CREST and Systemic Sclerosis: What you need to know

9/19/2020

CREST and Systemic Sclerosis: What You Need to Know

Ohio Association of Rheumatology 2020 Vickie Sayles, MHA, BSN-RN, RN-BC, CRNI

Objectives

? * To recognize the differences between CREST and systemic

sclerosis

? * To learn about the pathophysiology of scleroderma ? * To review the the clinical features present in CREST and systemic

sclerosis

? * To identify risk factor associated with these diseases ? * To identify current and future treatment options for patients

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Pathophysiology of Scleroderma (SSc)

? Scleroderma is a rare connective tissue disease with unknown and complex

pathogenesis. The exact etiology of Scleroderma is not completely understood, but both genetic and environmental factors are thought to play a part

Genetic Association

Very rare genetic association/more common in females/30-50 years

? The risks of Scleroderma and other autoimmune diseases are increased in relatives

of people with SSc Familial clustering: excess occurrence of a disease within a family compared to occurrence in the general population More common in African Americans and Native Americans Having family members with an autoimmune condition increases risk May be exposure to environmental factors shared by members of some families

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Environmental Factors

? Several environmental triggers have been associated with development of SSc ? Infectious agents such as Cytomegalovirus (CMV), Epstein-Barr virus (EBV) and

parvovirus B19.

? Exposure to silica dust ? Exposure to certain solvents (epoxy resins, organic solvents) ? Some drugs such as Bleomycin and cocaine have been associated with the

development of SSc like illness (graft vs host disease, Werner's syndrome)

Werner's Syndrome

? Dramatic, rapid appearance of features associated with normal aging.

? Grow and develop normally until puberty ? Do not have a normal growth spurt ? Aged appearance begins in twenties (graying and loss of hair; a

hoarse voice; and thin, hardened skin) ? Facial appearance described as "bird-like" ? As Werner syndrome progresses, cataracts, skin ulcers, diabetes

infertility, severe hardening of arteries, osteoporosis, cancer ? Life expectancy 40-50

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Systemic Sclerosis vs. CREST

? Scleroderma can be divided into two distinct forms, Localized Scleroderma

or Systemic Sclerosis. These can be further be classified as either Limited Cutaneous Systemic Sclerosis (CREST syndrome) or Diffuse Cutaneous Systemic Sclerosis. Diagnosis is based on clinical and serological criteria (Adigun et al., 2020). It primarily affects women (female: male ratio of 4:110:1, depending on age and ethnicity)

CREST

? CREST syndrome, also known as limited or localized scleroderma, is a

diffuse connective tissue disease which is differentiated by changes in the skin and subcutaneous tissue, blood vessels, skeletal muscles, and internal organs. Biopsy of the skin reveals dermal fibrosis like the changes seen in the thickened skin of Systemic Sclerosis. Although the skin changes approximate the changes with Systemic Sclerosis, Limited Scleroderma is not associated with some of the more serious pathology seen in Systemic Sclerosis.

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CREST is an acronym for the clinical features that are identified in a patient with this disease.

Calcinosis

Raynaud's phenomenon

Esophageal disfunction

Sclerodactyly

Telangiectasias

CREST

Calcinosis

? Abnormal collection of calcium salts in or under the skin and

in muscles or tendons

? Appear frequently in dermatomyositis but also may be

present in patients with overlapping autoimmune diseases, including systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease.

? Hard, irregular nodules in or under the skin in any area of the

body

? Very uncomfortable when they appear on the face, around

joints, or on pressure points

? Can cause functional disability, contractures, skin ulcers, and

pain.

? Have a significantly negative impact on quality of life.

? Why nodules develop is not well-understood

? Tissue damage from inflammation and blood vessel

changes?

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