Case 1: 52 Year Old Male

patient-case-1

Male, 52 years of age.

Presents with 1 month history of dyspnea.

Referred from PCP who ordered chest X-ray.

X-ray came back abnormal.

 

Physical Exam & Review
of Symptoms

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Case Review

  • Male, 52 years of age.
  • Presents with 1 month history of dyspnea.
  • Completed comprehensive medical exam, family, social and medical history.
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Key Findings

  • Has restrictive pattern on PFTs
  • SS-B - Positive (mild)
  • Increased blood calcium
  • Elevated ACE levels
  • Abundant mid-lung nodules
  • Tight and well-formed, confluent granulomas
Case study courtesy of and used with permission from Marilyn Glassberg, MD.

Patient history

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Past Medical/Surgical History

  • GERD
  • Hypertension
  • Sinusitis
  • Knee Surgery
  • Tonsillectomy
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Past Social and Family History

  • No Family History noted
  • Married
  • Never smoker
  • Social ETOH, no drug use
  • Owns a roofing contracting company
Medications Prescribed Prior to Initial Visit

Medications Prescribed Prior to Initial Visit

  • Insomnia medication
  • Beta blocker
  • Quinolone antibiotic
  • Corticosteroid nasal spray
Case study courtesy of and used with permission from Marilyn Glassberg, MD.

HRCT

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Your radiologist tells you the CT scan has the following features:

Small nodules abundant in the mid-lung.

Case study courtesy of and used with permission from Marilyn Glassberg, MD.

 

Back

Physical Exam & Review
of Symptoms

Physical Exam

Physical Exam

  • BP: 152/106 mmHg, HR: 88, RR: 20
  • Temperature: 98.1
  • Weight: 194 lbs
  • Height: 5’8”
  • SpO2: 98%
  • Well appearing
  • Lungs: CTA&P
  • Physical exam unremarkable
PFTs

PFTs

  • FVC 3.46 (76%)
  • FEV1 2.77 (79%)
  • FEV1/FVC Ratio 103%
  • TLC 5.05 (76%)
  • VC 3.48 (77%)
  • RV 1.57 (74%)
  • DLCO 29.07 (94%)
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Case study courtesy of and used with permission from Marilyn Glassberg, MD.

Histopathology

VATS Pathology
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VATS Pathology

Images courtesy of and used with permission from Vijayalakshmi Ananthanarayanan, MD.

VATS Pathology

  • Extensive non-caseating granulomatous inflammation that follows the lymphatic routes along the bronchovascular bundles and reaching the overlying pleura
  • Stains for fungi and mycobacteria are negative

VATS Pathology

  • Extensive non-caseating granulomatous inflammation that follows the lymphatic routes along the bronchovascular bundles and reaching the overlying pleura

Stains for fungi and mycobacteria are negative

VATS Pathology

  • Extensive non-caseating granulomatous inflammation that follows the lymphatic routes along the bronchovascular bundles and reaching the overlying pleura
  • Stains for fungi and mycobacteria are negative

NOTE: Here is a lymph node section with tight and well-formed, confluent granulomas. The magnified image shows epithelioid histiocytes (arrowheads): Folded, bland, “bathroom slipper/ flip flap/ insole” shaped nuclei.

Serology Testing

Serologies

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) - positive (mild)
  • Anti-nuclear antibody (ANA) - negative
  • Scleroderma-70 (SCL-70; Topoisomerase I) - negative

Additional information from bloodwork:

  • Hypercalcemia
  • Elevated ACE levels
    (Angiotensin converting enzyme)

What conditions can a positive SS-B be associated with?

SS-B, also known as anti-La antibody, is an autoantibody that is commonly associated with the CTDs Sjögren's syndrome and systemic lupus erythematosus, but can be found in other conditions as well.1

ANA, Anti-nuclear antibody; CCP, Cyclic citrullinated peptide; RF, rheumatoid factor; SS-A, Sjögren's syndrome-A (Anti Ro); SS-B, Sjögren's syndrome-B (anti-La); SOL-70, Scleroderma-70 (topoisomerase I); ACE, Angiotensin converting enzyme.

Case study courtesy of and used with permission from Marilyn Glassberg, MD.  

References

  1. Mayo Clinic Laboratories website. Available at https://www.mayocliniclabs.com/test-catalog/Clinical+and+Interpretive/81359. Accessed April 22, 2019.
  2. 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.
  3. du Bois RM. Fibroblastic foci: time to be counted? Chest. 2006;130(1):3-5. doi: 10.1378/chest.130.1.3.
  4. 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?

  • Sarcoidosis

    Sarcoidosis

  • Rheumatoid Arthritis

    Rheumatoid Arthritis

  • LAM

    LAM

  • IPF

    IPF

Correct!

Correct

Sarcoidosis

This is correct

Incorrect!

Incorrect X Clipart

Rheumatoid Arthritis

This is incorrect. The patient had negative CCP and RF antibodies, both of which are associated with rheumatoid arthritis. Further, the patient had no joint involvement or other signs and symptoms of rheumatoid arthritis.

Select another test

Incorrect!

Incorrect X Clipart

LAM

This is incorrect. A diagnosis of LAM is incorrect because it occurs exclusively in women. Furthermore, normal TLC, RV, DLCO, along with the lack of cysts on HRCT, suggests an alternative diagnosis.

Select another test

Incorrect!

Incorrect X Clipart

IPF

This is incorrect. A diagnosis of IPF is unlikely because nodules are present on HRCT, which is consistent with an alternative diagnosis of IPF. Also, this patient is younger than the expected age of >60 years and is a never smoker.

Select another test

Find out the answer

Correct

Sarcoidosis