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. 2014 May;21(3):120-127.
doi: 10.1097/cpm.0000000000000034.

Familial Interstitial Pneumonia (FIP)

Janet L Talbert  1 , David A Schwartz  2 , Mark P Steele  3
Affiliations

Familial Interstitial Pneumonia (FIP)

Janet L Talbert et al. Clin Pulm Med. 2014 May.
No abstract available

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Conflict of interest statement

Conflicts of Interest

M. Steele and J. Talbert report no conflicts of interests or disclosures. D. Schwartz has a patent pending on utilizing MUC5B for the diagnosis and prognosis of pulmonary fibrosis.

Figures

Figure 1
Figure 1
The pedigree demonstrates multiple affected individuals in the second generation and male to male transmission consistent with autosomal dominant inheritance. The third generation is unaffected at the time of ascertainment reflecting late age of disease onset. Phenotypic heterogeneity is reflected by both NSIP and UIP in two siblings.
Figure 2
Figure 2
The pedigree demonstrates the pleiotropic manifestations of telomere-mediated disease in FIP. Family members may be affected with one or more phenotypes, with IPF being the most common manifestation. A thorough family history should include inquiries regarding telomere-mediated diseases as seen in Dyskeratosis Congenita (DC).
See this image and copyright information in PMC

References

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