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'''Usual interstitial pneumonia''', abbreviated '''UIP''', is common [[diffuse lung disease]]. Overall, it is uncommon. | {{ Infobox diagnosis | ||
| Name = {{PAGENAME}} | |||
| Image = Fibroblast focus.jpg | |||
| Width = | |||
| Caption = Fibroblast focus in usual interstitial pneumonia. [[H&E stain]]. | |||
| Synonyms = | |||
| Micro = fibroblast foci, interstitial inflammation, microscopic honeycombing (typically peripheral & lined by ciliated epithelium), spatial heterogeneity - patchy lesional distribution (areas of abnormal and normal lung may appear beside one another), temporal heterogeneity - lesions of differing age side-by-side | |||
| Subtypes = | |||
| LMDDx = [[asbestosis]], [[chronic hypersensitivity pneumonitis]], collagen vascular disease (e.g. [[systemic lupus erythematosus]], [[rheumatoid arthritis]], [[scleroderma]]), chronic drug toxicity | |||
| Stains = [[iron stain]] -ve | |||
| IHC = | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = lower lobe & periperal predominant: fibrosis, peripheral cysts | |||
| Grossing = | |||
| Site = [[lung]] - see ''[[diffuse lung diseases]]'' | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = | |||
| Signs = signs of right heart failure | |||
| Symptoms = shortness of breath | |||
| Prevalence = uncommon | |||
| Bloodwork = | |||
| Rads = interstitial pattern, lower lobe predominant, peripheral cysts | |||
| Endoscopy = | |||
| Prognosis = usually poor, dependent on amount of fibrosis | |||
| Other = histologic correlate of ''idiopathic pulmonary fibrosis'' | |||
| ClinDDx = [[asbestosis]], [[chronic hypersensitivity pneumonitis]], collagen vascular disease (history missing), chronic drug toxicity (history missing), idiopathic pulmonary fibrosis | |||
| Tx = lung transplantation | |||
}} | |||
'''Usual interstitial pneumonia''', abbreviated '''UIP''', is a relatively common pattern in [[diffuse lung disease]]s. Overall, it is an uncommon pathology. | |||
''Idiopathic pulmonary fibrosis'' (abbreviated ''IPF'') redirects here. | |||
==General== | ==General== | ||
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Note: | Note: | ||
*Cysts - have thin walls (think of emphysema, [[lymphangioleiomyomatosis]] et cetera). | *Cysts - have thin walls (think of [[emphysema]], [[lymphangioleiomyomatosis]] et cetera). | ||
**Cysts may be isolated/not close to a neighbour. | **Cysts may be isolated/not close to a neighbour. | ||
**Medcyclopaedia defines it as: thin-walled, well-demarcated and >1 cm.<ref>[http://www.medcyclopaedia.com/library/topics/volume_v_1/l/lung_cyst.aspx http://www.medcyclopaedia.com/library/topics/volume_v_1/l/lung_cyst.aspx]</ref> | **Medcyclopaedia defines it as: thin-walled, well-demarcated and >1 cm.<ref>[http://www.medcyclopaedia.com/library/topics/volume_v_1/l/lung_cyst.aspx http://www.medcyclopaedia.com/library/topics/volume_v_1/l/lung_cyst.aspx]</ref> | ||
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DDx of UIP:<ref name=leslie>{{cite book |author=Wick, Mark R.; Leslie, Kevin |title=Practical pulmonary pathology: a diagnostic approach |publisher=Churchill Livingstone |location=Edinburgh |year=2005 |pages= |isbn=0-443-06631-0 |oclc= 156861539|doi= |accessdate=}}</ref> | DDx of UIP:<ref name=leslie>{{cite book |author=Wick, Mark R.; Leslie, Kevin |title=Practical pulmonary pathology: a diagnostic approach |publisher=Churchill Livingstone |location=Edinburgh |year=2005 |pages= |isbn=0-443-06631-0 |oclc= 156861539|doi= |accessdate=}}</ref> | ||
*Idiopathic pulmonary fibrosis (UIP not otherwise specified). | *Idiopathic pulmonary fibrosis (UIP not otherwise specified). | ||
