SYSTEMIC SCLEROSIS (SCLERODERMA) Flashcards
What is systemic sclerosis?
Hardening or thickening of the skin owing to abnormal dermal collagen.
What is the difference between limited scleroderma and diffuse scleroderma?
Limited scleroderma involves cutaneous manifestations that mainly affect the hands, arms and face. (Also CREST syndrome)
Diffuse scleroderma is rapidly progressing and affects a large area of the skin and one or more internal organs, frequently the kidneys, esophagus, heart and/or lungs. This form of scleroderma can be quite disabling.
Are males or females more affected by systemic sclerosis and what is the ratio?
Females 3:1
What is the peak incidence in terms of age?
30 to 50 years
What are the components of CREST in systemic sclerosis?
Calcinosis Raynaud's phenomenon Esophageal dysmotility Sclerodactyly Telangiectasia
What is the difference between limited cutaneous scleroderma and diffuse cutaneous scleroderma?
Limited cutaneous scleroderma involves cutaneous manifestations that mainly affect the hands, arms and face. (Also CREST syndrome)
Diffuse cutaneous scleroderma is rapidly progressing and affects a large area of the skin and one or more internal organs, frequently the kidneys, esophagus, heart and/or lungs. This form of scleroderma can be quite disabling.
What are the components of CREST in systemic sclerosis?
Calcinosis Raynaud's phenomenon Esophageal dysmotility Sclerodactyly Telangiectasia
What does the C of CREST in systemic sclerosis refer to? Describe the feature in more detail.
Calcinosis - thickening and tightening of the skin with deposition of calcific nodules
What does the R of CREST in systemic sclerosis refer to? Describe the feature in more detail.
Raynaud’s phenomenon is frequently the first manifestation of CREST/lcSSc, preceding other symptoms by years. Stress and cold temperature induces an exaggerated vasoconstriction of the small arteries, arterioles, and thermoregulatory vessels of the skin of the digits. Clinically this manifests as a white-blue-red transitions in skin color. Underlying this transition is pallor and cyanosis of the digits, followed by a reactive hyperemia as they rewarm. In contrast to uncomplicated primary Raynaud phenomenon, this occurrence is often painful.[2] Frequently this phenomenon leads to digital ulcerations, gangrene, or amputation. Ulceration can predispose to chronic infections of the involved site
What does the R of CREST in systemic sclerosis refer to? Describe the feature in more detail.
Raynaud’s phenomenon is frequently the first manifestation of CREST, preceding other symptoms by years. Stress and cold temperature induces an exaggerated vasoconstriction of the small arteries, arterioles, and thermoregulatory vessels of the skin of the digits. Clinically this manifests as a white-blue-red transitions in skin color. Underlying this transition is pallor and cyanosis of the digits, followed by a reactive hyperemia as they rewarm. In contrast to uncomplicated primary Raynaud phenomenon, this occurrence is often painful. Frequently this phenomenon leads to digital ulcerations, gangrene, or amputation.
What does the R of CREST in systemic sclerosis refer to? Describe the feature in more detail.
Raynaud’s phenomenon is frequently the first manifestation of CREST, preceding other symptoms by years. Stress and cold temperature induces an exaggerated vasoconstriction of the small arteries, arterioles, and thermoregulatory vessels of the skin of the digits. Clinically this manifests as a white-blue-red transitions in skin color. Underlying this transition is pallor and cyanosis of the digits, followed by a reactive hyperemia as they rewarm. In contrast to uncomplicated primary Raynaud phenomenon, this occurrence is often painful. Frequently this phenomenon leads to digital ulcerations, gangrene, or amputation.
What does the E of CREST in systemic sclerosis refer to? Describe the feature in more detail.
Esophageal dysmotility - Presents as a sensation of food getting stuck (dysphagia) in the mid or lower esophagus, atypical chest pain, or cough. Patients often state they must drink liquids to swallow solid food. This motility problem results from atrophy of the gastrointestinal tract wall smooth muscle. This change may occur with or without pathologic evidence of significant tissue fibrosis.
What does the E of CREST in systemic sclerosis refer to? Describe the feature in more detail.
Esophageal dysmotility - Presents as a sensation of food getting stuck (dysphagia) in the mid or lower esophagus, atypical chest pain, or cough. Patients often state they must drink liquids to swallow solid food. This motility problem results from atrophy of the gastrointestinal tract wall smooth muscle. This change may occur with or without pathologic evidence of significant tissue fibrosis.
What does the S of CREST in systemic sclerosis refer to? Describe the feature in more detail.
Though it is the most easily recognizable manifestation, it is not prominent in all patients. Thickening often only involves the skin of the fingers distal to the metacarpophalangeal joints. Early in the course of the disease, the skin may appear edematous and inflamed. Eventually, dermal fibroblasts overproduce extracellular matrix leading to increased tissue collagen deposition in the skin. Collagen cross-linking then causes a progressive skin tightening. Digital ischemic ulcers commonly form on the distal fingers in 30-50% of patients.
What does the S of CREST in systemic sclerosis refer to? Describe the feature in more detail.
Though it is the most easily recognizable manifestation, it is not prominent in all patients. Thickening often only involves the skin of the fingers distal to the metacarpophalangeal joints. Early in the course of the disease, the skin may appear edematous and inflamed. Eventually, dermal fibroblasts overproduce extracellular matrix leading to increased tissue collagen deposition in the skin. Collagen cross-linking then causes a progressive skin tightening. Digital ischemic ulcers form in 30-50% of patients.
What does the S of CREST in systemic sclerosis refer to? Describe the feature in more detail.
Sclerodactyly - Though it is the most easily recognizable manifestation, it is not prominent in all patients. Thickening often only involves the skin of the fingers distal to the metacarpophalangeal joints. Early in the course of the disease, the skin may appear edematous and inflamed. Eventually, dermal fibroblasts overproduce extracellular matrix leading to increased tissue collagen deposition in the skin. Collagen cross-linking then causes a progressive skin tightening. Digital ischemic ulcers form in 30-50% of patients.
What does the T of CREST in systemic sclerosis refer to? Describe the feature in more detail.
Telangiectasis - dilated capillaries occur in the skin of the face, the palmar surface of the hands, and the mucous membranes. Telangiectasias tend to be more numerous in those patients with other scleroderma related vascular disease (i.e. pulmonary arterial hypertension). The number of telangiectasias and the sites involved tend to increase over time.
What are the complication of organ involvement in patients with diffuse cutaneous systemic sclerosis?
Lethargy Anorexia Weight loss Heartburn, reflux or dysphagia Anal incontinence Malabsorption - related to bacterial overgrowth due to dilatation and atony of small bowel Pseudobstruction of bowel Acute hypertensive renal crisis Chronic kidney disease Fibrosis of the lungs Pulmonary hypertension Myocardial fibrosis - leading to arrythmias and conduction defects
