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Article

Severe Pulmonary Fibrosis as the First Manifestation of Interferonopathy (TMEM173 Mutation).

Chest - 1 Sept 2016

Picard Cécile, Thouvenin Guillaume, Kannengiesser Caroline, Dubus Jean-Christophe, Jeremiah Nadia, Rieux-Laucat Frédéric, Crestani Bruno, Belot Alexandre, Thivolet-Béjui Françoise, Secq Véronique, Ménard Christelle, Reynaud-Gaubert Martine, Reix Philippe

Abstract excerpt

We report three cases of pulmonary disease suggesting fibrosis in two familial and one sporadic case. Pulmonary symptoms were associated with various clinical features of systemic inflammation and vasculitis involving the skin, and appeared at different ages. A strong interferon signature was found in all three cases. Disease was not responsive to corticosteroids, and lung transplantation was considered for all...

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Identifiers

PMID: 27613991
DOI: 10.1016/j.chest.2016.02.682

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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