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Fig. 5 | Orphanet Journal of Rare Diseases

Fig. 5

From: Pilot experience of multidisciplinary team discussion dedicated to inherited pulmonary fibrosis

Fig. 5

(a) A 44-year-old non-smoking woman with rheumatoid arthritis and familial pulmonary fibrosis. (b, c) The CT pattern was considered indeterminate for UIP and not suggestive of a specific diagnosis. Genetic analysis revealed a heterozygous SFTPA2 mutation (c.532G > A, p.Val178Met) classified as pathogenic, and genetic counseling was proposed for the relatives. A double lung transplantation was proposed and performed in April 2017. d Histology of lung transplant tissue was considered indeterminate for UIP: patchy fibrosis with both subpleural and centrilobular fibrosis with dense inflammatory infiltrates (*).b: bronchiole, C: subpleural cyst. Hematoxylin Eosin Saffron stain, bar = 3000 μm

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