro R, Rosen A, Casciola-Rosen L.

Arthritis Rheumatol. 2016 Oct;68(10):2540-9. doi: 10.1002/art.39743.


Frequency of circulating topoisomerase-I-specific CD4 T cells predicts presence and progression of interstitial lung disease in scleroderma.

Fava A, Cimbro R, Wigley FM, Liu QR, Rosen A, Boin F.

Arthritis Res Ther. 2016 May 4;18(1):99. doi: 10.1186/s13075-016-0993-2.


Thrombotic complications after radial arterial line placement in systemic sclerosis: A case series.

Paik JJ, Hirpara R, Heller JA, Hummers LK, Wigley FM, Shah AA.

Semin Arthritis Rheum. 2016 Oct;46(2):196-199. doi: 10.1016/j.semarthrit.2016.03.015. Epub 2016 Mar 31.


Genetic susceptibility loci of idiopathic interstitial pneumonia do not represent risk for systemic sclerosis: a case control study in Caucasian patients.

Wu M, Assassi S, Salazar GA, Pedroza C, Gorlova OY, Chen WV, Charles J, Taing ML, Liao K, Wigley FM, Hummers LK, Shah AA, Hinchcliff M, Khanna D, Schiopu E, Phillips K, Furst DE, Steen V, Baron M, Hudson M, Zhou X, Pope J, Jones N, Docherty P, Khalidi NA, Robinson D, Simms RW, Silver RM, Frech TM, Fessler BJ, Fritzler MJ, Molitor JA, Segal BM, Movahedian M, Martín J, Varga J, Mayes MD.

Arthritis Res Ther. 2016 Jan 20;18:20. doi: 10.1186/s13075-016-0923-3.


Anti-Interferon-Inducible Protein 16 Antibodies Associate With Digital Gangrene in Patients With Scleroderma.

McMahan ZH, Shah AA, Vaidya D, Wigley FM, Rosen A, Casciola-Rosen L.

Arthritis Rheumatol. 2016 May;68(5):1262-71. doi: 10.1002/art.39558.


Development and Validation of the Body Concealment Scale for Scleroderma.

Jewett LR, Malcarne VL, Kwakkenbos L, Harcourt D, Rumsey N, Körner A, Steele RJ, Hudson M, Baron M, Haythornthwaite JA, Heinberg L, Wigley FM, Thombs BD; Canadian Scleroderma Research Group.

Arthritis Care Res (Hoboken). 2016 Aug;68(8):1158-65. doi: 10.1002/acr.22819.


Symptomatic and Electrodiagnostic Features of Peripheral Neuropathy in Scleroderma.

Paik JJ, Mammen AL, Wigley FM, Shah AA, Hummers LK, Polydefkis M.

Arthritis Care Res (Hoboken). 2016 Aug;68(8):1150-7. doi: 10.1002/acr.22818.


Validation of the Self-Efficacy for Managing Chronic Disease Scale: A Scleroderma Patient-Centered Intervention Network Cohort Study.

Riehm KE, Kwakkenbos L, Carrier ME, Bartlett SJ, Malcarne VL, Mouthon L, Nielson WR, Poiraudeau S, Nielsen K, Baron M, Frech T, Hudson M, Pope J, Sauve M, Suarez-Almazor ME, Wigley FM, Thombs BD; Scleroderma Patient-Centered Intervention Network Investigators.

Arthritis Care Res (Hoboken). 2016 Aug;68(8):1195-200. doi: 10.1002/acr.22807.


Editorial: Scleroderma: Bringing a Disease From Black-and-White Into Technicolor.

Ligon CB, Wigley FM.

Arthritis Rheumatol. 2015 Dec;67(12):3101-3. doi: 10.1002/art.39310. No abstract available.


Biomarkers of pulmonary hypertension in patients with scleroderma: a case-control study.

McMahan Z, Schoenhoff F, Van Eyk JE, Wigley FM, Hummers LK.

Arthritis Res Ther. 2015 Aug 6;17:201. doi: 10.1186/s13075-015-0712-4.


Pilot study to determine whether transient receptor potential melastatin type 8 (TRPM8) antibodies are detected in scleroderma.

Shah AA, Montagne J, Oh SY, Wigley FM, Casciola-Rosen L.

Clin Exp Rheumatol. 2015 Jul-Aug;33(4 Suppl 91):S123-6. Epub 2015 Aug 5.


Management of Raynaud Phenomenon and Digital Ulcers in Scleroderma.

Cappelli L, Wigley FM.

Rheum Dis Clin North Am. 2015 Aug;41(3):419-38. doi: 10.1016/j.rdc.2015.04.005. Epub 2015 May 23. Review.


Spectrum of Muscle Histopathologic Findings in Forty-Two Scleroderma Patients With Weakness.

Paik JJ, Wigley FM, Lloyd TE, Corse AM, Casciola-Rosen L, Shah AA, Boin F, Hummers LK, Mammen AL.

Arthritis Care Res (Hoboken). 2015 Oct;67(10):1416-25. doi: 10.1002/acr.22620.


Prediction and impact of attacks of Raynaud's phenomenon, as judged by patient perception.

