Case ReportOrganizing Diffuse Alveolar Damage Associated With Progressive Systemic Sclerosis
Section snippets
REPORT OF CASES
Case 1.—A previously healthy 53-year-old woman sought medical attention because of swelling of her right hand 16 months before hospital admission. Subsequently, pain and swelling developed in her right ankle. Serologic test results for antinuclear antibodies, rheumatoid factor, and Scl-70 antibody were negative. Ten months before admission, she complained of skin tightening, and a repeated Scl-70 antibody test was positive. PSS was diagnosed, and she was treated with 250 mg of penicillamine per
PATHOLOGY
Lung biopsy specimens from both patients were similar and showed acute and organizing diffuse alveolar damage (DAD). Eosinophilic hyaline membranes were present in both specimens and were most conspicuous along alveolar ducts and alveolar septa (Fig. 3). There was associated alveolar septal thickening due to a combination of edema and inflammation, intra-alveolar edema, patchy hyperplasia of type II pneumocytes, and early intra-alveolar and interstitial fibroblast proliferation. Special stains
DISCUSSION
Our report of two patients with acute ILD associated with PSS and a histologic pattern of DAD suggests that DAD may be another manifestation of lung disease in patients with PSS. Unlike the other more common types of ILD (Table 1), however, DAD has a rapid, fulminant deteriorating course.
Pulmonary parenchymal abnormalities are common in PSS, even in the absence of classic cutaneous involvement,4 and radiographic and high-resolution computed tomographic evidence of ILD has been reported in up to
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