CLINICAL PROBLEMS: The Lungs
Section snippets
PULMONARY VASCULAR DISEASE
Pulmonary hypertension may occur as a primary process or secondary to interstitial lung disease and is found in 35% to 80% of patients with SSc, depending upon the study population and method of detection. Severe pulmonary hypertension is clinically detectable in 9% of limited cutaneous SSc patients.81 Pathologic studies, either autopsy or biopsy, reveal that 65% of such patients have evidence of pulmonary hypertension.93 Pulmonary hypertension is defined as a resting pulmonary artery pressure
INTERSTITIAL LUNG DISEASE
The major clinical feature of SSc interstitial lung disease is a restrictive ventilatory defect manifest by complaints of dyspnea on exertion and nonproductive cough with bibasal crackles on auscultation. Pulmonary function tests reveal a reduced forced vital capacity (FVC) and forced expiratory volume in one second (FEV1); the residual volume (RV) and total lung capacity (TLC) are reduced, as is DLco. Routine chest radiographs may show linear and reticular shadows (Fig. 1) and in more advanced
SUMMARY
A recent follow-up study found that patients with pulmonary involvement (with no renal or cardiac involvement) survived a median of 78 ± 17 months.4 Another study found death from SSc was most frequently due to pulmonary hypertension.39 Improved diagnostic modalities and better understanding of the pathophysiology of SSc lung disease are essential, because mortality from this SSc lung disease remains high.
During the past decade, advances have been made in the understanding of the alveolitis of
ACKNOWLEDGMENTS
The author acknowledges years of collaboration with Charlie Strange, MD, Anna Ludwicka, PhD, Marcy Bolster, MD, Maria Trojanowska, PhD, and Edwin Smith, MD in studies of SSc lung disease conducted at the Medical University of South Carolina, as well as the secretarial assistance of Vicki Kivett.
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Cited by (0)
Address reprint requests to Richard M. Silver, MD, Division of Rheumatology and Immunology, Medical University of South Carolina, 171 Ashley Avenue, Charleston, SC 29425–2229
This work was supported in part by the RGK Foundation (Austin, TX) and a General Clinical Research Center grant from the National Institutes of Health (RR01070-18).
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From the Medical University of South Carolina, Charleston, South Carolina