© Springer International Publishing Switzerland 2016 287
Y. El Miedany (ed.), Patient Reported Outcome Measures in Rheumatic Diseases,
DOI 10.1007/978-3-319-32851-5_11
Chapter 11
PROMs for Systemic Sclerosis (Scleroderma)
Russell E. Pellar , Theresa M. Tingey , and Janet E. Pope
Introduction
Systemic sclerosis, otherwise known as scleroderma or SSc, is a rare multisystem
connective tissue disease of unknown cause with fi brosis of the skin and internal
organs, vascular disruption and damage, and the production of autoantibodies.
These characteristic features lead to the development of many heterogeneous clini-
cal features depending on the extent of disease and organs involved [ 1 ]. SSc is
chronic in its course and is associated with signifi cant morbidity and mortality and
is one of the most severe connective tissue diseases [ 2 – 4 ].
Perhaps the most well recognized and universal physical manifestation of SSc is
the progressive skin thickening and fi brosis [ 1 ]. This distinctive element of SSc clas-
sically involves the hands, but may also affect the arms, trunk, face, legs, and essen-
tially any skin and carries the potential to cause substantial disfi gurement and disability
[ 5 – 7 ]. Due to the fi brosis, infl ammation, pain, and joint contractures of SSc, patients
often struggle with essential and basic tasks such as holding objects, reaching, eating,
bathing, grooming, dressing, climbing stairs, and/or walking at all [ 8 ]. Because SSc is
chronic and progressive, impaired function tends to worsen with time [ 9 ].
Although the clinical course is quite variable, SSc is typically classifi ed as either
limited cutaneous SSc (lcSSc) or diffuse cutaneous SSc (dcSSc), depending on the
extent of skin involvement. In lcSSc, skin fi brosis not only is found distal to the
R. E. Pellar , B.E.Sc. (*) • T. M. Tingey , B.Arts.Sc.
Schulich School of Medicine and Dentistry , University of Western Ontario , London , ON , Canada
e-mail: [email protected]
J. E. Pope , M.D., M.P.H., F.R.C.P.C.
Division of Rheumatology, Schulich School of Medicine and Dentistry ,
University of Western Ontario , London , ON , Canada
Division of Rheumatology, Department of Medicine , St. Joseph’s Health Care London ,
London , ON , Canada