Case 2: 67 Year Old Male

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MALE, 67 YEARS OF AGE

Presents with 2-3 years history of cough and progressive dyspnea.

 

Physical Exam & Review
of Symptoms

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Case Review

  • Male, 67 years of age.
  • Presents with 2-3 years history of cough and progressive dyspnea.
  • No clinically relevant symptoms.
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Key Findings

  • Has restrictive pattern on PFTs
  • Serologies - unremarkable
  • HRCT - traction bronchiectasis; honeycombing
  • Histopathology: microcystic honeycombing, patchwork distribution
Case study courtesy of and used with permission from Gregory Cosgrove, MD.

Patient history

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Past Medical/Surgical History

  • GERD
  • Seasonal allergies
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Past Social and Family History

  • No Family History noted
  • Ex-smoker, 20 pack-years
  • No Drug use
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Medications Prescribed Prior to Initial Visit

  • Proton Pump Inhibitors
Case study courtesy of and used with permission from Gregory Cosgrove, MD.

HRCT

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HRCT Traction Bronchiectasis & Honey Combing Features
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CT scan traction bronchiectasis
 
 
 
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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.

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Physical Exam & Review
of Symptoms

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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

  • 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%)
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Case study courtesy of and used with permission from Gregory Cosgrove, MD.

Surgical lung biopsy

High magnification fibroblast focus
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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

  1. Visscher D and Myers J. Proc Am Thorac Soc. 2006;3(4):322-9.
  2. Du Bois. CHEST. 2006;130(1):3-5.
  3. 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

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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

    Sarcoidosis

  • Rheumatoid Arthritis

    Rheumatoid Arthritis

  • LAM

    LAM

  • IPF

    IPF

Correct!

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Idiopathic Pulmonary Fibrosis

This is correct! The clinical and HRCT results are characteristics of IPF.

Incorrect!

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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.

Select another test

Incorrect!

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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.

Select another test

Incorrect!

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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.

Select another test

Find out the answer

Final Answer Icon

Idiopathic Pulmonary Fibrosis

The clinical and HRCT results are characteristics of IPF.