1. Scleroderma Lab Data and Diagnosis Iraj Salehi-Abari MD., Internist
Rheumatologist

2. Lab. Data: CBC: Anemia ESR Renal FT, Liver FT FANA: 95%, speckled CXR
Salehi I.
Lab. Data:
CBC: Anemia
ESR
Renal FT, Liver FT
FANA: 95%, speckled
Scl-70
ACA
CXR
Scleroderma

3. Lab. Data: AOCD; mild, most common IDA: GAVE, chronic esophagitis
Salehi I.
Lab. Data:
AOCD; mild, most common
IDA: GAVE, chronic esophagitis
Macrocytic anemia:
B12, Folate deficiency
Drugs: MTX
Microangiopathic hemolytic anemia:
SRC
Scleroderma

4. Lab. Data: Pancytopenia: Elevated ESR: Drugs SRC Malignancy Myositis
Salehi I.
Lab. Data:
Pancytopenia:
Drugs
SRC
Malignancy
Elevated ESR:
Myositis
Scleroderma

5. Lab. Data: Antitopoisomerase I (Scl-70)
Highly specific for SSc
High risk of ILD
Associated with dcSSc
Anti-centromere antibodies (ACA)
Associated with lcSSc
Salehi I.
Lab. Data:
Scleroderma

6. Lab. Data: AntiU3-RNP (Fibrillarin): SSc + PAH
Salehi I.
AntiPM-Scl: PM + Scl
AntiU3-RNP (Fibrillarin): SSc + PAH
Anti RNA polymerase I & III: only in SSc
Anti RNA polymerase II: in SLE or SSc
RF: 25%
Lab. Data:
Scleroderma

7. Attention please: Scl-70 & ACA &
Salehi I.
Attention please:
Scl-70 &
ACA &
Anti-RNA polymerase III antibody tests
are highly specific (> 99.5%) but only moderately sensitive (20-50%)
Over 95% of SSc patients have at least one autoantibodies
Scleroderma

8. Lab. Data: GI Endoscopy Doppler Echocardiography HRCT of lung
Salehi I.
Lab. Data:
GI Endoscopy
Doppler Echocardiography
HRCT of lung
Hand X-Ray
Manometry, Dlco
Spirometry, Pletismography
Thyroid FT
Skin biopsy
Scleroderma

9. Skin biopsy: Not necessary for diagnosis of SSc
Salehi I.
Skin biopsy:
Not necessary for diagnosis of SSc
Only done in doubtful cases
For DD with Scleroderma-like disorders
May be necessary for localized Scleroderma
Should include subcutaneous tissue, fascia, and muscle
Scleroderma

10. Scleroderma-like disorders:
Salehi I.
Scleroderma-like disorders:
Scleredema
Scleromyxedema
Diabetes Mellitus
Hypothyroidism
Nephrogenic systemic fibrosis
Amyloidosis
Eosinophilic fasciitis
Chronic GVHD
Scleroderma

11. Limited Cutaneous SSc (lcSSc):
Salehi I.
Limited Cutaneous SSc (lcSSc):
Raynaud’s ph. For years
Acro-facial skin sclerosis
Dilated nailfold capillary loops( no drop-out )
Accompanied by CREST syndrome
Pulmonary Artery Hypertension( PAH )
ILD lately & SRC rarely occurs
ACA: %
Scleroderma

12. Diffuse Cutaneous SSc (dcSSc):
Salehi I.
Diffuse Cutaneous SSc (dcSSc):
Raynaud’s ph. Followed, within one year, by puffy skin changes
Trunkal & acro-facial skin sclerosis
Tendon friction rubs
Nailfold capillary dilatation (with drop-out)
Early & significant incidence of SRC, ILD, GI & cardiac disease
Anti-Scl-70: 30%
Scleroderma

13. Pre-Scleroderma Subclinical Scleroderma Early Scleroderma
Salehi I.
Pre-Scleroderma Subclinical Scleroderma Early Scleroderma
Scleroderma

14. Pre-Scleroderm Raynaud’s phenomenon plus
Salehi I.
Pre-Scleroderm
Raynaud’s phenomenon plus
Nailfold capillary changes &/or
Autoantibodies:
Scl-70
ACA
Anti-RNA polymerase
No skin involvement
Scleroderma