Hughes M, Snapir A, Wilkinson J, Snapir D, Wigley FM, Herrick AL.

Rheumatology (Oxford). 2015 Aug;54(8):1443-7. doi: 10.1093/rheumatology/kev002. Epub 2015 Mar 9.


Examination of autoantibody status and clinical features associated with cancer risk and cancer-associated scleroderma.

Shah AA, Hummers LK, Casciola-Rosen L, Visvanathan K, Rosen A, Wigley FM.

Arthritis Rheumatol. 2015 Apr;67(4):1053-61. doi: 10.1002/art.39022.


Intravenous immunoglobulin may be an effective therapy for refractory, active diffuse cutaneous systemic sclerosis.

Poelman CL, Hummers LK, Wigley FM, Anderson C, Boin F, Shah AA.

J Rheumatol. 2015 Feb;42(2):236-42. doi: 10.3899/jrheum.140833. Epub 2014 Nov 29.


Severity of systemic sclerosis-associated pulmonary arterial hypertension in African Americans.

Blanco I, Mathai S, Shafiq M, Boyce D, Kolb TM, Chami H, Hummers LK, Housten T, Chaisson N, Zaiman AL, Wigley FM, Tedford RJ, Kass DA, Damico R, Girgis RE, Hassoun PM.

Medicine (Baltimore). 2014 Jul;93(5):177-85. doi: 10.1097/MD.0000000000000032.


Myopathy in scleroderma, its identification, prevalence, and treatment: lessons learned from cohort studies.

Paik JJ, Mammen AL, Wigley FM, Gelber AC.

Curr Opin Rheumatol. 2014 Mar;26(2):124-30. doi: 10.1097/BOR.0000000000000024. Review.


Immunochip analysis identifies multiple susceptibility loci for systemic sclerosis.

Mayes MD, Bossini-Castillo L, Gorlova O, Martin JE, Zhou X, Chen WV, Assassi S, Ying J, Tan FK, Arnett FC, Reveille JD, Guerra S, Teruel M, Carmona FD, Gregersen PK, Lee AT, López-Isac E, Ochoa E, Carreira P, Simeón CP, Castellví I, González-Gay MÁ; Spanish Scleroderma Group, Zhernakova A, Padyukov L, Alarcón-Riquelme M, Wijmenga C, Brown M, Beretta L, Riemekasten G, Witte T, Hunzelmann N, Kreuter A, Distler JH, Voskuyl AE, Schuerwegh AJ, Hesselstrand R, Nordin A, Airó P, Lunardi C, Shiels P, van Laar JM, Herrick A, Worthington J, Denton C, Wigley FM, Hummers LK, Varga J, Hinchcliff ME, Baron M, Hudson M, Pope JE, Furst DE, Khanna D, Phillips K, Schiopu E, Segal BM, Molitor JA, Silver RM, Steen VD, Simms RW, Lafyatis RA, Fessler BJ, Frech TM, Alkassab F, Docherty P, Kaminska E, Khalidi N, Jones HN, Markland J, Robinson D, Broen J, Radstake TR, Fonseca C, Koeleman BP, Martin J.

Am J Hum Genet. 2014 Jan 2;94(1):47-61. doi: 10.1016/j.ajhg.2013.12.002.


Association of the autoimmune disease scleroderma with an immunologic response to cancer.

Joseph CG, Darrah E, Shah AA, Skora AD, Casciola-Rosen LA, Wigley FM, Boin F, Fava A, Thoburn C, Kinde I, Jiao Y, Papadopoulos N, Kinzler KW, Vogelstein B, Rosen A.

Science. 2014 Jan 10;343(6167):152-7. doi: 10.1126/science.1246886. Epub 2013 Dec 5.


Novel investigational agents for the treatment of scleroderma.

McMahan ZH, Wigley FM.

Expert Opin Investig Drugs. 2014 Feb;23(2):183-98. doi: 10.1517/13543784.2014.848852. Epub 2013 Nov 22. Review.


Integrin-modulating therapy prevents fibrosis and autoimmunity in mouse models of scleroderma.

Gerber EE, Gallo EM, Fontana SC, Davis EC, Wigley FM, Huso DL, Dietz HC.

Nature. 2013 Nov 7;503(7474):126-30. doi: 10.1038/nature12614. Epub 2013 Oct 9.


Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension.

Tedford RJ, Mudd JO, Girgis RE, Mathai SC, Zaiman AL, Housten-Harris T, Boyce D, Kelemen BW, Bacher AC, Shah AA, Hummers LK, Wigley FM, Russell SD, Saggar R, Saggar R, Maughan WL, Hassoun PM, Kass DA.

Circ Heart Fail. 2013 Sep 1;6(5):953-63.


Race and association with disease manifestations and mortality in scleroderma: a 20-year experience at the Johns Hopkins Scleroderma Center and review of the literature.

Gelber AC, Manno RL, Shah AA, Woods A, Le EN, Boin F, Hummers LK, Wigley FM.

Medicine (Baltimore). 2013 Jul;92(4):191-205. doi: 10.1097/MD.0b013e31829be125. Review.

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