1. Virginia Steen, MD Professor of Medicine
SCLERODERMA
Virginia Steen, MD
Professor of Medicine

2. Scleroderma Localized Scleroderma Morphea Linear Scleroderma
En Coup de Sabre (Progressive Hemi-atrophy)
Pansclerotic, Deep subcutaneous

3. Linear Scleroderma Hyperpigmented, Linear, Muscle atrophy
but normal strength
Linear,
single extremity

4. Severe contractures, growth disturbances, atrophy
Linear Scleroderma
Severe contractures, growth disturbances, atrophy

5. Systemic Sclerosis
An uncommon disease 250/million population, new cases/ million per year, about ,000 in US
Age onset usually 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

6. 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

7. 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

9. 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 FINGERs Swollen, puffy HANDs Limited skin thickening Early diffuse skin
Anti-centromere antibody Anti-Scl 70 antibody
Anti-RNA polymerase III

10. NATURAL HISTORY OF SCLERODERMA SUBSETS Diffuse scleroderma
Contractures
Renal crisis
Myocardial failure 50
SKI N
THICKNESS
Pulmonary fibrosis 40
Diffuse scleroderma 30 20
Pulmonary hypertension Malabsorption
Limited scleroderma 10 5 10
DISEASE DURATION (YEARS)

11. RAYNAUD’S

12. Digital Ulcers

13. ... and can lead to auto-amputation

14. Skin Thickening

15. Swollen Hands

16. Early Scleroderma Puffy Phase

17. Evaluation of Skin Thickening
Rodnan Skin Score
17 different sites-
Score 0 to 3
Total 51
Limited - <12
Diffuse >12
Measuring skin thickness.
Rodnan Skin Score
Other Measures- Health Assessment Questionnaire
Medsger Severity Scale (only research)

18. 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

19. Sclerodactyly

20. Hand Contractures

21. CALCINOSIS

22. ACROOSTEOLYSIS

24. Systemic Sclerosis- Multisystem Disease

25. 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

26. 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)

27. 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

28. 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

29. Heart and Kidney Less common but more serious
Heart- Pericarditis, pericardial effusion, cardiomyopathy, rhythm problems, heart failure
Kidney- Malignant hypertension, kidney failure, dialysis.

30. 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.

31. 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

32. Disability in Diffuse Scleroderma
Early in Disease- mostly from progressive skin thickening, pain, fatigue, weight loss, contractures, digital ulcers.
Pulmonary fibrosis
Heart and Kidney

33. 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.