15. Diagnosis of SSc: Clinical: Upon No skin biopsy: only for History &
Salehi I.
Diagnosis of SSc:
Clinical: Upon
History &
Physical examination
No skin biopsy: only for
Localized Scl.
DD with Scl. Like disorders
Scleroderma

16. The ACR/EULAR criteria for the classification of SSc
Salehi I.
The ACR/EULAR criteria for the classification of SSc
1. Skin thickening of both hands proximal to MCPs p
2. Puffy fingers p
2. Sclerodactyly p
3. Digital tip ulcers p
3. Pitting ulcer p
4. Facial, lip telangiectasia p
5. Abnormal nailfold capillaries p
6. PAH p
6. ILD p
7. Raynaud’s ph p
8. Autoantibodies: ACA, Scl-70, Anti-RNA polymerase III p
Scleroderma

17. Attention please: The presence of : Calcinosis cutis
Salehi I.
Attention please:
The presence of :
Calcinosis cutis
Hyperpigmentation &/or Skin telangiectasia and
Facial and Fingers features
Can differentiate SSc from other DD
Scleroderma

18. Prognosis & Mortality:
Salehi I.
Prognosis & Mortality:
Mortality rate:
in dcSSc # 5-8 fold higher than nl population
In lcSSc # 2 fold
The most common cause of death in dcSSc:
Pulmonary fibrosis &/or PAH
SRC
Cardiac
Scleroderma

19. Management: No cure, No remission Localized or Systemic Scl.
Salehi I.
Management:
No cure, No remission
Localized or Systemic Scl.
Which type of systemic Scl.
Nonpharmacologic therapy
Pharmacologic
Symptomatic therapy
Systemic therapy:
Vascular + Immunologic + Fibrotic
Minor vs major
Scleroderma

20. Salehi I.
Attention please:
Elucidation of the precise interplay between the Vascular, Immunologic and Fibrotic components of SSc is likely to be an important first step towards more effective therapy
Scleroderma

21. Disease modifying therapy:
Salehi I.
Disease modifying therapy:
Immunosuppressive therapy:
Glucocorticoids (LDS)
MTX
Mycophenolate mofetil
Cyclophosphamide
Autologous stem cell transplantation
Scleroderma

22. Disease modifying therapy:
Salehi I.
Disease modifying therapy:
Anti-fibrotic therapy:
D-penicillamine:
Standard-dose: 750 mg/day
Low-dose: 125 mg qod
Scleroderma

23. Disease modifying therapy:
Salehi I.
Disease modifying therapy:
Vascular therapy:
Calcium channel blockers: Diltiazem
Angiotensin II receptor blockers: Losartan
Alpha-1 blocker: prazocin
5-phosphodiesterase inhibitors: Sildenafil
SSRI: Fluoxetine
Topical nitroglycerine
IV prostaglandine: Iloprost
Anti-platelet agent: low-dose Aspirin
Endothein-1 receptor antagonist: Bosentan
Scleroderma

24. Pulmonary therapy: Severe acute ILD: Cyclophosphamide plus
Salehi I.
Pulmonary therapy:
Severe acute ILD:
Cyclophosphamide plus
Medium dose Glucocorticoids
Symptomatic PAH:
Bosentan
Sildenafil
Scleroderma

25. Gastrointestinal therapy:
Salehi I.
Gastrointestinal therapy:
GERD: PPI
Omeprazole, Pantozol
Nexium (esomeprazole)
GI bleeding: Watermelon, GAVE
Laser photocoagulation
Bacterial overgrowth:
Metronidazole
Erythromycin & Tetracycline
Chronic hypomotility:
Octreotide
Scleroderma

26. Scleroderma Renal Crisis therapy:
Salehi I.
Scleroderma Renal Crisis therapy:
ACE inhibitors: 90% of cases
Hemodialysis: 2/3 of cases
In ½ of cases: recovery
In ½ of cases who are unable to D/C dialysis after 2 years: Renal transplantation
Recurrency of SRC in transplanted kidney is rare
Scleroderma

27. Symptomatic therapy: Pruritus Dry skin Dyspareunia
Salehi I.
Symptomatic therapy:
Pruritus
Dry skin
Dyspareunia
Facial telangiectasis
DOE
Scleroderma