Case 2: 67 Year Old Male
![patient-case-2 patient-case-2](/us/insights-in-ild/themes/custom/ild/img/case2-67-Year-Old-Male.png)
MALE, 67 YEARS OF AGE
Presents with 2-3 years history of cough and progressive dyspnea.
Physical Exam & Review
of Symptoms
![Case Review Case review](/us/insights-in-ild/themes/custom/ild/img/page-summary-plus.png)
Case Review
- Male, 67 years of age.
- Presents with 2-3 years history of cough and progressive dyspnea.
- No clinically relevant symptoms.
![Clipboard Checkmark Clipart Clipboard Checkmark Clipart](/us/insights-in-ild/themes/custom/ild/img/page-summary-correct.png)
Key Findings
- Has restrictive pattern on PFTs
- Serologies - unremarkable
- HRCT - traction bronchiectasis; honeycombing
- Histopathology: microcystic honeycombing, patchwork distribution
Patient history
![Past Medical/Surgical History Past Medical/Surgical History](/us/insights-in-ild/themes/custom/ild/img/page-summary-plus.png)
Past Medical/Surgical History
- GERD
- Seasonal allergies
![family-history Past Social and Family History](/us/insights-in-ild/themes/custom/ild/img/patient-past-social-and-family-history.png)
- No Family History noted
- Ex-smoker, 20 pack-years
- No Drug use
![Medications Prescribed Prior to Initial Visit Medications Prescribed Prior to Initial Visit](/us/insights-in-ild/themes/custom/ild/img/patient-medications-prescribed-prior-to-initial-visit.png)
Medications Prescribed Prior to Initial Visit
- Proton Pump Inhibitors
HRCT
![Bitmap Bitmap](/us/insights-in-ild/sites/default/files/aprimo/images/b6ada312-fa14-4827-bce5-ad9800d8e401.jpg)
![Diagnosing ILDs: perspectives on current challenges in emerging tech. Diagnosing ILDs: perspectives on current challenges in emerging tech.](/us/insights-in-ild/sites/default/files/aprimo/images/20926241-57ae-4b96-8e67-ad9800d8e56f.jpg)
![Bitmap Bitmap](/us/insights-in-ild/themes/custom/ild/img/f54c0ec2-af23-4cb3-bb08-ad9800d8e32d.jpg)
![Bitmap Bitmap](/us/insights-in-ild/themes/custom/ild/img/ec0d00b0-4afa-4784-875b-ad9800d8e35f.jpg)
Your radiologist tells you the CT scan has the following features:
- ➔ Traction bronchiectasis
- Honeycombing
Case study courtesy of and used with permission from Robert Suh, MD.
Physical Exam & Review
of Symptoms
![Physical exam Physical exam](/us/insights-in-ild/themes/custom/ild/img/Group 9@2x.png)
Physical Exam
- BP: 148/82 mmHg, HR: 75, RR: 18
- Temperature: 98.1
- Weight: 194 Ibs
- Height: 5’10”
- SpO2: 92%
- Well appearing
- Lungs: mid-late inspiratory crackles
- Digital clubbing
![PFTs PFTs](/us/insights-in-ild/themes/custom/ild/img/physical-exam-pft-image.png)
PFTs
- FVC 2.97 (67%)
- FEV1 2.64 (82%)
- FEV1/FVC Ratio 89%
- TLC 4.48 (67%)
- VC 3.48 (77%)
- RV 1.27 (47%)
- DLCO (73%)
SURGICAL LUNG BIOPSY
Image used with permission from Visscher D and Myers J. Proc Am Thorac Soc. 2006;3(4):322-9.
Low magnification image
Image used with permission from Visscher D and Myers J. Proc Am Thorac Soc. 2006;3(4):322-9.
Histopathology:
- High magnification: Fibroblast focus (arrows)
What are fibroblastic foci?
Fibroblastic foci -aggregates within the lung consisting of proliferating fibroblasts and myofibroblasts with a localized edge of spindle cells these indicate areas of active fibrosis.1-3
References
- Visscher D and Myers J. Proc Am Thorac Soc. 2006;3(4):322-9.
- Du Bois. CHEST. 2006;130(1):3-5.
- Jones et al. JCI Insight. 2016;1(5):e86375.
Pathology:
Low magnification
- Honeycomb change
- Columnar respiratory epithelium outline the cystic spaces
- Cystic spaces contain mucus and inflammatory cells
See additional note from pathologist:
NOTE: Additional sample shows patchy distribution of abnormalities
Serology Testing
![Serologies Serologies](/us/insights-in-ild/themes/custom/ild/img/Serologies.png)
Serologies
- Specific immunoglobulin panel - negative
- Cyclic citrullinated peptide (CCP) - negative
- Rheumatoid factor (RF) - negative
- Sjögren’s syndrome-A (Anti Ro) (SS-A) - negative
- Sjögren’s syndrome-B (anti-La) (SS-B) - negative
- Anti-nuclear antibody (ANA) - negative
- Scleroderma-70 (SCL-70; Topoisomerase 1) - negative
Case study courtesy of and used with permission from Gregory Cosgrove, MD.
Which is the correct
Diagnosis?
-
Sarcoidosis
-
Rheumatoid Arthritis
-
LAM
-
IPF
Correct!
![Correct Correct](/us/insights-in-ild/themes/custom/ild/img/logo1.png)
Idiopathic Pulmonary Fibrosis
This is correct! The clinical and HRCT results are characteristics of IPF.
Incorrect!
![Incorrect X Clipart Incorrect X Clipart](/us/insights-in-ild/themes/custom/ild/img/test-your-eyes-q1-answer-img1.png)
Rheumatoid Arthritis
The patient had negative CCP and RF antibodies, both of which are associated with rheumatoid arthritis. Furthermore, the patient had no joint involvement or other signs and symptoms of rheumatoid arthritis.
Incorrect!
![Incorrect X Clipart Incorrect X Clipart](/us/insights-in-ild/themes/custom/ild/img/test-your-eyes-q1-answer-img1.png)
Lymphangioleiomyomatosis
A diagnosis of LAM is incorrect because it occurs exclusively in women. Furthermore, reduced RV and TLC suggests an alternative diagnosis. The cysts found in this patient‘s HRCT are consistent with lower-lobe honeycombing, not the small diffusely distributed cysts seen in LAM.
Incorrect!
![Incorrect X Clipart Incorrect X Clipart](/us/insights-in-ild/themes/custom/ild/img/test-your-eyes-q1-answer-img1.png)
Sarcoidosis
A diagnosis of sarcoidosis is unlikely because the HRCT shows lower lobe honeycombing. In sarcoidosis, nodules in the upper or middle lobes are common. Also, pathology does not show non-caseating granulomas, which is a key feature of sarcoidosis.
Find out the answer
![Correct Correct](/us/insights-in-ild/themes/custom/ild/img/logo2.png)
Idiopathic Pulmonary Fibrosis
The clinical and HRCT results are characteristics of IPF.