example, Black et al. reported that 33 (43%) of 76 SCLE patients followed for between 2 and 10 years had features of SjS as determined by physician assessment at the time of study analysis [50]. The rates at which SCLE develops in patients initially diagnosed as having SjS has not been examined thoroughly.

11.4.2Skin Changes Associated with Rheumatoid Arthritis, Systemic Sclerosis, and the Idiopathic Inflammatory Myopathies

It is beyond the scope of this presentation to address each of the other autoimmune connective tissue diseases that can overlap with SjS. The more common skin findings that are found in these disorders are listed in the Table 11.1.

The reader is also referred to Table 11.1 for: (1) skin changes associated with the 8.1 ancestral haplotype that can be encountered in SjS patients; (2) skin changes reported anecdotally in SjS patients for which an association has yet to be confirmed; and (3) skin changes that can result from the treatment of SjS.

Acknowledgments There was no extramural support for this project. Dr. Sontheimer’s effort on this project were facilitated by internal support from the University of Utah Department of Dermatology in return for his work in strengthening the departmental academic mission. We express our appreciation to the following individuals who generously provided clinical images to illustrate this chapter: Jan P. Dutz, M.D. (Department of Dermatology, University of British Columbia, Canada) as well as Glen M. Bowen, M.D. and Scott R. Florell, M.D. (Department of Dermatology, University of Utah School of Medicine, USA).

Conflicts of interest None to declare.

External funding sources None.

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Chapter 12

Vasculitis and Sjögren’s Syndrome

George E. Fragoulis, Haralampos M. Moutsopoulos, and John H. Stone

12.1Introduction

Sjögren’s syndrome (SjS) is a chronic autoimmune disorder characterized by dysfunction and destruction of exocrine glands, principally the salivary and lacrimal glands. Vasculitis, one of the major extraglandular manifestations of SjS, frequently heralds the need for systemic therapy and serves as an indicator of a risk for poor outcomes in many patients. Poor outcomes associated with the development of vasculitis may include direct complications of the vascular inflammation, such as complications of cutaneous ulceration and vasculitic neuropathy, as well as the side

G.E. Fragoulis • H.M. Moutsopoulos

Department of Pathophysiology, School of Medicine,

National University of Athens, Athens, Greece

J.H. Stone (*)

Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital,

Boston, MA, USA