Case 3: 40 Year Old Female
![Patient Case Study 3 Patient Case Study 3](/us/insights-in-ild/themes/custom/ild/img/Patient-case-3.png)
FEMALE, 40 YEARS OF AGE
Patient referred to the lung disease clinic after reporting shortness of breath.
Physical Exam & Review
of Symptoms
![Case review Case review](/us/insights-in-ild/themes/custom/ild/img/page-summary-plus.png)
Case Review
- Female, 40 years of age.
- Presents with shortness of breath and Raynaud's Phenomenon.
![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
- HRCT-NSIP
- Skin thickening
- Serology positive for ANA and Scl - 70
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
- Melanoma
- Bilateral breast reduction
- Poor skin healing
- Mouth tightening
![Person with Heart Clipart Person with Heart Clipart](/us/insights-in-ild/themes/custom/ild/img/patient-past social-and-family-history.png)
- Mother: Arthritis, hypertension (HTN), high cholesterol, Diabetes
- Father: HTN, MI, Arthritis
- Never Smoker
- Rare ETOH
- Hispanic
![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
- Non-steroidal anti-inflammatory drug (NSAID)
- Non-NSAID analgesic
- Proton pump inhibitor
HRCT
![CT scan CT scan](/us/insights-in-ild/themes/custom/ild/img/ba938531-79fd-443a-b543-ad9800d8ec44.jpg)
![CT scan CT scan](/us/insights-in-ild/themes/custom/ild/img/3715b4e0-ce54-4ef6-ba00-ad9800d8d794.jpg)
![CT scan CT scan](/us/insights-in-ild/themes/custom/ild/img/ce08c90b-a683-4482-b7e5-ad9800d8d7b0.jpg)
![CT scan Example CT scan Example](/us/insights-in-ild/sites/default/files/aprimo/images/e50520ce-0897-4ae0-8629-ad9800d8d854.jpg)
Your radiologist tells you the CT scan has the following features:
- [RED] Bilateral ground glass opacities
- [BLUE] Subpleural sparing
- [YELLOW] Traction bronchiectasis
Case study courtesy of and used with permission from Marilyn Glassberg, 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: 150/92 mmHg, HR: 73, RR: 18
- Temperature: 98.6
- Weight: 189 lbs
- Height: 5’5”
- SpO2: 94%
- Raynaud’s Phenomenon
![CTD Hands Example CTD Hands Example](/us/insights-in-ild/sites/default/files/aprimo/images/6d2f902c-20d2-4e72-9153-ad9800dae87a_0.jpg)
![PFTs PFTs](/us/insights-in-ild/themes/custom/ild/img/physical-exam-pft-image.png)
PFTs
- FVC 3.75 (63%)
- FEV1 3.08 (67%)
- FEV1/FVC Ratio 87%
- TLC 5.26 (53%)
- VC 3.70 (63%)
- RV 1.65 (25%)
- DLCO 24.4 (43%)
Serology Testing
![Serologies Serologies](/us/insights-in-ild/themes/custom/ild/img/Serologies.png)
Serologies
- lgG subclasses - normal
- dsDNA 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) - 1:640 speckled
- Scleroderma-70 (SCL-70; Topoisomerase I) - positive
- TPO (thyroid peroxidase) 20.7 (high)
- Centromere B - negative
- Myositis panel — negative
- JO-1 - negative
- Aldolase — negative
- CK (creatine kinase) - normal
ANA, Anti-nuclear antibody; CCP, Cyclic citrullinated peptide; HP, hypersensitivity pneumonitis; RF, rheumatoid factor; SS-A, Sjögren's syndrome-A (anti-Ro); SS-B, Sjögren's syndrome-B (anti-La); SCL-70, Scleroderma-70 (topoisomerase I).
Case study courtesy of and used with permission from Marilyn Glassberg, MD.
References
- Mayo Clinic Laboratories website. Available at https://www.mayocliniclabs.com/test-catalog/Clinical+and+Interpretive/81359. Accessed April 22, 2019.
- Visscher D and Myers J. Histologic spectrum of idiopathic interstitial pneumonias. Proc Am Thorac Soc. 2006;3(4):322-9. doi: 10.1513/pats.200602-019TK.
- du Bois RM. Fibroblastic foci: time to be counted? Chest. 2006;130(1):3-5. doi: 10.1378/chest.130.1.3.
- Jones MG, Fabre A, Schneider P, et al. Three-dimensional characterization of fibroblast foci in idiopathic pulmonary fibrosis. JCI Insight. 2016;1(5):e86375. doi: 10.1172/jci.insight.86375.
Which is the correct
Diagnosis?
-
Antisynthetase Syndrome
-
Rheumatoid Arthritis
-
Interstitial Lung Disease Associated with Systemic Sclerosis
-
IPF
Correct!
![Correct Correct](/us/insights-in-ild/themes/custom/ild/img/logo1.png)
Interstitial Lung Disease Associated with Systemic Sclerosis
Reynaud‘s phenomenon, NSIP on HRCT, positive ANA and Scl-70 are consistent with systemic sclerosis.
Incorrect!
![Incorrect X Clipart Incorrect X Clipart](/us/insights-in-ild/themes/custom/ild/img/test-your-eyes-q1-answer-img1.png)
Antisynthetase Syndrome
While restrictive defects of PFTs, NSIP on HRCT, and Reynaud‘s phenomenon are consistent with antisynthetase syndrome, the serology test is not. Elevated levels of anti-J01 are consistent with antisythetase syndrome, but the serology in this patient is negative for anti-Jo 1.
Incorrect!
![Incorrect X Clipart Incorrect X Clipart](/us/insights-in-ild/themes/custom/ild/img/test-your-eyes-q1-answer-img1.png)
Idiopathic Pulmonary Fibrosis
HRCT is consistent with an NSIP pattern that would not be found in HRCT from patients with 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
While restrictive defects of PFTs and NSI P on HRCT are consistent with rheumatoid arthritis, the serology test and skin manifestation is not. Elevated levels of anti—RF or anti-CCP are consistent with rheumatoid arthritis, but the serology in this patient is negative for anti-RF.
Find out the answer
![Correct Correct](/us/insights-in-ild/themes/custom/ild/img/logo2.png)
Interstitial Lung Disease Associated with Systemic Sclerosis
Reynaud‘s phenomenon, NSIP on HRCT, positive ANA and Scl-70 are consistent with systemic sclerosis.