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The pulmonary function and chest roentgenograms were evaluated in 88 patients with the CREST syndrome variant of progressive systemic sclerosis (PSS or scleroderma). Seventy-two percent of the patients had abnormal pulmonary function. An isolated decrease in diffusing capacity was the most common abnormality noted, followed by restrictive abnormalities and airway obstruction. Chest roentgenograms revealed interstitial infiltrates consistent with pulmonary fibrosis in 33 percent. When compared to a contemporaneous group of 77 patients with PSS and diffuse scleroderma, patients with the CREST syndrome had similar abnormalities on pulmonary function testing and chest roentgenogram. However, patients with the CREST syndrome had a lower mean diffusing capacity despite a higher mean vital capacity; this combination of findings suggests primary pulmonary vascular disease. Calcified granulomata were identified significantly more often in PSS-CREST patients, while superior rib notching occurred exclusively in patients with PSS and diffuse scleroderma. The CREST variant of PSS is associated with frequent roentgenographic and pulmonary function abnormalities similar to those seen in PSS with diffuse scleroderma.
G R Owens, and
G J Fino, and
D L Herbert, and
V D Steen, and
T A Medsger, and
B E Pennock, and
J J Cottrell, and
G P Rodnan, and
R M Rogers
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G R Owens, and
G J Fino, and
D L Herbert, and
V D Steen, and
T A Medsger, and
B E Pennock, and
J J Cottrell, and
G P Rodnan, and
R M Rogers
September 1984,
The American journal of medicine,
G R Owens, and
G J Fino, and
D L Herbert, and
V D Steen, and
T A Medsger, and
B E Pennock, and
J J Cottrell, and
G P Rodnan, and
R M Rogers
November 1979,
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G R Owens, and
G J Fino, and
D L Herbert, and
V D Steen, and
T A Medsger, and
B E Pennock, and
J J Cottrell, and
G P Rodnan, and
R M Rogers
August 1989,
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G R Owens, and
G J Fino, and
D L Herbert, and
V D Steen, and
T A Medsger, and
B E Pennock, and
J J Cottrell, and
G P Rodnan, and
R M Rogers
January 1984,
Clinical cardiology,
G R Owens, and
G J Fino, and
D L Herbert, and
V D Steen, and
T A Medsger, and
B E Pennock, and
J J Cottrell, and
G P Rodnan, and
R M Rogers
March 1957,
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G R Owens, and
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V D Steen, and
T A Medsger, and
B E Pennock, and
J J Cottrell, and
G P Rodnan, and
R M Rogers
November 1991,
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G R Owens, and
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G P Rodnan, and
R M Rogers
August 1989,
Angiology,
G R Owens, and
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V D Steen, and
T A Medsger, and
B E Pennock, and
J J Cottrell, and
G P Rodnan, and
R M Rogers
January 1984,
The New England journal of medicine,
G R Owens, and
G J Fino, and
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V D Steen, and
T A Medsger, and
B E Pennock, and
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G P Rodnan, and
R M Rogers
September 1988,
Southern medical journal,
