Abstract
Study objectives: To reassess the association between neurofibromatosis and pulmonary fibrosis. Design: Retrospective single-center study with analysis of patients' chest radiographs, CT scans, and medical records. Setting: Tertiary care, referral medical center. Patients: One hundred fifty-six adult patients with neurofibromatosis seen over a 6-year period between 1997 and 2002. Results: A review of chest radiographs revealed abnormal findings in 70 patients (44.9%). The most common radiographic abnormalities were extrapulmonary nodules or masses seen in 22 patients (14.1%), followed by skeletal abnormalities in 16 patients (10.3%). Bilateral interstitial infiltrates were noted in only three patients (1.9%), all of whom had potential causes other than neurofibromatosis for their lung infiltrates, including smoking-related interstitial lung disease, rheumatoid lung disease, recurrent pneumonias, and a history of ARDS. CT scans were available in two of these patients and revealed nonspecific patterns of abnormalities with no honeycombing. Six patients had bullae or cystic airspaces demonstrated on chest radiography or CT scan; all of these findings occurred in the context of smoking-related emphysema. Combined together, bilateral interstitial lung infiltrates or cystic airspaces were demonstrated in five patients (3.2%) by chest radiography, and in eight patients (5.1%) by chest radiography or CT scanning; one patient had both findings on the CT scan. Conclusions: We found little evidence to support an association between neurofibromatosis and pulmonary fibrosis or any other form of parenchymal lung disease. Interstitial lung disease and bullae described in association with neurofibromatosis in previous reports may have, in part, represented smoking-induced manifestations.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 2381-2386 |
| Number of pages | 6 |
| Journal | Chest |
| Volume | 128 |
| Issue number | 4 |
| DOIs | |
| State | Published - Oct 2005 |
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Keywords
- Genetics
- Interstitial lung disease
- Pulmonary fibrosis
ASJC Scopus subject areas
- Pulmonary and Respiratory Medicine
Cite this
Lack of evidence for an association between neurofibromatosis and pulmonary fibrosis. / Ryu, Jay H; Parambil, Joseph G.; McGrann, Pamela S.; Aughenbaugh, Gregory L.
In: Chest, Vol. 128, No. 4, 10.2005, p. 2381-2386.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Lack of evidence for an association between neurofibromatosis and pulmonary fibrosis
AU - Ryu, Jay H
AU - Parambil, Joseph G.
AU - McGrann, Pamela S.
AU - Aughenbaugh, Gregory L.
PY - 2005/10
Y1 - 2005/10
N2 - Study objectives: To reassess the association between neurofibromatosis and pulmonary fibrosis. Design: Retrospective single-center study with analysis of patients' chest radiographs, CT scans, and medical records. Setting: Tertiary care, referral medical center. Patients: One hundred fifty-six adult patients with neurofibromatosis seen over a 6-year period between 1997 and 2002. Results: A review of chest radiographs revealed abnormal findings in 70 patients (44.9%). The most common radiographic abnormalities were extrapulmonary nodules or masses seen in 22 patients (14.1%), followed by skeletal abnormalities in 16 patients (10.3%). Bilateral interstitial infiltrates were noted in only three patients (1.9%), all of whom had potential causes other than neurofibromatosis for their lung infiltrates, including smoking-related interstitial lung disease, rheumatoid lung disease, recurrent pneumonias, and a history of ARDS. CT scans were available in two of these patients and revealed nonspecific patterns of abnormalities with no honeycombing. Six patients had bullae or cystic airspaces demonstrated on chest radiography or CT scan; all of these findings occurred in the context of smoking-related emphysema. Combined together, bilateral interstitial lung infiltrates or cystic airspaces were demonstrated in five patients (3.2%) by chest radiography, and in eight patients (5.1%) by chest radiography or CT scanning; one patient had both findings on the CT scan. Conclusions: We found little evidence to support an association between neurofibromatosis and pulmonary fibrosis or any other form of parenchymal lung disease. Interstitial lung disease and bullae described in association with neurofibromatosis in previous reports may have, in part, represented smoking-induced manifestations.
AB - Study objectives: To reassess the association between neurofibromatosis and pulmonary fibrosis. Design: Retrospective single-center study with analysis of patients' chest radiographs, CT scans, and medical records. Setting: Tertiary care, referral medical center. Patients: One hundred fifty-six adult patients with neurofibromatosis seen over a 6-year period between 1997 and 2002. Results: A review of chest radiographs revealed abnormal findings in 70 patients (44.9%). The most common radiographic abnormalities were extrapulmonary nodules or masses seen in 22 patients (14.1%), followed by skeletal abnormalities in 16 patients (10.3%). Bilateral interstitial infiltrates were noted in only three patients (1.9%), all of whom had potential causes other than neurofibromatosis for their lung infiltrates, including smoking-related interstitial lung disease, rheumatoid lung disease, recurrent pneumonias, and a history of ARDS. CT scans were available in two of these patients and revealed nonspecific patterns of abnormalities with no honeycombing. Six patients had bullae or cystic airspaces demonstrated on chest radiography or CT scan; all of these findings occurred in the context of smoking-related emphysema. Combined together, bilateral interstitial lung infiltrates or cystic airspaces were demonstrated in five patients (3.2%) by chest radiography, and in eight patients (5.1%) by chest radiography or CT scanning; one patient had both findings on the CT scan. Conclusions: We found little evidence to support an association between neurofibromatosis and pulmonary fibrosis or any other form of parenchymal lung disease. Interstitial lung disease and bullae described in association with neurofibromatosis in previous reports may have, in part, represented smoking-induced manifestations.
KW - Genetics
KW - Interstitial lung disease
KW - Pulmonary fibrosis
UR - http://www.scopus.com/inward/record.url?scp=27144443327&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=27144443327&partnerID=8YFLogxK
U2 - 10.1378/chest.128.4.2381
DO - 10.1378/chest.128.4.2381
M3 - Article
C2 - 16236898
AN - SCOPUS:27144443327
VL - 128
SP - 2381
EP - 2386
JO - Chest
JF - Chest
SN - 0012-3692
IS - 4
ER -