SCLERODERMA WITH PULMONARY FIBROSIS

John R. Murphy

JAMA · 1941 · 52 citations · 0 references

Concepts

Abstract

The pathologic changes in diffuse scleroderma are not confined to skin and subcutaneous tissue alone but involve the connective tissue system throughout the entire body. Muscle, fascia, tendon, bone and at times even the endocrine glands and the internal organs participate in the disease process. Everywhere the same changes occur, namely edema followed by sclerosis of the collagenous bundles. This may occur in the connective tissue between the alveoli of the lung, the acini of the liver, the glomeruli of the kidney and the muscle fibers of the heart and within the pulp of the spleen.<sup>1</sup>The smaller arteries within the regions involved show corresponding sclerotic degeneration and thickening of the intima which may proceed to obliteration of the lumen and subsequent focal necrosis.<sup>2</sup> The case to be presented is of interest because it has been possible to demonstrate visceral pathologic changes, namely pulmonary fibrosis or sclerosis as