Familial idiopathic pulmonary fibrosis. Evidence of lung inflammation in unaffected family members
We evaluated 17 clinically unaffected members of three families with an autosomal dominant form of idiopathic pulmonary fibrosis for evidence of alveolar inflammation. Each person in the study was examined by gallium-67 scanning for a general estimate of pulmonary inflammation, and by bronchoalveolar lavage for characterization of the types of recovered cells and their state of activation. Eight of the 17 subjects had evidence of alveolar inflammation on the lavage studies. Supporting data included increased numbers of neutrophils and activated macrophages that released one or more neutrophil chemoattractants, and growth factors for lung fibroblasts--findings similar to those observed in patients with overt idiopathic pulmonary fibrosis. Four of these eight also had a positive gallium scan; in all the other clinically unaffected subjects the scan was normal. During a follow-up of two to four years in seven of the eight subjects who had evidence of inflammation, no clinical evidence of pulmonary fibrosis has appeared. These results indicate that alveolar inflammation occurs in approximately half the clinically unaffected family members at risk of inheriting autosomal dominant idiopathic pulmonary fibrosis. Whether these persons with evidence of pulmonary inflammation but no fibrosis will proceed to have clinically evident pulmonary fibrosis is not yet known.
- Research Organization:
- National Institutes of Health, Bethesda, MD
- OSTI ID:
- 5653750
- Journal Information:
- N.Engl. J. Med.; (United States), Journal Name: N.Engl. J. Med.; (United States) Vol. 21; ISSN NEJMA
- Country of Publication:
- United States
- Language:
- English
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62 RADIOLOGY AND NUCLEAR MEDICINE
ANIMAL CELLS
BETA DECAY RADIOISOTOPES
BIOLOGICAL MATERIALS
BIOPSY
BLOOD
BLOOD CELLS
BODY
BODY FLUIDS
CONNECTIVE TISSUE CELLS
COUNTING TECHNIQUES
DAYS LIVING RADIOISOTOPES
DIAGNOSIS
DIAGNOSTIC TECHNIQUES
DISEASES
ELECTRON CAPTURE RADIOISOTOPES
FIBROSIS
GALLIUM 67
GALLIUM ISOTOPES
INFLAMMATION
INTERMEDIATE MASS NUCLEI
ISOTOPES
LAVAGE
LEUKOCYTES
LUNGS
MACROPHAGES
MATERIALS
NEUTROPHILS
NUCLEI
ODD-EVEN NUCLEI
ORGANS
PATHOLOGICAL CHANGES
PATIENTS
PHAGOCYTES
RADIOISOTOPE SCANNING
RADIOISOTOPES
RESPIRATORY SYSTEM
RESPIRATORY SYSTEM DISEASES
SOMATIC CELLS
SYMPTOMS