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Items: 12

1.

Idiopathic fibrosing alveolitis, chronic form

Familial pulmonary fibrosis (FPF in this GeneReview) is defined as idiopathic interstitial pneumonia (IIP) in two or more first-degree relatives (parent, sib, or offspring). Up to 20% of cases of IIP cluster in families. The clinical findings of IIP are bibasilar reticular abnormalities, ground glass opacities, or diffuse nodular lesions on high-resolution computed tomography and abnormal pulmonary function studies that include evidence of restriction (reduced VC with an increase in FEV1/FVC ratio) and/or impaired gas exchange (increased P(A-a)O2 with rest or exercise or decreased diffusion capacity of the lung for carbon monoxide [DLCO]). FPF usually presents between ages 50 and 70 years. FPF may be complicated by lung cancer; bronchoalveolar cell carcinoma, small-cell carcinoma, and adenocarcinoma have been described. [from GTR]

MedGen UID:
321462
Concept ID:
C1800706
Disease or Syndrome
2.

Pulmonary fibrosis

A process in which normal lung tissues are progressively replaced by FIBROBLASTS and COLLAGEN causing an irreversible loss of the ability to transfer oxygen into the bloodstream via PULMONARY ALVEOLI. Patients show progressive DYSPNEA finally resulting in death. [from MeSH]

MedGen UID:
11028
Concept ID:
C0034069
Disease or Syndrome
3.

Fibrosis

Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury. [from MeSH]

MedGen UID:
5179
Concept ID:
C0016059
Pathologic Function
4.

Exacerbation

A worsening of a problem, condition, or disease. [from NCI]

MedGen UID:
898763
Concept ID:
C4086268
Finding
5.

Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a nonneoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known cause. [from ORDO]

MedGen UID:
832773
Concept ID:
CN200819
Finding
6.

Furriers lung

MedGen UID:
538594
Concept ID:
C0264476
Disease or Syndrome
7.

Coffee-workers lung

MedGen UID:
538589
Concept ID:
C0264468
Disease or Syndrome
8.

Malt-workers lung

MedGen UID:
510125
Concept ID:
C0155888
Disease or Syndrome
9.

Cheese-washers lung

MedGen UID:
507549
Concept ID:
C0007969
Disease or Syndrome
10.

Idiopathic pulmonary fibrosis

MedGen UID:
39340
Concept ID:
C0085786
Disease or Syndrome
11.

Slowly Progressive Clinical Course

Clinical course characterized by slow progression without signs of aggressive disease. [from NCI]

MedGen UID:
857160
Concept ID:
C3897566
Finding
12.

Progressive Clinical Course

MedGen UID:
233349
Concept ID:
C1335498
Finding
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