CLINICAL PROBLEMS: The Lungs

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Pulmonary manifestations of systemic sclerosis (SSc, scleroderma) are manifold (Table 1) and often have a profound influence on the course of the disease. A discussion of all the pulmonary complications of SSc is beyond the scope of this article but is presented in a recent review.10 The two major pulmonary conditions occurring among SSc patients, pulmonary hypertension and interstitial fibrosis (Table 2), will be discussed in detail. Isolated pulmonary hypertension occurs primarily in patients with limited cutaneous SSc (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia [CREST] variant) in the absence of significant pulmonary fibrosis, whereas interstitial fibrosis occurs in either limited or diffuse cutaneous SSc.

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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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    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).

    *

    From the Medical University of South Carolina, Charleston, South Carolina

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