Inclusion Criteria:

1. All patients must fulfill the criteria for SSc by ACR criteria (Subcommittee for
Scleroderma Criteria 1980).

2. Age of entry into the study ≥ 18 yrs

3. FVC <85% of predicted.

4. Able to complete the 6MWT with a walking distance ≥ 150 m

5. Patients must have dyspnea on exertion (grade ≥ 2 on the Magnitude of Task component
of the Mahler Modified Dyspnea Index).

6. SSc for ≤ 10 years, with onset defined as the date of the first non-Raynaud
manifestation typical of systemic sclerosis.

7. Patients may have limited (cutaneous thickening distal but not proximal to elbows and
knees, with or without facial involvement) or diffuse (cutaneous thickening proximal
to elbows and knees, often involving the chest or abdomen) cutaneous SSc (Medsger
1995).

8. Patients must show some evidence of alveolitis as defined by an HRCT of the lung
which shows ground glass opacification as a radiographic marker of "alveolitis" or
finely reticulated fibrosis or they must have alveolitis by BAL ( ≥ 3% PMN's or ≥ 2%
eosinophils).

9. Female patients of childbearing potential must have negative pregnancy test within 7
days before initiation of study drug dosing. Postmenopausal women must be amenorrheic
for at least 12 months to be considered of non-childbearing potential. Male and
female patients of reproductive potential must agree to employ an effective barrier
method of birth control throughout the study and for up to 3 months following
discontinuation of study drug.

10. Patients must be able to provide written voluntary informed consent.

Exclusion Criteria:

1. FVC ≤ 50% of predicted or DLCO (corrected for Hgb but not for alveolar volume) ≤ 35%
of predicted (suggesting severe probably irreparable disease and/or significant
pulmonary vascular involvement by SSc).

2. FEV1/FVC ratio <65% (to exclude significant airflow obstruction)

3. Clinically significant abnormalities on HRCT not attributable to SSc (e.g., lung
mass, extensive scarring due to previous infection, etc.)

4. Clinically significant pulmonary hypertension documented on right heart
catheterization (i.e., right ventricular systolic pressure of >50 mm Hg and/or mean
PAP ≥30 mm Hg) pulmonary pressure or echocardiographic evidence of PAH (if echo
cardiographic systolic pressure ≥ 55 mmHg) or FVC/DLCO ratio >1.6 on pulmonary
function testing

5. Persistent unexplained hematuria (>10 RBCs/hpf).

6. History of persistent leukopenia (white blood cell count <3500), neutropenia
(absolute neutrophil count < 1500) or thrombocytopenia (platelet count <100,000).

7. Clinically significant anemia (<9.0 gm/dl)

8. Serum creatinine >ULN.

9. Pregnancy (documented by urine pregnancy test), breast feeding

10. If of child-bearing potential, failure regularly to be employing a reliable means of
contraception (i.e., condom, abstinence, IUD, tubal ligation, vasectomy)

11. Active infection of the lung or elsewhere, whose management would be compromised by
Imatinib

12. Unreliability, drug abuse (including active alcoholism)

13. Any chronic, debilitating illness (other than SSc) which might compromise the
patient's participation in the trial.

14. Smoking of cigars, pipes or cigarettes during the past 6 months

15. Baseline liver function tests (ALT or AST or bilirubin >1.5 x upper limit of normal

16. Previous use of prednisone > 10 mg per day. If on prednisone ≤10 mg/d, dose must have
been stable for > 1 month.

17. All other medication with putative disease-modifying properties (e.g.,
D-penicillamine, cyclophosphamide, azathioprine, methotrexate, colchicine, Potaba)
must be discontinued 1 month prior to beginning study medication.

18. Patient is < 5 years since she/he had a primary malignancy except: if the other
primary malignancy is not currently clinically significant nor requiring active
intervention, or if other primary malignancy is a basal cell skin cancer or a
cervical carcinoma in situ. Existence of any other malignant disease is not allowed
except after consultation with the PI.

19. Patient with Grade III/IV cardiac problems as defined by the New York Heart
Association Criteria. (i.e., congestive heart failure, myocardial infarction within 6
months of study)

20. Patient has a severe and/or uncontrolled medical disease (i.e., uncontrolled
diabetes, chronic renal disease, or active uncontrolled infection).

21. Patient has known chronic liver disease (i.e., chronic active hepatitis and
cirrhosis).

22. Patient has a known diagnosis of human immunodeficiency virus (HIV) infection.

23. Use of contraindicated medications at baseline. If used, need to be discussed with
PI, attempts made to find a substitute, documented and kept stable throughout. (see
appendix 1).