*Asbestosis = UIP pattern + ferruginous bodies with asbestos fibers. | *[[Asbestosis]] = UIP-like pattern + ferruginous bodies with asbestos fibers. | ||
*Chronic hypersensitivity pneumonitis ([[AKA]] extrinsic allergic alveolitis) - classically centrilobular predominant +/- granulomas. | *Chronic [[hypersensitivity pneumonitis]] ([[AKA]] extrinsic allergic alveolitis) - classically centrilobular predominant +/- granulomas. | ||
*Collagen vascular disease - includes [[systemic lupus erythematosus]], [[rheumatoid arthritis]], [[scleroderma]].<ref name=Ref_PCPBoD8_374>{{Ref PCPBoD8|374}}</ref> | *[[Collagen vascular disease]] - includes [[systemic lupus erythematosus]], [[rheumatoid arthritis]], [[scleroderma]].<ref name=Ref_PCPBoD8_374>{{Ref PCPBoD8|374}}</ref> | ||
*Chronic drug toxicity.<ref name=pmid10992015>{{cite journal |author=Rossi SE, Erasmus JJ, McAdams HP, Sporn TA, Goodman PC |title=Pulmonary drug toxicity: radiologic and pathologic manifestations |journal=Radiographics : a review publication of the Radiological Society of North America, Inc |volume=20 |issue=5 |pages=1245-59 |year=2000 |pmid=10992015 |doi=}}</ref> | *Chronic drug toxicity.<ref name=pmid10992015>{{cite journal |author=Rossi SE, Erasmus JJ, McAdams HP, Sporn TA, Goodman PC |title=Pulmonary drug toxicity: radiologic and pathologic manifestations |journal=Radiographics : a review publication of the Radiological Society of North America, Inc |volume=20 |issue=5 |pages=1245-59 |year=2000 |pmid=10992015 |doi=}}</ref> | ||
===Images=== | ===Images=== | ||
<gallery> | <gallery> | ||
Image:Honeycomb change.jpg|UIP - honeycomb change in UIP - low mag. (WC) | |||
Image:UIPlungbiopsy.jpg|UIP - fibrosis - low mag. (WC) | Image:UIPlungbiopsy.jpg|UIP - fibrosis - low mag. (WC) | ||
Image:Fibroblast focus.jpg|UIP - fibroblast focus - high mag. (WC) | Image:Fibroblast focus.jpg|UIP - fibroblast focus - high mag. (WC) | ||
</gallery> | </gallery> | ||
==Sign out== | |||
<pre> | |||
A. Lung, Right Lower Lobe, Wedge Biopsy: | |||
- Usual interstitial pneumonia, see comment and microscopic. | |||
B. Lung, Right Middle Lobe, Wedge Biopsy: | |||
- Usual interstitial pneumonia, see comment and microscopic. | |||
C. Lung, Right Upper Lobe, Wedge Biopsy: | |||
- Usual interstitial pneumonia, see comment and microscopic. | |||
Comment: | |||
There are no findings to specifically suggest hypersensitivity. Clinical and radiologic | |||
correlation is suggested. | |||
</pre> | |||
===Alternate=== | |||
<pre> | |||
Right Lower Lobe of Lung, Wedge Resection: | |||
- Usual interstitial pneumonia (UIP) pattern (fibroblast foci, bronchiolization, | |||
extensive fibroelastotic scarring and focal calcification/bone formation), see comment. | |||
Comment: | |||
UIP pattern may be idiopathic. It is also be seen in the context of collagen vascular diseases, hypersensitivity reactions, and drugs; these need to be considered in clinical context along with the imaging findings. | |||
</pre> | |||
===Micro=== | |||
<pre> | |||
Sections show lung parenchyma with: | |||
Fibrosis (severity; lobar location): present (moderate-to-severe; peripheral predominant). | |||
Fibrosis - lung field predominance: lower lobe > middle lobe > upper lobe. | |||
Fibroblast foci: present. | |||
Temporal heterogeneity: present. | |||
Spatial heterogeneity: present. | |||
Peripheral cyst formation: present. | |||
Inflammation: present - patchy, lymphocyte predominant. | |||
Granulomatous inflammation: absent. | |||
Blood vessel changes: present, moderate-to-severe. | |||
Airspace changes: mucous plugs. | |||
</pre> | |||
==See also== | ==See also== | ||