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Positive Antinuclear Factor in Patients with Unexplained Pulmonary Fibrosis

HIROSHI NAGAYA, M.D.; C. EDWARD BUCKLEY III, M.D.; and HERBERT O. SIEKER, M.D., F.A.C.P.
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Durham, North Carolina


Ann Intern Med. 1969;70(6):1135-1145. doi:10.7326/0003-4819-70-6-1135
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SUMMARY:

Twenty patients with unexplained pulmonary fibrosis and circulating antinuclear factor were identified. Diseases commonly associated with antinuclear factor, such as systemic lupus erythematosus or scleroderma, were excluded. Half of the patients showed typical restrictive ventilatory defects and X-ray evidence of severe fibrotic changes. The remaining patients predominantly had evidence of obstructive airway diseases.

Four lung biopsies were available. Three showed histological evidence of interstitial pulmonary fibrosis. Immunofluorescent study of one of the biopsy sections with pulmonary fibrosis from a patient with only IgM serum antinuclear factor showed IgM in the alveolar septa. Relatively little fibrosis was present in immunoglobulin-laden areas whereas no immunoglobulin could be demonstrated in the adjacent alveolar septa showing marked fibrosis. Seven out of 10 patients with X-ray evidence of severe interstitial pulmonary fibrosis and typical restrictive ventilatory defects had only IgM antinuclear factor in their sera. Altered immunologic reactions associated with the generation of autoantibody may play a role in the pathogenesis of interstitial pulmonary fibrosis.

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