scleroderma virginia steen, md professor of medicine
TRANSCRIPT
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SCLERODERMA
Virginia Steen, MD
Professor of Medicine
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Scleroderma
• Localized Scleroderma–Morphea– Linear Scleroderma– En Coup de Sabre (Progressive
Hemi-atrophy)– Pansclerotic, Deep subcutaneous
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Linear Scleroderma
Linear, single extremity
Hyperpigmented, Muscle atrophy
but normal strength
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Linear Scleroderma
Severe contractures, growth disturbances, atrophy
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Systemic Sclerosis
An uncommon disease 250/million population, 20 new cases/ million per year, about 80- 150,000 in US
Age onset usually 30-50 years, rare under 10.
Female 3-5: 1; Increased in African-Americans
Multisystem disease - Raynauds, digital ulcers, arthritis, tendon inflammation, skin thickening, myopathy, gastrointestinal, lung, heart and kidney involvement
Survival – decreased primarily from severe lung involvement, pulmonary fibrosis and pulmonary hypertension
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Diagnosis
• Clinical diagnosis by Rheumatologist– Raynaud’s– Swollen fingers and/or skin thickening of
hands/face– Esophageal symptoms-GERD. – Other organs-
• Small intestines• Pulmonary Fibrosis• Pulmonary Hypertension• Cardiac or Kidney involvement
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Laboratory Diagnosis
• Laboratory - not required– Antibodies helpful for prognosis, but not
necessary for diagnosis ( even ANA can be negative)
– There can be false positives, particularly slightly positive tests
– GI x-rays supportive but not required for diagnosis
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Clinical features associated with limited and diffuse scleroderma
Limited cutaneous Diffuse cutaneous Raynaud’s -1st symptom, Raynaud’s often delayed
alone for many years Acute onset, a lot of
Milder general symptoms constitutional symptoms
Milder joint symptoms Arthralgias, carpal tunnel
Tendon friction rubs
Puffy FINGERsFINGERs Swollen, puffy HANDs HANDs Limited skin thickening Early diffuse skin
Anti-centromere antibody Anti-Scl 70 antibody
Anti-RNA polymerase III
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SKI N
THICKNESS
50
40
30
20
10
0
DISEASE DURATION (YEARS)5 10 15 20
ContracturesRenal crisisMyocardial failure
Pulmonary hypertension Malabsorption
Limited sclerodermaLimited scleroderma
Pulmonary fibrosis
Diffuse sclerodermaDiffuse scleroderma
NATURAL HISTORY OF SCLERODERMA SUBSETS
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RAYNAUD’S
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Digital Ulcers
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... and can lead to auto-amputation
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Skin Thickening
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Swollen Hands
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Early Scleroderma Puffy Phase
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Evaluation of Skin Thickening
Measuring skin thickness. Rodnan Skin Score
Rodnan Skin Score17 different sites-Score 0 to 3 Total 51Limited - <12Diffuse >12
Other Measures- Health Assessment Questionnaire Medsger Severity Scale (only research)
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Joint and Tendon
• Hand swelling, joint pain and stiffness- fingers, wrists, swelling/puffiness, other joints also
• Contractures- hands, wrists, hips, shoulders, elbows.
• Acroosteolysis- deformity causing loss of function
• Tendon rubs- painful- hands, arms, ankles, knees
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Sclerodactyly
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Hand Contractures
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CALCINOSIS
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ACROOSTEOLYSIS
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Systemic Sclerosis- Multisystem Disease
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Gastrointestinal Involvement
• Esophageal- trouble swallowing, heartburn, reflux, potential aspiration
• Stomach –bloating, inability to eat full meals, need to eat small frequent meals
• Small Intestine- malabsorption, diarrhea, pseudo obstruction, bacterial overgrowth, weight loss, need for hyperalimentation.
• Large Intestine- constipation, rectal prolapse• Rectum- fecal incontinence
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PULMONARY PROBLEMS IN SYSTEMIC SCLEROSIS
• Pleurisy, pleural effusions, pleural scarring• Spontaneous pneumothorax (bronchiectasis)• Aspiration pneumonia• Malignancy-all cell types• BOOP• Interstitial fibrosis• Pulmonary vascular disease (PHT)
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Pulmonary Fibrosis
• Shortness of breath with activity
• Fatigue with activity
• Pulmonary function tests- Decreased FVC, TLC and DLCO, restrictive disease
• CT scan of lung- scarring, honeycombing
• Begins early in disease and progresses slowly or rapidly, major cause of death
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Pulmonary Hypertension
• Shortness of breath and fatigue with exercise
• Occurs later in illness
• More common in limited scleroderma
• Low DLCO on PFTs, and high PAP on echo
• Most common cause of death
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Heart and Kidney
• Less common but more serious
• Heart- Pericarditis, pericardial effusion, cardiomyopathy, rhythm
problems, heart failure
• Kidney- Malignant hypertension, kidney failure, dialysis.
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Disability in Limited Scleroderma
– Usually after a long history of Raynaud’s, (unless digital ulcers)
– Pain, fatigue, GI symptoms– Loss of hand function – digital ulcers,
loss of mobility/dexterity, fine motion– Fatigue/shortness of breath- anemia,
weight loss, GI, pulmonary fibrosis/hypertension.
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Special Situations
• Raynaud’s – can be disabling without ulcers, if job is outdoors, requires cold exposure (meat cutter)
• Limited skin- can be disabling even without contractures if very swollen, late acroosteolysis, need for fine motion
• GI –can be very disabling - with severe reflux, vomiting, difficulty swallowing, fatigue, inability to eat, weight loss
•
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Disability in Diffuse Scleroderma
– Early in Disease- mostly from progressive skin thickening, pain, fatigue, weight loss, contractures, digital ulcers.
– Pulmonary fibrosis– Heart and Kidney
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Special Consideration
• Most diffuse scleroderma patients have enough problems that they are disabled.
• While some diffuse scleroderma patients are able to continue working, it is usually because they are professionals and have a very flexible work situation.